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Manual for People Living with ALS

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Manual for People Living with ALS Powered By Docstoc
					ALS               SLA          AMYOTROPHIC LATERAL SCLEROSIS
                               SOCIETY OF CANADA

Maladie Lou Gehrig’s Disease   SOCIÉTÉ CANADIENNE DE
                               LA SCLÉROSE LATÉRALE AMYOTROPHIQUE




                               A MANUAL
                               FOR
                               PEOPLE
                               LIVING
                               WITH ALS
LIVING WITH ALS                                            TABLE OF CONTENTS
        page description

           iii Acknowledgement

               INTRODUCTION
           1 Introduction
   1           WHAT IS ALS?
           2   General Description
           2   Types of ALS
           3   Symptoms, Signs and Diagnosis
           4   Research
           7   Drug Trials
   2           COPING WITH ALS
          13   Coping with Diagnosis
          13   Coping Strategies for the Person with ALS
          14   Coping Strategies for Family Members
          14   Coping Strategies for Caregivers
          15   Friends and Family
          16   Helping Children to Cope
   3           MANAGING YOUR SYMPTOMS AND TREATMENT
          21   Meeting the Challenge
          23   Treating ALS Symptoms:
          23   • Mouth and Throat
          24   • Daily Living
          25   • Mobility
          26   Assistive Equipment
          27   Sexual Concerns
          28   Alternative Treatment Options
          29   Meditation
          30   Alternative Therapies
          31   Dealing With Dying
   4           YOUR HEALTHCARE TEAM
          35   Your Primary Doctor
          35   Neurologist
          35   Nurse Clinician
          36   Occupational Therapist & Physiotherapist
          37   Dietitian
          37   Respiratory Therapist
          37   Speech-language pathologist
          37   Social Worker
          38   Pastoral Care Worker
   5           MOBILITY STRATEGIES                              ALS                 SLA
          41   The Role of Exercise                             SOCIETY OF CANADA
          42   Dealing with Problems                            Maladie Lou Gehrig’s Disease
          43   Assistive Devices                                1(800)267-4257
          43   Transferring a person with ALS                   http://www.als.ca
          43   Travel                                           alscanada@als.ca
          43   Support Services

v 2.0                                                           PAGE i
TABLE OF CONTENTS                                      LIVING WITH ALS
                                    page description
                               6          DEALING WITH SWALLOWING DIFFICULTIES
                                     47   Mechanics of Swallowing
                                     48   Making Eating Easier
                                     49   Changes to Your Diet
                                     50   Pills
                                     51   Nutrition
                                     51   Tube Feeding
                               7          MOUTH CARE
                                     57 Mouth Care
                                     58 Managing Saliva
                                     59 Managing a Dry Mouth
                               8          DEALING WITH SPEAKING DIFFICULTIES
                                     63   Dealing with Speaking Difficulties
                                     64   Communication Devices
                                     64   High-Tech Strategies
                                     65   Low-Tech Strategies
                               9          ADAPTING TO BREATHING DIFFICULTIES
                                     69   Mechanics of Breathing
                                     70   Symptoms of Weakened Breathing Muscles
                                     72   Two Simple Strategies
                                     72   Respiratory Diseases
                                     73   Ventilators
                               10         ASSISTIVE EQUIPMENT
                                     77   Before Purchasing Equipment
                                     77   Bathroom Equipment
                                     78   Beds and Mattresses
                                     78   Body Supports
                                     78   Communication Equipment
                                     79   Lifts and Stair Glides
                                     80   Tube-feeding Equipment
                                     80   Walking – Canes and Walkers
                                     81   Wheelchairs
                                     84   Other Equipment
                               11         FINANCIAL & LEGAL CONSIDERATIONS
                                     87   Costs of Living with ALS
                                     88   Dealing with your Employer
                                     89   Insurance Issues
                                     93   Pension Plans
                                     94   Tax Issues
ALS              SLA                 96
                                     97
                                          Powers of Attorney
                                          Wills
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease
                               12   101 A MESSAGE OF HOPE
          1(800)267-4257
          http://www.als.ca
                               13   103 ABOUT THE ALS SOCIETY
           alscanada@als.ca
                               14   105 A LIST OF REFERENCES


                   PAGE ii                                                         v. 2.0
LIVING WITH ALS
ACKNOWLEDGEMENT

This manual has been the dream of many         Bob’s drive and spirit are evident in this
people across this country who are             manual and at times the project seemed to
touched by ALS, including those with the       keep the disease at bay. Unfortunately he
disease, their families, and caregivers.       did not see the publication of his work as a
                                               national publication.
This was especially true for its original
author Bob McDonald.                           This manual is dedicated to the memory of
                                               all those who have succumbed to ALS, but
Bob was no stranger to ALS as he suffered      in particular to Bob McDonald who held a
through the illness and deaths of his          vision for it and inspired its completion.
grandmother and his father with the
disease. When the rare familial line of                             y
ALS was diagnosed in his body he
plunged full force into the preparation of     ALS Canada wishes to acknowledge the
the first version of this patient manual.      efforts and foresight of the ALS Society of
                                               Toronto and Area and their partner Arthur
His search for more information on the         Andersen & Co. in compiling the first
disease to expand on what he already           manual and distributing it widely, both
knew led him around the world through          nationally and internationally. Also, the
his computer. The fruits of his search are     ALS Society of Ontario – Windsor
evident here.                                  Chapter for their generous contribution
                                               towards the printing of this edition.
The caring nature of Bob, his wife Sandy
and daughters Jennifer and Katy, can be                                       – March 2000
seen beyond this work in their tireless
efforts on behalf of the ALS Society,
especially their faithful attendance at many
support group meetings.




                                                                                              ALS                 SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE iii
                               LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                   PAGE iv                    v. 1.0
LIVING WITH ALS
INTRODUCTION

INTRODUCTION                                  ALS affects people in many different ways.     INTRODUCTION
                                              People’s needs can be very different,
As all people with ALS soon learn, ALS        depending both on their attitude and on
is a journey of continuous change and         how ALS has affected them. The primary
personal challenge. To meet the challenge     source of information for you comes from
presented when parts of your body don’t       your doctors and other healthcare
function the way they used to, you will       professionals. This manual should
need to find new ways of doing things,        therefore be considered complementary to
and although some may be of little help,      professional healthcare advice.
others may change your life.
                                              The main purpose of this manual is to
ALS is not an easy disease to have.           provide information and helpful hints that
Writing about it is not easy, and reading     will assist people with ALS to manage
about it won’t be. We suggest that you do     their new challenges.
not try to read this manual in one sitting.
                                              Information about some aspects of ALS
Instead, familiarize yourself with its
                                              changes from time to time, and many
content and then read sections at your own
                                              people want to be kept up-to-date with the
pace or as they become relevant to you.
                                              latest information that may affect them.
This is not a self-help book or a do-it-
                                              This information manual is in a format
yourself manual on how to take care of
                                              that can be updated. Any suggestions for
yourself with ALS. Reading it cannot
                                              updates from readers are welcome. Please
replace consultation with your doctor and
                                              send your suggestions to the ALS Society
with professionals who have a special
                                              of Canada.
interest in caring for patients with ALS.
                                              See Section 14 for the Society’s address and
We hope that reading this manual will
                                              fax number.
provide you with strength and
encouragement. Strength from                  The information for this publication has
understanding the specifics of the            come from sources which we deem
challenges you may need to face.              reliable and is submitted for general
Encouragement from knowing that many          information purposes only.
patients have found that even as the
disease has taken its physical toll, their    The ALS Society of Canada disclaims any
spirits have remained firm, surrounded by     liability for the accuracy thereof, and does
the love of their families and those who      not intend to disseminate either medical or
care for them.                                legal advice. Throughout this publication,
                                              people with ALS are advised to consult
                                              with their healthcare professionals.




                                                                                             ALS                 SLA
                                                                                             SOCIETY OF CANADA
                                                                                             Maladie Lou Gehrig’s Disease

                                                                                             1(800)267-4257
                                                                                             http://www.als.ca
                                                                                             alscanada@als.ca


v 1.0                                                                                        PAGE 1
 SECTION         1                                        LIVING WITH ALS
                                                                                                      WHAT IS ALS?

                                GENERAL DESCRIPTION                             TYPES OF ALS
    WHAT IS ALS?
GENERAL DESCRIPTION             Amyotrophic Lateral Sclerosis:                  The most common form of ALS is called
                                                                                Sporadic ALS. In addition, about 5% of
           TYPES OF ALS                   A    =   absence of                   patients have an inherited variety called
                                       myo     =   muscle                       Familial ALS. Until recently, an
      SYMPTOMS, SIGNS
       AND DIAGNOSIS                trophic    =   nourishment                  abnormally high incidence of ALS was
                                    Lateral    =   side (of spine)              observed in the Western Pacific (Guam,
               RESEARCH            Sclerosis   =   hardening                    Kii Peninsula of Japan, Papua New
           DRUG TRIALS                                                          Guinea). As a result of extensive research
                                Dr. Jean-Martin Charcot, a French
                                                                                into Guamanian ALS, it is now thought
                                neurologist, published the first full account
                                                                                that these high incidence rates were due to
                                of this disease in 1874. ALS, also known
                                                                                specific dietary toxins (for example,
                                as Charcot’s Disease, Lou Gehrig’s
                                                                                Aluminium, cycasin). With the
                                Disease and Motor Neuron Disease, is a
                                                                                Westernization of this region, and the
                                major neurological disorder. In Canada it
                                                                                reduced dependency on traditional
                                is the most common cause of neurological
                                                                                foodstuffs, dramatic declines in the
                                death on an annual basis.
                                                                                incidence of ALS in this region have
                                ALS destroys motor neurons that are an          occurred.
                                important link in the nervous system, and
                                through which the brain controls the
                                voluntary muscles throughout the body.

                                Leg and foot muscles are controlled by
                                motor neurons in the lower spinal cord.
                                Arm, hand and finger muscles are
                                controlled by motor neurons in the upper
                                spinal cord. Speaking, swallowing and
                                chewing are controlled by motor neurons
                                in the brain stem.

                                ALS does not affect the five senses of
                                sight, hearing, taste, smell and touch, nor
                                does it normally affect the mind, eye
                                muscles, heart, bladder, bowel, or sexual
                                muscles. There is no possibility that ALS is
                                contagious.

                                ALS strikes about 6 people per 100,000 per
                                year. Between 1,500 and 2,000 people in
                                Canada currently have ALS. Although it
                                most commonly occurs between the ages
                                of 40 and 70, it can also occur in older
 ALS              SLA           people, and rarely in teenagers.
 SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                    PAGE 2                                                                                              v. 1.0
LIVING WITH ALS                                                                                      SECTION          1
WHAT IS ALS?

SYMPTOMS, SIGNS AND DIAGNOSIS                 As such, clinicians familiar with ALS
                                              usually see the following signs of lower
                                                                                              WHAT IS ALS?
Symptoms are what you experience, while       and upper motor neuron degeneration:            GENERAL DESCRIPTION
signs are what your doctor can see. The
early symptoms of ALS may seem rather         Lower Motor Neuron Degeneration                 TYPES OF ALS
vague. They can include tripping, dropping                                                    SYMPTOMS, SIGNS
things, slurred or “thick” speech, and          • muscle weakness and atrophy                 AND DIAGNOSIS
muscle cramping, weakening, and                 • involuntary contraction of
                                                                                              RESEARCH
twitching. Some people with these early           muscle fibres
symptoms may tend to assume that they           • muscle cramps                               DRUG TRIALS
are normal signs of aging. As the disease
                                                • weakened reflexes
progresses, the muscles of the trunk of the
body are affected. Weakness of the              • flaccidity (decreased muscle tone)
breathing muscles develops slowly over          • difficulty swallowing
months or years.                                • disordered articulation
For some people, the muscles for speaking,      • shortness of breath at rest
swallowing or breathing are the first to be
affected. This is known as Bulbar ALS.        Upper Motor Neuron Degeneration
The term “bulbar” refers to the motor           • muscle stiffness or rigidity
neurons located in the bulb region of the
                                                • emotional lability (decreased
brain stem, that control the muscles used
                                                  ability to control emotions)
for chewing, swallowing, and speaking.
ALS symptoms, and the order in which
they occur, vary from one person to           Some may assume that these are signs of
another.                                      normal aging. Over time, as muscles
                                              continue to weaken, and the weakening
The rate of muscle loss can vary              spreads throughout the body, it becomes
significantly from person to person, with     more apparent that the cause is ALS.
some patients having long periods with
very slow degeneration. Although the          In addition to a physical examination,
average life expectancy of newly diagnosed    people are often given an EMG, blood
people is between 3 and 5 years, 20% live     tests, a MRI (Magnetic Resonance
more than 5 years, and 10% live more than     Imaging), and other tests to search for the
10 years. Being a progressive disease, ALS    presence of other diseases that can look
may spread throughout the body over time,     like ALS.
and at some point in this process, may
involve the muscles required for breathing.   Doctors who do not usually see people
                                              with ALS may be reluctant to suggest it as
ALS can be difficult to diagnose in the       a possible diagnosis. They normally refer
early stages, when symptoms may mimic         the person to a neurologist. Telling
other conditions. However, there are          someone and his or her family that the
clinical signs that can be indicative of a    person may have ALS requires a sensitive,
wasting of motor neurons in either the        understanding and compassionate manner.         ALS                 SLA
upper or lower portion of the spine.          People should also be told about the            SOCIETY OF CANADA
                                              disease, current research and drug trials, as   Maladie Lou Gehrig’s Disease
                                              well as about support services and the
                                                                                              1(800)267-4257
                                              ALS Society in their province.                  http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 3
 SECTION         1                                      LIVING WITH ALS
                                                                                                     WHAT IS ALS?

                                RESEARCH                                      In an alternate hypothesis, the ingestion of
    WHAT IS ALS?                                                              the false sago palm cycad circinalis or its
GENERAL DESCRIPTION             It has been more than a century since the     utilization as a medicinal poultice has also
                                first complete description of ALS by Dr.      been incriminated as an environmental
           TYPES OF ALS         Charcot. Since then, although theories        toxin that may have brought about the
      SYMPTOMS, SIGNS           with regard to the cause of ALS have been     abnormally high rates of ALS in the
       AND DIAGNOSIS            numerous, very few have found validation      Western Pacific. Regardless, with dietary
                                through scientific study. While several       and medicinal changes brought about by
               RESEARCH                                                       the Westernization of this culture, the
                                current hypotheses for the cause of ALS
           DRUG TRIALS          exist, researchers now believe that ALS       incidence rates of ALS in the Western
                                can be a disease of multiple causes sharing   Pacific have now declined to levels
                                a common pathway leading to the               approaching North American rates. The
                                destruction of the motor neurons. By          importance of these observations has been
                                understanding the mechanisms by which         to demonstrate that, in a susceptible
                                this common pathway is triggered, we will     population, certain dietary toxins can
                                ultimately understand ALS. Through such       induce ALS. Although to date no evidence
                                understanding, desperately needed             for such a process has been found for
                                therapeutic options will be developed. In     sporadic ALS, worldwide epidemiological
                                this section, we will highlight these         studies consistently point towards
                                hypotheses and their relevance to our         environmental triggers as being important.
                                understanding of ALS.
                                                                              Inherited Variants of ALS
                                Environmental Hypothesis                      In 5 to 10% of people with ALS the
                                The exceedingly high incidence rates of       disease is inherited. Approximately 15% of
                                ALS observed during the mid 40s and 50s       patients with dominant familial ALS have
                                on the island of Guam in the Western          abnormalities in a key cellular enzyme
                                Pacific and the Kii Peninsula of Japan        termed the copper/zinc superoxide
                                have been attributed to dietary toxins.       dismutase (SOD-1). Although initially
                                Individuals living in these regions were      postulated that this abnormality alone
                                found to be calcium-deficient and because     would be sufficient to cause ALS, it is
                                of the hyperparathyroid state induced as a    becoming increasingly clear that
                                normal response to this deficiency, coupled   abnormalities in SOD-1 function alone
                                with significantly elevated soil and water    may be insufficient to produce ALS in all
                                aluminium levels, a state of aluminium        individuals carrying this genetic mutation.
                                toxicity was induced. That aluminium          In addition to SOD-1 abnormalities,
                                exposure in this specific population could    genetic abnormalities in the neurofilament
                                bring about ALS has been subsequently         genes have been discovered, as have
                                demonstrated in animal models utilizing       impairments in cell surface receptors in
                                aluminium supplementation of calcium          specific forms of motor neuron disease
                                and magnesium-deficient diets to produce      that can mimic ALS (for example
                                a syndrome very similar to ALS.               Kennedy’s syndrome).
 ALS              SLA
 SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                    PAGE 4                                                                                             v. 1.0
LIVING WITH ALS                                                                                           SECTION          1
WHAT IS ALS?

Free Radical Hypothesis                            Researchers in Vancouver have proposed
                                                   that ALS, similar to other
                                                                                                   WHAT IS ALS?
All cells generate toxic metabolites (think        neurodegenerative disorders such as
of these as the “exhaust” from your car                                                            GENERAL DESCRIPTION
                                                   Parkinson’s and Alzheimer’s diseases,
and the consequences to yourself and your          possesses a prominent local immune              TYPES OF ALS
passengers should your car not be properly         response, mediated by microglial cells, that
vented). In the cell, these “exhausts” (“free                                                      SYMPTOMS, SIGNS
                                                   adds further to the injury of motor             AND DIAGNOSIS
radicals”) are a destructive form of oxygen        neurons and may be responsible for the
that can also be used by the cell under            disease continuing.
                                                                                                   RESEARCH
normal circumstances to fight disease.                                                             DRUG TRIALS
However, an overproduction of free
                                                   Hypothesis of Neurotrophic Factor
oxygen radicals can result in cell damage
and death. As might be expected, several           Deficiency
defenses against such a process exist. A           These important factors for the growth and
major one is the superoxide dismutase              maintenance of motor neurons in humans
enzyme (SOD-1). As discussed above,                have been shown to enhance motor
mutations in the SOD-1 gene have been              neuron survival in mice with a variety of
postulated to allow excessive free radical         motor neuron disorders. Although it is not
production and the destruction of the              clear how deficiencies of neurotrophic
neuron. Canadian researchers have                  factors may affect human motor neurons,
documented the existence of excessive              several attempts have been made to
levels of proteins damaged by free oxygen          determine whether neurotrophic factors
radicals within neurons in ALS, suggesting         can slow the rate of progression of ALS
that either the neuron synthesizes excessive       by first testing these agents in animal
levels of free radicals, or that it is incapable   models of the disease. Although initial
of “venting” those that are normally               clinical trials with ciliary neurotrophic
produced.                                          factor (CNTF) or brain-derived
                                                   neurotrophic factor (BDNF) failed to alter
Immunological Hypothesis                           the course of ALS, this may have been due
                                                   to a failure of these neurotrophic factors to
There has, to date, been very little evidence
                                                   reach the damaged motor neurons.
to suggest that ALS can be due to a
                                                   Current clinical trials in Edmonton,
primary abnormality of the immune
                                                   London, and Montreal are attempting to
system as we might classically think of it.
                                                   circumvent this problem, by using either
However, the nervous system possesses its
                                                   direct application of the neurotrophic
own capacity to mount an immune
                                                   agent to the spinal fluid, or by
response to the injury of neurons. The
                                                   administering large quantities of the agent
response can be either advantageous in
                                                   systemically (for example, by injections
removing the debris from damaged
                                                   like those given for diabetes). In addition
regions, or it can be harmful in inducing
                                                   to these studies, novel neurotrophic factors
even further damage. Recently,
                                                   continue to be described that will impact
investigators have become increasingly
                                                   on the survival of motor neurons (for
aware of the extent of the immunological
response of the central nervous system in
                                                   example, insulin-like growth factor [IGF-
                                                   1], cardiac derived neurotrophic factor,
                                                                                                   ALS                 SLA
ALS.                                                                                               SOCIETY OF CANADA
                                                   etc). Combination studies are also being
                                                                                                   Maladie Lou Gehrig’s Disease
                                                   developed in Hamilton.
                                                                                                   1(800)267-4257
                                                                                                   http://www.als.ca
                                                                                                   alscanada@als.ca


v 1.0                                                                                              PAGE 5
 SECTION         1                                     LIVING WITH ALS
                                                                                                      SECTION 1

                                Hypothesis of altered                        Excitatory Amino Acid
    WHAT IS ALS?                neurofilament metabolism                     Toxicity Hypothesis
GENERAL DESCRIPTION             A particular hallmark of the damage to       Abnormalities in the handling of
                                motor neurons in ALS is the accumulation     excitatory amino acids by the nervous
           TYPES OF ALS         of neurofilaments – key neuronal proteins    system, particularly glutamate, may be
      SYMPTOMS, SIGNS           believed to be responsible for maintaining   critical to the occurrence of ALS. Through
       AND DIAGNOSIS            the normal neuronal structure and shape.     damage to the normal “transporter”
               RESEARCH         Through research funded by the ALS           mechanisms by which glutamate is
                                Society of Canada, it has become clear       removed from the nervous system,
           DRUG TRIALS          that abnormalities in the metabolism of      excessive glutamate accumulates. As
                                neurofilaments can be critical to the        demonstrated by researchers in Vancouver,
                                production of a disease bearing all of the   this excessive glutamate can directly
                                hallmarks of ALS. Such studies have been     damage motor neurons.
                                undertaken through the use of transgenic
                                models of altered neurofilament
                                expression and through aluminium-
                                induced neurotoxicity. Using mouse
                                models in which individual neurofilament
                                proteins have been either removed
                                completely, or forced to be present in
                                excess, researchers in Montreal have
                                shown that a simple alteration in the
                                amount of neurofilament within the
                                neuron can lead to an ALS-like syndrome.
                                Others in Montreal have shown that
                                interfering with the way in which
                                neurofilaments interact with each other in
                                cell cultures produces aggregates of
                                neurofilament similar to those observed in
                                ALS. In London, researchers have shown
                                that changing the interactions between
                                neurofilaments and other key proteins can
                                similarly lead to ALS.




 ALS              SLA
 SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                    PAGE 6                                                                                          v. 1.0
LIVING WITH ALS                                                                                      SECTION          1
 WHAT IS ALS?

DRUG TRIALS                                   R-metHuBDNF
                                              (recombinant brain-derived
                                                                                              WHAT IS ALS?
The preceding discussion highlights just      neurotrophic factor)                            GENERAL DESCRIPTION
some of the exciting advances that have       While the initial study of BDNF
occurred in the last decade in our            (r-metHuBDNF) administered                      TYPES OF ALS
understanding of ALS. A tremendous and        subcutaneously failed to slow the               SYMPTOMS, SIGNS
long overdue explosion of interest in the     progression of ALS, this may have been          AND DIAGNOSIS
design of novel treatments of ALS has         related to an inability of the drug to cross    RESEARCH
also accompanied this unparalleled growth     the blood-brain barrier. Two trials are
of knowledge. This includes an                attempting to overcome this barrier. The        DRUG TRIALS
understanding of the factors that are         first involves the intrathecal administration
required to keep motor neurons surviving,     of BDNF.
or to enhance their ability to repair
themselves. Although there have been a        Using an implantable pump BDNF can be
number of failed trials in the treatment of   administered directly to the cerebrospinal
ALS over the last two decades, several        fluid. This route of administration allows
medications have shown promise. The           for smaller, better tolerated, amounts of
following represents a summary of these       BDNF to be given directly to the site
medications.                                  where it is needed the most. Stable levels
                                              of the drug can be established. The
Neurotrophic Factors                          implantation of the pump is safe and well
                                              tolerated. The objective of the current
Neurotrophic factors are naturally            study is to demonstrate a 25% reduction in
occurring proteins required for the           the rate of progression in pulmonary
development, maintenance and maturation       decline. The second trial involves the
of neurons. As discussed earlier, there is    administration of high-dose r-
evidence to suggest that these agents will    metHuBDNF subcutaneously. Enrolment
retard the rate of neuron loss in models of   commenced in the 1st quarter of 1999 and
spontaneous motor neuron degeneration.        is restricted to American sites.
In models of neuronal injury, these agents
have the capability of enhancing the rate     GDNF (glial derived neurotrophic
of recovery. It has been postulated,          factor)
therefore, that this class of agents may
                                              This is another naturally occurring trophic
promote neuronal recovery and enhance
                                              factor that has been shown to be protective
survival in ALS. Several agents have been,
                                              and survival promoting to motor neurons.
or are currently, under consideration.
                                              This trial again involves the direct
                                              administration of the medication to the
                                              CSF. But in contrast to that of the
                                              intrathecal BDNF trial, this involves
                                              implantation of a catheter directly into the
                                              ventricles of the brain, thereafter followed
                                              by administration of the drug through an
                                              access port. The current study involves a       ALS                 SLA
                                              small number of patients in a phase I           SOCIETY OF CANADA
                                              study designed to establish the safety of       Maladie Lou Gehrig’s Disease
                                              the drug and of the route of
                                                                                              1(800)267-4257
                                              administration. Enrolment is complete.
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 7
 SECTION         1                                       LIVING WITH ALS
                                                                                                       WHAT IS ALS?

                                Myotrophin                                      Small molecule neurotrophic agents
    WHAT IS ALS?
                                The final decision regarding the use of         Amgen and Guilford Pharmaceuticals are
GENERAL DESCRIPTION             myotrophin (insulin-like growth factor;         in the process of developing a new class of
           TYPES OF ALS         rhIGF-1) by the Food and Drug                   neurotrophic agents defined as small
                                Administration has not been rendered. On        molecule neurotrophic agents. These are
      SYMPTOMS, SIGNS           May 12th, 1998, the FDA issued an               orally active neurotrophic agents that will
       AND DIAGNOSIS
                                “approvable” letter to Cephalon, the            cross the blood–brain barrier, and they
               RESEARCH         parent company, indicating the additional       possess activity similar to nerve growth
           DRUG TRIALS          steps that were required by Cephalon in         factor (NGF), BDNF and GDNF. They
                                order for myotrophin to gain approval. To       are expected to have a direct effect on
                                date, these “approvable” criteria have not      injured neurons, and promote repair. Trials
                                been met. Although rhIGF-1 may slow             to date have been restricted to preclinical
                                progression of functional impairment and        trials.
                                the decline in health-related quality of life
                                in ALS patients, only one of two valid          Riluzole
                                randomized clinical trials of rhIGF-1
                                demonstrated results favouring                  Although not yet approved for release in
                                improvement in mortality, rate of clinical      Canada, Riluzole has been approved for
                                decline, and quality of life. In the North      release in the United States and several
                                American trial, the numbers needed to           other countries as a treatment for ALS.
                                treat (NNT) to progress less than 20 points     Riluzole appears to improve survival
                                on ALS disability scale over 9 months           although the degree of improvement is
                                using 0.1 mg/day rhIGF-1 subcutaneously         small. In the pivotal phase III study, the
                                was 6, and to survive 30 months using           improvement in survival ranged from 4.9%
                                0.05 or 0.1mg/day subcutaneously was 8.         (50 mg dose) to 7.4% (200 mg daily dose),
                                This suggests that this trophic factor may      with little difference between the 100 mg
                                have a significant beneficial effect in ALS.    and 200 mg dosage. The number needed
                                However, only 53% of patients completed         to treat value (NNT representing the
                                the North American study protocol and           number of patients needing treatment
                                the results were highly variable. In            before a statistically significant benefit is
                                contrast, the European protocol failed to       observed) ranged from 20 to 14,
                                show a significant difference in either         respectively, although the 95% confidence
                                measure. These last two issues are at the       intervals were very broad (approaching
                                heart of the controversy surrounding this       infinity). This suggested a wide range of
                                drug.                                           responses. In a retrospective study, utilizing
                                                                                novel staging techniques of ALS, there is a
                                SR57746A                                        suggestion of a prolongation of time spent
                                                                                within a stage of ALS at which functional
                                 A phase III study of SR 57746A,                benefit would have been gained. This
                                sponsored by Sanofi Pharmaceuticals, is         remains to be further studied through
                                nearing completion. This oral therapeutic       prospective studies. Recently, it has been
                                may work at a number of different levels,       demonstrated by Canadian investigators,
 ALS              SLA           by increasing the synthesis of a number of      that the use of Riluzole may retard the rate
 SOCIETY OF CANADA              neurotrophic factors. It is anticipated that    of neuronal loss as measured by magnetic
 Maladie Lou Gehrig’s Disease   the study will be completed by December         resonance (MR) spectroscopy analysis.
                                1999, with the analysis being completed by
           1(800)267-4257
           http://www.als.ca    the summer of the year 2000. There is no
            alscanada@als.ca    information available at this time.



                    PAGE 8                                                                                                v. 1.0
LIVING WITH ALS                                                                                          SECTION          1
WHAT IS ALS?

Novel anti-glutamergic agents                    explored actively. In a naturally occurring
                                                 mouse model of motor neuron disease, the
                                                                                                  WHAT IS ALS?
Two American universities are currently          initial studies demonstrated a 50% increase
participating in a small 50-patient study of                                                      GENERAL DESCRIPTION
                                                 in life span.
a glutamate antagonist (LY300164) versus                                                          TYPES OF ALS
placebo in the treatment of ALS. No              Being told that you have ALS is a shock
results are as yet available from this.          for which most people are totally                SYMPTOMS, SIGNS
                                                                                                  AND DIAGNOSIS
                                                 unprepared. When a healthy person starts
Gabapentin (Neurontin)                           to have problems with muscle weakness,           RESEARCH
                                                 and ultimately learns that he or she may         DRUG TRIALS
It is anticipated that the results of the        have a terminal disease, shock is normally
current phase III, placebo-controlled study      part of the first reaction as are many other
of Neurontin, currently being conducted          feelings such as anger, frustration,
by the Western ALS Research Consortium           disbelief, and denial. These emotions are
(WALS), will be completed and an                 normal for both the individual and their
analysis will be presented at the 1999           family.
International ALS/MND symposium.
The focus of the study is to attempt to          Everyone must find his or her own way of
confirm a previously observed (but not           dealing with grief. Communication is
statistically significant) reduction in the      especially important. Sharing emotions
rate of arm muscle weakening in people           and feelings normally helps, as does having
with ALS.                                        empathy and respect for the way others are
                                                 handling this shock.
Creatine therapy
                                                 Denial, in various forms, is a common
Although the exact mechanism by which            reaction to the diagnosis of a terminal
this agent may work is not entirely clear, it    disease. If a person wishes to deny his or
is likely that Creatine will function            her condition, respect his or her opinion
predominantly as an antioxidant, and in          and be ready to help when he or she wants
the presence of steroids, may also increase      help. Often it is the family that suffers as a
muscle bulk. Based on a study                    result of an individual’s denial. Denial can
demonstrating a delay in the onset of            be an effort to keep control of managing
disease in mutant SOD1 mice, a placebo-          one’s life. On the other hand, “meeting the
controlled trial of Creatine is currently        challenge” can lead to managing life
being formulated.                                within the realities of the disease, and
                                                 taking action can minimize the effect of
Gene Therapy                                     the symptoms.

There are currently no studies evaluating        Anger may also be part of your response.
the efficacy of gene therapy in ALS.             You may feel angry with medical
However, purine analogues have the ability       personnel because they cannot find a cure.
to modify gene expression of proteins that       You may also be angry with members of
are involved in nerve regeneration. A novel      your family because they are not ill and
class of such agents has been developed by
NeoTherapeutics. These agents are
                                                 cannot really understand what it is like to
                                                 face ALS. You may later respond by
                                                                                                  ALS                 SLA
                                                                                                  SOCIETY OF CANADA
currently in preclinical studies. In addition,   feeling withdrawn and depressed, as you
                                                                                                  Maladie Lou Gehrig’s Disease
the use of adenovirus vectors to deliver the     face up to all of the changes that will occur
genes encoding neurotrophic factors              in your life.                                    1(800)267-4257
directly to injured motor neurons is being                                                        http://www.als.ca
                                                                                                  alscanada@als.ca


v 1.0                                                                                             PAGE 9
SECTION         1              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 10                     v. 1.0
LIVING WITH ALS          SECTION          1
NOTES




                  ALS                 SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 11
SECTION         1              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 12                     v. 1.0
LIVING WITH ALS                                                                                       SECTION          2
COPING WITH ALS

COPING WITH DIAGNOSIS                          A person’s life with ALS can be looked
                                               upon as a slow death, or as an opportunity
                                                                                               Coping with ALS
Many people with a serious disease feel a      for enrichment. The choice is yours.            COPING WITH DIAGNOSIS
conflict between keeping a positive attitude   If you choose enrichment, it can take
to ward off disease while knowing that at      many forms. The choices are very                COPING STRATEGIES FOR
                                                                                               THE PERSON WITH ALS
some time they will have to accept that        personal. You can develop closer
they do have a serious disease. When the       relationships with family and friends;          COPING STRATEGIES
existence of disease is acknowledged,          make new friends with others who are            FOR FAMILY MEMBERS
“fighting it” is another attitude some         sharing your experiences and with many          COPING STRATEGIES
people adopt. Others prefer to avoid a win-    other people who are involved with ALS;         FOR CAREGIVERS
or-lose approach, and, instead, think of       learn about computers and how to
                                                                                               FRIENDS AND FAMILY
themselves and their bodies as one, a team     communicate with others through the
that will work together to deal with life’s    Internet and other similar services; learn to   HELPING CHILDREN
                                               appreciate other things in the world            TO COPE
needs, day by day.
                                               around you, things that you previously
A terminal disease has the power to            took for granted; take time to read and
strengthen healthy family relationships, or    listen to music and learn more about
shatter already weakened ones. And yet it      spiritual awareness. The list can go on and     The ultimate tragedy
can unpredictably bring out the best in        on. It won’t likely include everything          is to die without
some people and in others, awaken              you’ve always wanted to do, but it can          discovering the
emotions they can’t handle. Some people        include enough to give you a full and           possibilities of
                                                                                               full growth.
are uncomfortable discussing death, and        satisfying life. It all depends on your
others dislike witnessing emotional            attitude and imagination.                       The approach of death
displays of grief. The ALS Society of                                                          need not be denial
                                               Hope, faith, love, and a strong will to live    of that growth.
Canada has published a helpful booklet on
this subject, Coping With Grief, which is      offer no promise of immortality, only                       – Head First,
                                               proof of our uniqueness as human beings,             The Biology of Hope
available through the ALS Society in your                                                       Norman Cousins, Dutton
region.                                        and the opportunity to experience full
                                               growth, even under the grimmest
COPING STRATEGIES FOR                          circumstances. The clock provides only a
THE PERSON WITH ALS                            technical measure of how long we live.
                                               Far more real than the ticking of time is
God grant me the serenity                      the way we open up the minutes and invest
to accept the things I cannot change,          them with meaning. Death is not the
the courage to change the things I can,        ultimate tragedy in life.
and the wisdom to know the difference.

Social workers, psychologists, counsellors,
and support groups can be very helpful.
You can learn from those who are familiar
with what you are going through, share
understanding with others who have the
same problems as you, and learn how
people with more advanced ALS are
                                                                                               ALS                 SLA
dealing with problems you might have in                                                        SOCIETY OF CANADA
                                                                                               Maladie Lou Gehrig’s Disease
the future. The ALS Society, hospices, and
other organizations provide this type of                                                       1(800)267-4257
support service to many terminally ill                                                         http://www.als.ca
patients and their families.                                                                   alscanada@als.ca


v 1.0                                                                                          PAGE 13
   SECTION         2                                      LIVING WITH ALS
                                                                                               COPING WITH ALS

                                  COPING STRATEGIES                             COPING STRATEGIES
   Coping with ALS                FOR FAMILY MEMBERS                            FOR CAREGIVERS
COPING WITH DIAGNOSIS
                                  It is important to be aware that members      Members of your immediate family will
COPING STRATEGIES FOR             of your family and your friends are           likely become involved in your daily care
  THE PERSON WITH ALS             experiencing emotional reactions to your      and assistance. Most people with ALS
    COPING STRATEGIES             disease. They may feel guilty that you have   remain in the home for as long as possible,
   FOR FAMILY MEMBERS             ALS and they are healthy. They may also       and the demands upon family members
    COPING STRATEGIES             be short-tempered because of the extra        can be great. Your primary caregivers, such
      FOR CAREGIVERS              daily responsibilities that they face –       as your spouse, partner or grown children,
                                  banking, raising children with less help      may find that your care, especially in the
    FRIENDS AND FAMILY
                                  from you, more chores – all this in           later stages of the disease, is taking up
     HELPING CHILDREN             addition to the care they give you. They      most of their lives. It is natural for them to
              TO COPE             may feel that it is not fair and then feel    want to give all that they can to help you,
                                  guilty about reacting this way.               but there should be limits to self-sacrifice.

                                  Keeping the lines of communication open       Caregivers must continue to have lives of
                                  is the best way to work out these feelings.   their own. Time in the company of friends
                                  Talk openly to your family about how you      or family who are not sick, in the pursuit
                                  feel and encourage them to share their        of hobbies or activities in addition to care
                                  feelings with you. If you find this hard to   giving, are important refreshers. They
                                  do, you may find it useful to talk with a     allow the caregiver to recover from the
                                  social worker or other member of your         stresses of care giving and make him or
                                  ALS team. He or she may be able to help       her a better, more cheerful helper. Do not
                                  you and your family to solve                  hesitate to ask other family members to fill
                                  communication problems. It is most            in for your primary caregiver while he or
                                  important that you have someone to listen     she takes regular breaks or, if necessary,
                                  who will not be upset by what you are         arrange for paid help. A social worker may
                                  saying. The person you need may be one        be able to tell you about what types of
                                  of your healthcare professionals, a friend    help and funding arrangements are
                                  or a family member.                           available .

                                                                                The needs of a caregiver tend to take a
                                                                                back seat to the needs of the sick person. It
                                                                                is hard to have a serious illness, but it is
                                                                                also difficult to care for someone with a
                                                                                serious illness. Most people, sick or well,
                                                                                feel a combination of some or all of the
                                                                                following emotions at different times when
                                                                                dealing with illness. Most importantly, no
                                                                                one should feel guilty about any of these
                                                                                feelings. They are perfectly normal and to
                                                                                be expected.
   ALS              SLA
   SOCIETY OF CANADA
   Maladie Lou Gehrig’s Disease

             1(800)267-4257
             http://www.als.ca
              alscanada@als.ca


                     PAGE 14                                                                                              v. 1.0
LIVING WITH ALS                                                                                           SECTION          2
COPING WITH ALS

Initial emotions                                   FRIENDS AND FAMILY
                                                                                                   Coping with ALS
• Curiosity
                                                   Changes will take place for everyone.           COPING WITH DIAGNOSIS
• Love                                             Relationships with family and friends,
                                                   children and colleagues, will undergo a
                                                                                                   COPING STRATEGIES FOR
• Closeness and openness to others                                                                 THE PERSON WITH ALS
• Hope                                             transition period. Close friends and
                                                   relatives, including children, should be told   COPING STRATEGIES
• Disbelief                                        the truth about your ALS. For most              FOR FAMILY MEMBERS
• Loss                                             people, not knowing what is happening is        COPING STRATEGIES
• Anticipatory grief                               worse than knowing the truth. Children in       FOR CAREGIVERS
                                                   particular imagine frightening things if        FRIENDS AND FAMILY
• Guilt
                                                   they can see that someone is sick and no
• Erosion of trust                                 one talks about it in front of them.            HELPING CHILDREN
                                                                                                   TO COPE
• Denial                                           Knowing what is happening also makes it
• Responsibility                                   easier for people to offer support and to
                                                   help. Don’t be afraid to ask for help when
• Long-term emotions                               it is not offered; people often don’t know
• Persistence                                      what to say or what would be helpful.
• Hope                                             Most people, especially relatives, are glad
                                                   to be of assistance.
• Love
• Appreciation of the value of life                Telling others about your situation often
  and of others                                    means that you will discover who your
• Sadness                                          true friends are. Some people find it very
                                                   hard to be around sickness at any time,
• Guilt                                            and others simply do not want to commit
• Loneliness                                       themselves to helping in a serious
• Jealousy                                         situation. Expect that some people may
                                                   break off relationships. Although this is
• Annoyance
                                                   hurtful, it should not be taken personally.
• Feeling trapped                                  Most people will try to help and be
• Feeling overwhelmed                              supportive.

                                                                  – ALS: Strategies for Living
Remember, everyone has the right to:                  ALS Society of British Columbia, 1993
• put him or herself first
• sometimes make mistakes
• have his or her own opinions and
  convictions
• change his or her mind or decide on
   a different course of action
• protest unfair treatment or criticism                                                            ALS                 SLA
                                                                                                   SOCIETY OF CANADA
                   – ALS: Strategies for Living,                                                   Maladie Lou Gehrig’s Disease
        ALS Society of British Columbia, 1993
                                                                                                   1(800)267-4257
                                                                                                   http://www.als.ca
                                                                                                   alscanada@als.ca


v 1.0                                                                                              PAGE 15
   SECTION         2                                       LIVING WITH ALS
                                                                                                COPING WITH ALS

                                  HELPING CHILDREN                               Talking to Children
   Coping with ALS
                                                                                 In dealing with children experiencing
COPING WITH DIAGNOSIS             Although ALS is not primarily a disease of
                                                                                 sickness and big changes in their families
                                  the young, it sometimes happens that the
COPING STRATEGIES FOR                                                            and lives, it is necessary to explain
  THE PERSON WITH ALS             family of the person with ALS includes
                                                                                 everything as fully as possible. Because
                                  young children. It is easy to forget that
    COPING STRATEGIES                                                            adults tend not to explain to children what
                                  they need to know how the disease will
   FOR FAMILY MEMBERS                                                            is happening, children often blame
                                  affect you - a loved parent or grandparent
                                                                                 themselves for the sadness in the house
    COPING STRATEGIES             and the whole family. It is important that
      FOR CAREGIVERS                                                             and feel very guilty. They may want to
                                  your family members and counsellors
                                                                                 know what exactly it is that the sick person
    FRIENDS AND FAMILY            helping your family take time to work
                                                                                 has, whether they can catch it too, what
                                  through the problems children may have in
     HELPING CHILDREN                                                            will happen to the sick person, and
                                  coming to terms with ALS in their family.
              TO COPE                                                            whether that person will die. These
                                  The feelings of adults and children in         questions should be answered as positively
                                  upsetting situations are quite similar.        as possible. For example, instead of telling
                                  Anger, helplessness, fear, hope, and despair   the child that a person is very sick or
                                  are commonly experienced. Children,            dying, something like: “Well, they are not
                                  however, have fewer ways of expressing         doing very well right now, but the doctors
                                  their emotions and dealing with them.          say that there’s lots that we can do. We
                                  Younger children tend not to express           can.....” gives hope without being
                                  themselves in words and will act out their     dishonest.
                                  feelings. Older children can find it hard to
                                                                                 Children should know that they can ask
                                  talk about how they feel, and teenagers
                                                                                 questions and that someone will try to
                                  often do not have friends with whom they
                                                                                 answer them. If their parents do not feel
                                  can talk about serious situations. Contact
                                                                                 able to talk to them, then a relative, friend,
                                  your regional ALS Society to request an
                                                                                 or doctor should make sure that the child
                                  ALS Canada booklet prepared for young
                                                                                 knows that someone is always available to
                                  people whose lives have been touched by
                                                                                 talk, not only about the illness but also
                                  ALS.
                                                                                 about anything that is worrying the child.
                                  For children of all ages, feelings can be
                                                                                 A major concern of children is often what
                                  expressed through emotional and
                                                                                 will happen if the sick person becomes
                                  behavioural changes. Children may have
                                                                                 very sick or dies. They may want to know
                                  questions that they may be afraid to ask
                                                                                 if they will have to move, who will look
                                  because everyone is so upset, and they do
                                                                                 after them, if they will have to give up
                                  not know how to find things out by
                                                                                 their favourite things, change schools, and
                                  themselves. Although many people feel
                                                                                 so on. They may be afraid to ask about
                                  that children should be protected from
                                                                                 these things, so they should be reassured
                                  sickness, this does not help the child. The
                                                                                 and told about any planned changes before
                                  child will know that something is wrong
                                                                                 a crisis interrupts their normal routine.
                                  and will get upset anyway.
   ALS              SLA                                                          Very young children may be upset by what
                                                                                 is going on, but will probably not be able
   SOCIETY OF CANADA
                                                                                 to understand your explanation. Giving
   Maladie Lou Gehrig’s Disease
                                                                                 them lots of hugs, love and attention helps
             1(800)267-4257                                                      until they are old enough to understand
             http://www.als.ca                                                   the situation in more detail.
              alscanada@als.ca


                     PAGE 16                                                                                                v. 1.0
LIVING WITH ALS                                                                                      SECTION          2
COPING WITH ALS

What the child knows                           Long-term ways to cope
                                                                                              Coping with ALS
People should talk as openly as possible       Once the shock of sickness has worn off,
around children, as secrets make everyone      children may begin to feel bored with all      COPING WITH DIAGNOSIS
uncomfortable. Children should be              the changes and their new responsibilities,    COPING STRATEGIES FOR
encouraged to talk about how they feel, to     embarrassed by someone who is sick and         THE PERSON WITH ALS
cry if they want to, and to talk about the     different from everyone else and frightened    COPING STRATEGIES
situation to anyone with whom they feel        because they don’t get the attention that      FOR FAMILY MEMBERS
comfortable. Expressing feelings makes         they’re used to. They may start to fight
                                                                                              COPING STRATEGIES
them easier to cope with and understand.       with family and friends and be rude and        FOR CAREGIVERS
Children may hide their feelings at times,     disobedient at home. Making sure that
but that does not mean that they do not        someone will pay adequate attention to         FRIENDS AND FAMILY
care, or are not feeling anything.             him or her is the best way to prevent this     HELPING CHILDREN
                                               behaviour. If parents cannot do so,            TO COPE
Children will often want to help, and this     grandparents, neighbours, and friends of
should be encouraged. Being useful will        the family may be good choices to involve
make them feel that they are involved and      in this area. Having someone there who
valuable. They do not have to help with        understands how they are feeling helps.
medical care: Doing more chores at home,
getting a paper route, or just reading and     Children can also find it hard to deal with
spending time with the sick person are all     the physical changes of sickness. Explain
possibilities.                                 to children that a change in appearance
                                               does not mean that the person’s feelings
Life Goes On                                   toward them have changed.

Life for the child should go on as normally    Serious illness can mean mood changes. If
as possible once they know what is             possible, children should be protected from
happening. Neighbours and parents of the       the emotional swings of the sick person.
child’s friends usually do not mind picking    Visiting at times when the sick person feels
children up from school or taking them to      good should be discussed. Children should
after-school activities. If money is a         also be allowed to go away with friends
problem, many community centres and            and family for a break.
schools offer inexpensive programs for
children. Children should be encouraged        Children, like adults, will continue to feel
to spend time with friends as well as at       upset and unhappy for long periods of
home. Allow the child to help in making        time in these situations. Not showing their
plans for weekends and free time; this is      feelings does not mean that they have
another way for them to feel useful.           none. They should not be told to “get over
Planning activities to include both the sick   it” or that they have been upset for too
person and children helps everyone to feel     long. Children should be able to express
better about lifestyle changes.                their feelings for as long as they want to.

                                                             – ALS: Strategies For Living
                                                  ALS Society of British Columbia, 1993
                                                                                              ALS                 SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 17
SECTION         2              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 18                     v. 1.0
LIVING WITH ALS          SECTION          2
NOTES




                  ALS                 SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 19
SECTION         2              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 20                     v. 1.0
LIVING WITH ALS                                                                                        SECTION          3
MANAGING YOUR SYMPTOMS AND TREATMENT

MEETING THE CHALLENGE                           Managing ALS is a continually changing
                                                challenge. Although it is a degenerative
                                                                                                Managing Your
Acceptance of ALS does not mean giving          disease, the rate at which muscles              Symptoms and
up. It should be the first step in making the   degenerate is unpredictable and varies          Treatment
most of life with ALS. There is much that       greatly. In some cases the disease seems to
can be done to help you to continue to live     have reached a plateau, while in others it      MEETING THE CHALLENGE
a productive and enjoyable life. Be positive    reaches a standstill for varying lengths of     TREATING ALS SYMPTOMS
but do not trivialize the situation. It is      time. Also, it can progress steadily at a
                                                rapid or slow rate. Whatever the rate of        ASSISTIVE EQUIPMENT
foolish to pretend that everything will be
fine or that ALS is not a very serious          muscle degeneration, you should remain          SEXUAL CONCERNS
condition.                                      as active as possible, without causing
                                                fatigue in affected muscles. It also helps to   ALTERNATIVE TREATMENT
                                                                                                OPTIONS
On the other hand, there is no need to          focus on what you can do, rather than on
dwell on the negative aspects of the            what you can’t do.                              MEDITATION
disease. It is true that 20% of people with                                                     ALTERNATIVE THERAPIES
ALS live more than five years and that          Although there are no known medications
nearly 10% live ten years or more. It is also   that will cure ALS, there are medical           DEALING WITH DYING
true that neurological research moves           treatments and several treatment therapies
steadily ahead and that no one knows            which can help patients maintain an active
when a breakthrough may occur. These            and independent life for as long as
facts give hope and hope is a crucial part      possible. These treatments should help the
of life. You must try to achieve a correct      well-being of both you and your family.
balance between hope and realism, which         Some treatments mentioned below are
is not an easy task.                            discussed in more detail in other sections
                                                of this manual. The success of most of
                                                these treatments depends on the will of the
                                                patient and their ability to communicate
                                                openly with their caregivers, therapists and
                                                doctors.




                                                                                                ALS                 SLA
                                                                                                SOCIETY OF CANADA
                                                                                                Maladie Lou Gehrig’s Disease

                                                                                                1(800)267-4257
                                                                                                http://www.als.ca
                                                                                                alscanada@als.ca


v 1.0                                                                                           PAGE 21
   SECTION         3                                      LIVING WITH ALS
                                                    MANAGING YOUR SYMPTOMS AND TREATMENT

                                  If you are a person with ALS who has           Contact your family doctor
      Managing Your               decided to take charge of your condition,
      Symptoms and                here are a few things you can consider         When doctors find out that one of their
         Treatment                doing.                                         patients has ALS, some might say that
                                                                                 there is nothing they can do to help. In
MEETING THE CHALLENGE                                                            fact, they probably can help but they don’t
                                  Register with the ALS Society
                                                                                 know it! Request that your family doctor
TREATING ALS SYMPTOMS                                                            refer you to an ALS Clinic and ALS
                                  As described in Section 14 of this manual,
   ASSISTIVE EQUIPMENT            the ALS Society provides various services      Rehab team. Your doctor will also be able
                                  to assist people with ALS. Let your local      to help you in other ways in the future,
     SEXUAL CONCERNS
                                  Society know that you are a person with        such as signing your application for a CPP
ALTERNATIVE TREATMENT             ALS in their area, and that you would like     disability pension, and Revenue Canada’s
              OPTIONS             to register with them and receive any          Disability Credit Certificate for your
              MEDITATION          helpful information they provide.              annual tax deduction, or by approving
                                                                                 your application for home care.
 ALTERNATIVE THERAPIES
                                  Apply for a Disabled Parking Permit            You may want to suggest that your doctor
   DEALING WITH DYING
                                  Your local city or provincial government       access the resources from the ALS Society
                                  will issue a disabled parking permit to        by requesting a copy of this manual or
                                  people with ALS. Get the application           other information the ALS Society makes
                                  through your local ALS Society or ALS          available. You need to discuss with your
                                  Clinic.                                        family doctor his or her availability to do
                                                                                 home visits as ALS progresses.
                                  Obtain a Medic Alert Bracelet
                                                                                 Contact your auto insurance provider
                                  A Medic Alert bracelet can be useful to tell
                                  others about your condition, in the event      If you don’t report your disability to your
                                  that you are unable to speak. This may be      insurance company, your auto insurance
                                  particularly useful for those with Bulbar      coverage may not be valid. Driving
                                  symptoms who are unable to speak or            sometimes requires fast foot and hand
                                  have slurred speech. For further               reactions. Enquire about a driving test
                                  information and an order form call the         service in your community that will certify
                                  Canadian Medic Alert Foundation at 416-        your current abilities for insurance
                                  696-0267 or 1-800-668-1507.                    purposes.




   ALS              SLA
   SOCIETY OF CANADA
   Maladie Lou Gehrig’s Disease

             1(800)267-4257
             http://www.als.ca
              alscanada@als.ca


                     PAGE 22                                                                                             v. 1.0
LIVING WITH ALS                                                                                     SECTION          3
MANAGING YOUR SYMPTOMS AND TREATMENT

TREATING ALS SYMPTOMS                          Build-up of Saliva
                                                                                             Managing Your
MOUTH AND THROAT
                                               People with Bulbar ALS commonly see a         Symptoms and
                                               reduced ability to manage saliva. This        Treatment
Speaking and Swallowing                        problem is even greater for those using
                                               tube feeding.                                 MEETING THE CHALLENGE
As mentioned before, when the onset
symptoms of ALS are speaking or                See Managing Saliva in Section 8.             TREATING ALS SYMPTOMS
swallowing problems, it is known as                                                          ASSISTIVE EQUIPMENT
Bulbar ALS. For other people with ALS,         Saliva and Mucus in the Lungs
these problems don’t generally occur until                                                   SEXUAL CONCERNS
the later phases of the disease.               If you are immobile, a problem can            ALTERNATIVE TREATMENT
                                               develop coughing up saliva and mucus.         OPTIONS
See Sections 7 and 9 of this manual for        You should see a doctor as soon as
                                               possible. There is a technique for freeing    MEDITATION
detailed comments and suggestions regarding
swallowing and speaking problems.              saliva and mucus from the throat and          ALTERNATIVE THERAPIES
                                               lungs, which involves lying on a slope with
People with Bulbar ALS are vulnerable to                                                     DEALING WITH DYING
                                               your head on the down side so that the
significant weight loss, and should            saliva flows down when the technique is
consider a feeding tube in the earlier         applied. This technique should be taught
phases of the disease. For those who lose      by a trained professional who is familiar
their ability to speak, there is now a range   with and can demonstrate the correct
of communication aids on the market.           position and procedures. This is especially
                                               the case if you are experiencing shortness
Choking                                        of breath. Also, a suction unit can help by
                                               removing saliva from the mouth, and
Those with swallowing problems are             preventing it from flowing back into the
vulnerable to choking. Because this can be     lungs.
very dangerous, get advice from your
doctor and other healthcare professionals
about how to deal with this problem.
                                               Coughing
                                               Coughing can occur for more than one
See Dealing With Swallowing Difficulties,
                                               reason. For example the lungs can clear a
Section 7.
                                               passage by automatically and unexpectedly
                                               expelling air, while at the same time
Drooling                                       irritations in the throat can also cause
People with Bulbar ALS often develop an        unexpected coughing.
excessive drooling problem. Your doctor        See Section 7 regarding coughing caused by
can offer various medications to reduce        tube feeding, and Section 10.
drooling.

See Section 8 for more details
regarding excess saliva.
                                                                                             ALS                 SLA
                                                                                             SOCIETY OF CANADA
                                                                                             Maladie Lou Gehrig’s Disease

                                                                                             1(800)267-4257
                                                                                             http://www.als.ca
                                                                                             alscanada@als.ca


v 1.0                                                                                        PAGE 23
   SECTION         3                                       LIVING WITH ALS
                                                    MANAGING YOUR SYMPTOMS AND TREATMENT

                                  DAILY LIVING                                    There are a large number of aids that can
      Managing Your                                                               help you to do the things you now find
      Symptoms and                Eating                                          difficult. It is essential that you consult
         Treatment                Continue to feed yourself as long as            your therapist before buying an aid in
                                  possible. However, if you feel too weak to      order to avoid expensive mistakes.
MEETING THE CHALLENGE
                                  manage a whole meal by yourself, then           See Section 10 for further information.
TREATING ALS SYMPTOMS             compromise, and seek help for half of it.
   ASSISTIVE EQUIPMENT            Get input from a dietitian regarding
                                  modifying the texture of the food you eat.
                                                                                  Sleeping
     SEXUAL CONCERNS                                                              People who are unable to move get very
                                  See Section 7 for more information.
ALTERNATIVE TREATMENT                                                             uncomfortable lying in the same position
              OPTIONS                                                             while sleeping for several hours. There are
                                  Excess Emotions                                 special beds, which help an immobile
              MEDITATION
                                  An unusual and often misunderstood              person sleep without being manually
 ALTERNATIVE THERAPIES                                                            turned during the night. A satin bottom
                                  symptom of ALS is experiencing
   DEALING WITH DYING             uncontrollable emotions such as excessive       sheet and nightwear also facilitates
                                  laughing or crying. This is called              turning.
                                  emotional lability, and can be particularly     See Section 11 for more information about
                                  frustrating for the person with ALS,            this type of equipment.
                                  because the outburst is often caused by
                                  something very trivial and may be
                                  misunderstood by other people. This
                                                                                  Use of Alcohol and Medication
                                  symptom usually catches people by               Many people try a little wine or spirits. Be
                                  surprise when it first happens; however,        very careful. The combination of alcohol
                                  over time, many learn how to modify these       and many medications can cause serious
                                  emotional outbreaks and how to avoid            problems. This is not always predictable.
                                  some of the situations in which they occur.     For example, alcohol with some
                                                                                  medications, such as a simple cough
                                  Constipation                                    remedy, even taken several hours apart,
                                                                                  can restrict a patient’s ability to breathe,
                                  This can be caused by a lack of adequate        and at the same time cause a coughing
                                  fibre and/or water through tube feeding.        spell, both lasting for hours. Also, reduced
                                  Ask a dietitian about ways to add more          motor control can be accentuated by
                                  fibre to your diet. If adding fibre to your     alcohol.
                                  diet doesn’t solve the problem, see a doctor
                                  about an appropriate medication.

                                  Fatigue
                                  One of the best ways to combat fatigue is
                                  to conserve your energy for really
                                  important tasks or activities that you really
   ALS              SLA           enjoy. An occupational therapist can plan
   SOCIETY OF CANADA              a daily routine with you that will help you
   Maladie Lou Gehrig’s Disease   to adapt to life with ALS. Some tasks can
             1(800)267-4257
                                  be done in different ways that will save
             http://www.als.ca    some of your energy.
              alscanada@als.ca


                     PAGE 24                                                                                                  v. 1.0
LIVING WITH ALS                                                                                         SECTION          3
MANAGING YOUR SYMPTOMS AND TREATMENT

Other ALS Symptoms                              Leg and Foot Swelling
                                                                                                 Managing Your
You should consult your doctor for drugs        You may experience mild leg and foot             Symptoms and
that are available to treat other common        swelling, which is best reduced by moving        Treatment
ALS symptoms, such as muscle spasms,            the toes and ankle, if possible, and by
cramps, pains and sleeping problems.            elevating the leg and/or using an elastic        MEETING THE CHALLENGE
                                                stocking.
The use of sedatives and tranquillizing                                                          TREATING ALS SYMPTOMS
medication depresses respiration and should                                                      ASSISTIVE EQUIPMENT
                                                Muscle Cramping
be used cautiously by those with impaired
pulmonary function. People with                 Cramps are not uncommon in people with           SEXUAL CONCERNS
pseudobulbar affect may be helped by            ALS. They can be alleviated to some extent       ALTERNATIVE TREATMENT
antidepressant drugs, such as amitriptyline     by keeping the affected muscle warm and          OPTIONS
hydrochloride.                                  by stretching it or having your caregiver        MEDITATION
                                                stretch it until the pain is eased. Severe or
MOBILITY                                        frequent cramps should be discussed with         ALTERNATIVE THERAPIES
                                                your doctor. There are a number of               DEALING WITH DYING
Joint and Muscle Pain                           medications available to reduce cramping.
Range-of-motion exercises are designed to
prevent the sort of joint pain that results     Posture Changes
from stiffness because of lack of use.          If the muscles that maintain your posture
Careful attention to your exercise regime,      weaken, you may have discomfort in your
whether active or passive, will eliminate       lower back, neck and shoulder blade region.
much potential joint pain. However, there       Special cushions, chair backs, lumbar
are still a number of common pains that         (lower back) and cervical (neck) rolls are
can develop. If your arms are weak and you      available to help you to maintain correct
allow them to hang unsupported from the         sitting posture. It may be necessary for you
shoulder, there is a tendency for the           to sit in a reclined position or to use a neck
shoulder joint to become painful. It is         collar to maintain proper positioning. Your
helpful to support weak arms whenever           occupational therapist can assist you in
possible on pillows, armrests or on a table.    choosing the right device.
A shoulder sling will also give the arm some
support and decrease strain on the shoulder
joint while you are walking. Hip pain can
result from prolonged sitting in a sagging
seat or chair. A firm seat on a regular chair
or wheelchair will relieve strain on the hip
joints.

See Section 6 for more information on these
exercises.

                                                                                                 ALS                 SLA
                                                                                                 SOCIETY OF CANADA
                                                                                                 Maladie Lou Gehrig’s Disease

                                                                                                 1(800)267-4257
                                                                                                 http://www.als.ca
                                                                                                 alscanada@als.ca


v 1.0                                                                                            PAGE 25
   SECTION         3                                       LIVING WITH ALS
                                                    MANAGING YOUR SYMPTOMS AND TREATMENT

                                  Walking                                          ASSISTIVE EQUIPMENT
      Managing Your
      Symptoms and                As leg and ankle muscles weaken,
                                                                                   As ALS progresses, there are various types
         Treatment                unexpected fatigue, tripping and falling
                                                                                   of equipment available to assist your
                                  become problems. You should begin to use
                                                                                   mobility. At first you may need a cane or a
MEETING THE CHALLENGE             a cane or walker as soon as you are
                                                                                   walker, then a scooter or a manual
                                  threatened by unexpected falls. Also,
TREATING ALS SYMPTOMS                                                              wheelchair, and ultimately a power
                                  various leg and ankle splint devices are
                                                                                   wheelchair, to assist in maintaining a level
   ASSISTIVE EQUIPMENT            available to provide added support to
                                                                                   of independence. There are eating utensils
                                  weakened muscles.
     SEXUAL CONCERNS                                                               that can assist and increase independence -
ALTERNATIVE TREATMENT             For more information about these devices see     cutlery with large easy-grip handles, non-
              OPTIONS             Section 6.                                       slip mats and special plastic plates. Also,
                                                                                   there are raised chairs and toilet seats
              MEDITATION                                                           which are easier to get up from, and
                                  Gripping and Holding
 ALTERNATIVE THERAPIES                                                             portable hoists to move a person around.
                                  People with ALS eventually lose strength         Choosing the best assistive equipment for
   DEALING WITH DYING
                                  in the hand and wrist muscles, losing the        your situation involves many
                                  ability to manage small hand movements           considerations, including an understanding
                                  like holding a pen to write or cutlery to        of longer-term needs. It is advised that you
                                  eat, turning a key to start a car or turning a   get advice from a healthcare professional
                                  handle to open a door. There are a variety       such as an occupational or physiotherapist
                                  of products designed to assist you with          before acquiring assistive equipment.
                                  weakened gripping strength.

                                  Section 6 provides more information about
                                  these devices.

                                  As these weaknesses continue to develop,
                                  you can lose your ability to hold even
                                  lighter weight articles. Getting dressed and
                                  undressed becomes more and more
                                  difficult, and then impossible to do alone.
                                  As this process occurs, dressing can be
                                  made easier with Velcro fasteners, elastic
                                  waistbands, and other features that make
                                  clothing easier to put on and take off.

                                  Cold or hot hands can weaken the grip of
                                  most people. For persons with ALS,
                                  however, this is even more pronounced. If
                                  your fingers get cold you will probably be
                                  surprised by how much harder it is to do
                                  such things as undo buttons, or turn a
   ALS              SLA           doorknob.
   SOCIETY OF CANADA
   Maladie Lou Gehrig’s Disease

             1(800)267-4257
             http://www.als.ca
              alscanada@als.ca


                     PAGE 26                                                                                                v. 1.0
LIVING WITH ALS                                                                                       SECTION          3
MANAGING YOUR SYMPTOMS AND TREATMENT

SEXUAL CONCERNS -                                The following suggestions may prove
                                                 helpful in dealing with sexual concerns:
                                                                                               Managing Your
Sexuality and intimacy are basic aspects of                                                    Symptoms and
                                                 • Using techniques, assistive devices,        Treatment
human life. Sexual desires and abilities
                                                   and positioning to accommodate
may not be affected by the disease process,
                                                   increasing muscle weakness and              MEETING THE CHALLENGE
except for the physical limitations imposed
                                                   other symptoms of ALS
by physical discomfort, muscle weakness,                                                       TREATING ALS SYMPTOMS
and fatigue or low energy levels. Anxiety        • Identifying techniques and assistive
                                                   devices that enable you to maintain         ASSISTIVE EQUIPMENT
may cause impotence. The person with
ALS may worry about not being able to              good grooming and personal cleanliness      SEXUAL CONCERNS
please a healthy partner.                        • Maintaining communication (the open         ALTERNATIVE TREATMENT
                                                   expression of affection and need is         OPTIONS
Other factors that may contribute to               important.)
unsatisfactory sexual relations are:                                                           MEDITATION
                                                 • Respecting the boundaries of the other
• Adjustment to assistive devices                  partner                                     ALTERNATIVE THERAPIES
  or support systems                             • Altering the living environment to          DEALING WITH DYING
• Dealing with everyday survival                   provide adequate privacy and reduce
• Decreasing self-image                            embarrassment
• Grooming                                       • Scheduling of “adult time” if there are
                                                   young children in the family
• Level of communication
                                                 • Wearing street clothes rather than night
• Mobility                                         attire whenever possible during the day
• Physical appearance                              to stress normality
                                                 • Re-channelling interests and energies
The following issues may also be of                into other areas
  concern:
• Altered role                                   Partners should realize that touching is as
• Emotional state                                important as sexual performance in
                                                 reducing tension and maintaining
• Functional level
                                                 emotional intimacy. Preservation of
• Mobility level                                 personal integrity should be an overriding
• Range of motion                                concern. If you have any questions or
• Self-care level                                concerns, speak with your doctor and
                                                 request a referral to a sexual health
• Self-image                                     clinician in your area.

You and your partner may need
counselling with an empathetic healthcare
provider in order to deal openly with
mutual concerns and expectations. If you
are both willing, you could explore                                                            ALS                 SLA
different sexual behaviours, role flexibility,                                                 SOCIETY OF CANADA
and alternative methods of sexual                                                              Maladie Lou Gehrig’s Disease
expression.                                                                                    1(800)267-4257
                                                                                               http://www.als.ca
                                                                                               alscanada@als.ca


v 1.0                                                                                          PAGE 27
   SECTION         3                                      LIVING WITH ALS
                                                   MANAGING YOUR SYMPTOMS AND TREATMENT

                                  ALTERNATIVE TREATMENT OPTIONS                 Aromatherapy
      Managing Your
      Symptoms and                Massage and Touch Therapies
                                                                                Aromatherapy is a massage that involves
         Treatment                                                              the use of fragrant oils, which penetrate
                                  Touch therapies can be very relaxing and      the skin, adding another sense of pleasure
MEETING THE CHALLENGE             comforting for people with ALS. They can      to relaxation.
                                  warm up limbs, which often feel cold
TREATING ALS SYMPTOMS
                                  because of inactivity, low circulation and    Cranio-Sacral
   ASSISTIVE EQUIPMENT            decreased muscle mass. Though touch
                                  therapies are usually given by a caregiver    A cranio-sacral massage is a very light and
     SEXUAL CONCERNS                                                            gentle massage of the back of the head
                                  or therapist, self-massage can also be very
ALTERNATIVE TREATMENT             beneficial.                                   and neck. It is non-invasive and very
              OPTIONS                                                           soothing.
              MEDITATION          The easiest touch therapy to learn is
                                  massage. For the most beneficial massage,     Tens Machine
 ALTERNATIVE THERAPIES            body oil is used to lubricate the skin,
   DEALING WITH DYING             which aids in soothing and relaxing the       A Tens Machine stimulates muscles
                                  muscles. Massage involves systematically      through electronic impulses, and is often
                                  stroking, kneading and pressing the soft      used by physiotherapists.
                                  tissues of the body with fingers and the
                                  palm of the hand, working on the muscles,     Costs
                                  ligaments and tendons. A complete
                                                                                The cost of most massage treatments
                                  massage covers the entire body, inducing a
                                                                                ranges between $40 and $75 for sessions
                                  state of warmth and relaxation. However a
                                                                                up to an hour in length. Most of these
                                  massage of parts of the body, such as
                                                                                treatments are not covered by provincial
                                  arms, legs or shoulders is also beneficial
                                                                                healthcare plans, but may be covered in
                                  and will help improve circulation.
                                                                                whole or in part by private or group
                                                                                insurance if they are prescribed and/or
                                  Shiatsu                                       delivered by a registered therapist,
                                  Shiatsu is a Japanese system of touch
                                  therapy and is given using fingers, thumbs
                                  and hands. The forearm, knees, and feet
                                  can also be used, applying pressure to
                                  specific points on the body.

                                  Reflexology
                                  Reflexology is based on the theory that
                                  pressing and massaging certain points in
                                  the feet will affect the whole body and
                                  induce relaxation.

   ALS              SLA
   SOCIETY OF CANADA
   Maladie Lou Gehrig’s Disease

             1(800)267-4257
             http://www.als.ca
              alscanada@als.ca


                     PAGE 28                                                                                               v. 1.0
LIVING WITH ALS                                                                                         SECTION          3
MANAGING YOUR SYMPTOMS AND TREATMENT

Meditation                                      Before starting to meditate it is usually best
                                                to let other people in your house know that
                                                                                                 Managing Your
Meditation is a way to relax and revitalize     you don’t want to be disturbed for 20            Symptoms and
both mind and body, and can be practised        minutes. Many meditation teachers                Treatment
in many ways. Technically, meditation is        suggest that, for the best results, meditation
deliberately entering into an inner silence     should be done twice a day, for about            MEETING THE CHALLENGE
and stillness for a certain period of time,     twenty minutes, with your eyes closed.
usually fifteen to twenty minutes. It has                                                        TREATING ALS SYMPTOMS
                                                When you think about 20 minutes is up,
been practised over the centuries by many       gently open your eyes and look at your           ASSISTIVE EQUIPMENT
different cultures and in many different        watch. Two common problems for those             SEXUAL CONCERNS
ways. Referring to the results of               new to meditation are being unable to
researching meditation in his book The          relax as their minds are so full of thoughts     ALTERNATIVE TREATMENT
Relaxation Responses, (Avon Books,                                                               OPTIONS
                                                and/or when they are finally relaxed, they
N.Y.), Dr. Herbert Benson writes that           fall asleep.                                     MEDITATION
meditation creates an overall awareness of
relaxation and a feeling of well-being.
                                                                                                 ALTERNATIVE THERAPIES
                                                It takes daily practice and time to develop
Breathing and heart rate slow down, blood       the ability to achieve a state of conscious      DEALING WITH DYING
pressure drops, and there is an increase of     relaxation. Sometimes quiet, peaceful
healing alpha waves from the brain. In          music can help the mind calm down, but
other words, meditation can reduce stress,      in later phases of meditation that may be
and relax muscles.                              too distracting.

Meditation techniques can be divided into       There are several other techniques to help
two categories. One type of meditation          clear the mind, and achieve a state of
involves concentrating on something, such       relaxation, including music therapy and
as your breathing, a candle flame, a            visualization therapy. Books on this subject
crucifix, even rolling surf, while peacefully   can provide patients with knowledge of
and silently repeating a mantra, a special      these techniques, instructions, exercises
word or sound. Any word that contributes        and many hours of quiet pleasure.
to inner peacefulness can work. The other
type of meditation also involves repeating
a mantra, but the objective is to relax your
mind entirely. When thoughts come into
your mind, you try to relax, observe the
thoughts and let them flow, without
participating in the thought process. For
most people, this takes practice.




                                                                                                 ALS                 SLA
                                                                                                 SOCIETY OF CANADA
                                                                                                 Maladie Lou Gehrig’s Disease

                                                                                                 1(800)267-4257
                                                                                                 http://www.als.ca
                                                                                                 alscanada@als.ca


v 1.0                                                                                            PAGE 29
   SECTION         3                                        LIVING WITH ALS
                                                     MANAGING YOUR SYMPTOMS AND TREATMENT

                                  Alternative Medications                          If you are considering alternative
      Managing Your                                                                medicines, here are some points to keep in
      Symptoms and                Because vitamins, herbs and other so-            mind.
         Treatment                called complementary or alternative
                                  medicines have become so popular over            Medicines that come from nature are in
MEETING THE CHALLENGE             the last few years, it is appropriate to say a   fact drugs, and can be harmful, particularly
                                  few things about these potential remedies        in large doses. Different individuals have
TREATING ALS SYMPTOMS             in the context of research. People with          different tolerance levels for drugs,
   ASSISTIVE EQUIPMENT            ALS can be particularly attracted to these       including so-called natural remedies. There
                                  remedies either because drug trials so far       are many products from nature that are in
     SEXUAL CONCERNS
                                  have been unsuccessful, or because of the        fact poisonous.
ALTERNATIVE TREATMENT             perception that alternative medicines are
              OPTIONS             natural.                                         Alternative medicines are not regulated.
              MEDITATION                                                           The consumer is subject to domestic and
                                  While extensive research is done for             foreign manufacturers who could dilute
 ALTERNATIVE THERAPIES            pharmaceutical drugs, virtually no research      the strength, or change the composition of
   DEALING WITH DYING             has been done on alternative medicines           their products without disclosing this
                                  with the exception of vitamins, which have       information on their labels.
                                  been extensively researched. On the other
                                  hand many alternative medicines have             If you want more information about these
                                  been used by some cultures for hundreds          types of medicines, you should discuss
                                  and even thousands of years. Stories             your options with professionals such as
                                  circulate about people who have been             your family doctor, pharmacist, and
                                  cured by these medicines. Also, it is well       naturopathic and homeopathic doctors. Be
                                  known that animal and plant by-products          careful: some people with ALS have been
                                  have provided the basis for many                 enticed by false claims into spending many
                                  successful drugs. Unfortunately, the             thousands of dollars on alternative
                                  hundreds of millions of dollars required to      medicines and therapies.
                                  thoroughly test pharmaceutical medicines
                                  are not available to test alternative
                                  medicines. Scientifically, very little is
                                  known about the effect of alternative
                                  medicines on the human body.




   ALS              SLA
   SOCIETY OF CANADA
   Maladie Lou Gehrig’s Disease

             1(800)267-4257
             http://www.als.ca
              alscanada@als.ca


                     PAGE 30                                                                                               v. 1.0
LIVING WITH ALS                                                                                     SECTION          3
MANAGING YOUR SYMPTOMS AND TREATMENT

DEALING WITH DYING                              Dying at Home
                                                                                             Managing Your
Death is something most of us would
                                                Most people with ALS would prefer to die     Symptoms and
                                                in the peace of their own home. You          Treatment
prefer not to talk about. How we deal with
                                                should communicate this wish to your
death can be deeply affected by our
                                                family while you are still able to do so.    MEETING THE CHALLENGE
cultural values. Some cultures view death
                                                Factors that might not make dying at
as a welcomed spiritual transition. Others                                                   TREATING ALS SYMPTOMS
                                                home possible include your family’s
believe that death and rebirth are a
                                                financial or emotional inability to cope,    ASSISTIVE EQUIPMENT
continual process. During our lives, the
                                                caregiver burnout, respiratory problems or
cells in our bodies die at an ever-increasing                                                SEXUAL CONCERNS
                                                insufficient home care services. Remember,
rate, while at the same time our learning,                                                   ALTERNATIVE TREATMENT
                                                calling 911 will bring a medical team
intelligence and spiritual awareness can                                                     OPTIONS
                                                obligated to keep you alive, including
continue to grow.
                                                artificial life support that you may not     MEDITATION
As your physical activity decreases, you        want. Some provinces may have a death at
                                                home policy which allows bypassing of
                                                                                             ALTERNATIVE THERAPIES
will have more time to think about and to
become at peace with death.                     calling 911. There will be forms to          DEALING WITH DYING
                                                complete. Please check with your regional
Even though we may be uncomfortable             ALS Unit.
talking about death, important and
inevitable decisions will have to be made.       Instructions in a Power of Attorney and
It is best to discuss and plan for these        Living Will should help to ensure that you
decisions well before death is near.            are treated according to your wishes.

                                                See Section 12 for more about Powers of
Practical Planning                              Attorney.
As ALS progresses, there are a lot of
practical problems to deal with and
arrangements to be made. At some point
someone has to deal with financial
changes, tax issues, insurance, a will and
funeral arrangements, as well as
distribution of assets and belongings. It is
often difficult for a family to talk openly
about these practical matters.

You can usually make it much easier for
your family if you bring up these subjects,
and see to it that appropriate planning and
action are taken. Inadequate
communication and planning of these
practical concerns could force family
members to make decisions without
knowing your wishes, and could also cause                                                    ALS                 SLA
unnecessary financial hardship.                                                              SOCIETY OF CANADA
                                                                                             Maladie Lou Gehrig’s Disease
See Section 12 of this manual for
information about dealing with financial and                                                 1(800)267-4257
legal concerns.                                                                              http://www.als.ca
                                                                                             alscanada@als.ca


v 1.0                                                                                        PAGE 31
SECTION         3              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 32                     v. 1.0
LIVING WITH ALS          SECTION          3
NOTES




                  ALS                 SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 33
SECTION         3              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 34                     v. 1.0
LIVING WITH ALS                                                                                     SECTION          4
YOUR HEALTHCARE TEAM

There are a number of ALS clinics across         NURSE CLINICIAN
Canada that treat ALS through a team
                                                                                             YOUR
approach. In other communities, the same         A nurse clinician generally formulates
                                                                                             HEALTHCARE
healthcare professionals are available as        nursing goals after analyzing assessment    TEAM
individuals or through homecare                  data, then draws up and implements a care   YOUR PRIMARY
community programs. A variety of                 plan. Other nursing functions may include   DOCTOR
medical personnel can help you to cope           the following:                              NEUROLOGIST
with the limitations that the disease places
upon you.                                        • explaining terminology and techniques;    NURSE CLINICIAN
                                                 • teaching skills and providing             OCCUPATIONAL
YOUR PRIMARY DOCTOR                                demonstrations;                           THERAPIST &
                                                                                             PHYSIOTHERAPIST
                                                 • evaluating skill levels and reviewing
You can ask your primary doctor for                procedures;                               DIETITIAN
referrals to these healthcare professionals if
                                                 • addressing questions and concerns;        RESPIRATORY
a specific problem needs to be addressed.                                                    THERAPIST
                                                 • ensuring the comfort of people living
It is important that all of your primary           with ALS;                                 SPEECH-LANGUAGE
caregivers meet with members of your                                                         PATHOLOGIST
                                                 • encouraging and promoting decision-
healthcare team in order to be trained in          making by the people with ALS and         SOCIAL WORKER
the various helping techniques.                    their families;                           PASTORAL CARE
Your doctor’s tasks may include the              • liaising with community organizations;    WORKER
following:                                       • making referrals;
• Explaining the diagnosis and possible          • limiting the development of
  progression of ALS;                              complications;
• Making necessary referrals to, and             • providing nursing interventions
  mediating with, other healthcare                 if problems arise.
  providers.


NEUROLOGIST

Your doctor will refer you to a neurologist,
a specialist in diseases of the nervous
system who can confirm a diagnosis of
ALS. Between them they will:
• outline types of treatment options
  available;
• encourage the setting of short-term
  goals;
• help you preserve a positive self and
  body image and maintain your morale;
                                                                                             ALS                 SLA
                                                                                             SOCIETY OF CANADA
• facilitate problem-solving by identifying                                                  Maladie Lou Gehrig’s Disease
  specific needs and concerns and finding
  solutions.                                                                                 1(800)267-4257
                                                                                             http://www.als.ca
                                                                                             alscanada@als.ca


v 1.0                                                                                        PAGE 35
SECTION         4                                       LIVING WITH ALS
                                                                                YOUR HEALTHCARE TEAM

         YOUR                  OCCUPATIONAL THERAPIST                        The PT is generally more concerned with
                               & PHYSIOTHERAPIST                             gross-motor function and anatomical
   HEALTHCARE                                                                impairments associated with abnormal
         TEAM                  The occupational therapist (OT) and the       movement. The PT’s tasks may include the
        YOUR PRIMARY           physical therapist/physiotherapist (PT)       following:
            DOCTOR             formulate management strategies that
                               enable people with ALS to continue to         • providing a detailed analysis of
         NEUROLOGIST                                                           abnormal movement (for example, gait
                               carry out work and leisure activities in a
     NURSE CLINICIAN           safe and efficient manner. This is              analysis);
      OCCUPATIONAL             accomplished through the following:           • optimizing the person’s strength,
         THERAPIST &                                                           function, and comfort;
     PHYSIOTHERAPIST           • teaching;
                                                                             • designing and monitoring a therapeutic
               DIETITIAN       • ongoing assessment/evaluation;                exercise regimen when appropriate;
                               • ordering appropriate equipment in a         • assisting with respiratory management;
           RESPIRATORY
             THERAPIST           timely manner;
                                                                             • providing training in energy
                               • prevention of complications;                  conservation and time management
  SPEECH-LANGUAGE
       PATHOLOGIST             • utilization of community resources;           techniques;
      SOCIAL WORKER            • provision of psychological support.         • preventing needless purchases of
       PASTORAL CARE                                                           unsuitable equipment;
             WORKER            The OT is generally more concerned with       • making a home assessment.
                               fine-motor control and the application of
                               movement to function. The OT’s tasks          The physiatrist generally evaluates the
                               may include the following:                    extent of disability and functioning, and
                               • assessing fine-motor functional abilities   gauges the level of residual muscle
                                 (for example, hand use);                    function. On the basis of these findings,
                                                                             treatment plans may be designed. Other
                               • evaluating positioning and seating          functions may include:
                                 requirements;
                               • assisting in choosing and accessing         • recommending preventative and
                                 suitable augmentative mobility and            supportive treatment;
                                 communications strategies and any           • preventing complications;
                                 required assistive devices;                 • determining which diagnostic tests are
                               • giving instruction concerning exercise,       necessary;
                                 positioning, correct body mechanics for     • helping maintain maximum function;
                                 lifting and transferring, and use of
                                 assistive devices to the person with ALS,   • consulting with therapists concerning
                                 family members, and caregivers;               orthoses and equipment.
                               • teaching energy conservation and time
                                 management techniques;

ALS               SLA          • making a home assessment.

SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 36                                                                                                v. 1.0
LIVING WITH ALS                                                                                        SECTION          4
YOUR HEALTHCARE TEAM

DIETITIAN                                         SPEECH-LANGUAGE PATHOLOGIST                   YOUR
A dietitian’s primary considerations are to       A speech-language pathologist provides
                                                                                                HEALTHCARE
keep your quality of life as high as possible     advice on techniques and strategies to        TEAM
by maintaining safe and adequate nutrition        allow the person with ALS to continue to      YOUR PRIMARY
and hydration, in order to prevent life-          communicate throughout life. Some             DOCTOR
threatening nutritional deficits from             functions of the speech-language              NEUROLOGIST
developing. The dietitian may assess or           pathologist may include:
evaluate the following:                                                                         NURSE CLINICIAN
                                                  • evaluating the individual’s motivation      OCCUPATIONAL
• functional abilities (ability to self-feed or     and potential for learning new              THERAPIST &
  to administer tube feedings);                     techniques;                                 PHYSIOTHERAPIST
• nutritional status and present intake.          • evaluating functional abilities, such as    DIETITIAN
                                                    oral motor function, cognitive-linguistic
The dietitian may recommend the                                                                 RESPIRATORY
                                                    function, augmentative communication        THERAPIST
following strategies:                               function, and swallowing function;
• appropriate changes in food texture and                                                       SPEECH-LANGUAGE
                                                  • determining the most efficient              PATHOLOGIST
  consistency;                                      communication function;
• appropriate methods of food                                                                   SOCIAL WORKER
                                                  • training people with ALS and family
  preparation;                                      members in techniques of effective          PASTORAL CARE
• substitutions for hard-to-manage foods;           communication and energy
                                                                                                WORKER
• meals of a manageable size and                    conservation, and safe eating, drinking
  frequency;                                        and swallowing techniques.
• strategies for improving nutritional
  deficiencies.
                                                  SOCIAL WORKER

RESPIRATORY THERAPIST                             In addition to the doctors and nurses, you
                                                  may be referred to a social worker. The
The respiratory therapist generally devises       social worker’s tasks may include
strategies to optimize remaining muscle
                                                  • promoting an understanding of, and
function and reduce discomfort, and
                                                    adjustment to, ALS;
institutes a program of chest care if
necessary. Other tasks may include:               • sharing information about available
                                                    community resources;
• evaluating pulmonary function status;           • providing information on legal matters;
• maintaining pulmonary hygiene;                  • assisting in assigning priorities and
• providing suggestions for managing                making long-range plans;
  decreasing function;                            • giving emotional support to the person
• offering information on body                      with ALS and to family members.
  positioning, energy conservation,
  relaxation, and compensatory                                                                  ALS                 SLA
  techniques to improve breath support for                                                      SOCIETY OF CANADA
  nutrition and for speech;                                                                     Maladie Lou Gehrig’s Disease

• facilitating a home ventilation program                                                       1(800)267-4257
  if appropriate;                                                                               http://www.als.ca
                                                                                                alscanada@als.ca
• making suggestions concerning a course
  of action when respiratory failure occurs.
v 1.0                                                                                           PAGE 37
SECTION         4                                       LIVING WITH ALS
                                                                               YOUR HEALTHCARE TEAM

        YOUR                   PASTORAL CARE WORKER
  HEALTHCARE                   A minister, priest, chaplain or pastoral care
        TEAM                   worker may perform the following
        YOUR PRIMARY           functions:
            DOCTOR
                               • listening to and empathizing with those
        NEUROLOGIST
                                 who want to vent concerns;
    NURSE CLINICIAN            • assisting in making decisions;
     OCCUPATIONAL              • giving support during emotional or
        THERAPIST &
    PHYSIOTHERAPIST              physical crises;
                               • acting as an advocate for those who have
              DIETITIAN
                                 no voice;
           RESPIRATORY         • reassuring people with ALS that their
             THERAPIST
                                 lives have meaning;
  SPEECH-LANGUAGE
       PATHOLOGIST             • encouraging people with ALS to
                                 discover their personal strengths;
     SOCIAL WORKER
                               • encouraging people with ALS to
      PASTORAL CARE              recapture positive experiences from the
            WORKER               past by recall or reminiscence;
                               • celebrating the individual’s humanity
                                 and worth;
                               • arranging and conducting services when
                                 appropriate.




ALS               SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 38                                                                        v. 1.0
LIVING WITH ALS          SECTION          4
NOTES




                  ALS                 SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 39
SECTION         4              LIVING WITH ALS
                                           NOTES




ALS               SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 40                     v. 1.0
LIVING WITH ALS                                                                                         SECTION          5
MOBILITY STRATEGIES

THE ROLE OF EXERCISE                             Moderation in Exercise
                                                                                                 MOBILITY
The purpose of exercise for people with
                                                 It is important that all exercise be            STRATEGIES
                                                 performed in moderation. Fatigue will
ALS is                                                                                           THE ROLE OF EXERCISE
                                                 only increase your weakness and rob you
• to maintain or improve the flexibility of      of energy that you need for your daily          DEALING WITH
  muscles not affected by ALS;                   routines and the activities you enjoy.          PROBLEMS
                                                 If you find that your prescribed set of
• to maintain the flexibility of muscles                                                         ASSISTIVE DEVICES
                                                 exercises tires you, talk to your therapist.
  that have been affected;                                                                       TRANSFERS
                                                 Changes can be made that will eliminate
• to maintain the flexibility of joints in the   the risk of fatigue.                            TRAVEL
  neck, trunk and limbs.
                                                 Similarly, none of your exercises should        SUPPORT SERVICES
                                                 cause you pain. If you do experience pain
It is important to realize that exercise will    when exercising, stop that exercise and talk
not strengthen muscles that have been            to your therapist. It may be that you are
weakened by ALS. Once the supply of              not doing the exercise correctly, or perhaps
motor neurons that control a particular          some modification to your exercise
muscle has degenerated, it cannot be             program must be made.
regenerated by exercise or anything else.
The right exercise program can minimize
                                                 Recreational Exercise
joint and muscle stiffness.
                                                 If you enjoy such activities as walking,
Range-of-Motion (ROM) Exercises                  stationary bicycling and especially
                                                 swimming, keep them up for as long as
A person with ALS needs to move each             you can do them safely. If you experience
affected joint through a series of range-of-     cramping or fatigue, do not continue the
motion (ROM) exercises every day to              exercise until you have consulted your
prevent joint stiffening. Exercise will help     doctor or therapist.
to keep your body as flexible as possible
and your joints mobile. ROM exercises are
                                                 Active, Active-Assisted
usually done systematically, meaning that
the joints of one limb are exercised in a        and Passive Exercises
particular order before the next limb is         The objective of ROM exercises is to move
exercised and so on.                             each affected joint through its full range of
                                                 motion every day. Not every person with
Each person with ALS needs a program of
                                                 ALS can do a full set of active exercises.
exercise tailored to his or her individual
needs and abilities. Your doctor and             An active exercise is one you do yourself
therapist can prescribe the exercises that       without any assistance, when your muscles
are right for you at any given time. Your        can perform the full movement.
physiotherapist can demonstrate the
exercises and ensure that you are                Muscles that are able to move a joint only
performing them correctly.                       partially need active-assisted exercise.
                                                 A helper may assist the muscle through the      ALS                 SLA
                                                 movement, or you may be shown a way to          SOCIETY OF CANADA
                                                 do a self-assisted range of motion.             Maladie Lou Gehrig’s Disease

                                                                                                 1(800)267-4257
                                                                                                 http://www.als.ca
                                                                                                 alscanada@als.ca


v 1.0                                                                                            PAGE 41
 SECTION         5                                       LIVING WITH ALS
                                                                                         MOBILITY STRATEGIES

                                Passive exercises are done completely by a      DEALING WITH PROBLEMS
        MOBILITY                helper when muscles can no longer
      STRATEGIES                perform any of the movement. The helper         Falls
                                moves the joints through their range of
THE ROLE OF EXERCISE                                                            To avoid head injuries when falling, it is
                                motion by manipulating your limb. Passive
        DEALING WITH            exercises work the joints but not the           better to drop straight down, and not fall
            PROBLEMS            muscles. Your therapist can train your          forward or backward. The best way to get
     ASSISTIVE DEVICES          caregiver(s) to do these exercises properly.    up from a fall depends on what muscles
                                                                                you can still use. If you still have enough
              TRANSFERS         The transition from active to passive           arm and hand strength, you can hold on to
                   TRAVEL       exercise is seldom abrupt. You may find         something firm, such as furniture, for
                                that you can do some exercises actively,        example and pull yourself up and into a
    SUPPORT SERVICES            some with assistance and still others only      chair.
                                passively.
                                                                                When you have fallen, the most important
                                Some Exercising Tips                            thing to do is to get help to sit in an
                                                                                upright position. The level of assistance
                                Exercises should be done daily and should       needed will depend on the level of muscle
                                become a routine. You may wish to break         weakness. You may need only some
                                up your exercise routine into parts to avoid    support while rising. Or you may need to
                                fatigue. If you experience fatigue, consult     be lifted from behind until you can push
                                your therapist for a change in your             against the ground with your legs. Or
                                program.                                        you will need two people to assist you into
                                                                                a chair or wheelchair. It is important that
                                Do as many active exercises as you can.         caregivers not strain themselves, but make
                                It may be necessary to switch to active-        you comfortable until enough help is
                                assisted or passive exercises later. Your       available. Ask your therapist to teach you
                                therapist can help you to make decisions        and your caregivers the best method of
                                about the correct limits of your exercise.      recovering from falls.
                                Ask your therapist which exercises are the
                                most important ones to do if you have a         Joint Pain
                                busy day ahead of you.                          If you are unable to move yourself, you
                                Some exercises can be done while you            may spend too long in one position. This
                                either sit or lie down. Passive exercises are   can be very uncomfortable for both the
                                usually performed while you lie down.           skin and the joints. Arrange for your helper
                                Your therapist can advise you on which          to change your position every couple of
                                exercise positions are best for you.            hours throughout the day and to turn you
                                                                                at night. Some people with ALS improve
                                Stop doing any exercise that hurts, and         their comfort in bed by using a sheepskin,
                                consult your therapist if this happens.         egg crate foam, a satin bottom sheet or a
                                                                                vibrating air mattress. Your nurse or
                                                                                therapist can discuss the options with you
ALS                SLA                                                          and help you to decide what to try.
 SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease                                                   If you experience joint pain, discuss this
                                                                                with a doctor or physiotherapist.
           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                   PAGE 42                                                                                                   v. 1.0
LIVING WITH ALS                                                                                         SECTION          5
MOBILITY STRATEGIES

ASSISTIVE DEVICES                               TRANSFERRING A PERSON WITH ALS
                                                                                                 MOBILITY
Canes and Walkers                               All your caregivers should be instructed in
                                                                                                 STRATEGIES
                                                safe and effective transferring techniques by    THE ROLE OF EXERCISE
Most ALS patients will require a cane or        professional healthcare providers as soon as
walker sooner or later. The choice of a         possible. It is important that proper body       DEALING WITH
cane or walker should be made in                                                                 PROBLEMS
                                                mechanics be taught to decrease the risk of
consultation with your doctor and               injury to both you and your caregiver.           ASSISTIVE DEVICES
physiotherapist.
                                                                                                 TRANSFERS
For information on canes and walkers,                                                            TRAVEL
see Section 11.
                                                TRAVEL                                           SUPPORT SERVICES
Orthotic Devices
                                                If you have problems walking, or are in a
Orthotic devices are aids that are attached     wheelchair, telephone ahead to find out
to your body. They help to support your         what accessible facilities are available. Some
joints in certain positions when your           places are very accessible to the disabled,
muscles no longer can. Your doctor              others are not. Be very specific about what
usually prescribes such devices, and your       your needs are – “accessible” means
therapist will fit and instruct you in proper   different things to different people.
use of the aid.
                                                Air Travel
For more information about orthotic devices,
see Section 11.                                 Most airports provide wheelchairs that you
                                                can take to and from the plane. Make sure
Wheelchairs                                     that the airline knows in advance that you
                                                require a wheelchair. If you need to be
Many people with ALS will find                  accompanied by an attendant, and have the
themselves in need of a wheelchair at           appropriate document signed by a doctor,
some time. Some will use it only for long       some airlines let an accompanying
excursions outside the home, some for           attendant fly at half-price. Airlines also
activities only and some people will spend      provide other services for the disabled
most of their day in a wheelchair.              including special meals. When inquiring
                                                about reservations, it is also a good idea to
The decision about when to acquire a            make sure that the airline can
wheelchair is one that you will make with       accommodate your disability requirements.
your doctor. If you always need another
person to help you to rise from a chair, or
if you stumble and fall a lot, or if you are
fatigued when you walk a distance, a            SUPPORT SERVICES
wheelchair could be useful. No one is
eager to use a wheelchair and this is
                                                As your mobility becomes more of a
natural, but a wheelchair should be viewed
as an opportunity to increase your
                                                problem for family and other caregivers,         ALS                 SLA
                                                please access the ALS Society nearest you.       SOCIETY OF CANADA
independence and your ability to get
                                                We are here to provide support and               Maladie Lou Gehrig’s Disease
around. It will also help you to conserve
                                                resources.
your energy.                                                                                     1(800)267-4257
                                                                                                 http://www.als.ca
For more information on wheelchairs, see                                                         alscanada@als.ca
Section 11.

v 1.0                                                                                            PAGE 43
SECTION         5              LIVING WITH ALS
                                           NOTES




ALS               SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 44                     v. 1.0
LIVING WITH ALS          SECTION          5
NOTES




                  ALS                 SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 45
SECTION         5              LIVING WITH ALS
                                           NOTES




ALS               SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 46                     v. 1.0
LIVING WITH ALS                                                                                       SECTION         6
DEALING WITH SWALLOWING DIFFICULTIES

MECHANICS OF SWALLOWING                         Because of the variety of muscles used to
                                                speak and swallow, the problems that
                                                                                              DEALING WITH
Eating is a process of chewing and              occur as a result of degeneration depend      SWALLOWING
swallowing. Chewing is done primarily           on which muscles have been affected.          DIFFICULTIES
with jaw muscles that, in people with ALS,      Also, as ALS progresses, conditions
                                                                                              MECHANICS OF
do not always weaken when other mouth           change and eating and drinking procedures     SWALLOWING
and swallowing muscles are weakening.           usually have to change as well. As these
                                                problems occur it is important that you       MAKING EATING EASIER
Weakened jaw muscles are more likely to
occur in the latter stages of ALS.              consult a doctor, a speech-language           CHANGES TO
                                                pathologist, and/or other professionals       YOUR DIET
Swallowing is a three-phase process:            who have had training and experience in       PILLS
                                                this area.
First, the tongue moves the food back                                                         NUTRITION
against the soft palate. If these muscles are   If you have your swallowing assessed by
                                                                                              TUBE FEEDING
weakened, food may get stuck in the             videofluoroscopy or other procedures, it is
mouth, or fall back to the throat before it     worth asking that you and your caregiver
has been fully chewed.                          also have a look. That will give you a
                                                better understanding of what happens
Second, the upper and lower throat              inside your throat when you swallow.
muscles move the food down the passage
to the stomach (oesophagus), as well as         Different people can have different
close off passages to the nose (nasal cavity)   problems and remedies, depending on
and the air passage to the lungs (trachea).     their physical, mental and emotional
If throat muscles are weakened three types      conditions.
of problems can occur. Food can:

• get into the nose cavity, especially if the
  patient is lying down;
• get stuck in the throat;
• cause choking by blocking off the
  airway to the lungs (trachea).

In the third phase of swallowing, the top
of the muscle system that moves food
down to the stomach (the upper
oesophageal sphincter muscle) is usually
closed. It normally relaxes and opens to
receive food and pass it down to the
stomach. People with ALS can find that
this muscle doesn’t relax, in which case
food gets stuck, feeling as if it is stuck in
the back of throat.
                                                                                              ALS                 SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 47
 SECTION         6                                       LIVING WITH ALS
                                                       DEALING WITH SWALLOWING DIFFICULTIES

                                MAKING EATING EASIER                           Concentrate on eating
 DEALING WITH
 SWALLOWING                     Eating can be a dangerous activity for
                                                                               Eating in a group may result in not being
   DIFFICULTIES                 people with ALS with weakened throat
                                                                               able to concentrate on swallowing or
                                                                               feeling that you cannot take your own
        MECHANICS OF            muscles. Don’t take risks. Learn as much
                                                                               time, resulting in an adequate meal not
         SWALLOWING             as you can about your own swallowing
                                                                               being eaten or being rushed, causing
                                limitations from a speech-language
MAKING EATING EASIER                                                           coughing and distress. Eating alone may
                                pathologist, a dietitian and other
                                                                               make it easier, though it may also be
           CHANGES TO           healthcare professionals. Become aware of
            YOUR DIET                                                          important to have at least one other person
                                swallowing changes as they occur in your
                                                                               nearby should you encounter problems.
                      PILLS     throat muscles, and ensure that your diet is
                                adapted to your changing abilities.
             NUTRITION                                                         Focus on eating position
         TUBE FEEDING           Take more time                                 With chewing and swallowing problems
                                                                               you should try eating in an upright
                                Eating and drinking may be a very slow
                                                                               position, with the chin tucked towards the
                                and labour intensive process. Allow more
                                                                               chest to close off the airway to the lungs
                                time to eat meals and never hurry.
                                                                               when swallowing and to prevent coughing.
                                                                               Drinking may be easier with the use of a
                                Be relaxed when eating                         cut out Styrofoam cup. Tilt the chair back
                                A person experiencing difficulty with          as far as is comfortable. Again use a chin
                                eating and drinking may feel acute             tuck.
                                embarrassment. Anxiety and distress may
                                accompany embarrassment, and anxiety           Take small bites
                                itself impairs the ability to relax. Being
                                                                               With weakened tongue and lip muscles,
                                relaxed and feeling confident is of
                                                                               smaller bites are easier to handle in your
                                tremendous assistance.
                                                                               mouth and will reduce the chances of
                                Some people find it easier to relax if there   choking if the food falls into the throat
                                are no distractions. For example, they may     before being fully chewed.
                                turn off the TV or radio, and discourage
                                visitors from calling at meal times. Others
                                find distractions helpful in allowing them
                                to relax and feel less anxious.




 ALS               SLA
  SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                   PAGE 48                                                                                              v. 1.0
LIVING WITH ALS                                                                                        SECTION         6
DEALING WITH SWALLOWING DIFFICULTIES

Be prepared                                   Take food and liquid separately
                                                                                               DEALING WITH
Learn what to do before an emergency          It may be difficult for the throat muscles to    SWALLOWING
happens. When the throat gets irritated or    switch between eating and drinking, which        DIFFICULTIES
blocked, it can close around the irritant     require slightly different muscle activity. It
making it difficult to breathe. Even your     may help to eat food separately from             MECHANICS OF
saliva can cause coughing and choking.        drinking, rather than switching back and         SWALLOWING
This may also happen when a few small         forth.                                           MAKING EATING EASIER
particles from a previous meal are still
                                                                                               CHANGES TO
stuck in the throat. Practice the following   When food sits in your throat                    YOUR DIET
so that you will be prepared when this
happens:                                      If you feel food sitting in your throat, try     PILLS
                                              swallowing two or three times to clear it,       NUTRITION
Try to relax                                  before taking more food. It may be that the
                                              upper oesophageal sphincter muscles at the       TUBE FEEDING
Lean forward, the further the better,         bottom of your throat, which are normally
depending on the seriousness of the           closed, are not relaxing and opening as
problem. If possible, stand up and bend       actively as they should to allow
over as if to touch your toes                 swallowing.

Try to take small breaths                     Never use liquid to wash down food
through the nose
                                              If food is stuck in the throat and liquid is
Expel the food by breathing in slowly, and    added, the liquid can very easily be
exhaling or coughing quickly. For more        channelled into the airway leading to your
force “splint” you’re abdomen by crossing     chest, and cause choking.
your arms over your lower abdomen and
pressing firmly in and down as you cough
out. A caregiver can also do this by
standing behind you and pressing their        CHANGES TO YOUR DIET
hands into your abdomen as you cough.
Ask a nurse or other professional to show     As chewing and swallowing problems
you this technique, known as the Heimlich     develop, it will be necessary to make
maneuver, and practice it with your           dietary changes to cope with these reduced
caregivers as soon as possible so that you    eating abilities.
will know what to do should choking
occur.                                        Consult with your dietitian or doctor
                                              regarding changes to your diet. With a
Never hit a choking person                    weakened tongue and lips, it is not only
on the back                                   difficult to chew, it is difficult to position
When a person is choking from food,           food in your mouth so it won’t either fall
hitting on the back can cause the food to     out between your lips, or fall down your
jam tighter in the throat.                    throat before you have finished chewing.
                                                                                               ALS                 SLA
Ask a member of your ALS team about                                                            SOCIETY OF CANADA
this or contact your regional ALS Unit,                                                        Maladie Lou Gehrig’s Disease
local Red Cross Society or St. John’s
Ambulance branch for CPR and First Aid                                                         1(800)267-4257
courses in your area which some of your                                                        http://www.als.ca
caregivers can take.                                                                           alscanada@als.ca


v 1.0                                                                                          PAGE 49
 SECTION         6                                        LIVING WITH ALS
                                                         DEALING WITH SWALLOWING DIFFICULTIES

                                When eating or drinking consider the         Specific foods
 DEALING WITH                   following:                                   that may cause problems
 SWALLOWING
                                                                             • Extra-spicy, “hot” or acidic foods
   DIFFICULTIES                 Smaller and softer
                                                                             • Soft fresh bread
        MECHANICS OF            Food should be softer and cut into smaller
         SWALLOWING             pieces that can slide down the throat with   • Cookies, crackers, dry cereal,
                                minimum chewing.                               graham crackers
MAKING EATING EASIER
                                                                             • Dry muffins, cake
           CHANGES TO           Not too runny                                • Dry, fibrous, or bony meats and fish
            YOUR DIET
                                If food or drinks are too runny, some of     • Coconut, pineapple
                      PILLS     the liquid can run into the airway to the
                                lungs and cause coughing.                    • Sticky foods (for example, peanut butter)
             NUTRITION
                                                                             • Stringy vegetables
         TUBE FEEDING           Not too dry                                    (for example, lettuce, celery)
                                If food is too dry, such as dry toast, it    • Fried noodles, rice
                                tends to irritate the throat and causes      • Popcorn, potato chips, nuts
                                coughing. This problem can often be
                                                                             • Fruits and vegetables with skin or seeds
                                solved by adding butter, jam etc.
                                                                               (for example, peas, corn, apples, berries)
                                Not too sticky
                                Foods that are too sticky, such as a thick   A dietitian can make helpful suggestions
                                sticky sauce, will be a problem if they      about the following:
                                don’t flow in the mouth and throat.
                                Thinning the sauce can help.                 • Suitable methods of cooking;
                                                                             • Substitutions for suitable foods;
                                Foods that may be easier to manage           • Ways to thicken thin liquids (for
                                                                               example, use commercial thickeners,
                                • Custards, applesauce, sherbets,              mashed potato, arrowroot, gelatin,
                                  puddings, plain yogurt                       sour cream, egg, cornstarch, flour,
                                • Crustless toast with butter,                 infant cereals);
                                  crustless French toast                     • Types of semi-solid foods (pudding,
                                • Dark chicken meat with gravy                 custard, cottage cheese, pureed fruit).
                                • Canned fruit, soft fruit
                                  (for example, bananas)
                                                                             PILLS
                                • Eggs (scrambled, poached, omelettes)
                                • Cooked cereals (with milk)                 If pills are a problem to swallow, grind
                                • Casseroles (macaroni and cheese)           them into powder and mix them with
                                • Milkshakes, frozen ice cream bars          applesauce or another easy-to-eat food.
                                                                             Some medications are available in liquid
                                • Mashed potatoes with gravy
                                                                             form. Ask your pharmacist about this
 ALS               SLA          • Gelatin with yogurt/cottage cheese         possibility.
  SOCIETY OF CANADA             • Salmon/tuna/egg salad
 Maladie Lou Gehrig’s Disease
                                • Thick soups
           1(800)267-4257       • Juice nectars, tomato juice, vegetable
           http://www.als.ca    juice
            alscanada@als.ca
                                • Pasta with sauce

                   PAGE 50                                                                                               v. 1.0
LIVING WITH ALS                                                                                     SECTION         6
DEALING WITH SWALLOWING DIFFICULTIES

NUTRITION                                      What type of tube should you have?
                                                                                            DEALING WITH
It is difficult to maintain a fully balanced
                                               There are three main places to insert        SWALLOWING
diet if you have chewing and swallowing
                                               feeding tubes:                               DIFFICULTIES
difficulties. There are many ways to add       • through an incision into the stomach;      MECHANICS OF
nutrients to food - this is not the time to                                                 SWALLOWING
                                               • through an incision
worry about fat and cholesterol! Special
                                                 into the side of the neck;                 MAKING EATING EASIER
high-nutrient powders, drinks and
puddings are available commercially in         • through the nose.                          CHANGES TO
some drug and health food stores. Check                                                     YOUR DIET
                                               Each route has its own advantages and
out how you like the taste before spending                                                  PILLS
                                               disadvantages. Also, there are different
too much money on these products. When
                                               types and diameters of tubes. Tubes wider    NUTRITION
you are unable to continue eating normal
                                               in diameter are less likely to clog with
meals, you should consult a dietitian for                                                   TUBE FEEDING
                                               food. The decision regarding which route
recommendations regarding nutritional
                                               to choose should be made in conjunction
supplements. Also, if you haven’t already
                                               with your family and doctor.
done so, now is the time to consider
having a feeding tube.
                                               Stomach Tube
TUBE FEEDING                                   To install a stomach feeding tube requires
                                               a simple 30-minute operation under mild
When to consider a feeding tube                sedation. There are several types of
                                               stomach tubes. Discuss which type is best
Consider getting a feeding tube as soon as     for you with your doctor. Unlike neck and
your weight goes 10% to 15% below              nasal tubes, a stomach tube remains in
normal. An important advantage of a            place, and is unplugged and plugged at
feeding tube for people, who are unable to     each feeding. Initially, a dressing is
drink, is the ability to have liquids again,   required around the incision. A small
and avoid dehydration. Having a feeding        amount of leakage around the incision is
tube does not mean that you have to stop       not unusual. When the incision heals,
eating normal food. If you decide to have      leaving it open to the air may be the best
a feeding tube, do it while you are still      way to keep it healthy.
relatively healthy, and able to maintain a
diet of normal meals, supplemented by
tube feeding, thereby minimizing weight
loss and maintaining better health. Also, it
may take time for your body to get used to
significant dietary changes, such as a high
volume of nutritional supplements, and it
is better to go through that change when
you are relatively healthy.

                                                                                            ALS                 SLA
                                                                                            SOCIETY OF CANADA
                                                                                            Maladie Lou Gehrig’s Disease

                                                                                            1(800)267-4257
                                                                                            http://www.als.ca
                                                                                            alscanada@als.ca


v 1.0                                                                                       PAGE 51
 SECTION         6                                       LIVING WITH ALS
                                                       DEALING WITH SWALLOWING DIFFICULTIES

                                An Adjustment Period                           Excessive Coughing
 DEALING WITH
 SWALLOWING                     After the operation, you will probably have    You may find that tube feeding causes
   DIFFICULTIES                 to stay in the hospital for a few days so      excessive coughing. This may happen for a
                                that healthcare professionals can assess       number of reasons, including excess saliva,
        MECHANICS OF            your body’s ability to get the nutrition it    not sitting up enough, the feeding rate is
         SWALLOWING             needs. They will prescribe the quantity and    too fast, or because of various other
MAKING EATING EASIER            type of nutritional supplements you should     stomach problems.
                                receive through tube feedings. During this
           CHANGES TO                                                          If this happens to you, consult your
            YOUR DIET           period, learn how your new tube-feeding
                                system works, how you initially react to       dietitian who may suggest reducing your
                      PILLS     liquid food, and how to control the flow,      feeding rate. Another option is to lower the
             NUTRITION          particularly if you start coughing.            height of the feeding container to just
                                                                               above the height of your stomach and fully
         TUBE FEEDING                                                          open the valve. This will allow your
                                Feeding Pump
                                                                               stomach to take in the food at its natural
                                When you first get a feeding tube, a           rate rather than a rate determined by
                                feeding pump may be necessary. However         higher gravity or a pump. The ability to
                                when your body gets used to tube feeding,      digest liquid nutrients varies greatly from
                                gravity feeding from an IV pole without a      person to person.
                                pump will probably be sufficient.
                                                                               Lying Down
                                Nasal Feeding Tube
                                                                               After tube feeding you should not lie down
                                No surgery is required for nasal feeding.      for at least one to two hours. When lying
                                Some nasal tubes can be taken out after        down it is best to lie on your right side.
                                each feeding, while others are left in place   The stomach empties into the intestines
                                for weeks. Changing nasal tubes requires       through the right side of the stomach
                                special training and cleanliness. Nasal        cavity; therefore when lying on your right
                                tubes must be relatively thin, and therefore   side, liquid that has remained in your
                                are only suitable for thin liquids. Nasal      stomach will more likely flow into the
                                tubes are generally only used for people       intestines, rather than back up the throat.
                                who are immobile.




 ALS               SLA
  SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                   PAGE 52                                                                                             v. 1.0
LIVING WITH ALS                                                                                       SECTION         6
DEALING WITH SWALLOWING DIFFICULTIES

Home care                                      Tube-Feeding Diet
                                                                                              DEALING WITH
After you have a feeding tube installed,       A dietitian at the hospital will usually       SWALLOWING
and before leaving the hospital, arrange for   work out your tube-feeding requirements        DIFFICULTIES
appropriate home care assistance while         based on what you can still eat normally
you are getting used to tube feeding. You      and your estimated calorie requirements.       MECHANICS OF
will probably have some questions              Because your eating abilities will change,     SWALLOWING
regarding tube feeding, cleaning               your tube-feeding requirements will            MAKING EATING EASIER
procedures, supplies, suppliers,               probably increase over time. When this
                                                                                              CHANGES TO
government assistance programs, etc. Also,     occurs, increased feeding tube                 YOUR DIET
hooking up the feeding bag requires a few      requirements should be recalculated by a
tricks to prevent sending a tube full of air   dietitian. Note that most dietitians, and      PILLS
into your stomach before the food.             many doctors, are not familiar with the        NUTRITION
                                               effects of ALS and may prescribe volumes
                                                                                              TUBE FEEDING
Dehydration                                    of food in excess of your body’s ability to
                                               absorb it. This can cause severe coughing if
It is a serious mistake to assume that you     the liquid is increased to a level beyond
are getting enough liquid because you are      what your system can tolerate. If you find
living on liquid nutrients. Concentrated       the liquid nutrition you are taking causes
liquid nutrients do not have much liquid!      thick mucus in your throat, try mixing
Take lots of water or other liquids to avoid   some thinner milk with it.
dehydration, which will cause a lot of
problems you don’t need.                       Commercial Prepared Food
Choosing Tube-Feeding Equipment                You should discuss the various
                                               commercially prepared alternatives
When you first have your feeding tube          with a dietitian.
installed, you can learn in the hospital
what your equipment needs will be. When
you are at home you may first be using
equipment supplied by your regional ALS
Society Unit or home care for a few weeks,
after which you will need to get your own
equipment. You will need a method to
hold up the container while feeding, such
as an IV pole, a supply of feeding
containers, some feeding syringes and a
supply of liquid food.

See Section 11 for more details about tube-
feeding equipment.


                                                                                              ALS                 SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 53
SECTION         6              LIVING WITH ALS
                                           NOTES




ALS               SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 54                     v. 1.0
LIVING WITH ALS          SECTION          6
NOTES




                  ALS                 SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 55
SECTION         6              LIVING WITH ALS
                                           NOTES




ALS               SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 56                     v. 1.0
LIVING WITH ALS                                                                                      SECTION          7
MOUTH CARE

MOUTH CARE                                    Going to the dentist
                                                                                              MOUTH CARE
                                              Not many dentists will know about ALS,
Weakened mouth and throat muscles,                                                            MOUTH CARE
                                              its effect on your ability to keep your teeth
weakened hands and arms to brush teeth,
                                              clean, excess saliva, or the precautions they   MANAGING SALIVA
and tube feeding all are changes which
                                              should take because of a patient’s
require special treatment for mouth care.                                                     MANAGING A
                                              swallowing problems. Make your dentist          DRY MOUTH
Food can easily collect in pockets in the
                                              fully aware of these problems.
mouth as well as between teeth. It is
important that you take care of your
mouth to prevent harmful bacteria from        Suction
developing.                                   Another method for moving food caught
                                              in pockets of the mouth is a suction unit,
Freshness                                     which is also used for removing excess
                                              saliva to reduce drool.
An applicator with lemon and water can
be used to freshen the mouth if other
methods are a problem.                        Toothbrushes
                                              For those with weakened hands and arms,
Furry Tongue                                  an electric toothbrush can be very useful
                                              for brushing your teeth. Others may find
Some ALS patients with bulbar symptoms
                                              that a hard toothbrush is very helpful for
develop a furry tongue. Water may be used
                                              removing build-up which occurs on the
to clean the tongue with an applicator or
                                              teeth, particularly after tube feeding.
washcloth. If the tongue appears white for
any length of time, consult your ALS team
or your doctor. Sometimes a condition
called ‘thrush’ can develop. It is a fungal
infection and is easily treated.




                                                                                              ALS              SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 57
SECTION         7                                       LIVING WITH ALS
                                                                                                      MOUTH CARE

MOUTH CARE                     MANAGING SALIVA                                 The following have proven helpful for
                                                                               treating thick mucus:
MOUTH CARE                     Build-up of saliva is a common problem
                                                                               • Apple juice or hot tea with lemon
                               among people with ALS who have tongue
MANAGING SALIVA                                                                • SSK1 (potassium iodide) 10 drops in a
                               and throat muscles that are weak and not
MANAGING A                     able to automatically swallow the saliva          glass of water 2–3 times per day. This
DRY MOUTH                      that builds up in the mouth. A tenacious          may take 1–2 weeks to be effective. A
                               mucus can also build up in the mouth,             prescription is required;
                               compounding the problem.                        • An expectorant cough syrup;
                               This build-up of saliva can cause choking       • Meat tenderizer mixed with a little
                               and disrupt sleep. Relief may come from           water can be used to coat the tongue or
                               home remedies, over-the-counter products,         can be placed under the tongue.
                               and prescription drugs and, in extreme          Cautions
                               cases, even surgical procedures. Advice
                               should be obtained from your doctor             For over-the-counter medication, generic
                               and/or your local pharmacist. An obvious        labels are usually cheaper.
                               side effect of drugs to relieve a build-up of
                               saliva is a dry mouth. It’s a question of       Sleepiness is often the side effect of
                               finding the right balance for you.              medications for a dry mouth.

                               The following have proven helpful for           Beware: anything containing alcohol can
                               managing saliva:                                increase muscle weakness, at least
                                                                               temporarily.
                               • Portable suction machine;
                                                                               Beware of anything that slows breathing.
                               • Decongestants and over-the-counter
                                 medications used for colds and allergies;     Beware: before putting any medication,
                               • Antidepressants - Amitriptyline may           crushed or otherwise in a feeding tube,
                                 reduce saliva and enable sleep;               check with a pharmacist to ensure that it
                               • Anticholinergic drugs to reduce spasms        won’t harden and clog the tube.
                                 of smooth muscle in the bronchi and           Excess Saliva - has one advantage! If you
                                 decrease gastric, bronchial and salivary      have excess saliva, and are still able to eat,
                                 secretions;                                   mixing more saliva with your food, makes
                               • Transderm V patches usually used for          it both easier to swallow, and easier to
                                 motion sickness also tend to dry up           digest.
                                 saliva.




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 58                                                                                                 v. 1.0
LIVING WITH ALS                                                                                     SECTION          7
MOUTH CARE

MANAGING A DRY MOUTH                           The following have proven helpful for a dry
                                                                                             MOUTH CARE
                                               mouth:
Although management of saliva is                                                             MOUTH CARE
                                               • Make a conscious effort to breathe
common in many people with ALS,
                                                 through your nose;                          MANAGING SALIVA
especially those with difficulty swallowing,
some are troubled by excessive mouth           • If you sleep with your mouth open,          MANAGING A
dryness. A dry mouth can cause thick             ask your doctor or occupational therapist   DRY MOUTH
mucus to form, which may in turn cause           about the possibility of a chin-strap to
serious choking problems.                        wear at night;
                                               • If you suffer from nasal congestion,
Excessive dryness is usually caused by one       ask your doctor for assistance.
or more of the following:                        Nasal congestion leading to a dry mouth
• Breathing primarily through the mouth,         can cause thick mucus to form, which
  rather than the nose can dry out the           in turn may cause serious choking
  lining in your mouth. If you consistently      problems;
  wake up with a dry mouth, thick mucus        • Increase your liquid intake. Ask your
and/or a sore throat you may be                  speech pathologist which consistency is
  breathing through your mouth while             best for you;
  sleeping;                                    • Use a vaporizer. (Clean the vaporizer
• A side effect from some surgical               once a week with vinegar and water.)
  operations;
• Stress from chronic anxiety or
  depression;
• A side effect from some medications;
• The natural process of aging;
• Smoking.




                                                                                             ALS              SLA
                                                                                             SOCIETY OF CANADA
                                                                                             Maladie Lou Gehrig’s Disease

                                                                                             1(800)267-4257
                                                                                             http://www.als.ca
                                                                                             alscanada@als.ca


v 1.0                                                                                        PAGE 59
SECTION         7              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 60                     v. 1.0
LIVING WITH ALS          SECTION          7
NOTES




                  ALS              SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 61
SECTION         7              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 62                     v. 1.0
LIVING WITH ALS                                                                                         SECTION          8
DEALING WITH SPEAKING DIFFICULTIES

When the muscles of the face, throat, neck      Conserve energy, speak slowly with short
and tongue are weakened, you may have           sentences and use non-verbal signals. If
                                                                                                 DEALING WITH
difficulty with speaking, chewing,              forming words is a problem, try using            SPEAKING
swallowing and controlling mucus and            words that are easy to pronounce. Also, it       DIFFICULTIES
saliva. The onset of ALS with these types       can be very helpful to work out hand             COMMUNICATION
of symptoms is known as Bulbar ALS.             signals for frequently used phrases with         DEVICES
                                                your immediate family and other
Losing your ability to speak is a very                                                           HIGH-TECH
                                                caregivers. If you can still use your fingers,   STRATEGIES
significant change for most people. It can      it will be helpful to always carry a pad and
be frustrating for both you and your            paper with you so that you can write out         LOW-TECH
listener. For you, it is the end of off-the-                                                     STRATEGIES
                                                your messages.
cuff remarks and participating in quick
conversation. For your listener it becomes      Patients on ventilation with respirators
a question of trying to interpret. Some         have to contend with the type of breathing
people are much better at it than others.       device that has been inserted into their
Speech therapy is helpful to determine          throat. They may be able to speak, but will
which muscles are weak and how to best          need to be trained to do so by a respiratory
use the muscles still available for speaking.   therapist.

Speaking problems are generally caused by
weaknesses in one or more of the
following muscle groups:

• Breathing muscles – resulting in
  weakened air pressure required to make
  sound;
• Vocal cords – resulting in soft or
  monotonous sounds;
• Soft palate and throat muscles –
  resulting in a nasal sound;
• Tongue and lip muscles – resulting in
  difficulty forming word sounds.




                                                                                                 ALS              SLA
                                                                                                 SOCIETY OF CANADA
                                                                                                 Maladie Lou Gehrig’s Disease

                                                                                                 1(800)267-4257
                                                                                                 http://www.als.ca
                                                                                                 alscanada@als.ca


v 1.0                                                                                            PAGE 63
SECTION         8                                       LIVING WITH ALS
                                                               DEALING WITH SPEAKING DIFFICULTIES

                               COMMUNICATION DEVICES                           HIGH-TECH STRATEGIES
DEALING WITH
    SPEAKING                   Communication devices facilitate sharing        Microcomputers
  DIFFICULTIES                 information, self-expression, signalling for
                               attention, maintaining contact with others,     If you are willing to use a high-tech
   COMMUNICATION
          DEVICES              and problem solving.                            communication system, information about
                                                                               systems that meet your preferences, needs
            HIGH-TECH          What communication equipment is best for        and functional abilities should be discussed
            STRATEGIES
                               you is very much a personal decision. Some      with a speech-language pathologist. There
             LOW-TECH          people want to avoid electronic equipment       is a wide range of alternative-input
             STRATEGIES        while others want the latest, most              computers available. Some word
                               sophisticated equipment available. Each         processors, designed to compensate for
                               device should be chosen with the unique         specific disabilities, have specialized
                               needs of the individual in mind. You            keyboards that accommodate a limited
                               should also be aware of how long the            range of motion or poor hand control.
                               chosen system will be useful to you. It is      Some software supports a variety of input
                               most likely that you will need several          methods, for example, Morse code. Other
                               devices as the disease progresses.              systems work in response to laser beams,
                                                                               eye blinks, or other eye movements.
                               It is also essential that you get advice from
                               an assistive technology clinic, from a          In the later stages of ALS, a system that
                               speech-language pathologist or from             can be operated by a user trained in switch
                               another professional source before              use and scanning may be more practical.
                               purchasing any equipment. You can also          Single-switch systems feature options that
                               visit local suppliers and try out the various   allow control of the total environment.
                               options.
                                                                               For example, one technique is to have the
                               Think through what your needs are, and          cursor slowly scan a keyboard displayed on
                               will be in the future.                          the screen, so the operator just has to press
                                                                               the on/off switch when the cursor is on the
                               • Will you need a device mostly for             key they want. Another assistive technique,
                                 communicating commonly used phrases?          called word prediction, will display 10
                               • Are you able to write messages?               commonly used words on the screen that
                               • Will you have to make presentations to        start with the letters that the operator is
                                 groups of people?                             typing in. Each predicted word has a
                                                                               number beside it. If the computer predicts
                               When choosing a device, some of the             the word the operator wants, the operator
                               following factors may be relevant:              can enter the whole word by pressing the
                                                                               switch when the cursor is on the correct
                               • Level of training needed and the              number. Some of this software also has a
                                 complexity of the system                      feature which can be used with a computer
                               • Cost                                          to turn on and off a TV, VCR, light
                               • Portability                                   switches and other electronic equipment.
ALS              SLA           • Versatility
                                                                               As these machines are highly sophisticated,
SOCIETY OF CANADA                                                              their cost is considerable.
Maladie Lou Gehrig’s Disease   • Speed of communication
                               • Circumstances of use
          1(800)267-4257
          http://www.als.ca    • Amount of follow-up needed
           alscanada@als.ca


                  PAGE 64                                                                                               v. 1.0
LIVING WITH ALS                                                                                       SECTION          8
DEALING WITH SPEAKING DIFFICULTIES

Voice Synthesizers and Computers                LOW-TECH STRATEGIES
                                                                                               DEALING WITH
A voice synthesizer allows communication
                                                Often simple manual systems are preferred
                                                                                               SPEAKING
over the telephone with a group of people,
                                                because they permit continuing human           DIFFICULTIES
as well as in face-to-face interaction. The
                                                contact without requiring the higher levels    COMMUNICATION
artificial voice is linked to a computer, and
                                                of energy, technical knowledge and skill       DEVICES
is activated when you type, operate a
                                                and motivation needed to operate more          HIGH-TECH
switch, move a cursor, or use Morse code
                                                sophisticated equipment.                       STRATEGIES
to translate thoughts into speech. The
switching mechanisms are often extremely                                                       LOW-TECH
sensitive, and need minimal user                Communication Boards                           STRATEGIES
movement to operate; a head turn or eye         Writing boards (for example, white boards,
movement is often adequate.                     magic slates) are often the preferred choice
                                                when you can still write. Once grip is
Voice Amplifiers                                impaired, your gaze is often an efficient
                                                and reliable method of communicating.
These devices enhance the volume of the
                                                Communication boards that use gaze
voice. They may be considered if
                                                usually feature alphabet letters, symbols
articulation is adequate, and respiratory
                                                and/or complete words, phrases, or
weakness is the cause of decreased
                                                sentences. To use the board, you point to,
speaking volume.
                                                look at, or use a device or otherwise
                                                indicate the desired communication.
                                                Words usually included on
                                                communication boards are lists of foods,
                                                comfort items and positioning requests.
                                                The more information there is, the larger
                                                the board must be.

                                                When only face-to-face communication is
                                                needed and you can still indicate selections
                                                on a board by pointing or eye movements,
                                                a word or letter board is a good low-tech
                                                communication choice.




                                                                                               ALS              SLA
                                                                                               SOCIETY OF CANADA
                                                                                               Maladie Lou Gehrig’s Disease

                                                                                               1(800)267-4257
                                                                                               http://www.als.ca
                                                                                               alscanada@als.ca


v 1.0                                                                                          PAGE 65
SECTION         8                                       LIVING WITH ALS
                                                              DEALING WITH SPEAKING DIFFICULTIES

                               Signalling Systems                             Communication Assistive Devices
DEALING WITH
    SPEAKING                   These no-cost systems are usually worked       If you cannot speak you may prefer to
  DIFFICULTIES                 out between you and your regular               write or type out messages as long as you
                               communication partners. Signalling makes       are able to do so. Assistive devices to aid
   COMMUNICATION               use of facial expressions, eye contact, eye    manual communication include pencil
          DEVICES
                               movements, gestures, touch and body            grips, magic slates and other portable
            HIGH-TECH          language. Some of this system’s drawbacks      boards, book holders, tilt-top tables,
            STRATEGIES         include the limited nature of possible         pointers, typing sticks (held in the mouth,
            LOW-TECH           responses and the inability to                 hand, or foot), writer’s splints, TTY
            STRATEGIES         communicate with people who are                (teletyper), TTD (telephone for the deaf),
                               unfamiliar with the system.                    and page turners. If you leave home
                                                                              without a caregiver, it may be wise to wear
                               Personal signalling systems are a valuable     a medical emergency bracelet.
                               backup to high-tech systems that are
                               vulnerable to system failure.                  A palatal lift or other hard-palate
                                                                              prosthesis may be considered as a short-
                               However, a 24-hour monitoring alarm (for       term option in cases where existing speech
                               example, a bell, intercom, or buzzer) is       can be improved. A prosthesis may
                               necessary to enable you to request             improve swallowing and reduce problems
                               attention.                                     associated with excessive salivation.
                                                                              However, oral devices cannot compensate
                               You and your family may want to apply
                                                                              for a lack of speech caused by weakness or
                               for an emergency response service. Those
                                                                              paralysis of the lips, tongue, larynx or
                               who join are given a small (about the size
                                                                              breathing muscles. Also, as ALS is
                               of a small box of matches) transmitter
                                                                              dynamic, a prosthesis may not be a
                               with an emergency button on it.
                                                                              realistic option in cases where there is
                               When joining this program people advise        rapid change in communication function
                               the service of :                               and control. You should consult a speech-
                                                                              language pathologist or prosthodontist to
                               • Facts about their illness;                   determine your best option.
                               • The names and phone numbers of
                                 people who should be contacted in case       Specialized Telephone Equipment
                                 of emergency.
                                                                              Special phone services are available. If you
                               When the person presses the emergency          are unable to lift a phone receiver and/or
                               button on the small transmitter, a signal is   dial a phone number, there is a hands-free
                               sent to the service that tells them who has    phone, activated by blowing on a switch,
                               the emergency. They will know the              plus an operator dialing service. Speaker
                               patient’s illness and immediately call the     phones may also be helpful. See your local
                               names given to them for emergency              phone book for the specialized services in
                               purposes.                                      your area.

ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 66                                                                                              v. 1.0
LIVING WITH ALS          SECTION          8
NOTES




                  ALS              SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 67
SECTION         8              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 68                     v. 1.0
LIVING WITH ALS                                                                                    SECTION          9
ADAPTING TO BREATHING DIFFICULTIES

As with other muscle weaknesses in people   MECHANICS OF BREATHING
with ALS, the point at which breathing
                                                                                            ADAPTING TO
muscles begin to weaken is unpredictable.   Normal “easy” breathing involves two main
                                                                                            BREATHING
With some, these are the first muscles to   muscle groups. When you breathe in, the
                                                                                            DIFFICULTIES
weaken; for others they are the last. For   diaphragm moves down; at the same time          MECHANICS OF
most people with ALS, death comes                                                           BREATHING
                                            the intercostal muscles between your ribs
peacefully as a result of weakened          contract to pull your rib cage up and out.      SYMPTOMS OF
breathing muscles.                          These two actions cause a partial vacuum.       WEAKENED BREATHING
                                                                                            MUSCLES
                                            Fresh air rushes down the windpipe
                                            through the bronchi, the largest air passages   TWO SIMPLE
                                            in your lungs, and then into the small air      STRATEGIES
                                            sacs which pass fresh oxygen to the blood.      RESPIRATORY DISEASES
                                            This process is called inspiration.
                                                                                            VENTILATORS
                                            When you breathe out, both diaphragm
                                            and intercostal muscles relax, decreasing
                                            the size of your chest cavity. Used air that
                                            contains waste carbon dioxide is pushed out
                                            of your lungs.

                                            If you are breathing heavily, two additional
                                            muscle groups come into play. When you
                                            take a deep breath in, muscles in the neck
                                            that attach to the collarbone and upper ribs
                                            assist in breathing. When you force a breath
                                            out, your abdominal muscles help to push
                                            up the diaphragm.




                                                                                            ALS              SLA
                                                                                            SOCIETY OF CANADA
                                                                                            Maladie Lou Gehrig’s Disease

                                                                                            1(800)267-4257
                                                                                            http://www.als.ca
                                                                                            alscanada@als.ca


v 1.0                                                                                       PAGE 69
 SECTION         9                                       LIVING WITH ALS
                                                              ADAPTING TO BREATHING DIFFICULTIES

                                SYMPTOMS OF WEAKENED                            When you stand upright, the diaphragm
  ADAPTING TO                   BREATHING MUSCLES                               moves down when you breathe in. When
    BREATHING                                                                   you lie down, the organs in your abdomen
   DIFFICULTIES                 Fatigue                                         press against the diaphragm, and more
        MECHANICS OF                                                            strength is required for the diaphragm to
           BREATHING            Fatigue is a common symptom of ALS. It          move down during breathing. Therefore,
       SYMPTOMS OF              is caused by a number of factors.               you might breathe less effectively at night if
WEAKENED BREATHING                                                              you are lying flat.
           MUSCLES              As ALS attacks your motor neurons, they
                                become unable to send commands from             To help cope with this problem, you
            TWO SIMPLE
             STRATEGIES         your brain to the muscle cells that they        should try to raise your head and
                                control. A smaller number of muscle cells       shoulders during sleep. You can
 RESPIRATORY DISEASES           must then try to perform jobs usually done      accomplish this by using two or more
           VENTILATORS          by the full number. The result is that your     pillows under your head and shoulders, by
                                muscles tire before they normally would.        choosing a wedge-shaped pillow, or by
                                                                                raising the head of your bed on blocks 4”
                                As your respiratory muscles are affected by     to 6” high.
                                ALS, they are able to draw less air into
                                your lungs. When activity increases, it
                                                                                Shortness of Breath with Activity
                                becomes more difficult for the lungs to
                                supply enough oxygen to the body. Other         Shortness of breath may be the first
                                metabolic changes take place and you feel       symptom of the weakening of breathing
                                fatigued.                                       muscles. You may notice shortness of
                                                                                breath after rushing upstairs, carrying a
                                ALS produces many changes in your life.
                                                                                load, etc. Shortness of breath can also
                                Change often results in general stress that
                                                                                occur with no particular exertion; you may
                                can manifest itself as fatigue.
                                                                                not be able to walk or talk for long without
                                Don’t let yourself become fatigued. Rest        becoming winded. You should tell your
                                when you begin to feel tired. Try to keep       doctor about these symptoms. When you
                                your exertions within the limits that your      experience shortness of breath, stop what
                                body is now imposing. Pay attention to          you are doing. Shortness of breath will
                                signals like fatigue. It may be necessary for   disappear when activity is reduced. Relax
                                you to change your priorities and forego        in one of the following positions if you are
                                less important activities. Plan your day to     able and breathe slowly and deeply.
                                include regular rest periods.
                                                                                Sit at a table, lean forward with a straight
                                                                                back and rest your head and shoulders on
                                Morning Fatigue                                 a pillow on the table.
                                Some people with ALS experience                 Sit on a chair or the edge of your bed, lean
                                morning fatigue. You may wake up feeling        forward, keep your back straight and rest
                                tired, sometimes with a headache, and the       your elbows and forearms on your thighs.
                                feeling that you slept poorly. This may be
                                caused by a weakness in the diaphragm.
 ALS              SLA                                                           Lean against a wall with your feet about
                                                                                12 inches from the wall. Rest your lower
  SOCIETY OF CANADA                                                             back against the wall and lean your upper
 Maladie Lou Gehrig’s Disease
                                                                                back away from the wall.
           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                   PAGE 70                                                                                                v. 1.0
LIVING WITH ALS                                                                                        SECTION          9
ADAPTING TO BREATHING DIFFICULTIES

Weak Cough                                      Excess Mucus and Secretions
                                                                                                ADAPTING TO
When you cough, your stomach muscles            In some people, excess mucus and                BREATHING
and intercostal muscles between the ribs        secretions can build up to the point where      DIFFICULTIES
contract quickly. This forces air from the      they cannot be easily cleared by coughing.      MECHANICS OF
lungs and up the airway. Any mucus or           This is particularly the case first thing in    BREATHING
food in one of the air passages is forced       the morning when secretions have built up
                                                                                                SYMPTOMS OF
out as well. People with ALS have normal        during the night. If you experience this        WEAKENED BREATHING
cough reflexes, but the muscles may be          problem, ask your physiotherapist to            MUSCLES
weakened and unable to produce a strong         instruct you in methods to loosen
                                                                                                TWO SIMPLE
cough. You can strengthen your cough            secretions and bring them to the mouth to       STRATEGIES
using a technique known as “splinting the       be spit out.
abdomen.”                                                                                       RESPIRATORY DISEASES
                                                One method of dealing with mucus and            VENTILATORS
                                                saliva build-up is suctioning. Suction
Splinting the Abdomen
                                                equipment acts very much like a dentist’s
First, take a deep breath, then clasp your      suction tube; it consists of a motor,
arms firmly across your lower abdomen           collection bottle, tubing and catheters. Talk
and bend over a high-backed chair. Just as      to your nurse or physiotherapist about
you are coughing out, squeeze your arms         suction equipment; he or she will be able
in tightly so that you push your abdomen        to tell you and your caregiver where to get
in and down. It is important to time the        the equipment and how to use it properly.
bending and squeezing to coincide with
the expelling phase of your cough.

A variation on the self-assisted cough can
be performed while sitting on a hard
surface. Clasp your arms over your
abdomen and as you are about to cough
out, bend forward quickly and squeeze the
abdomen in and down.

If your arms are too weak to perform this
maneuver, ask someone to help by
pressing your forearms firmly into your
abdomen, bending you forward as you
cough. Ask your healthcare professional to
assist you and your caregiver to perfect this
technique.




                                                                                                ALS              SLA
                                                                                                SOCIETY OF CANADA
                                                                                                Maladie Lou Gehrig’s Disease

                                                                                                1(800)267-4257
                                                                                                http://www.als.ca
                                                                                                alscanada@als.ca


v 1.0                                                                                           PAGE 71
 SECTION         9                                      LIVING WITH ALS
                                                             ADAPTING TO BREATHING DIFFICULTIES

                                TWO SIMPLE STRATEGIES                         RESPIRATORY DISEASES
  ADAPTING TO
    BREATHING                   Exercises to use full lung capacity can       It is a good idea to avoid people who have
   DIFFICULTIES                 compensate for weakened muscles. This         colds or flu if you can. People with ALS
        MECHANICS OF            can be achieved with the following deep       are no more likely to catch these infections
           BREATHING
                                breathing exercise:                           than other people, but they are at more risk
       SYMPTOMS OF                                                            of having simple respiratory infections
WEAKENED BREATHING              Sit at a table, hunched over slightly with    develop into pneumonia.
           MUSCLES              your weight supported on your hands or
            TWO SIMPLE          elbows. This position expands the rib cage    If you do get a fever with bloody mucus
             STRATEGIES         for larger breaths. Also, supporting the      coughed from the chest or if you notice
                                shoulders helps the neck muscles assist       that the mucus has changed from clear
 RESPIRATORY DISEASES
                                breathing. Breathe in as much as possible,    white to yellowish, tell your doctor
           VENTILATORS          hold the breath for a few seconds, and then   immediately. These could be symptoms of
                                exhale. Repeat several times.                 a respiratory infection that could lead to
                                                                              pneumonia.
                                Stop smoking. In addition to all of the
                                other problems smoking causes, it reduces
                                lung capacity. It can also cause increased
                                phlegm that some patients will have
                                difficulty clearing from their airways.

                                Some doctors believe that patients who do
                                deep-breathing exercises are less prone to
                                lung infections and partial lung collapse.
                                Ask your physiotherapist to demonstrate
                                an exercise routine and advise you about
                                the use of exercise devices.




 ALS              SLA
  SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                   PAGE 72                                                                                            v. 1.0
LIVING WITH ALS                                                                                        SECTION          9
ADAPTING TO BREATHING DIFFICULTIES

VENTILATORS                                      There are various devices that can assist
                                                 breathing. It is best to ask a doctor about
                                                                                                ADAPTING TO
Failure of the respiratory muscles is an         each of the alternatives available in your     BREATHING
eventual result of ALS. Modern medical           area, and the long-term implications of        DIFFICULTIES
technology can offer portable life-support       each. Two common alternatives are non-         MECHANICS OF
                                                 invasive ventilation and tracheostomy.         BREATHING
equipment, but the choice to use such
equipment will have important                                                                   SYMPTOMS OF
consequences for your way of life and that       Non-Invasive Ventilation                       WEAKENED BREATHING
                                                                                                MUSCLES
of your family and caregivers.
                                                 This system does not require an operation.     TWO SIMPLE
Ventilators are “controlling” devices that       It uses a pump called a BI Pap which           STRATEGIES
take over your breathing. The decision of        produces two levels of pressure, and is
                                                 attached to a small mask which fits over the   RESPIRATORY DISEASES
whether or not to use a ventilator is yours,
but it is a decision that you should make        mouth and nose. The two-level pressure         VENTILATORS
only after consulting with your family,          system keeps the airways open, and air
your doctor, and other healthcare workers.       going to the lungs.

Your doctor can tell you that you need a
respirator. Only you can decide if you
want one. You will have to decide whether
you are willing to accommodate your life
and that of your family to dependence on
a respirator. Can you count upon the
assistance of family members, including a
primary caregiver? It is important to
realize that technology has produced
portable, lightweight ventilators that allow
considerable mobility including travel. You
should discuss the available options with
both your medical practitioners and your
family. If at all possible, the decision about
a respirator should be made before a
respiratory emergency develops, and you
should ensure that your family is aware of
your decision in case an emergency does
occur. If you do decide that you are
interested in ventilation, you will need to
be referred to a respirologist.




                                                                                                ALS              SLA
                                                                                                SOCIETY OF CANADA
                                                                                                Maladie Lou Gehrig’s Disease

                                                                                                1(800)267-4257
                                                                                                http://www.als.ca
                                                                                                alscanada@als.ca


v 1.0                                                                                           PAGE 73
 SECTION         9                                       LIVING WITH ALS
                                                              ADAPTING TO BREATHING DIFFICULTIES

                                Tracheostomy                                    Tracheostomy -
  ADAPTING TO                                                                   The Decision-Making Process
    BREATHING                   A tracheostomy is a surgical procedure
   DIFFICULTIES                 that makes an opening in the neck which         ALS is a disease that will ultimately
        MECHANICS OF            connects the throat’s passageway to the         disable all body movement, and yet a
           BREATHING            lungs, known as the trachea. In the             person with ALS can continue to be kept
                                surgical opening a medical or plastic tube      alive artificially. It is best to make a
       SYMPTOMS OF
WEAKENED BREATHING              is put in place. This tube is connected via a   decision about the use of ventilation before
           MUSCLES              hose to a volume ventilator, which assists      a medical crisis. You should decide what
                                with breathing. When necessary a                level of inactivity you wish to endure, and
            TWO SIMPLE
             STRATEGIES         suctioning device can be inserted into the      give guidance to others about
                                tube in the trachea to remove secretions.       implementing artificial life support
 RESPIRATORY DISEASES                                                           systems. Will your quality of life make it
           VENTILATORS          Before making a decision about having a         worthwhile? This should be thought
                                tracheostomy or similar operation, both         through and discussed with family
                                you and your family should learn from a         members, long before a decision is
                                doctor about the full implications of           required. Factors in this decision should
                                having this type of an artificial breathing     include family and financial concerns, as
                                device. For example, this will require full-    well as your wishes. If you are considering
                                time, 24-hour support from trained              a tracheostomy, try to arrange a discussion
                                caregivers. To ensure that the equipment is     with family members of a person who is
                                always running properly, dressings are          living on a ventilator. Learn what they are
                                changed as required, feedings are attended      going through so that your family will be
                                to, etc. It is a full-time job for 3 people,    prepared. An extremely helpful review of
                                assuming 8-hour shifts.                         these factors is covered in the videotape
                                                                                Ventilation - The Decision Making Process
                                                                                produced by the Les Turner Foundation, a
                                                                                leading ALS centre in Skokie, Illinois,
                                                                                USA. Also, the GF Strong Rehabilitation
                                                                                Centre in Vancouver has a brochure called,
                                                                                Making a Decision on Ventilators.




 ALS              SLA
  SOCIETY OF CANADA
 Maladie Lou Gehrig’s Disease

           1(800)267-4257
           http://www.als.ca
            alscanada@als.ca


                   PAGE 74                                                                                              v. 1.0
LIVING WITH ALS          SECTION          9
NOTES




                  ALS              SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 75
SECTION         9              LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 76                     v. 1.0
LIVING WITH ALS                                                                                  SECTION          10
ASSISTIVE EQUIPMENT

BEFORE PURCHASING EQUIPMENT                   Equipment Suppliers
                                                                                             ASSISTIVE
Before purchasing assistive equipment, you
                                              Most suppliers of equipment for disabled       EQUIPMENT
                                              patients are listed in the phone book under
are advised to consult with professionals                                                    BEFORE PURCHASING
                                              “Hospital Equipment and Supplies.”
who are familiar with the advantages and                                                     EQUIPMENT
disadvantages of the equipment that is        HELP YOURSELF! Hints for Persons               BATHROOM
available to serve your needs.                with Disabilities is a very useful book        EQUIPMENT
                                              published by Human Resources                   BEDS AND
Think through what your needs are, both
                                              Development Canada, and distributed by         MATTRESSES
immediate and longer term.
                                              the Ministry of Supply and Services
                                                                                             BODY SUPPORTS
Discuss your needs with a doctor, nurse,      Canada. Cat. No. H21-95/1994E. It is
physiotherapist, occupational therapist or    filled with helpful hints, including many      COMMUNICATION
                                              easy to make devices. The book has             EQUIPMENT
other healthcare professional with
knowledge of your condition, and              sections on Personal Care; In the Kitchen;     LIFTS AND
familiarity with the available equipment to   Cooking Techniques; Living Easier;             STAIR GLIDES
serve those needs.                            Reading, Writing and Telephone; Safety;        TUBE-FEEDING
                                              In the Car; and Recreation Activities for      EQUIPMENT
Find out from healthcare professionals        the Disabled.                                  WALKING –
what loan equipment or financial                                                             CANES AND WALKERS
assistance might be available.                Home Care                                      WHEELCHAIRS
Visit local suppliers and test out the        Home Care personnel are another source         OTHER EQUIPMENT
various options where possible.               of information about assistive equipment.
                                              Many of them have probably visited other
Financial Support                             people with similar needs, and have seen
                                              how useful different types of equipment
Check with your local healthcare              have been.
professional and equipment suppliers to
find out what government support              BATHROOM EQUIPMENT
programs are available in your area for
equipment, and for home renovations
                                              There are several devices to help disabled
required to accommodate your disability.
                                              people in the bathroom. Raised toilet seats
Some programs will fund only one project
                                              are a standard requirement for those with
per patient. Therefore, consider carefully
                                              weakened leg muscles. These range from
what your longer term needs are likely to
                                              homemade and low-cost seats to more
be before applying for this type of
                                              expensive models. Another option is to
assistance program.
                                              have a plumber raise the level of the toilet
                                              by putting it on a low platform. Also, there
ALS Society Equipment                         are a number of alternatives for assisting
Some ALS Units and Chapters in your           with bathing. There are several models of
area may have a limited inventory of          bathtub seats and lifting equipment that
equipment acquired through the generosity     can assist you in sliding or rotating over
of contributors and for use by needy          the tub for showering. Retail stores that      ALS              SLA
people with ALS.                              handle equipment for the disabled often        SOCIETY OF CANADA
                                              have a variety of bathroom aids on display.    Maladie Lou Gehrig’s Disease
See Section 14 for details regarding          Check with your occupational therapist or      1(800)267-4257
equipment-lending programs.                   other healthcare professional for their        http://www.als.ca
                                              assistance in choosing these aids.             alscanada@als.ca


v 1.0                                                                                        PAGE 77
SECTION         10                                      LIVING WITH ALS
                                                                                          ASSISTIVE EQUIPMENT

                               BEDS AND MATTRESSES                               Hand and Wrist Supports
       ASSISTIVE
     EQUIPMENT                 A common problem during the advanced
                                                                                 A wrist and thumb splint can stabilize the
                                                                                 wrist and thumb, helping you to grasp eating
BEFORE PURCHASING              stages of ALS is being unable to roll over in
                                                                                 utensils and other objects. Additional
        EQUIPMENT              bed. Lying in one position becomes
                                                                                 supports can be added for positioning the
                               intolerably painful, or requires a caregiver to
            BATHROOM                                                             fingers, enabling you to make finer
            EQUIPMENT          move you every few hours. There are now
                                                                                 movements with weakened fingers, such as
                               specially segmented, air filled mattresses, (a
             BEDS AND                                                            are required for writing.
                               KVC bed) which are attached to an
            MATTRESSES
                               electronic pumping device which                   For those in wheelchairs, there are more
      BODY SUPPORTS            continually changes the air levels in different   sophisticated supporting devices that help a
   COMMUNICATION               cells in the mattress, thereby gradually          person with ALS eat. This requires adapting
       EQUIPMENT               rotating the sleeper during the night.            these devices to each person’s individual
             LIFTS AND                                                           situation.
           STAIR GLIDES        BODY SUPPORTS
         TUBE-FEEDING                                                            Shoulder and Neck Supports
           EQUIPMENT           The various types of body supports available
                               are called orthotic devices. Available            Weakened shoulder muscles can cause the
        WALKING –                                                                arms to “hang” more than normal, which
CANES AND WALKERS              through hospitals and clinics, orthotic
                               devices help to support your joints in certain    can become very painful. A shoulder sling
         WHEELCHAIRS                                                             can pull the arm up, reducing the pressure
                               positions when your muscles weaken.
  OTHER EQUIPMENT                                                                on arm muscles and ligaments. There are
                               The universal cuff may be the most familiar       also cervical collars that can support the
                               orthotic aid. This straps over the hand and       head when neck muscles are weak.
                               allows you to grasp such objects as cutlery,
                               hairbrushes and other small personal              COMMUNICATION EQUIPMENT
                               objects. A thumb splint helps in squeezing
                               the fingers in opposition to the fingers and      There are many different types of assistive
                               makes it easier to grasp and hold an object       devices available to help the speaking
                               for use. Many people with ALS experience          impaired communicate. These devices range
                               “foot drop.” This problem leads to                from communication letter boards based on
                               stumbling on stairs or curbs. The answer          tracing the person’s eye movements, to throat
                               may be a simple ankle-foot splint made of         contact devices for those on a respirator, to
                               plastic that is inconspicuous under trousers      small hand held electronic speaking devices,
                               or slacks.                                        to computer based systems with
                                                                                 sophisticated software and speech
                               There are more orthotic aids available than
                                                                                 synthesizers. Your choice will depend on
                               we can discuss in this brief overview. An aid
                                                                                 your budget and/or other funding available,
                               or a combination of aids can be prescribed
                                                                                 your specific needs, and your adaptability to
                               by your doctor. A physical or occupational
                                                                                 new ways of communicating. Also keep in
                               therapist can teach you how to integrate
                                                                                 mind that assistive technology is continually
                               these devices into your daily routine.
                                                                                 improving. You will need to ask your local
ALS              SLA                                                             sources about what is currently available in
SOCIETY OF CANADA              Foot and Ankle Supports                           your area and can be serviced locally if
Maladie Lou Gehrig’s Disease                                                     needed, to determine what type is best for
                               When weakened muscles cause “foot drop”
          1(800)267-4257       and lead to stumbling on stairs or curbs, a       you.
          http://www.als.ca    lightweight plastic or metal ankle splint can
                                                                                 For further information, see Dealing with
           alscanada@als.ca    be a big help.
                                                                                 Speaking Difficulties, Section 9.


                  PAGE 78                                                                                                    v. 1.0
LIVING WITH ALS                                                                                        SECTION          10
ASSISTIVE EQUIPMENT

LIFTS AND STAIR GLIDES                            Portable Lifts
                                                                                                   ASSISTIVE
This type of equipment can be expensive
                                                  Portable hoists or lifts, which use a sling to   EQUIPMENT
                                                  lift a person up, can be rolled around on
and requires a lot of consideration and                                                            BEFORE PURCHASING
                                                  one level. Also, most of them are light, can
pre-planning to determine what is the best                                                         EQUIPMENT
                                                  be broken down into two pieces, and easily
solution for your residence and longer-
                                                  moved to a different level, or put in a car.     BATHROOM
term situation. The types of lifting                                                               EQUIPMENT
                                                  The minimum door size for moving a
equipment to consider include stair glides,
                                                  person through in a portable lift is about       BEDS AND
portable lifts, ceiling-track lifts and                                                            MATTRESSES
                                                  26”. Using a portable lift requires training,
wheelchair lifts. It is best to get a trained
                                                  during which both you and caregiver              BODY SUPPORTS
professional to assess your residence and
                                                  should be lifted, so that the caregiver can
situation when making your plans                                                                   COMMUNICATION
                                                  understand what you are experiencing.            EQUIPMENT
regarding lifting devices.
                                                  Stair Glides                                     LIFTS AND
Chairs                                                                                             STAIR GLIDES
                                                  Most stair glides use a track that is            TUBE-FEEDING
People with weakened legs will find it                                                             EQUIPMENT
                                                  fastened securely to the wall side of a
difficult to get up out of chairs. Higher
                                                  stairway. An automated chair can then be         WALKING –
chairs with arms to push on should make                                                            CANES AND WALKERS
                                                  moved up and down the track. Stair glides
it easier. Ultimately, an automated easy-lift
                                                  can go on curving stairs, and even around        WHEELCHAIRS
chair may be required. These are
                                                  corners. However, there must be adequate
motorized chairs that have a switch                                                                OTHER EQUIPMENT
                                                  room and help at the top and at the
enabling the person to adjust the incline,
                                                  bottom of the stairway, for assisting you
and to raise the seat and lift themselves out
                                                  on and off the stair glide chair. Stair glides
of a sitting position and into a standing
                                                  should only be installed by a professional
position. More expensive models come
                                                  familiar with the safety aspects of this
with heaters, a massage device and other
                                                  equipment.
features.
                                                  Wheelchair Lifts
Ceiling Lifts
                                                  Portable wheelchair lifts are the most
Ceiling lifts are usually installed to move a
                                                  economical solution, and can be used on
person between the bedroom, bathroom
                                                  various stairways. Built-in wheelchair lifts
and living areas. In this case a rail is
                                                  are often used when it is not practical to
installed in the ceiling, and the person is
                                                  have a wheelchair ramp from an outside
lifted in a sling, similar to the sling used on
                                                  door to ground level. Again, installing a
a portable lift. There are also turntable
                                                  wheelchair lift system is a major project
tracks that allow the running tracks to
                                                  which requires advice and installation by a
intersect or cross. Installing a ceiling lift
                                                  professional who is familiar with the safety
system is a major project that requires
                                                  aspects of wheelchair lifts. (An amateur
professional advice and installation.
                                                  installation of a wheelchair lift was once
                                                  responsible for the death of a small child
                                                  in Ontario.)                                     ALS              SLA
                                                                                                   SOCIETY OF CANADA
                                                                                                   Maladie Lou Gehrig’s Disease

                                                                                                   1(800)267-4257
                                                                                                   http://www.als.ca
                                                                                                   alscanada@als.ca


v 1.0                                                                                              PAGE 79
SECTION         10                                      LIVING WITH ALS
                                                                                        ASSISTIVE EQUIPMENT

                               TUBE FEEDING EQUIPMENT                          When choosing an IV pole, you should
       ASSISTIVE                                                               consider the following options:
     EQUIPMENT                 Tube feeding normally requires two pieces
                                                                               The more expensive models tend to be
BEFORE PURCHASING              of equipment:
                                                                               more sturdy and stable
        EQUIPMENT
                               • a container with a tube and clamp
            BATHROOM                                                           Floor models or other models that attach
                                 for the liquid food
            EQUIPMENT                                                          to a table, chair or a bed. There is also a
                               • a pole to hang the container on               pocket model available for travelling. It has
             BEDS AND
            MATTRESSES         A pump may also be used instead of a            fittings that will attach to a variety of
                               clamp to regulate the liquid flow for a         surfaces, including a suction cup for
      BODY SUPPORTS
                               short period (2 to 4 weeks) while you get       windows, a magnet for metal surfaces, an
   COMMUNICATION               used to tube feeding. A special syringe can     eye and two hooks, one of which is for the
       EQUIPMENT               also be used to inject liquid through a tube,   food container.
             LIFTS AND         or to check if your stomach has too much
           STAIR GLIDES        liquid.
         TUBE-FEEDING
           EQUIPMENT           Containers                                      WALKING
        WALKING –
CANES AND WALKERS              Food containers come in the form of             Canes and Walkers
                               plastic bags or bottles with a tube attached.
         WHEELCHAIRS           Some containers have a stretchable section,     Most people with ALS will require a cane
  OTHER EQUIPMENT              which is required if a pump is being used.      or walker. These are especially useful when
                               These containers need to be thoroughly          one leg is stronger than the other. Canes
                               cleaned every day and replaced with a new       should always be used on the stronger side,
                               container approximately every 7 days.           with the cane moving forward with the
                                                                               weaker leg. For more cane support, there
                               When choosing a container you will              are canes with four spread tips to give
                               probably have the following options:            more stability. Another type of cane
                                                                               extends up the lower arm, almost to the
                               • Format: A plastic bag or plastic bottle;
                                                                               elbow, with a ring that fits around the arm
                               • Capacity:Common sizes range from              for added support. Walkers provide
                                 500 to 1000 ml.;                              maximum support because they spread the
                               • Tube: You may need the type of tube           weight over a wide area.
                                 that works with a pump
                                                                               The choice of cane or walker should be
                               IV Poles                                        made in consultation with your doctor and
                                                                               physiotherapist and you should not try to
                               It may be practical to rig up your own          use it until you have received instruction
                               method for hanging the feeding container.       from your therapist.
                               One way is to use a pole held up by a
                               Christmas tree stand. If you wish to
                               purchase a pole, the commonly available
                               models are priced from under $100 to over
ALS              SLA           $400. Most models are extendible, have
SOCIETY OF CANADA              casters, and 2 or 4 hooks.
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 80                                                                                                v. 1.0
LIVING WITH ALS                                                                                SECTION            10
                                                                                        ASSISTIVE EQUIPMENT

WHEELCHAIRS                                    For the more advanced stages of ALS, a
                                               battery-powered wheelchair may be
                                                                                              ASSISTIVE
The decision about when to acquire a           required. These can be operated using a        EQUIPMENT
wheelchair is one that you will make with      joystick or other switching devices that can
                                                                                              BEFORE PURCHASING
your doctor. A wheelchair will be              be controlled by almost any part of the        EQUIPMENT
prescribed for you by your therapist.          body that you can move.
                                                                                              BATHROOM
                                               Getting fitted with a wheelchair requires      EQUIPMENT
There are different types of wheelchairs
and wheelchair features that require           careful consideration and professional         BEDS AND
consideration. Straps may be needed to         advice. First, your size will determine the    MATTRESSES
hold in arms and feet, and a seat belt is      appropriate height, depth and width of         BODY SUPPORTS
also a good idea. A reclining back can give    seat, as well as the height and width of the
                                               back.
                                                                                              COMMUNICATION
you added flexibility or comfort. Hand-rim                                                    EQUIPMENT
pegs are helpful if you have trouble
                                               You should try different chairs, and ensure    LIFTS AND
gripping with your fingers, but have                                                          STAIR GLIDES
                                               that:
strength in your arms. There are also
wheelchairs that have a double hand rim                                                       TUBE-FEEDING
                                               • Both feet touch the ground squarely          EQUIPMENT
for operating from one side, useful if you       when you are properly seated;
have strength in one side only. Anti-tipping                                                  WALKING –
                                               • The foot rests are adjusted so that when     CANES AND WALKERS
bars may be extended out the front or back
                                                 your feet are on the rests, the back of      WHEELCHAIRS
to prevent the wheelchair from tipping
                                                 your knee is about 3" away from the
forward or backward. A commode                                                                OTHER EQUIPMENT
                                                 edge of the seat;
attachment is available when you are alone
for long periods. Tray- or table-like          • The back of the seat touches your
platforms should be a standard feature that      shoulder blades;
can be attached to your wheelchair.            • There is at least 1" space between your
Folding wheelchairs are useful because           hips and the wheelchair on both sides to
they can be put in a car. Ask the                leave room for bulky clothing, such as
professionals who help you with your             an overcoat;
wheelchair decision about other available
                                               • The arm-rests are at a height which
features.
                                                 enables your arms to bend naturally.




                                                                                              ALS              SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 81
SECTION         10                                      LIVING WITH ALS
                                                                                       ASSISTIVE EQUIPMENT

       ASSISTIVE               It is very important that you purchase the     Tilt-and-Recline Wheelchairs
     EQUIPMENT                 right chair. Many factors will have to be
                                                                              A tilt-and-recline wheelchair is usually
                               considered, such as your physical
BEFORE PURCHASING              condition now and in the future, your          recommended as a person becomes
        EQUIPMENT              financial situation and the availability of    weaker. Many people with ALS find
                               financial assistance, your level of help       breathing easier when in a reclining
            BATHROOM                                                          position. This chair positions the body in
            EQUIPMENT          from family and friends, and the sorts of
                               services that your community provides for      ways that use gravity, instead of working
             BEDS AND                                                         against it. The tilt feature allows gravity to
            MATTRESSES         transportation. A wheelchair is a major
                               purchase, and if it is going to enhance        pull the hips to the back of the chair to
      BODY SUPPORTS            your life now and in the future, it must be    prevent a continual forward sliding
   COMMUNICATION               a careful choice.                              movement, which is a common problem
       EQUIPMENT                                                              with a reclining chair. The tilt feature also
                                                                              relieves pressure on the ischials and coccyx
             LIFTS AND         Manual Chairs
           STAIR GLIDES                                                       (tailbone), preventing pressure sores from
                               Lightweight manual wheelchairs are often       forming.
         TUBE-FEEDING
           EQUIPMENT           used by people who retain trunk stability
                               and the ability to self-position themselves    No matter what type of chair is used, skin
        WALKING –              in the chair. These chairs are light to push   breakdown is more likely to occur if
CANES AND WALKERS                                                             regular repositioning techniques are not
                               and may be easily transported by car.
         WHEELCHAIRS           Because ALS is a degenerative disease, it      used.
  OTHER EQUIPMENT              may be more cost-effective to rent or
                               borrow this type of chair, leaving funds
                               available for other equipment.




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 82                                                                                                v. 1.0
LIVING WITH ALS                                                                                    SECTION          10
ASSISTIVE EQUIPMENT

Electric Wheelchairs                            As physical mobility decreases, more
                                                pressure-relieving cushions will be
                                                                                                ASSISTIVE
Electric wheelchairs allow a person to          necessary. Many people with ALS report          EQUIPMENT
remain mobile and independent for a             that gel cushions are uncomfortable, while
longer period of time than manual                                                               BEFORE PURCHASING
                                                air-filled cushions seem to provide more        EQUIPMENT
wheelchairs do. A standard electric chair       comfort and pressure relief. More than one
(or scooter if muscles are relatively           type of cushion is usually required since       BATHROOM
unimpaired) will increase your outdoor                                                          EQUIPMENT
                                                no cushion will provide both comfort and
mobility while reducing overall fatigue.        relief all the time.                            BEDS AND
Because the progression of the disease will                                                     MATTRESSES
necessitate ongoing changes, borrowing or                                                       BODY SUPPORTS
                                                Home Modifications
renting this chair may be more cost-
effective than buying it.                                                                       COMMUNICATION
                                                Listed below are some areas to consider         EQUIPMENT
                                                when modifying an environment for the
As you weaken and more support and              wheelchair user:                                LIFTS AND
better positioning are needed, a scooter                                                        STAIR GLIDES
will no longer be a viable option. Electric     Rearrange furniture to make wheelchair          TUBE-FEEDING
wheelchairs with manual or electric tilt-       access and movement easier.                     EQUIPMENT
and-recline functions should be                                                                 WALKING –
considered. The wheelchair should support       Install ramps (12:1 in slope) and guardrails    CANES AND WALKERS
your back and head. Specialty backs             or lifts in place of stairs.
                                                                                                WHEELCHAIRS
provide pressure relief and lateral support,
which improves stability and positioning.       Doorways should be at least 76 cm (30 in.)      OTHER EQUIPMENT
Headrests are available in a variety of sizes   wide. They can be widened by removing
and styles.                                     the door and hinges, or removing inner
                                                mouldings and installing a sliding door or
Some electric chairs can be controlled by       curtain. A “narrower” may be necessary to
using sip-and-puff or rim controls. For         decrease the width of the wheelchair by 5-
many people with ALS, these features            10 cm (2-4 in.) temporarily to permit
significantly prolong independence. Other       passage through a doorway.
people do not choose this kind of
alternative control as they may not be able     Hallways should be 92 cm (36 in.) wide to
to maintain a seal around a straw or have       accommodate the chair’s turning radius. A
adequate breath control for sip-and-puff,       cleared turning space of 1.6m2 (5 sq. ft.) is
and consistent head movements are               necessary for safe and easy turns.
needed to drive a chair with head or rim        Space under sinks and counters to
control.                                        accommodate wheelchairs can be made by
Cushions should be chosen for comfort           removing cupboards and exposed pipes
and pressure relief, as many positions          must be insulated.
demanded by prolonged wheelchair use            Carpets can hinder the movement of a
can cause skin breakdown. If you can            wheelchair. Hardwood, vinyl or Berber
independently reposition every 30 minutes,      flooring are good choices for wheelchair
a high-density foam cushion should be
adequate to relieve pressure.
                                                use.                                            ALS              SLA
                                                                                                SOCIETY OF CANADA
                                                                                                Maladie Lou Gehrig’s Disease

                                                                                                1(800)267-4257
                                                                                                http://www.als.ca
                                                                                                alscanada@als.ca


v 1.0                                                                                           PAGE 83
SECTION         10                                      LIVING WITH ALS
                                                                                        ASSISTIVE EQUIPMENT

                               OTHER EQUIPMENT                                 Modifications to radio, light and television
       ASSISTIVE                                                               switches allow a person with ALS to turn
     EQUIPMENT                 Many people with ALS have trouble with          the set on or off with the palm of the
                               grasping and manipulating objects. It is        hand, a head movement or even a puff of
BEFORE PURCHASING
        EQUIPMENT              often possible to modify everyday tools or      breath. Telephones that do not require the
                               to substitute specially designed versions of    use of hands are also available.
            BATHROOM
            EQUIPMENT          such tools to compensate for weakness in
                                                                               We have mentioned just a few of the
                               the muscles of the fingers, hands and
             BEDS AND                                                          modified or special tools available to make
                               wrists. For example, a knife, fork and
            MATTRESSES                                                         everyday life easier for you. There are
                               spoon with extra-thick handles can make
      BODY SUPPORTS                                                            many more such tools that your therapist
                               eating much easier. There are also sets of
                                                                               can tell you about. It is important to
   COMMUNICATION               cutlery available with thick, long handles
       EQUIPMENT                                                               discuss any tool with your therapist before
                               that compensate to some extent for
                                                                               making a purchase. Some devices are
             LIFTS AND         impaired shoulder movement.
                                                                               reasonably priced and some are expensive.
           STAIR GLIDES                                                        Some will assist you for a long period,
                               Mugs with oversized handles will allow
         TUBE-FEEDING          you to slip all fingers under the handle,       others may be useful only in the very short
           EQUIPMENT                                                           term. Your therapist can save you
                               reducing the danger of spills. A plate
        WALKING –              guard gives a slight vertical edge to a plate   disappointments as well as costly errors.
CANES AND WALKERS              so that food can be pushed against it onto
         WHEELCHAIRS           a spoon or fork. The double action of
  OTHER EQUIPMENT              gripping and turning a doorknob can be a
                               problem for you. A doorknob adapter
                               could be the answer since it allows the
                               door to be opened by pushing down or
                               pulling up the lever. A fat wooden or
                               plastic handle attached to your door key
                               can help with the turning motion
                               necessary to turn the key in the lock. Thick
                               pens or pencils are easier to grasp than the
                               usual thin variety, or a writing aid may be
                               used which consists of a block holding a
                               pen or pencil.

                               Clothing fasteners are finicky and difficult
                               to use if your hands and fingers are weak.
                               There are devices to assist in fastening
                               buttons. Velcro is a popular replacement
                               for both buttons and zippers. Best of all,
                               such items as pullover tops and pants or
                               skirts with elastic waistbands need no
                               fasteners at all.
ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 84                                                                                               v. 1.0
LIVING WITH ALS       SECTION          10
NOTES




                  ALS              SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 85
SECTION         10             LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 86                     v. 1.0
LIVING WITH ALS                                                                                   SECTION          11
FINANCIAL AND LEGAL CONSIDERATIONS

COSTS OF LIVING WITH ALS                          Below is a list of various types of         FINANCIAL
                                                  equipment required by a person with ALS.
                                                  This list is by no means complete as each
                                                                                              & LEGAL
ALS is a very expensive disease, whether
one lives at home or in an institution.           person may require additional items.        CONSIDERATIONS
Institutional care imposes a heavy burden                                                     FINANCIAL & LEGAL
                                                  •   Cervical collar                         COSTS OF LIVING
on taxpayers. Home care is much less costly,
                                                  •   Porch lift                              WITH ALS
but the expense falls principally on the
                                                  •   Leg splints
family, and often becomes exorbitant in                                                       DEALING WITH
                                                  •   Software/Switch computer                YOUR EMPLOYER
terms of their ability to pay. This is true
                                                  •   Raised toilet seat
even though various agencies and groups                                                       INSURANCE ISSUES
                                                  •   Switches
provide some form of help. The assistance
                                                  •   Scooter                                 PENSION PLANS
provided by the ALS Society becomes, for
                                                  •   Home renovations
many people, a critical factor in their                                                       TAX ISSUES
                                                  •   Custom seating
remaining in the much more desirable and
                                                  •   Communication devices                   POWERS OF ATTORNEY
less costly home setting.
                                                  •   Batteries
                                                                                              WILLS
The person with ALS may need some form            •   Bed
of supporting device when diagnosed, and          •   Walkers
in almost all cases, proceed through a            •   Special mattress
bewildering variety of equipment: scooter,        •   Manual wheelchair
walker, manual and power wheel chairs,            •   Ramping
transfer seat, ceiling lift, stair glide, porch   •   Electric wheelchair
lift, hands-free toilet, ventilator… The list     •   Sturdy Lift, installed
goes on and on. It may be that no other           •   Easy-lift chair
disease requires so much equipment or care.
At the same time the requirement for              Other in-home care, for example nursing
attendant and nursing care is increasing.         assistants, homemakers, etc., means
                                                  additional cost.
Funding assistance for these requirements
may be available through government and
other programs.

See Section 14 for more information regarding
assistive programs in your community.




                                                                                              ALS              SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 87
  SECTION         11                                      LIVING WITH ALS
                                                           FINANCIAL AND LEGAL CONSIDERATIONS

     FINANCIAL                   DEALING WITH YOUR EMPLOYER                     When do you tell your employer?
        & LEGAL                                                                 Here are some things to consider.
                                 When you have first been diagnosed with
CONSIDERATIONS                   ALS, there are many things to consider.        • Can you hide your symptoms from
    FINANCIAL & LEGAL            One is when and what to tell your                your employer?
       COSTS OF LIVING           employer. This is very much a personal
             WITH ALS                                                           • Are you working on a project that you
                                 decision depending on your condition and
                                                                                  want to finish, before telling your
         DEALING WITH            personality, as well as your employment
        YOUR EMPLOYER                                                             employer?
                                 situation.
                                                                                • Should you tell your employer so that
     INSURANCE ISSUES
                                 Checking out the benefits in your                they have adequate time to plan how to
         PENSION PLANS           employer’s group insurance is certainly a        accommodate you in the work place or
               TAX ISSUES        first step, though at this stage you may not     plan for your replacement? (In many
                                 yet be aware of the full range of your           provinces it is a legal requirement to
 POWERS OF ATTORNEY              potential needs. The pension plan may be         “accommodate” a person with a
                      WILLS      another area worth checking into. All of         disability such as ALS.)
                                 this can be done before telling your
                                 employer you have ALS.                         Do not sign anything regarding your
                                                                                termination without first getting legal
                                                                                advice. Legal termination minimums are
                                                                                usually linked to years of service.

                                                                                Be careful about doing anything that might
                                                                                restrict your access to group benefits.

                                                                                What protections do you have under
                                                                                provincial labour legislation?

                                                                                Consider getting advice from a lawyer who
                                                                                specializes in human rights, provincial
                                                                                employment standards and labour
                                                                                legislation.




  ALS              SLA
  SOCIETY OF CANADA
  Maladie Lou Gehrig’s Disease

            1(800)267-4257
            http://www.als.ca
             alscanada@als.ca


                    PAGE 88                                                                                            v. 1.0
LIVING WITH ALS                                                                                    SECTION          11
FINANCIAL AND LEGAL CONSIDERATIONS

INSURANCE ISSUES                               Ask if your policy offers case                 FINANCIAL
                                               management. Case managers either work
Understanding Your                             for the insurance company or are
                                                                                              & LEGAL
Insurance Coverage                             contracted by them to monitor and              CONSIDERATIONS
                                               advocate for patients whose costs are high     FINANCIAL & LEGAL
In order to maximize the benefits of your
                                               or who have complicated needs. They are        COSTS OF LIVING
insurance plan, it is important for you to                                                    WITH ALS
                                               often helpful in cutting internal “red tape”
keep a record of the name, phone number,
                                               (problems), gaining access to little-known     DEALING WITH
identification, and group number of your
                                               insurance benefits, and reducing your          YOUR EMPLOYER
insurance plan. You should also note who
                                               overall out-of-pocket expenses. Case
the subscriber is (you or your spouse),                                                       INSURANCE ISSUES
                                               management can be activated at various
his/her date of birth, and social insurance
                                               stages of your illness, depending on how       PENSION PLANS
number. Identify any other insurance
                                               your insurance company has defined the         TAX ISSUES
benefits you may have, such as a separate
                                               benefit. Having direct communication with
prescription benefit card or a long-term-                                                     POWERS OF ATTORNEY
                                               your insurance carrier will help you to gain
care policy.
                                               an overview of your policy and remove the      WILLS
If you have more than one insurance plan,      mystery of how your plan works.
determine which policy is primary and
                                               Use the following guide when contacting
which is secondary; confusion with this
                                               your insurance company. Be sure to have
matter can result in billing errors. Your
                                               all of the specific information about your
insurance company customer service
                                               insurance plan in front of you before
representative can be helpful in identifying
                                               calling. Do not hesitate to ask what certain
which plan is to be billed first. Also keep
                                               words or terms mean.
up to date on your insurance; if your
coverage changes in any way, notify all
your doctors, medical suppliers, and           What You Need to Know
pharmacists immediately so that the next       About Your Health Insurance Policy
time you access their services, you will
                                               General Questions
have helped to prevent billing errors.
                                               Is there an annual deductible?
Contact your insurance company directly,
and ask specific questions about your          Is there an annual out-of-pocket expense
benefits. Always note the date and the         limit or maximum? If I meet my limit,
person who provided the information.           does my coverage increase and to what
Remember, having your benefits described       extent?
over the phone does not guarantee
coverage. Your doctor may be asked to          Do I have a major medical plan? Is there
write a letter of medical necessity and        an annual or lifetime maximum?
complete specific forms to verify your
                                               Do I need to complete any claim forms?
medical condition and eligibility for
whatever item or service is being              Am I subject to preexisting condition
requested.                                     regulations?
                                                                                              ALS              SLA
                                                                                              SOCIETY OF CANADA
                                                                                              Maladie Lou Gehrig’s Disease

                                                                                              1(800)267-4257
                                                                                              http://www.als.ca
                                                                                              alscanada@als.ca


v 1.0                                                                                         PAGE 89
  SECTION         11                                      LIVING WITH ALS
                                                           FINANCIAL AND LEGAL CONSIDERATIONS

     FINANCIAL                   Durable Medical Equipment                      Home Health Questions
                                 (DME) Questions
        & LEGAL                                                                 Does my plan have home health coverage?
CONSIDERATIONS                   Does my plan cover DME? What about             How do I access this?
                                 ventilator coverage; is it under respiratory
    FINANCIAL & LEGAL            equipment or DME?                              Is there a preferred home health care
       COSTS OF LIVING                                                          agency I must use?
             WITH ALS            What is the percentage of my coverage?
         DEALING WITH                                                           Is there private-duty nursing coverage at
        YOUR EMPLOYER            Is there a preferred provider I must see?      home? Describe this benefit.
     INSURANCE ISSUES            Is pre-authorization or a medical review       Does my plan offer case management? At
         PENSION PLANS           required?                                      what point does case management get
                                                                                involved and for how long?
               TAX ISSUES        Prescription Questions
 POWERS OF ATTORNEY                                                             Group Medical Insurance
                                 Does my plan cover prescription drugs?
                      WILLS      What are the terms of this coverage, and is    Examine your group insurance policy
                                 coverage different based on using brand-       carefully and call your insurance provider
                                 name versus generic drugs?                     for detailed information. What are you
                                                                                entitled to?
                                 Is there a specific pharmacy/supplier
                                 network I must use?                            Loss of income support: How much and
                                                                                for how long?
                                 Are injectable medications covered under
                                 my plan?                                       Prescription Drugs: What drugs are
                                                                                covered? What is the maximum? What is
                                 Is there a limit on the amount of
                                                                                deductible?
                                 prescription drugs I can go through on this
                                 plan?                                          Alternative Therapies: Are any, such as
                                                                                massage, covered? Any limitations?
                                 Is there coverage for all Health Protection
                                 Branch-approved drugs, or is coverage          Home Care: How much and how long?
                                 provided only for those listed on your
                                 formulary (a list of drugs that an insurance   Assistive Equipment: What type and what
                                 policy covers)?                                limitations?

                                 Does my plan offer a mail-order pharmacy       Hospital: Private or semi-private coverage?
                                 option? How does this work?
                                                                                Are there any other benefits?




  ALS              SLA
  SOCIETY OF CANADA
  Maladie Lou Gehrig’s Disease

            1(800)267-4257
            http://www.als.ca
             alscanada@als.ca


                    PAGE 90                                                                                                 v. 1.0
LIVING WITH ALS                                                                                     SECTION          11
FINANCIAL AND LEGAL CONSIDERATIONS

Why you may need legal advice                   Life Insurance: Living Benefits                FINANCIAL
Group insurance can be very important to        Most life insurance companies offer a          & LEGAL
a person with ALS. If you have group            “living benefit” feature to people with a      CONSIDERATIONS
insurance through your employer, you may        terminal disease enabling them to get a
need to seek advice from a lawyer or            portion of their life insurance paid to them
                                                                                               FINANCIAL & LEGAL
                                                                                               COSTS OF LIVING
someone else you can trust to ensure that       in advance, during the years before their      WITH ALS
this insurance coverage continues to be         death. If you wish to benefit from such a
effective when you are unable to continue       plan, calculate how much the benefit is        DEALING WITH
                                                                                               YOUR EMPLOYER
working. Some employers attempt to              worth, relative to the resulting decrease in
terminate employees who become                  amount of your life insurance. If you do       INSURANCE ISSUES
disabled. They do so to cancel the disabled     not understand these calculations, get         PENSION PLANS
employee’s group insurance in an effort to      advice from an independent person you
keep company insurance premium costs            can trust. For more information on living      TAX ISSUES
down. Also there are many cases where           benefits see the following pages.              POWERS OF ATTORNEY
disabled patients have had to hire lawyers
                                                                                               WILLS
to force an insurance company to pay the        How Do Living Benefits Work?
benefits described in their group insurance
plan, particularly loss of income benefits.     Policies and procedures for living benefits
If you require legal advice for this purpose    vary slightly, depending on the insurance
find a lawyer who has experience with this      company. Typically, to receive living
area of the law.                                benefits an application must be made to
                                                the insurance company together with a
Driving and Property Insurance                  medical opinion that the policy holder is in
                                                the terminal stages of an illness, and has
If you don’t report your disability to your     approximately 24 months to live.
insurance company, your car insurance
coverage may not be valid. Driving can          The insurance company will then check to
sometimes require fast foot and hand            make sure the proceeds of the insurance
reactions to avoid an accident. Ask at your     have not been assigned to pay off a loan or
clinic about a driving test service that will   debt or left irrevocably to someone else
certify your current abilities for insurance    who might sue for the full proceeds once
purposes. Also, you may need additional         the policy holder has died.
property insurance regarding assistive
                                                Once these two conditions are met, the
equipment you have or renovations that
                                                insurance company will then pay a
have been done in your residence to
                                                percentage of the value of the policy —
accommodate your condition.
                                                ranging from one-third to half —as a
                                                living benefit. Again, the details vary
                                                depending on the company. Some set a
                                                maximum — such as $50,000 — for living
                                                benefits. Some charge interest, which is
                                                usually paid out of the policy. Some make
                                                special arrangements with the person to
                                                ensure that the regular premium payments
                                                                                               ALS              SLA
                                                                                               SOCIETY OF CANADA
                                                are made and the insurance policy stays in
                                                                                               Maladie Lou Gehrig’s Disease
                                                force.
                                                                                               1(800)267-4257
                                                                                               http://www.als.ca
                                                                                               alscanada@als.ca


v 1.0                                                                                          PAGE 91
  SECTION         11                                      LIVING WITH ALS
                                                           FINANCIAL AND LEGAL CONSIDERATIONS

     FINANCIAL                   What Happens to the Money                       A Word of Caution
        & LEGAL                  Left in the Policy?
                                                                                 There are organizations that buy life
CONSIDERATIONS                   When the policy-holder dies, the                insurance policies from people who are
                                 remainder will be paid to his or her estate     terminally ill. In return for a certain
    FINANCIAL & LEGAL                                                            amount of cash now, usually substantially
       COSTS OF LIVING           or designated beneficiary, less any interest
             WITH ALS            charges on the money paid out as living         less than the death claim value of the
                                 benefits.                                       policy, the person with a life insurance
         DEALING WITH                                                            policy names the organization as
        YOUR EMPLOYER
                                 Are Living Benefits Available                   beneficiary and gives up all rights under
     INSURANCE ISSUES                                                            the policy. When the person dies, the
                                 on All Life Insurance Policies?
         PENSION PLANS                                                           organization will receive the full value of
                                 Living benefits are usually available on        the policy.
               TAX ISSUES        individual life insurance policies but they
 POWERS OF ATTORNEY              are not always available on group life          In Ontario at the present time, it is illegal
                                 insurance plans. Practices vary, so be sure     for any organization other than an
                      WILLS                                                      insurance company to traffic in insurance
                                 to ask specifically about your policy.
                                                                                 policies. While there are no such
                                                                                 organizations operating in Ontario, many
                                 What Should I Do if I Am Interested
                                                                                 US firms actively try to recruit Canadian
                                 in Getting Living Benefits?
                                                                                 policy-holders. Talk to your lawyer or the
                                 Before you decide to apply for living           Canadian Life and Health Insurance
                                 benefits, talk to a lawyer or financial         Association before becoming involved with
                                 advisor about all the financial implications.   these organizations.
                                 At the present time, the federal
                                 government is not taxing living benefits,
                                 but anyone receiving a lump sum living
                                 benefit payment will no longer be eligible
                                 for social assistance benefits. Talk to
                                 someone who can look at your entire
                                 financial situation and advise you about
                                 the best course of action.

                                 If you decide to apply for living benefits,
                                 either you or your lawyer should talk to
                                 your insurance agent. Ask about the
                                 availability of living benefits under your
                                 policy. Because this is a relatively new
                                 program in most insurance companies,
                                 some agents and brokers may not be aware
                                 of it. If you are having trouble getting
                                 information about your policy or getting
                                 living benefits, contact the help line at the
  ALS              SLA           Canadian Life and Health Insurance
  SOCIETY OF CANADA              Association: in Toronto 777-2344; outside
  Maladie Lou Gehrig’s Disease   Toronto 1-800-268-8099.
            1(800)267-4257
            http://www.als.ca
             alscanada@als.ca


                    PAGE 92                                                                                               v. 1.0
LIVING WITH ALS                                                                                 SECTION          11
FINANCIAL AND LEGAL CONSIDERATIONS

 PENSION PLANS                                When you are already receiving a Canada      FINANCIAL
                                              Pension Plan retirement benefit and
                                              become disabled between 60 and 65 years
                                                                                           & LEGAL
Group Pension Plans                                                                        CONSIDERATIONS
                                              of age, you must have become disabled
If you are in a company or other group        before, or within six months after, the      FINANCIAL & LEGAL
pension plan, find out what will happen as    effective date of your retirement pension.   COSTS OF LIVING
a result of an ALS diagnosis.                                                              WITH ALS
                                              Apply in writing. (Applications can be
Can you receive some of your pension                                                       DEALING WITH
                                              obtained from your nearest federal           YOUR EMPLOYER
before age 65, if you are disabled, and       government office.)
unable to work?                                                                            INSURANCE ISSUES
                                              A Canada Pension Plan Disability Pension
Will payments be made to your spouse if
                                                                                           PENSION PLANS
                                              is payable from the fourth month after you
you are no longer alive? To be sure that      are deemed to have become disabled. You      TAX ISSUES
you get your full entitlement, have these     may receive up to a maximum of 12            POWERS OF ATTORNEY
questions answered by someone you trust.      months of retroactive payments. Like most
If problems occur, you may need advice        pensions, your Canada Pension Plan           WILLS
from a lawyer with experience with group      Disability Pension is considered taxable,
pension plans.                                subject to your total income.
To minimize tax on death, you may want
to make sure that your spouse is the
                                              Things that can change eligibility for a
beneficiary of your pension plan;             CPP Disability Pension
otherwise your pension plan’s value will be   Those receiving a CPP Disability Pension
part of your estate, and subject to probate   must notify the Canada Pension Plan of
fees.                                         any changes that might affect their
                                              continuing eligibility for benefits. This
Canada Pension Plan                           includes:
Patients with ALS may qualify for a CPP       An improvement in your medical
disability pension. To be eligible for a      condition
Canada Pension Plan Disability Pension,
you must:                                     A return to full, part-time, volunteer or
                                              trial period of work
Be between the ages of 18 and 66
                                              Attendance at school or university; trade
Have contributed to the CPP for a             or technical training
minimum qualifying period calculated as
follows:                                      Any rehabilitation.

If you have only 2 calendar years in your
contributory period, you must have
contributed in both of those years.

b. If you have more than two years in your
contributory period, then contributions
                                                                                           ALS              SLA
must have been made in 2 of the last 3                                                     SOCIETY OF CANADA
                                                                                           Maladie Lou Gehrig’s Disease
years, or 5 of the last 10 years of the
contributory period.                                                                       1(800)267-4257
                                                                                           http://www.als.ca
                                                                                           alscanada@als.ca


v 1.0                                                                                      PAGE 93
  SECTION         11                                     LIVING WITH ALS
                                                           FINANCIAL AND LEGAL CONSIDERATIONS

     FINANCIAL                   TAX ISSUES                                    Estate Freezing
        & LEGAL                                                                The purpose of estate freezing is to
CONSIDERATIONS                   Disability Tax Credit                         minimize the taxes due on death.
                                                                               Assuming assets will be at a taxable level,
    FINANCIAL & LEGAL            Form T2201, Revenue Canada’s Disability       taxes can be minimized by a financial plan
       COSTS OF LIVING           Credit Certificate, has a section that must
             WITH ALS                                                          that is structured so that future profits from
                                 be completed and signed by your doctor        your assets will go to someone else in a
         DEALING WITH            and sent in with your income tax return.      lower tax bracket, such as your children.
        YOUR EMPLOYER
                                                                               Consider getting advice from a
     INSURANCE ISSUES            Minimize Tax Through Income Splitting         professional accountant regarding estate
         PENSION PLANS           Income splitting reduces a family’s total     freezing.

               TAX ISSUES        income tax by dividing income among
                                 various family members, using up the low      Tax on Deemed Disposition
 POWERS OF ATTORNEY              tax rates of family members who have          of Assets on Death
                      WILLS      little other income. This has to be done in
                                                                               At the date of death, Revenue Canada
                                 ways that will “attribute” less income to
                                                                               requires one tax return for income earned
                                 the higher income earner(s) in the family,
                                                                               to the date during the year and to account
                                 according to Revenue Canada, such as the
                                                                               for the tax that is applicable on all of the
                                 following:
                                                                               increased values of the deceased person’s
                                 • Estate freezing                             properties and other assets. There are
                                                                               certain tax-free “roll over” provisions for
                                 • Transfer property at fair market value
                                                                               property left to a spouse, or spousal trust.
                                 • Lend or gift assets to generate business    Also, dividend-paying shares may incur a
                                   income, or income on income                 double tax unless appropriate action is
                                 • Gift funds to child turning 17              taken. Be sure to seek advice from a
                                 • Earn capital gains for children             professional accountant on these matters.

                                 • Reasonable salaries (for example, person
                                                                               Probate Fees
                                   with ALS paying spouse as a caregiver is
                                   a deductible expense to the person with     A Certificate of Appointment of Estate
                                   ALS)                                        Trustee With A Will validates a will. Upon
                                 • High taxpayer pays all household            issuing this certificate, the Estate Court
                                 expenses                                      charges a tax rate that varies from province
                                                                               to province. Probate fees are reduced by
                                 • Deposit child tax credits
                                                                               reducing the amount of the estate, such as
                                   in child’s bank account
                                                                               making life insurance payable to a spouse
                                 • Contribute to spousal RRSP                  instead of to the estate.
                                 • Assign one half of CPP benefits to
                                 spouse
                                 And there are others. If substantial funds
                                 are involved, or even if funds are minimal,
  ALS              SLA           see a professional tax expert who
  SOCIETY OF CANADA              specializes in disability issues.
  Maladie Lou Gehrig’s Disease

            1(800)267-4257
            http://www.als.ca
             alscanada@als.ca


                    PAGE 94                                                                                              v. 1.0
LIVING WITH ALS                                                                                     SECTION          11
FINANCIAL AND LEGAL CONSIDERATIONS

US Estate Tax                                  Tax-Effective Wills                             FINANCIAL
If you were born in the US and own             There are numerous estate planning              & LEGAL
property in the US, or own US securities       alternatives, including naming spouse as        CONSIDERATIONS
registered in your name, your beneficiaries    beneficiary to all life insurance and pension
may be in for an unpleasant surprise.          plans. Also multiple testamentary trusts, an
                                                                                               FINANCIAL & LEGAL
                                                                                               COSTS OF LIVING
                                               exclusive spousal trust and trustee powers      WITH ALS
Even if you have been a Canadian citizen       to authorize actions to minimize tax are
for most of your life, when the US Internal    other techniques. Mentioning these plans in     DEALING WITH
Revenue Service learns of a person’s death,                                                    YOUR EMPLOYER
                                               a will helps to ensure that your plan will be
they will apply an estate tax on the total     carried out according to your wishes.           INSURANCE ISSUES
estate, regardless of what countries the       Bequests to a voluntary organization can        PENSION PLANS
assets are in. Assets will then be held by     also reduce estate tax. Again if substantial
the US Internal Revenue Service until this     amounts are involved, it is probably wise to    TAX ISSUES
tax is paid. There are easy ways to avoid      consult with a professional estate planning     POWERS OF ATTORNEY
this tax. If this could happen to you, see a   accountant or estate planning lawyer.
professional accountant who is familiar                                                        WILLS
with US estate taxes.




                                                                                               ALS              SLA
                                                                                               SOCIETY OF CANADA
                                                                                               Maladie Lou Gehrig’s Disease

                                                                                               1(800)267-4257
                                                                                               http://www.als.ca
                                                                                               alscanada@als.ca


v 1.0                                                                                          PAGE 95
  SECTION         11                                     LIVING WITH ALS
                                                          FINANCIAL AND LEGAL CONSIDERATIONS

     FINANCIAL                   POWERS OF ATTORNEY                            For Property
        & LEGAL                                                                A Continuing Power of Attorney for
                                 What is a Power of Attorney and a Living
CONSIDERATIONS                   Will?
                                                                               property (including managing your
                                                                               financial affairs) will help to protect your
    FINANCIAL & LEGAL                                                          assets from being taken over by the
       COSTS OF LIVING           The purpose of a Power of Attorney and a
             WITH ALS            Living Will is to give someone else the       provincial public trustee. If that happens,
                                 authority to act on your behalf in matters    your family will have an easier time getting
         DEALING WITH                                                          back control of your assets if they have
        YOUR EMPLOYER            relating to your health. They may also
                                 make decisions that arise at the end stages   your Continuing Power of Attorney.
     INSURANCE ISSUES
                                 of life and about all matters relating to
         PENSION PLANS           personal property and finance. Consult        Appointing Your Decision Maker
               TAX ISSUES        with your ALS team social worker, your
                                                                               It is important that you appoint a person,
                                 financial advisor or lawyer for more
 POWERS OF ATTORNEY                                                            or persons, that you trust to make
                                 information. The particulars may vary
                                                                               decisions on your behalf, and make this
                      WILLS      from province to province.
                                                                               person, or these persons, aware of your
                                 Various Kinds of Documents:                   wishes. The person you appoint as your
                                                                               attorney must follow the wishes that you
                                 For Personal Care                             express unless it is impossible to do so.
                                                                               Your Powers of Attorney should be kept in
                                 A Power of Attorney for personal care         a safe place, and be easily accessible to the
                                 also known as a health care directive (for    person you have appointed.
                                 example, medical treatment, nutrition,
                                 safety, shelter) is necessary so that your
                                 wishes regarding medical treatment, when
                                 you are incapacitated, will be carried out
                                 according to your written instructions and
                                 not on the basis of someone else’s
                                 assumptions.




  ALS              SLA
  SOCIETY OF CANADA
  Maladie Lou Gehrig’s Disease

            1(800)267-4257
            http://www.als.ca
             alscanada@als.ca


                    PAGE 96                                                                                             v. 1.0
LIVING WITH ALS                                                                                     SECTION         11
FINANCIAL AND LEGAL CONSIDERATIONS

WILLS                                          Using Your Will to Make                         FINANCIAL
                                               That Special Gift                               & LEGAL
Why is a Will Necessary?                       Many people choose to make provision in         CONSIDERATIONS
                                               their will to contribute a special bequest to
An estate of a deceased person will not                                                        FINANCIAL & LEGAL
                                               a cause close to their heart. Bequests allow    COSTS OF LIVING
normally come under the control of the
                                               people to name a charity of their choice as     WITH ALS
Public Guardian and Trustee (formerly the
                                               a beneficiary and to make a financial
Public Trustee) if there are family                                                            DEALING WITH
                                               contribution from their estate to the charity   YOUR EMPLOYER
members who are entitled to the estate.
                                               that is often far greater than would have
The family members who are entitled to                                                         INSURANCE ISSUES
                                               been possible during their lifetime. You
receive the estate are also entitled to be
                                               may wish to consider the ALS Society in         PENSION PLANS
appointed administrator (or Estate
                                               your region, or ALS Canada, as a
Trustee).                                                                                      TAX ISSUES
                                               beneficiary for a fixed donation or a
If the family members are not entitled to      percentage of your estate.                      POWERS OF ATTORNEY
be named as administrators, or do not                                                          WILLS
wish to take on the administration, it is
possible for them to name the person who
will take on this responsibility. However,
having a will is a much better option.

Planning A Will
Planning your will can minimize the
additional income taxes, and probate fees
payable on your death. Also, planning can
minimize taxes payable by your
beneficiaries on future income.

To plan a will you will need to:

• Make a list of all your assets and
  liabilities including the additional taxes
  due on your death
• Determine who your beneficiaries will
  be, such as spouse, children, other
  relatives, friends, charities, etc.
• Consider tax reduction strategies, such
  as donations to charities
• Choose your estate trustee, such as your
  spouse or trusted friend. A lawyer,
  accountant or trust company could be
  considered for more complex situations                                                       ALS              SLA
                                                                                               SOCIETY OF CANADA
                                                                                               Maladie Lou Gehrig’s Disease

                                                                                               1(800)267-4257
                                                                                               http://www.als.ca
                                                                                               alscanada@als.ca


v 1.0                                                                                          PAGE 97
SECTION         11             LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                  PAGE 98                     v. 1.0
LIVING WITH ALS        SECTION          11
NOTES




                  ALS              SLA
                  SOCIETY OF CANADA
                  Maladie Lou Gehrig’s Disease

                  1(800)267-4257
                  http://www.als.ca
                  alscanada@als.ca


v 1.0             PAGE 99
SECTION         11             LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                PAGE 100                      v. 1.0
LIVING WITH ALS                                  SECTION          12
SUBJECT

A MESSAGE OF HOPE                             A MESSAGE
                                              OF HOPE
ALS may rob you of your body. It does
not rob you of your soul. You live in a
society that emphasizes patient autonomy.
You will be able to maintain yours. While
embarking on a difficult endeavour, you
are not going alone. Families and friends
come closer. You will discover new friends.
Other people with ALS are available and
willing to share in the ups and downs of
the journey. There are many dimensions to
treatment and many professionals available
to provide it. The resources available to
patients are without precedent.

You continue to be a valued member of
society who can contribute to your family
and your friends through the human
values in which you believe. You continue
to play a role in educating your children
and providing support to your family. Due
to the intense interest in people with ALS,
the options available to you exceed those
that were available to previous generations
and are expected to increase.

While no one has chosen this path, most
have traversed it with courage and with
dignity.




                                              ALS              SLA
                                              SOCIETY OF CANADA
                                              Maladie Lou Gehrig’s Disease

                                              1(800)267-4257
                                              http://www.als.ca
                                              alscanada@als.ca


v 1.0                                         PAGE 101
SECTION         12             LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                PAGE 102                      v. 1.0
LIVING WITH ALS                                                                               SECTION          13
                                                                                           THE ALS SOCIETY

ABOUT THE ALS SOCIETY                          For more information on ALS and the         ABOUT THE
                                               ALS Unit in your province, contact us at:
                                                                                           ALS SOCIETY
ALS Society of Canada
                                               The ALS Society of Canada
The ALS Society of Canada was founded          265 Yorkland Blvd. Suite #300
in 1977 as a national non-profit, charitable   Toronto, Ontario
organization dedicated solely to providing     M2J lS5
a variety of services to people with ALS
and their families. The Society has            Tel: (416) 497-2267
affiliated groups in every province in         Fax: (416) 497-1256
Canada and remains the only organization
in Canada to provide the following             website: www.als.ca
services.                                      E-mail: alscanada@als.ca

Our Mission
To provide care and find a cure for ALS


Our commitment in support
of our mission:
• To provide the best possible support
  for people with ALS, their families
  and caregivers.
• To support research into the cause of
  and cure of ALS
• To build public awareness of ALS
  and its impact
• To advocate for improved service,
  research and attention to ALS
  as a catastrophic disease
• To work collaboratively and with
  mutual respect for each other's role in
  meeting the above commitments.




                                                                                           ALS              SLA
                                                                                           SOCIETY OF CANADA
                                                                                           Maladie Lou Gehrig’s Disease

                                                                                           1(800)267-4257
                                                                                           http://www.als.ca
                                                                                           alscanada@als.ca


v 1.0                                                                                      PAGE 103
SECTION         13             LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                PAGE 104                      v. 1.0
LIVING WITH ALS                                                          SECTION          14
LIST OF REFERENCES

HELPFUL ALS PUBLICATIONS                                              REFERENCES
ALS: Strategies for Living.
 ALS Society of British Columbia. 1993, Vancouver:
 ALS Society of British Columbia.

An Overview for People with ALS.
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

Breathing Strategies.
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

Coping with Grief: Strategies for People Living with ALS
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

Communications Strategies
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

Eating, Drinking, and Swallowing Strategies
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

“How Can I Help?”
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

Mobility Strategies.
 ALS Society of Canada. 1990, Toronto. ALS Society of Canada.

Resources for ALS Healthcare Providers.
 ALS Society of Canada. 1994, Toronto. ALS Society of Canada.

Living with ALS: What’s it all about?
  Forshew, Dallas, and Sharon Hulihan, Edited by Ginny King Supple.
  1997, Woodland Hills, CA. The ALS Association.




                                                                      ALS              SLA
                                                                      SOCIETY OF CANADA
                                                                      Maladie Lou Gehrig’s Disease

                                                                      1(800)267-4257
                                                                      http://www.als.ca
                                                                      alscanada@als.ca


v 1.0                                                                 PAGE 105
SECTION         14             LIVING WITH ALS
                                           NOTES




ALS              SLA
SOCIETY OF CANADA
Maladie Lou Gehrig’s Disease

          1(800)267-4257
          http://www.als.ca
           alscanada@als.ca


                PAGE 106                      v. 1.0

				
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