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difficulty in breathing


									     Chapter 5

                                      HOW DOES
                                      ALS AFFECT
                                      R     espiratory prob-
                                            lems are perhaps
                                            the most serious
                                      of medical complica-
                                      tions in ALS. Breathing
                                      difficulties occur from
                                      the gradual deteriora-
                                      tion of muscles involved
                                      with breathing: the dia-
                                      phragm and the inter-
                                         The diaphragm is an
                                      arched muscle located
                                      just beneath the lungs

68   Chapter 5 — Respiratory Issues
that moves up and down and allows air to come        your breathing problems aren’t
in and move out. The intercostals are muscles        caused by problems with your
between the ribs that contract and relax and also    lungs; they’re caused by weak-
assist with air movement.                            ness of the muscles that oper-
   As ALS weakens these muscles, you’ll become       ate the lungs. Treatments for
conscious of the act of breathing, which is nor-     these two types of conditions
mally automatic, and it will consume additional      are quite different.
   Weakening respiratory muscles may increase           Your best approach is to have
your fatigue levels and deplete your energy. The     your ALS physician communi-
effort to breathe, when these muscles aren’t         cate with the pulmonary spe-
functioning well, is hard work.                      cialist, and to work closely with
   Weakening of the respiratory muscles can          a respiratory therapist who’s
also challenge your ability to respond to the        familiar with ALS.
stresses of colds, flu or pneumonia — illnesses

                                                     THE ROLE OF
that are caused by bacterial or viral infections.
Pneumonia also may be caused by aspiration of

                                                     THE RESPIRATORY
food or fluid into the lungs, which can be caused
by weakened muscles.

   But, as in many other areas, new knowledge
and technology make it possible for you to work,

travel and continue with normal activities while
obtaining the help you need with breathing.               he respiratory therapist on
   MDA health care experts recommend that                 your ALS health care team
people with ALS get a flu vaccine in the fall, get        is responsible for obtaining
a pneumonia vaccine, and be familiar with the        measurements of your respira-
symptoms of pneumonia:                               tory function and instructing
n	 coughing                                          you and your family in the use
n	 sputum production (may be clear, yellow
    or green)
n	 chest discomfort with breathing
n	 fever/chills

  People who are having difficulty with liquids
(choking or coughing) should use a thickener
(such as ThickIT or SimplyThick) in their diets
to prevent aspiration of fluids into the lungs.

SPECIAL NOTE: As your respiratory muscles
weaken, your neurologist may refer you to you
a pulmonologist, a doctor who specializes in
issues related to the lungs and respiratory sys-
tem. Be sure this specialist understands that

                                               Everyday Life with ALS: A Practical Guide   69
     of therapeutic measures and equipment        decrease the work of breathing.
     prescribed by the pulmonary specialist.         If oral or pharyngeal secretions (from
     The importance of careful monitoring of      the mouth or nose) become excessive,
     respiratory function and proper instruc-     drugs that decrease saliva production
     tion in therapeutic measures is essential    or suction devices to remove secretions
     in the overall care of people with ALS.      may be beneficial. If low blood oxygen
        Your RT or pulmonary specialist will      levels are documented, supplemental
     measure the forced vital capacity (FVC)      oxygen may be given. All infections
     (total amount of air that can be moved       should be promptly treated.
     in or out of the lung). This can easily be

     accomplished by exhaling into a spiro-
     meter. The FVC is easy to perform and is

     a meaningful indicator of changing respi-
     ratory status in the person with ALS.

        Evidence of respiratory involvement
     might include:                                     reathing, meaning the exchange
     n	 shortness of breath with or without             of oxygen and carbon dioxide that
         exertion                                       normally occurs, may become less
     n	 increased lethargy or loss of energy      effective for you in advanced stages of
     n	 poor cough                                ALS. The result may be respiratory dis-
     n	 difficulty breathing while lying flat     tress, which has many symptoms:
     n	 headaches, especially when you            n	 the inability to sing or shout
         awaken                                   n	 the inability to cough or sniff hard
                                                  n	 the ability to speak only in short sen-
        If any of these symptoms occur fre-           tences
     quently, you need a medical examination      n	 apparently labored breathing
     of your respiratory status.
        The RT assists in the instruction of
     therapeutic measures ordered by the
     pulmonary specialist such as incen-
     tive spirometry, assistive coughing and
     breathing exercises, suctioning, inter-
     mittent positive pressure breathing, and
     postural drainage.
        The therapy of respiratory dysfunction
     in ALS is primarily aimed at general sup-
     portive measures. Considerations may
     include ventilatory maneuvers (voluntary
     or positive pressure) to prevent atelecta-
     sis (lung collapse), a cessation of smok-
     ing program, instruction in maintaining
     nutrition and prevention of aspiration,
     flu vaccinations, and medications to

70   Chapter 5 — Respiratory Issues
n	 use of muscles in the neck or             SPECIAL NOTE: It’s wise to think
   abdomen to compensate for a weak-         ahead about your choices and needs
   ened diaphragm                            for respiratory help. Without planning,
n	 headaches at waking                       you could experience a respiratory cri-
n	 excessive daytime sleepiness              sis and have to make important deci-
n	 exhausted appearance or weight loss       sions in an emergency situation.
   owing to retained excess carbon

                                             TYPES OF VENTILATION

                                             AND EQUIPMENT
   Before these respiratory complications
emerge, your doctor will probably begin
to discuss various methods and steps of
respiratory support — ventilation. There
are more options for assisted breathing
today than ever before, some that can
                                             This form of ventilation has seen several
prolong life for several years.
                                             advances and wider use in recent years.
   Some experts say that assisted ventila-
                                             Noninvasive devices don’t involve surgi-
tion is the single most significant factor
                                             cal invasion of the body. Many times,
in the increased life expectancy of peo-
                                             ventilators can be used for several hours
ple with ALS in recent years.
                                             a day or just during sleeping hours.
   You should give the question of ven-
                                             People with ALS whose bulbar (mouth
tilatory support serious thought in
                                             and throat) muscles are extensively
advance and put your wishes in writing
                                             weakened, however, may need more
so they’ll be known to your caregivers
                                             extensive ventilation solutions.
and medical team if you have a respira-
                                                There are several forms of noninva-
tory emergency. You may want to state
                                             sive ventilation. One is pressure-cycled
your wishes formally in a medical direc-
                                             vent machines, which deliver air at a set
tive; you can alter this document any
time you change your mind.
   Ventilators are now small, portable
and quiet, but maintaining a person with
one at home can be very expensive and
taxing on caregivers.
   Remember that your health care team
and other professionals at the MDA clin-
ic are there to help you understand your
options and answer your questions as
you make difficult decisions about ven-
tilation. Be sure to discuss this subject
in detail, including the options described
here, so you can make your choices
clear to your doctor and loved ones.

                                              Everyday Life with ALS: A Practical Guide   71
     pressure level with a variable volume of    (such as a tracheostomy, see below),
     air on a timed cycle.                       some doctors now prescribe them for
        A CPAP (continuous positive airway       use with a mouthpiece, nasal or face
     pressure) machine is not indicated in       mask.
     ALS. CPAP increases the work of breath-
     ing by forcing the user to exhale against
     resistance. This can be dangerous for
     those with ALS.
        A BiPAP machine, on the other hand,
     is often prescribed in ALS. BiPAP (the
     trademark name of a machine distrib-
     uted by Respironics) is short for bilevel
     positive airway pressure and delivers air
     at two pressures, one for inspiration and
     one for expiration (inhalation and exha-
     lation). A number of nasal or face masks
     and attachments are available, and can
     be customized for the best fit.
        Volume-cycled ventilators deliver a
     pre-set amount of air. Volume vents
     can deliver air in far greater pressures
     and volumes than pressure vents can.
     Although these machines were tradition-
     ally used only with invasive interfaces

                                                    Another noninvasive form of ventila-
                                                 tion uses negative pressure through
                                                 a corset-like device that wraps around
                                                 the chest and creates negative pressure,
                                                 allowing the lungs to expand. With nega-
                                                 tive pressure applied on a timed cycle,
                                                 the lungs inflate and deflate alternately,
                                                 as in regular breathing. This is the same
                                                 technique that was used years ago in
                                                 “iron lungs.”


                                                 Invasive ventilation is delivered via a
                                                 tracheostomy, a surgically created hole
                                                 in the trachea (windpipe) through which
                                                 air is forced. The tube through which the

72   Chapter 5 — Respiratory Issues
                         air is delivered
                                               combat this problem.
                         also is called a
                                                  Not everyone with ALS will need or
                                               choose to have a tracheostomy, but
                         (trach) tube.
                                               there may come a time when it’s neces-
                            A ventilator
                                               sary for continued breathing.
                         delivers air on
                                                  In addition, most people relying on
                         a timed cycle
                                               invasive ventilation will need humidifica-
                         through the trach,
                                               tion because the nose, through which
                         and ensures that
                                               air is normally moisturized, has been
                         you’ll take a mini-
mum number of breaths per minute.
Many ventilators can then be adjusted to
respond to the person’s own efforts to
breathe, or to completely override these
                                               COUGHING AND
efforts. The decision to start tracheos-
tomy-delivered ventilation is often a per-
                                               CLEARING SECRETIONS

manent one because it’s usually impos-
                                                     oughing and clearing secretions
sible for people with ALS to recover the
                                                     from the lungs are activities that
ability to breathe on their own.
                                                     most people do automatically.
   Many people with ALS ultimately need
                                               Normally, the lungs constantly move
a trach because of the weakness of the
                                               excess mucus and inhaled particles up
mouth and throat (bulbar) muscles.
                                               toward the mouth to a spot where they
Invasive ventilation is thought to be a
                                               can be coughed up.
more reliable means of delivering air to
                                                 As ALS advances you may eventually
the lungs when the disease is advanced.
                                               have difficulty coughing and clearing
But one important drawback of a trach is
                                               your throat because of weakened abdom-
that it interferes with the body’s normal
                                               inal and throat muscles. Material that
mechanisms for clearing the respiratory
                                               would normally be coughed up instead
tract of mucus. Various solutions can
                                               will fall back down into your lungs
                                               where it can cause respiratory irritation
                                               and infection. This may occur whether
                                               or not you’re using assisted ventilation.
                                                 Certain techniques and medical equip-
                                               ment can enable you and your caregiv-
                                               ers to create or assist a cough, and then
                                               clear mucus secretions from your air-
                                               way. Talk with your doctor and your RT
                                               about these interventions.

                                                 Everyday Life with ALS: A Practical Guide   73
     Breath Stacking
                                                  OTHER EQUIPMENT
     An RT can show you this technique,
     which involves closing the throat after      Suction Machines
     each breath taken in through a mouth-
     piece, and then coughing.
                                                  Portable or stationary suction machines
                                                  can provide appropriate suction for remov-
     Abdominal Thrust                             ing the lungs’ mucus secretions.
                                                     Caregivers must be instructed in sterile
     Caregivers can be taught how to increase     techniques for suctioning, which usually
     coughing efficiency by pressing on your      involves inserting a tiny tube (catheter)
     abdomen.                                     several inches into the trachea via a trach
                                                  tube. In the absence of a trach, suction
     Assisted Coughing Devices                    devices similar to those seen in dentists’
                                                  offices can be directly inserted into the
                                                  mouth. Others can be inserted through
     An In-Exsufflator machine delivers a large
                                                  the nose via a tube with a soft catheter
     volume of air into the lungs and then
                                                  tip. The tube is attached to a suction
     quickly reverses the air flow to pull out
     secretions, just as a cough would.
                                                     In addition, most people relying on suc-
        An example is the CoughAssist. Used
                                                  tion machines will need humidification
                      with a facemask, with a
                                                  because the nose, through which air is
                      mouthpiece or with an
                                                  normally moisturized, has been bypassed.
                      adapter to a patient’s
                      endotracheal or tracheos-
                      tomy tube, this machine     Pulse Oximeter
                      can be used to clear your
                      airway as needed. This      Oximeters are electronic devices about
                      type of machine is often    the size of a small cell phone that mea-
                      recommended for use in      sure the amount of oxygen in the blood
                      conjunction with invasive   through a painless sensor that can be
                      ventilation, and can be     clipped to a finger or earlobe. If your RT
     demonstrated by your RT.                     or pulmonologist finds that your oxygen
        Another option that’s been recently       level is normal (at least 95 percent “satu-
     studied for its effectiveness for people     ration”) without any supplemental oxy-
     with ALS is The Vest. This system uses       gen, it’s likely that air exchange (of oxy-
     a technology called high-frequency chest     gen for carbon dioxide) is adequate.
     wall oscillation. During therapy, The Vest      If saturation levels dip below nor-
     inflates and deflates rapidly, applying      mal, you and your doctor have to decide
     gentle pressure to the chest wall. This      whether the problem is chronic under-
     loosens and thins mucus and moves it         ventilation because air exchange isn’t
     toward the larger airways, where it can be   adequate, or whether there’s mucus plug-
     cleared by coughing or suctioning.           ging the airways.

74   Chapter 5 — Respiratory Issues
                                             Spotlight on
These medications, such as those com-
                                             Feeding Tubes

monly used to treat asthma (albuterol,
                                                nsertion of a percutaneous endoscopic
Proventil, etc.), dilate (open) the airway
                                                gastrostomy tube, commonly called
and are sometimes prescribed for people
                                                a PEG tube or a feeding tube, some-
with ALS. However, many doctors don’t
                                             times coincides with respiratory support
think this type of drug is helpful because
                                             in ALS. This intervention may be neces-
it won’t improve the muscle weakness
                                             sary to prevent choking episodes and to
that’s the source of respiratory problems
                                             enhance the ease of feeding if swallowing
in ALS.
                                             problems are present. A PEG tube deliv-
                                             ers food directly into the stomach from
Expectorants                                 the outside and not down the throat.
                                                As a result of bypassing the mouth, a
This type of drug, commonly found in         PEG tube can reduce, but may not elimi-
some over-the-counter cough medicines,       nate completely, the danger of aspirating
can thin secretions and make them            food or liquid into the lungs. With better
easier to cough up. Doctors sometimes        nutrition and less energy used for eating,
prescribe them for people with ALS, as       weight loss may stabilize, or even partial-
well as recommend an increase in fluid       ly reverse, and respiratory function often
intake.                                      improves. Ideally, such a tube should be
                                             placed before serious respiratory com-
Saliva Management                            plications arise, and some doctors insist
                                             that it’s “never too early” to consider a
                                             PEG tube in ALS.
Weakened breathing muscles, along with
                                                Placement of a PEG tube is another
weaker muscles in the mouth, in ALS
                                             personal and important decision that
may result in drooling or sialorrhea. This
                                             should be made with the help of your
isn’t a case of excessive saliva produc-
                                             ALS health care team. For resources on
tion; it occurs when you’re unable to
                                             the topic, see Chapter 10.
swallow saliva as well as before. There
                                                Some doctors advocate proactive place-
are several medications and other treat-
                                             ment of a feeding tube to prevent any
ments that your physician may suggest to
                                             weight loss, choking episodes or nutri-
control drooling.
                                             tional deficits.
                                                For more detailed information about
                                             nutrition in ALS, see the MDA ALS
                                             Caregiver’s Guide.

                                              Everyday Life with ALS: A Practical Guide   75

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