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MND Education 2012 no pictures1 by Ic4bxY9


									Caring for People Living With
  Motor Neurone Disease

                   Dr Monika Wilson
           ReConnections Counselling Service

               0428 777809 / 5457 3329
Umbrella term – a group of diseases

 First described by Jean-Martin Charcot
  in 1869
 The cause of MND is still unknown and
  there is currently no cure
 A progressive neurological disease:
   motor nerve cells (neurones)
    degenerate and die
  muscles for voluntary movement,
   speech, breathing and swallowing
   gradually weaken and waste
  no nerves to activate them
  patterns of weakness vary from
   person to person
         What are Motor Neurones?

• Neurones are a network of nerve
  cells that are the electrical wires of
  the human body
• Motor neurones control the
  muscles used in voluntary
• Motor neurones -messages to
• Sensory neurones- messages to
  the brain
   Types of Motor Neurones

 Upper motor nerves (UMN)
  from motor cortex
  along spinal cord
  connect with LMN
 Lower motor nerves (LMN)
  in spinal cord (anterior horn cells)
  take message to muscles
 Incidence: Approx 1 in 37,500 people diagnosed
  each year
 Prevalence: Approx 1400 in Australia / 350-400 in
  Qld ?
 Each day more than one person dies from MND and
  another is diagnosed
 Duration: Average 2 to 3 years, but 10% live > 10
 Most common age of onset: 50-60s
 Gender: Men affected slightly more frequently
  (2:3 ratio)
 90% sporadic, 5%-10% familial

  amyotrophic lateral sclerosis (ALS)
    65% UMNs and LMNs
  progressive bulbar palsy (PBP)
    25% LMNs
  progressive muscular atrophy (PMA)
    <10% LMNs
  primary lateral sclerosis (PLS)
    rare UMNs
               Not Affected

 Muscles controlling bladder and bowels
    not directly affected
 Hearing, taste, smell and sensation
    sensory nerves
 Heart
    autonomic nerves
 Difficult to diagnose
 Mimics many other diseases
 Tests to exclude other conditions:
   blood tests
   electromyography (EMG)
   nerve conduction tests
   transcranial magnetic stimulation (TMS)
   Xray
   CAT scan / MRI
   Muscle biopsy
   Lumbar puncture
 Rapidly changing physical abilities
 Decreasing capacity of carer over time
 Increasing levels of support and care required
 Emotional and psychological demands of caring and
  being cared for

 MND affects each person differently, the rate of
  progression varies and our caring strategies need
  to be flexible and creative
        Multidisciplinary Care
                    Fact Sheets EB2/EB3

 Multidisciplinary care:
   Health care professionals being
    knowledgeable about MND
   Flexible, coordinated professional support
   Referrals in a coordinated way
   Regular review/assessment of symptoms
   Opportunities to get specialist advice
   Key worker role
                      Fact Sheet EB4

 Anti-glutamate medication (Rilutek)
 Blocks the release of glutamate from nerve
 May cause weariness, nausea, dizziness
 Research: prolongs median survival by 2-3
 Those taking riluzole early are more likely to
  remain in the milder stages of the disease for
           Non-Invasive Ventilation
                         Fact Sheet EB7

 Provides breathing support (positive pressure)
 Relief of symptoms - fatigue, breathlessness and
  disturbed sleep
 Does not prevent weakening of the muscles
 Research: prolongs median survival up to 7 – 12
 Suitability / availability
                                   Fact Sheet EB8

 Permanent feeding tube into the stomach
 Improved nutrition and QoL
 Early decision required
Information>National Information
              Goals of Care
 relief of symptoms    dignity and respect
 preservation of       quality relationships
  independence          peaceful dying
 quality of life        process
 support               listening, acceptance,
 choice and control     acknowledgement
 information and       minimise suffering
  education             comfort
            Common Symptoms
 Symptoms experienced:
       weakness/ fatigue    94%
       dysphagia            90%
       dyspnoea             85%
       pain                 73%
       weight loss          71%
       speech problems      71%
       constipation         54%
       poor sleep           29%
       emotional lability   27%
       drooling             25%
                             Oliver, 2008
Muscles: Lower Limb Weakness
•Often begins with foot drop
•Difficulty climbing stairs
•Difficulty arising from chairs
•Possibility of falls
•Eventually leading to hoist
•Fasciculation and cramps
              Care Strategies
 Ongoing assessment for equipment needs
   Home modifications
     Grab rails, chairs and beds on blocks, toilet raiser,
      shower chair, hoist
 Ongoing assessment for
     Movement and mobility
     Transfer belt, walker, wheelchairs
     Ankle / foot orthosis
 Manage swollen limbs
     Elevation, pressure stockings, recline chair,
      passive exercise, keep cool
Muscles: Upper Limb Weakness

 Hand weakness
   difficulty with fine motor tasks using hands
 Shoulder girdle weakness
   difficulty using arms
 Neck weakness
              Care Strategies
 Ongoing assessment for equipment needs
     hand and body functional aids
     alternative clothing
     home modifications
 Ongoing assessment for
     Splints / orthotic devices / neck collars
     Movement / light exercise
 Care when transferring, esp shoulder joint
 Massage, pressure garments, elevation
       Maintaining Comfort

 Repositioning
 Subtle adjustments (small moves)
 Satin sheets, kylies, bed stick
 Support – cushions
 Care for weakened limbs
                 Discomfort & Pain

1.   Musculoskeletal pain    Physiotherapy and
2.   Cramp/spasm pain         passive movement
3.   Skin pressure pain      Massage
                             Hydrotherapy
                             Use of splints and
                             Medications (initially non-
                              narcotic analgesics, anti-
                              inflammatory and anti-
                              spasticity agents)
                             Opioids
           Pain Management
 Similar to other advanced diseases:
   Careful assessment of pain
   Differing types of pain (cramps, spasticity,
    musculoskeletal discomfort)
   Severity
   Time course
   WHO guidelines
 Unique issues:
   Pain assessment with non-vocal plwMND
   Impaired swallowing and PEGs
              Creative Thinking

 Need an effective way of calling for assistance
       Door chimes
       Jelly bean switches
       Baby monitors
       Intercom systems
 Location of best position
 Minimise anxiety
 Physical body weakness, deterioration and
 Dysphagia (difficulty swallowing: eating,
  drinking, saliva, choking, aspiration
 Dysarthria (changes in speech: volume,
  slurred, weakness, no communication)
 Respiratory weakness (dyspnoea,
  orthopnoea, respiratory failure)
  Muscles: Bulbar Weakness
•Choking on thin liquids
•Slurring of speech
•Quiet voice
•Loss of speech
•Difficulty chewing and swallowing
•Weight loss
Signs and symptoms of weakness in the muscles
      involved in chewing and swallowing
 making an extra effort to chew
 coughing whilst eating or drinking or soon afterwards
 needing several swallows for each mouthful
 muffled or ‘wet’ sounding voice after eating
 eating or drinking appears tiring - the person may be
  breathless after a meal
 meal times take longer
 frequent chest infections - caused by food and liquid residue
  in the lungs
 difficulty clearing saliva

 Swallowing difficulties can lead to dehydration, malnutrition
  and constipation.
       Muscles: Swallowing
 Dysphagia requires:

  Thorough and regular mouth care / hygiene
  Regular assessment by speech pathologist
   and dietician
  Maximise hydration and nutrition
  Modify diet and consistency
  Time over meals – no distractions
  Correct posture – upright, chin tuck
  Conscious swallowing, food positioning
     Sialorrhoea: Saliva beyond the
       margin of the lip (drooling)

 We produce approx 600 ml each day
 Handling of saliva is affected due to:
   Weakness of the tongue
   Weakness of throat muscles
   Anatomical structure (poor lip seal)
   Poor head control
     The Impact of Drooling

 Social participation
   Withdrawal, embarrassment
 Emotional wellbeing
   Loss of independence and self esteem
 Physical function
   Speech
   Swallowing
   Oral health ie infection, odour
   Dehydration
                Saliva Care: Thin
 Strategies:                   Medications:
   Upright position
   More conscious swallow
                                  Glycopyrrolate
   Wipes and clothing            Amitriptylilne
                                  Benztropine
   Assisted cough technique
 Natural remedies:
   Golden rod drops            Suction
   Sage and hibiscus tea       Collar
   Horseradish tablets
                                Botox injections
             Saliva Care: Thick
 Natural remedies:
                              Nebulizer (with saline
   Dark grape, pineapple,
     apple or lemon juices
   Papaya extract            Steam inhalation
   Suck sugar-free citrus    Mouth care products
     lozenges                  i.e. Biotene
 Hydration ++++
                              Assisted cough
 Reduce / eliminate
  alcohol, caffeine, dairy
 Aspiration pneumonia       Reducing the risk
   Defined as the           Elevate the bed
    inhalation of either       Peg tube
    oropharyngeal or           Avoid eating 1 to 2 hours
    gastric contents into       before bedtime
    the lower airways          Saliva control
                               Oral hygiene
 Due to poor swallow
  weakness or gag reflex
 Choking – due to:
   Impaired respiration
   Muscle spasm (laryngospasm)
 Care strategies
   stay calm
   reassure person
   wait for attack to pass
 Seek advice from physiotherapist for assisted
  cough technique
 Medications i.e. Morphine, Benzodiazepines: Clonazepam (drops),
  Lorazepam (Ativan)
       Nutrition: Eating well

• Speech pathologist / dietician to assess
• Increasing dysphagia
• Modified diet – pureed food, thickened fluids,
  nutritional supplements, gravies
• Positioning, use of equipment
      Why Consider PEG?

• Stabilise weight loss
• Maximise nutrition and hydration
• Maximum energy
• Improve quality of life
• Prevent choking on thin fluids (safety)
• Prevent prolonged mealtimes (distress)
• Reduce risk of aspiration
         Placement of PEG
 Early placement recommended
   can be left un-used
   use as a ‘top up’
   some risks involved
 Respiratory assessment
   Should be inserted before vital capacity falls
    below 50% of predicted (for safe anaesthetic)
                   Muscles: Speech
 Dysarthria (motor speech disorder)    Affects:
 Slurred speech, quiet                   Vulnerability
  voice                                   Isolation
 Changes in vocal quality                Inability to express needs
 Requires coordinated                    Exclusion from decision
  movement of several                      making
  muscle groups                           Loss of independence and
                                           social role
 Speech pathologist to
                                          Loss of self identity
  review and advise
                                          Challenges relationships
            Care Strategies
 Key word of sentence first
 First letter of word
 Eye contact and signals
 Gestures
 Translation by carer
 Letter / phrase chart
 Yes/no questions
 Be patient – slow down
          Communication Aids
 Low tech aids:
     Writing
     Magna doddle / white boards
     Laser pointer and chart
     Etran boards
 High tech aids:
   Lightwriter / Polyanna / Alora
   VMax
   Essence Vantage Light

 Most common symptom
 Everything is exhausting
 Rest following activities (smaller rest periods)
 Small aids and equipment can help
 Conserve energy
 Be aware of insomnia
 Visit in the pre-lunch hours
 Bigger meals earlier in the day

 Due to lack of movement
 Legs elevated with cushion support
 Use of massage
 Elastic stockings
 Be aware of deep vein thrombosis
             Bladder & Bowels
 Fasciculation may irritate    Weak abdominal and
  the bladder                    chest muscles
 Hand weakness or              Diet / hydration
  mobility limitations          Privacy
   Use of pads                 Require adequate fibre,
   Uridomes                     fluid
   Catheter                    Routine, comfort, aids
                                Laxatives
           Emotional Lability –
           pseudo bulbar effect

 Unpredictable episodes of crying and laughing
 Disease damages the area of the brain that
  controls normal expression of emotion
 Anxiety and embarrassment, particularly in
 Explanation (part of the disease), reassurance
  (not going mad)
 Medication in more severe situations
          Cognitive Changes

 previously thought cognition was not
 research indicates up to 75% may have
  some frontal lobe dysfunction
 15% to 41% meet criteria for fronto-
  temporal dementia (FTD)
                          Miller & others, 2009
          Cognitive Changes

 Cognitive Impairment (CI): deficits in
  attention, word generation, cognitive
 Behavioural Impairment (BI): changes in
  social interaction
 Fronto-temporal Dementia (FTD):
  altered social conduct, emotional
  blunting, loss of insight, language
  change, poor self care, emotional
  recognition, lack of empathy
• Changes in decision-making
• Reduced awareness of risk, concerns about
  risk taking
• Frustration; forgetfulness
• Communication
• Obsessional behaviour; impulsiveness
• Lack of self care
          Issues for Professionals
Decision making
  • Assessment earlier to make decision –but person
    may not want to discuss the issues
  • Unsure if discussion retained and able to be
    involved in the discussion
Assessing symptoms
  • Pain / depression / swallowing problems
Coping with memory loss / confusion
                           Care Strategies
 Education for caregivers
 Give simple directions
 Establish a regular routine
 Possible medical management

Cognitive and behavioural challenges in caring for patients with frontotemporal
dementia and ALS (2010). Amyotrophic Lateral Sclerosis, 11: 298-302.
        Muscles: Respiratory
 Disturbed sleep       Reduced movement
 Daytime sleepiness     of the rib cage or
 Increased fatigue      abdominal muscles
 Morning headaches     Excessive use of the
                         muscles in the upper
 Quieter voice
                         chest and neck
 Fewer words per
                        Weakened cough and
 Shallow, faster
Respiratory muscle weakness can cause

 Breathlessness (dyspnoea) even at rest
 Breathlessness lying flat (orthopnoea)
 Impaired concentration or confusion
 Irritability and anxiety
 Decreased appetite
                    Care Strategies
 Be vigilant for symptoms      Improve ventilation – fans,
 Refer to a specialist          air flow, humidifier
  respiratory service for       Adjust room temperature
  regular assessment            Reclined or fully upright
 Avoid infections (people       position
  with coughs/cold)             Respiratory / breathing /
 Treat reversible causes of     relaxations exercises
  dyspnoea                      Medications: lorazepam,
 Discuss NIPPV support          midazolam, morphine
 Avoid crisis situations
Non-Invasive Positive Pressure Ventilation

 • The use of positive pressure to do some of the
   work of breathing
 • BIPAP (bi-level) or VPAP (variable)
 • Used overnight to improve symptoms
 • Does not prevent weakening of muscles
  Benefits of Assisted Ventilation

 Decreased daytime sleepiness
 Better appetite
 Rests fatigued respiratory muscles
 Improved sleep
 Quality of life
 More energy
 Improved defence against infections
       Implications to Consider

 Significant improvement in survival
 Mask issues, intolerance
 Costs, availability, accessibility, back up
 Increasing dependency
 Carer burden
 Advance care planning (AHD/POA)
 See NICE Clinical Guidelines for the use of non-
  invasive ventilation:
       Advance Care Planning
 75% preferred early discussion of Advanced
  Directives Oliver, International Symposium, 2007
 plwMND preferred that doctor initiates
 Communication issues
 Ventilation withdrawal issues
 Shown to change their preference for life-
  sustaining measures (e.g. ventilators) over a
  six month period Silverstein et al., 2006 = periodically re-
  evaluate AHD
 Cultural differences
Six Triggers for Initiating Discussion About
             End of Life Issues
1.   The plwMND or the            4. Dysphagia requiring a
     family asks - or ‘opens         feeding tube
     the door’ – for end of       5. Dyspnoea or symptoms
     life information and/or         of hypoventilation, a
     interventions                   forced vital capacity of
2.   Severe psychological            50% or less is present
     and/or social or spiritual   6. Loss of function in two
     distress or suffering           body regions (bulbar,
3.   Pain requiring high             arms or legs)
     dosages of analgesic            Promoting excellence in end of
     medications                     life ALS care, 2004
               Use of Oxygen

 Breathlessness is due to muscle weakness not
  low oxygen

  If oxygen is given inappropriately it can:
     Increase carbon dioxide retention
     Reduce the body’s spontaneous signals to
     Put increased pressure on weakened
                      Common Cause of Death
 Most people die of respiratory failure
   Without NIPPV
       Choose not to
       Intolerance
   With NIPPV
       Eventual failure or voluntary withdrawal
 The duration between an acute deterioration and
  death is less then 24 hours
 Choking rarely occurs
 Neurvert, C, Oliver D Journal of Neurology 2001: 248
           Other Causes of Death
• Malnutrition and
  • Without peg             • Aspiration pneumonia
    •Refusal                • Sepsis
    •Anatomical             • Pulmonary embolus
      considerations        • Head injury/falls
    •Respiratory status     • Suicide
  • With peg                • Co-morbidity
    •Voluntary stopping
    •Intolerance or other
 Terminal Phase is recognised by

 Increased, progressive weakness
 Deterioration over a few days
 Often proceeded by
   Reduction in chest expansion
   Quietening of the breath sounds
   Accessory muscles for breathing
   Morning headaches
          Medical Management during
                Terminal Phase

 Use range of routes: oral, peg, patch or continuous
  subcutaneous infusion (syringe driver)
   Morphine or diamorphine to reduce
   Lorazepam, Diazepam (Valium) or Midazolam, a
    sedative, to reduce agitation/restlessness
   Glycopyrronium bromide to reduce the chest
    secretions and saliva (or hyoscine hydrobromide)
 Ethically appropriate to sedate; no muscle-paralyzing
  agents should be used
 Used appropriately (start small & increase) these
  medications will not hasten death
Withdrawal of Ventilatory Support
 Major decision making as    Cultural or religious rituals
  to when to cease             discussed and planned
                              Location prepared
 Education of what to
  expect                      Medications ready
 Comfort maintained          Subcutaneous route is
 Physician should be          preferred
  present (established        Family and friends
  relationship)                present
 Planned event; no haste
                  End of Life Care
 Fear of choking (rarely      Build up of carbon-
  occurs) and                   dioxide will
  breathlessness                anesthetise
 Increasing immobility        Step-process of
 Discussions and               withdrawal of NIPPV
  anticipation of the final    Use of adequate
  time                          medications
 Advance care planning,       Support for the family
  directives and EPOA           and friends
      done ahead of time       (bereavement)
      regular review
Features of Optimal End of Life Care

  care plans and information are shared
  adequate nursing cover
  comprehensive symptom control
  Psychological, social & spiritual support
  family and friends are providing practical
   support for the primary carer
  the opportunity to find completion
    Psychological, Emotional & Social Issues
o A spiralling series of progressive losses
o Changes in ability to influence their external
  and internal environment (control)
o Changed relationship with body/self/identity
o Aware of what is happening, what will come
  and increasing dependency
o Carer burnout, relationship issues
o Many psychological, emotional, sexual,
  financial, spiritual adjustments to be made
          Family & Friends
 Create relationships: open and honest,
  ongoing communication, inclusion, non-
 Family also have needs
 Respite
 Involvement in care
 Discuss fears and concerns
    Health Care Professionals

 Stretches the physical, emotional and spiritual
  ‘resources’ of staff
 Acknowledgement and support
 Awareness of self reactions (buttons)
 Flexible approach to care (share the care)
 Remember self care and understanding of
  own loss, grief and death fatigue
 Be aware of the unique challenges of caring
  for a person living with MND
 Understand the disease and rapidly changing
 The disease is the problem, not the person
 Early contact, relationship building
 Ongoing, preemptive assessment and referral
 Well coordinated teamwork
• for information
 on MND care, symptom management
 and support for health professional
•MND Aware: online training modules

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