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Caring for People Living With
Motor Neurone Disease
Dr Monika Wilson
ReConnections Counselling Service
www.reconnectionscounselling.com
[email protected]
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
movement
• Motor neurones -messages to
muscles
• 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
years
 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
Diagnosis
 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
Riluzole
Fact Sheet EB4
 Anti-glutamate medication (Rilutek)
 Blocks the release of glutamate from nerve
cells
 May cause weariness, nausea, dizziness
 Research: prolongs median survival by 2-3
months
 Those taking riluzole early are more likely to
remain in the milder stages of the disease for
longer
 PBS
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
months
 Suitability / availability
Gastrostomy
Fact Sheet EB8
 Permanent feeding tube into the stomach
 Improved nutrition and QoL
 Early decision required
http://www.mndaust.asn.au/
Information>National Information
Goals of Care
 relief of symptoms
 preservation of
independence
 quality of life
 support
 choice and control
 information and
education
 dignity and respect
 quality relationships
 peaceful dying
process
 listening, acceptance,
acknowledgement
 minimise suffering
 comfort
Common Symptoms
 Symptoms experienced:
 weakness/ fatigue
 dysphagia
 dyspnoea
 pain
 weight loss
 speech problems
 constipation
 poor sleep
 emotional lability
 drooling
94%
90%
85%
73%
71%
71%
54%
29%
27%
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
transfer
•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.
2.
3.
Musculoskeletal pain
Cramp/spasm pain
Skin pressure pain
 Physiotherapy and
passive movement
 Massage
 Hydrotherapy
 Use of splints and
cushions
 Medications (initially nonnarcotic analgesics, antiinflammatory and antispasticity 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
immobility
 Dysphagia (difficulty swallowing: eating,
drinking, saliva, choking, aspiration
pneumonia)
 Dysarthria (changes in speech: volume,
slurred, weakness, no communication)
 Respiratory weakness (dyspnoea,
orthopnoea, respiratory failure)
Muscles: Bulbar Weakness
•Drooling
•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:
 Upright position
 More conscious swallow
 Wipes and clothing
protection
 Assisted cough technique
 Natural remedies:
 Golden rod drops
 Sage and hibiscus tea
 Horseradish tablets
 Medications:
 Glycopyrrolate
 Amitriptylilne
 Benztropine
 Suction
 Collar
 Botox injections
Saliva Care: Thick
 Natural remedies:
 Dark grape, pineapple,
apple or lemon juices
 Papaya extract
 Suck sugar-free citrus
lozenges
 Hydration ++++
 Reduce / eliminate
alcohol, caffeine, dairy
products
 Nebulizer (with saline
solution)
 Steam inhalation
 Mouth care products
i.e. Biotene
 Assisted cough
technique
Complications
 Aspiration pneumonia
 Defined as the
inhalation of either
oropharyngeal or
gastric contents into
the lower airways
 Due to poor swallow
weakness or gag reflex
 Reducing the risk
 Elevate the bed
 Peg tube
 Avoid eating 1 to 2 hours
before bedtime
 Saliva control
 Oral hygiene
Complications
 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)
 Slurred speech, quiet
voice
 Changes in vocal quality
 Requires coordinated
movement of several
muscle groups
 Speech pathologist to
review and advise
 Affects:




Vulnerability
Isolation
Inability to express needs
Exclusion from decision
making
 Loss of independence and
social role
 Loss of self identity
 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
Fatigue
 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
Swelling
 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
the bladder
 Hand weakness or
mobility limitations
 Use of pads
 Uridomes
 Catheter
 Weak abdominal and
chest muscles
 Diet / hydration
 Privacy
 Require adequate fibre,
fluid
 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
public
 Explanation (part of the disease), reassurance
(not going mad)
 Medication in more severe situations
Cognitive Changes
 previously thought cognition was not
affected
 research indicates up to 75% may have
some frontal lobe dysfunction
 15% to 41% meet criteria for frontotemporal dementia (FTD)
Miller & others, 2009
Cognitive Changes
 Cognitive Impairment (CI): deficits in
attention, word generation, cognitive
flexibility
 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
Communication
• 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
 Daytime sleepiness
 Increased fatigue
 Morning headaches
 Quieter voice
 Fewer words per
breath
 Shallow, faster
breathing
 Reduced movement
of the rib cage or
abdominal muscles
 Excessive use of the
muscles in the upper
chest and neck
 Weakened cough and
sneeze
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
 Refer to a specialist
respiratory service for
regular assessment
 Avoid infections (people
with coughs/cold)
 Treat reversible causes of
dyspnoea
 Discuss NIPPV support
 Avoid crisis situations
 Improve ventilation – fans,
air flow, humidifier
 Adjust room temperature
 Reclined or fully upright
position
 Respiratory / breathing /
relaxations exercises
 Medications: lorazepam,
midazolam, morphine
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 noninvasive ventilation:
http://guidance.nice.org.uk/CG105
Advance Care Planning
 75% preferred early discussion of Advanced
Directives Oliver, International Symposium, 2007
 plwMND preferred that doctor initiates
discussion
 Communication issues
 Ventilation withdrawal issues
 Shown to change their preference for lifesustaining measures (e.g. ventilators) over a
six month period Silverstein et al., 2006 = periodically reevaluate AHD
 Cultural differences
Six Triggers for Initiating Discussion About
End of Life Issues
1.
2.
3.
The plwMND or the
family asks - or ‘opens
the door’ – for end of
life information and/or
interventions
Severe psychological
and/or social or spiritual
distress or suffering
Pain requiring high
dosages of analgesic
medications
4. Dysphagia requiring a
feeding tube
5. Dyspnoea or symptoms
of hypoventilation, a
forced vital capacity of
50% or less is present
6. Loss of function in two
body regions (bulbar,
arms or legs)
Promoting excellence in end of
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
breath
 Put increased pressure on weakened
muscles
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
dehydration
• Without peg
•Refusal
•Anatomical
considerations
•Respiratory status
• With peg
•Voluntary stopping
•Intolerance or other
complications
• Aspiration pneumonia
• Sepsis
• Pulmonary embolus
• Head injury/falls
• Suicide
• Co-morbidity
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
pain/breathlessness
 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
to when to cease
ventilation
 Education of what to
expect
 Comfort maintained
 Physician should be
present (established
relationship)
 Planned event; no haste
 Cultural or religious rituals
discussed and planned
 Location prepared
 Medications ready
 Subcutaneous route is
preferred
 Family and friends
present
End of Life Care
 Fear of choking (rarely
occurs) and
breathlessness
 Increasing immobility
 Discussions and
anticipation of the final
time
 Advance care planning,
directives and EPOA
 done ahead of time
 regular review
 Build up of carbondioxide will
anesthetise
 Step-process of
withdrawal of NIPPV
 Use of adequate
medications
 Support for the family
and friends
(bereavement)
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
(grief)
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, nonabandonment
 Family also have needs
 Respite
 Involvement in care
planning
 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
Conclusion
 Be aware of the unique challenges of caring
for a person living with MND
 Understand the disease and rapidly changing
need
 The disease is the problem, not the person
 Early contact, relationship building
 Ongoing, preemptive assessment and referral
 Well coordinated teamwork
•www.mndcare.net.au for information
on MND care, symptom management
and support for health professional
•MND Aware: online training modules