Title: Caring for People Living With Motor Neurone Disease
1Caring for People Living With Motor Neurone
Disease
- Dr Monika Wilson
- ReConnections Counselling Service
- www.reconnectionscounselling.com
- reconnections_at_monikawilson.com.au
- 0428 777809 / 5457 3329
2- 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
3- 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
4 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
5Types 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
6- 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 gt 10
years - Most common age of onset 50-60s
- Gender Men affected slightly more frequently
- (23 ratio)
7 - 90 sporadic, 5-10 familial
- amyotrophic lateral sclerosis (ALS)
- 65 UMNs and LMNs
- progressive bulbar palsy (PBP)
- 25 LMNs
- progressive muscular atrophy (PMA)
- lt10 LMNs
- primary lateral sclerosis (PLS)
- rare UMNs
8 Not Affected
- Muscles controlling bladder and bowels
- not directly affected
- Hearing, taste, smell and sensation
- sensory nerves
- Heart
- autonomic nerves
9Diagnosis
- 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
10- 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
11Multidisciplinary CareFact 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
12RiluzoleFact 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
13Non-Invasive VentilationFact 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
14GastrostomyFact Sheet EB8
- Permanent feeding tube into the stomach
- Improved nutrition and QoL
- Early decision required
- http//www.mndaust.asn.au/
- InformationgtNational Information
15Goals 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
16Common 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
17Muscles 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
18Care 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
19Muscles Upper Limb Weakness
- Hand weakness
- difficulty with fine motor tasks using hands
- Shoulder girdle weakness
- difficulty using arms
- Neck weakness
20Care 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
21Maintaining Comfort
- Repositioning
- Subtle adjustments (small moves)
- Satin sheets, kylies, bed stick
- Support cushions
- Care for weakened limbs
22Discomfort Pain
- Physiotherapy and passive movement
- Massage
- Hydrotherapy
- Use of splints and cushions
- Medications (initially non-narcotic analgesics,
anti-inflammatory and anti-spasticity agents) - Opioids
- Musculoskeletal pain
- Cramp/spasm pain
- Skin pressure pain
23Pain 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
24Creative Thinking
- Need an effective way of calling for assistance
- Door chimes
- Jelly bean switches
- Baby monitors
- Intercom systems
- Location of best position
- Minimise anxiety
-
25- 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)
26Muscles Bulbar Weakness
- Drooling
- Choking on thin liquids
- Slurring of speech
- Quiet voice
- Loss of speech
- Difficulty chewing and swallowing
- Weight loss
27Signs 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.
28Muscles 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
29Sialorrhoea 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
30The 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
31Saliva 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
32Saliva 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
33Complications
- 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
34 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)
35Nutrition Eating well
- Speech pathologist / dietician to assess
- Increasing dysphagia
- Modified diet pureed food, thickened fluids,
nutritional supplements, gravies - Positioning, use of equipment
36Why 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
37Placement 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)
- Latest research evidence suggests an
- improvement in nutrition and QOL only
38Muscles 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
39Care 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
40Communication 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
41Fatigue
- 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
42Swelling
- Due to lack of movement
- Legs elevated with cushion support
- Use of massage
- Elastic stockings
- Be aware of deep vein thrombosis
43Bladder 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
44Emotional 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
45Cognitive Changes
- previously thought cognition was not affected
- research indicates up to 75 may have some
frontal lobe dysfunction - 15 to 41 meet criteria for fronto-temporal
dementia (FTD) - Miller others, 2009
46Cognitive 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
47- Changes in decision-making
- Reduced awareness of risk, concerns about risk
taking - Frustration forgetfulness
- Communication
- Obsessional behaviour impulsiveness
- Lack of self care
48Issues 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
49Care 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.
50Muscles 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
51Respiratory muscle weakness can cause
- Breathlessness (dyspnoea) even at rest
- Breathlessness lying flat (orthopnoea)
- Impaired concentration or confusion
- Irritability and anxiety
- Decreased appetite
52 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
53Non-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
54Benefits of Assisted Ventilation
- Decreased daytime sleepiness
- Better appetite
- Rests fatigued respiratory muscles
- Improved sleep
- Quality of life
- More energy
- Improved defence against infections
55Implications 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 http//guidance.nice.org
.uk/CG105
56Advance 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
life-sustaining measures (e.g. ventilators) over
a six month period Silverstein et al., 2006
periodically re-evaluate AHD - Cultural differences
57Six Triggers for Initiating Discussion About End
of Life Issues
- 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
- Dysphagia requiring a feeding tube
- Dyspnoea or symptoms of hypoventilation, a
forced vital capacity of 50 or less is present - Loss of function in two body regions (bulbar,
arms or legs) - Promoting excellence in end of life ALS care,
2004
58Use of Oxygen
- Breathlessness is due to muscle weakness not low
oxygen - If oxygen is given inappropriately it can
- Increase carbon dioxide retention
- Reduce the bodys spontaneous signals to breath
- Put increased pressure on weakened muscles
59Common 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
60 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
61Terminal 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
62Medical 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
63Withdrawal 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
64End of Life Care
- Build up of carbon-dioxide will anesthetise
- Step-process of withdrawal of NIPPV
- Use of adequate medications
- Support for the family and friends (bereavement)
- 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
65Features 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
66Psychological, Emotional Social Issues
- A spiralling series of progressive losses (grief)
- Changes in ability to influence their external
and internal environment (control) - Changed relationship with body/self/identity
- Aware of what is happening, what will come and
increasing dependency - Carer burnout, relationship issues
- Many psychological, emotional, sexual, financial,
spiritual adjustments to be made
67Family Friends
- Create relationships open and honest, ongoing
communication, inclusion, non-abandonment - Family also have needs
- Respite
- Involvement in care
- planning
- Discuss fears and concerns
68Health 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
69Conclusion
- 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
70- www.mndcare.net.au for information on MND care,
symptom management and support for health
professional - MND Aware online training modules