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Caring for People Living With Motor Neurone Disease
Dr Monika WilsonReConnections Counselling Servicewww.reconnectionscounselling.com
[email protected] 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
• 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
What are 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
Types of Motor Neurones
IncidenceIncidence: Approx 1 in 37,500 people diagnosed each year
PrevalencePrevalence: Approx 1400 in Australia / 350-400 in Qld ?
Each day more than one person dies from MND and another is diagnosed
DurationDuration: Average 2 to 3 years, but 10% live > 10 years
Most common age of onsetage of onset: 50-60s GenderGender: Men affected slightly more
frequently (2:3 ratio)
90% sporadic, 5%-10% familial
amyotrophic lateral sclerosisamyotrophic lateral sclerosis (ALS) 65% UMNs and LMNs
progressive bulbar palsyprogressive bulbar palsy (PBP) 25% LMNs
progressive muscular atrophyprogressive muscular atrophy (PMA) <10% LMNs
primary lateral sclerosisprimary lateral sclerosis (PLS) rare UMNs
Muscles controlling bladder and bowels
not directly affected Hearing, taste, smell and sensation
sensory nerves Heart
autonomic nerves
Not Affected
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
Diagnosis
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: 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
Multidisciplinary CareFact Sheets EB2/EB3
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
RiluzoleFact Sheet EB4
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
Non-Invasive VentilationFact Sheet EB7
Permanent feeding tube into the stomach
Improved nutrition and QoL Early decision required
http://www.mndaust.asn.au/Information>National Information
GastrostomyFact Sheet EB8
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
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
Common Symptoms
•Often begins with foot drop•Difficulty climbing stairs•Difficulty arising from chairs•Possibility of falls •Eventually leading to hoist transfer•Fasciculation and cramps
Muscles: Lower Limb Weakness
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
Care Strategies
Hand weakness difficulty with fine motor tasks using
hands Shoulder girdle weakness
difficulty using arms Neck weakness
Muscles: Upper Limb Weakness
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
Care Strategies
Repositioning Subtle adjustments (small moves) Satin sheets, kylies, bed stick Support – cushions Care for weakened limbs
Maintaining Comfort
Discomfort & Pain
1. Musculoskeletal pain
2. Cramp/spasm pain3. Skin pressure pain
Physiotherapy and passive movement
Massage Hydrotherapy Use of splints and
cushions Medications (initially
non-narcotic analgesics, anti-inflammatory and anti-spasticity agents)
Opioids
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
Pain Management
Need an effective way of calling for assistance
Door chimes Jelly bean switches Baby monitors Intercom systems
Location of best position Minimise anxiety
Creative Thinking
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)
•Drooling•Choking on thin liquids•Slurring of speech •Quiet voice •Loss of speech •Difficulty chewing and swallowing•Weight loss
Muscles: Bulbar Weakness
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.
Signs and symptoms of weakness in the muscles involved in chewing and
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
Muscles: Swallowing
We produce approx 600 ml each dayHandling of saliva is affected due to:
Weakness of the tongue Weakness of throat muscles Anatomical structure (poor lip seal) Poor head control
Sialorrhoea: Saliva beyond the margin of the lip (drooling)
Social participation Withdrawal, embarrassment
Emotional wellbeing Loss of independence and self esteem
Physical function Speech Swallowing Oral health ie infection, odour Dehydration
The Impact of Drooling
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
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)
Complications
• Speech pathologist / dietician to assess
• Increasing dysphagia• Modified diet – pureed food, thickened
fluids, nutritional supplements, gravies• Positioning, use of equipment
Nutrition: Eating well
• 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
Why Consider PEG?
* Latest research evidence suggests an
improvement in nutrition and QOL only
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
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
Care Strategies
Low tech aids: Writing Magna doddle / white boards Laser pointer and chart Etran boards
High tech aids: Lightwriter / Polyanna / Alora VMax Essence Vantage Light
Communication Aids
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
Fatigue
Due to lack of movement Legs elevated with cushion support Use of massage Elastic stockings Be aware of deep vein thrombosis
Swelling
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
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
Emotional Lability – pseudo bulbar effect
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
Cognitive Changes
Cognitive Impairment (CI):Cognitive Impairment (CI): deficits in attention, word generation, cognitive flexibility
Behavioural Impairment (BI):Behavioural Impairment (BI): changes in social interaction
Fronto-temporal Dementia (FTD):Fronto-temporal Dementia (FTD): altered social conduct, emotional blunting, loss of insight, language change, poor self care, emotional recognition, lack of empathy
Cognitive Changes
• Changes in decision-making • Reduced awareness of risk, concerns
about risk taking• Frustration; forgetfulness• Communication• Obsessional behaviour; impulsiveness• Lack of self care
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 discussionAssessing symptoms
• Pain / depression / swallowing problemsCoping with memory loss / confusion
Issues for Professionals
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.
Care Strategies
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
Breathlessness (dyspnoea) even at rest
Breathlessness lying flat (orthopnoea)
Impaired concentration or confusion Irritability and anxietyDecreased appetite
Respiratory muscle weakness can cause
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
• 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
Non-Invasive Positive Pressure Ventilation
Decreased daytime sleepiness Better appetite Rests fatigued respiratory muscles Improved sleep Quality of life More energy Improved defence against infections
Benefits of Assisted Ventilation
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
Implications to Consider
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
Advance Care Planning
Six Triggers for Initiating Discussion About End of Life Issues
1. The plwMND or the family asks - or ‘opens the door’ – for end of life information and/or interventions
2. Severe psychological and/or social or spiritual distress or suffering
3. 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
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
Use of Oxygen
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 occursNeurvert, C, Oliver D Journal of Neurology 2001: 248
Common Cause of Death
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
Increased, progressive weaknessDeterioration over a few daysOften proceeded by
Reduction in chest expansionQuietening of the breath soundsAccessory muscles for breathingMorning headaches
Terminal Phase is recognised by
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
Medical Management during Terminal Phase
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 carbon-dioxide will anesthetise
Step-process of withdrawal of NIPPV
Use of adequate medications
Support for the family and friends (bereavement)
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
Features of Optimal End of Life Care
oA spiralling series of progressive losses (grief)
oChanges in ability to influence their external and internal environment (control)
oChanged relationship with body/self/identityoAware of what is happening, what will come
and increasing dependencyoCarer burnout, relationship issuesoMany psychological, emotional, sexual,
financial, spiritual adjustments to be made
Psychological, Emotional & Social Issues
Create relationships: open and honest, ongoing communication, inclusion, non-abandonment
Family also have needs Respite Involvement in care
planning Discuss fears and concerns
Family & Friends
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
Health Care Professionals
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
Conclusion
•www.mndcare.net.au for information on MND care, symptom management and support for health professional
•MND Aware: online training modules