Caring for People Living With Motor Neurone Disease

Dr Monika WilsonReConnections Counselling Servicewww.reconnectionscounselling.com

reconnections@monikawilson.com.au0428 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 affectedHearing, taste, smell and sensation

sensory nervesHeart

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