Motor Neurone Disease; Challenges and

ResourcesA Summary for Primary Care Teams in Western

Area

This resource is based on MNDA Scotland’s Paper: Motor Neurone Disease, A Problem Solving Approach. Available on http://www.mndscotland.org.uk/wp-content/uploads/2011/08/A-Problem-Solving-Approach-2012.pdf Adapted for local use by: Alison Dick MND Network Coordinator Helen McClean MNDA Northern Ireland Primary Palliative Care Team, WHSCT Foyle Hospice Medical Team

Neurology Lead: Dr Colette Donaghy. ColetteG.Donaghy@westerntrust.hscni.net

MND Network Coordinator: Ms Alison Dick. alison.dick@belfasttrust.hscni.net

MND Association: Ms Helen Mc Clean helen.mcclean@mndassociation.org

Others, inc:• SALT• Dietetics• Specialist Palliative Care Community team • Physiotherapy• Respiratory Team• OT• Social worker

Key Contacts and Resources

Progressive degeneration of motor neurones in the following areas:

Anterior horn cells - resulting in Lower Motor Neurone Lesions (LMN)

Cortico spinal tract cells - resulting in Upper Motor Neurone Lesions (UMN)

Cortico bulbar tracts – resulting in UMN lesions in areas controlling the oropharyngeal muscles

Motor Nuclei in brain stem - resulting in both Upper and Lower Motor Lesions.

MND: Some Anatomy

Four Main Patterns. Commonest is

1. Amyotrophic Lateral Sclerosis (65%)

• Begins in a limb, trunk or respiratory muscles.

• Both upper and lower motor neurones (UMN & LMN) may be involved

• Characterised by: muscle weakness, spasticity, hyperreflexia , emotional lability, fasciculations, weight loss.

• Usually progresses (80% of cases) to include progressive bulbar palsy symptoms.

• Average survival 2-5 years from onset of first symptoms.

How does it present?

2. Progressive Bulbar Palsy (PBP) A form of ALS Affects ~ 22% of all MND diagnoses.

UMN & LMN may be involved. Typified by dysarthria and dysphagia.

LMN damage: nasal speech, regurgitation of fluids via nose, tongue atrophy and fasciculation, pharyngeal weakness.

UMN damage: tongue spasticity, explosive dysarthria, emotional lability.

Slightly more women than men affected.

Average survival 6 months to 3 years from onset of symptoms

Less Common

3. Progressive Muscular Atrophy (PMA) Affects about 10% of all MND diagnoses.

Defined as a disease of purely LMN degeneration, however a significant number of cases develop sub-clinical and, eventually, overt UMN signs. Characterised by: muscle weakness, wasting, weight loss and fasciculation. Affects men 5 times more commonly than women.

Younger age of onset: average survival five years plus.

Less Common

4. Primary Lateral Sclerosis (PLS)Approximately 2% of all MND diagnoses.Only upper motor neurones damaged. Characterised by muscle weakness, stiffness of limbs and increased reflex response. Men are affected twice as often as women.Onset usually after 50 years of age:Survival similar to normal life span.

Less Common

Considerable overlap between 4 clinical syndromes outlined as disease progresses towards more generalised weakness.

Onset is insidious.Early symptoms may include: stumbling, foot drop, weakened grip, slurred speech, cramp, muscle wasting and/or tiredness.Rarely, presents with acute respiratory weakness.

How Does it present?

1.Riluzole (Rilutek) is the only drug available to treat MND. It was licensed in 1996 and NICE approval was obtained for the treatment of MND in 2001. Riluzole acts by modulating the pre-synaptic release of glutamate.

2.A baseline respiratory assessment and discussion of future support options should be undertaken as soon as possible after diagnosis, by specialist team.

Treatments Available

Mobility e.g. walking and balancing Physiotherapist Swallowing Speech and Language Therapist Weight loss, lack of appetite Dietician Communication Speech and Language Therapist Coughing Physiotherapist and Speech and Language Therapist Breathlessness at rest or lying flat, sleep disturbances, daytime sleepiness, morning

headaches, tired or lethargic or difficult to rouse. Respiratory consultant,

Respiratory physiotherapist, Specialist palliative care services Fear, anxiety, depression Psychologist/counsellor/palliative care services Uncontrolled pain Specialist palliative care services Drooling and/or thick viscous mucus Physiotherapist for advice on positioning or

District Nurse/GP for suction unit/cough assist, GP for medication Difficulties in activities of daily living e.g. washing, dressing, cooking and leisure

Occupational Therapist or Social Worker Support for individual and family Social Work, MNDA, MND Care Team, Local

Hospice Service, Northern Ireland Rare Disease Partnership , Faith communities, Carer networks

3. Multi Disciplinary, problem solving approach, e.g:

1.Register patient on supportive/palliative care register, with key contact person.

2.Provide relationship based continuity as far as possible to patient and/or carer. (Monitoring and assessment)

3.Information gathering (give current and anticipatory information; liase with specialist teams)

4. Coordinate and refer as needed

5. Symptom management plans: coordinate, develop, share.

Role of Primary Care post diagnosis

1.Patient identified as having MND/flagged on IT system

2.Carers identified and flagged.

3.Key contact people in primary and secondary care teams easily identifiable on IT system

4.OOH informed of diagnosis and key issues with frequent update

5.ACP/ ADRT decisions clearly communicated

6.DNACPR status identified and communicated to ambulance and OOH

7.End of life care preferences communicated as and when appropriate.

Communication; a Key Issue in Primary Care

1.Environment: Will home be suitable? Adaptations? (OT)

2.Changes in ability to speak/communicate/swallow? (SALT)

3.Respiratory Compromise: Ventilatory support? Home oxygen?

4.Loss of function (physiotherapy)

5.Emotional implications (psychology, support resources)

6.Care and respite needs? (Social work)

7. End of life (advance care planning, ADRT, DNACPR)

Aim to Think Ahead. Support patient and family over time to anticipate and adapt to major change, e.g.:

80% affectedCauses:• Weakness & wasting of: Tongue lips facial

muscles pharynx and larynx• Reduced palatal elevation, which can lead to

“hyper-nasal voice quality.”• Reduced breath support, which can lead to

reduced volume or “breathy” voice quality.Impact:Progressive difficulty with articulation, slurred speech and/or loss of volume. May lead to anarthria

Symptom Burden: Dysarthria

Role of SALT:Examine the patient’s range of movement in their lips, tongue and palate or will carry out an oromotor examination; Advise on communication strategies; Arrange for reassessment and provision of communication aids such as Lightwriters and other aids.

Role of OT :Seating, positioning, wrist and head supports; Switches and pointers; Mobile arm supports, tables to access communication aids; Environmental controls.

Role of Everyone:Take time and create a quiet relaxed atmosphere; Ascertain individual’s own preferred communication strategy and/or equipment used; Position - face to face, watch lips, eyes, gestures.

Always be aware of the impact of emotion and fatigue on ability to communicate

Dysarthria

Cause: Weakness and paralysis of the oropharyngeal muscles resulting from affected gIossopharyngeal, vagus, accessory and hypoglossal nerves.

Effect: The first sign of early dysphagia may be problems when swallowing liquids resulting in severe coughing. This may progress to include: Loss of ability to form lip seal, chew, propel food with the tongue and/or form a bolus; Poor or absent swallow reflex; Weak pharyngeal muscles resulting in uncoordinated swallow; Failure to close airway; Muscle spasm; Acid reflux.

Resulting in Drooling; Dehydration and weight loss; Aspiration and recurrent chest infection; Impaired respiratory function.

Symptom Burden: Dysphagia and Nutrition

1.Food textures (SALT)

2.Fortification (Dietetics)

3.Head positioning and support (Physio)

4.Timing and quantity of food

5.Enteral feeding (PEG; RIG): can be used in combination with oral feeding; patient’s wishes should be sought at an early stage; (risks higher when illness progressed; weight loss affects prognosis)

6.Drooling/constipation (Prescribing)

Dysphagia: Issues to consider

Thin, runny saliva:

Suction Tricyclic antidepressants such as: Amitriptyline

Hyoscine (butylbromide or hydrobromide) sublingually, S.C., 24 hour syringe pump; Transdermal patches

Glycopyrronium (Robinul) (usually given PO or S.C. as intermittent injections or via a syringe pump).

Botox injection

Attention to head support and general posture - consult physiotherapist or OT.

Symptom Burden: Saliva Management

Thick tenacious saliva:

Check fluid intake, re-hydrate, avoid mouth breathing. Oral hygiene - consult district nurse.

Proteases such as Papain or Bromelaine, from health food stores sprinkled on the tongue can help break down thick saliva.

Pineapple, papaya, apple or lemon juices or flavoured ice cubes can also help.

Nebulise: saline

Mucodyne (Carbocysteine)

Expectorants (e.g.)Robitussin

Symptom Burden: Saliva Management

Tends to be due to forced inactivity, reduced peristalsis, low fluid intake, reduced fibre intake and/or weakness of pelvic floor/abdominal muscles, and use of analgesics. Maintain hydration and fibre intake.

Treatment Regular aperients: Movicol sachet Lactulose Docusate Sennoside B Co-danthramer (where prognosis is limited). Use of suppository, enemas, manual evacuation may be necessary on occasions. Also remedies such as bulking agents, prunes, linseed etc. available over the counter. Note: Diarrhoea may be due to constipation with overflow.

Symptom Burden: Constipation

Major cause of anxiety.Always review swallowMay lead to:• Fear, anxiety, panic & disturbed sleep; • Fatigue and reduced functional ability; • Daytime somnolence; • Poor appetite; • Vivid Dreams; Breathless with or without exertion; • Breathless when lying (orthopnoea); • Hypoxia or hypercapnia and possible confusion; • Inability to expectorate lung secretions.• Increased CO2 levels resulting in headaches, particularly on waking.

Caution: O2 should be prescribed by specialist and used with caution (hypercapnia: use 28% O2 only until specialist assessment)

Symptom Burden: Dyspnoea and Choking

Consider:• Referral to a consultant in respiratory medicine early on, to explore

options before respiratory problems become evident. • Involve the family;• Assisted ventilation; • Consider annual flu vaccination.• Referral to the palliative care team;• Medication Use : Humidifier; Nebuliser; • Low dose beta blockers e.g. Metoprolol, Propranolol, to reduce

production of lung secretions and thick tenacious saliva, mucus plugs (contra-indicated in asthmatics);

• Carbocisteine – increase mucodyne to relieve symptoms – use with caution as patient’s cough is weak; Midazolam S.C. or buccal; Lorazepam; Diazepam;

• Liquid morphine; Morphine Sulphate modified release tablets e.g. MST, Zomorph; Diamorphine.

At each contact with the patient assess their breathing and ask about symptoms (as below).

Early referral to a respiratory specialist is vital if one or more of the following occur.

o Breathlessness on exertion or when lying flat; Shallow rapid breathing or use of accessory muscles on inspiration;

o Weak cough/sniff; o Weak voice; o Sleep disturbances; o Unrefreshing sleep; o Daytime sleepiness; o Headaches on wakening; o Poor appetite/easily full; o Very tired/irritable/confused/drowsy.

Respiratory Support

National Guidelines on non-invasive ventilation http://guidance.nice.org.uk/CG105

Benefits Improved quality of life through improved sleep, less troublesome

symptoms during the day; Increased survival; Increased appetite.

Disadvantages Risk of aspiration if control of oropharyngeal secretions is poor; Skin breakdown if mask is used for long periods; Inability to tolerate mask or use machine; Less effective as MND progresses; Increasing dependency over time, which may have inverse impact on

quality of life; Usually requires assistance of a carer and can increase the carer burden.

Non-invasive ventilation

o Affects round half of sufferers

o High index of suspicion. May lead to agitation or restlessness.

o Muscle spasm, neuropathic pain, joint pain, immobility, constipation or colic

o Often requires a combination of agents

o Re assess and adjust management often.

Symptom Burden: Pain

Significant issue for some but may be subtle.

Fronto temporal cognitive loss (loss of inhibition, rigidity, repetitive behaviour) affects 15% or more; changes may be mild.

Behavioural or mild personality change commonly reported.

Symptom Burden: Cognitive Change

Key issue Major and progressive losses Feelings of helplessness common Fear of choking causes great anxiety Watch for depression Anticipate carer exhaustion and advise re self

care.

Psychological and Family Support

Is someone helping the patient develop an advance care plan? Do you need to involve a specialist palliative care service? Have you discussed the situation with patient and family so they can

prepare for what is likely to happen? Have you continued to reassure the patient and family that, contrary

to popular belief, death by choking or fighting for breath is not the norm?

Have you arranged for appropriate medications for the home to prevent a crisis admission?

Is there adequate physical and emotional support for the family if the person wishes to die at home?

Have you informed the ambulance service of any relevant advance planning, e.g. DNACPR?

Have you informed your local primary care out-of-hours service?

End of Life Care; Key Issues

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