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208 Chapter 14

The careful assessment of pain is essential, and later in

the disease progression specific scales may be necessary.

The DisDaT scale is used if the patient has limited com-

munication or severe cognitive change [23]. It compares

the cues that the patient is content and the cues of distress,

as described by close carers, so that the presence of distress

can be identified and then assessment undertaken of pos-

sible causes of pain, followed by appropriate reassessment

to ascertain if the distress has lessened [23]. The PAINAD

scale allows carers to assess various aspects of the patient

[24]. It is important to involve carers in these assessments

and where possible to differentiate pain from non-pain

distress, which may be due to many different causes. Close

assessment, examination, and re-evaluation are necessary.

See Chapter 10: Pain and Its Management for more detail.

Common causes of pain are:

Musculoskeletal Pain

This may occur due to altered muscle tone around joints –

particularly the shoulder and spine – or to restricted

movement. Management includes:

•

Careful positioning

•

Physiotherapy, including passive movements, to main-

tain joint mobility, splints, and support aids

• Non-pharmacological strategies such as heat pads,

massage, and acupuncture

• Non-steroidal anti-inflammatory drugs – although the

risks of gastrointestinal, renal, and cardiovascular toxicity

may limit their use in some patients

•

Intra-articular injections of steroid and local anaesthetic

may be helpful.

Muscle Cramps

If muscles are spastic – due to upper motor neurone

damage in MND or MS – cramp and spasm may be trou-

blesome. Careful positioning and physiotherapy are help-

ful and muscle relaxant drugs may be tried (see Box 2).

All these drugs may cause drowsiness and may reduce

mobility (as lower limb spasticity may allow some mobility

and if this is reduced walking may be more difficult).

Symptom Management

As there are no curative treatments for these progressive

neurological diseases the primary aims of management

should be quality of life, longevity for some, symptom

management, and addressing other issues – physical,

psychological, social, and spiritual. As outlined in Chapter 1,

good symptom management requires:

• Impeccable assessment of the problem (to identify,

where possible, causes and mechanisms and to inform

prioritisation)

Careful history of the symptom

Careful physical examination

Relevant investigation

• Discussion with the patient, and those close to them,

about possible options – including the advantages and dis-

advantages of treatments that might be offered.

Pain

Pain is common in people with progressive neurological

diseases and may be due to the disease process itself, or be

caused by the changes imposed by the disease, such as

immobility. It is important to be aware that there may be

coincidental pains, from other causes that may be wors-

ened by the progression of neurological dysfunction, such

as immobility increasing arthritic pain.

Pain is common in all neurological disease groups

(Box 1):

Box 1: Prevalence of Pain in people with

neurological disorders

Motor neurone disease 73% [17]

Parkinson’s disease 85% [18]

Multiple sclerosis 77% [19]

Multiple systems atrophy 73% [20]

MSA C 41%

MSA P 63%

Corticobasal syndrome 25% [20]

Progressive supranuclear palsy 52% [20]

Huntingdon’s disease 41% [21]

Duchenne muscular dystrophy 69% [22]

Box 2: Treatment of muscle cramps

Baclofen 5 mg–20 mg TDS/QDS

Tizanidine 2 mg–24 mg/day

Dantrolene 25 mg–100 mg QDS

Diazepam 5 mg nocte increasing slowly

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Palliative Care for People with Progressive Neurological Disorders 209

Management

•

Infection – appropriate antibiotics and physiotherapy. If

there is evidence of aspiration a full assessment of swallow-

ing and consideration of alternative feeding methods

should be considered, including gastrostomy.

• Respiratory muscle weakness – careful positioning: if

the diaphragm is affected, patients may find lying flat

distressing and raising the head of the bed may be help-

ful. The NICE Guideline on MND suggests early

discussion of the issues when monitoring of ventilatory

capacity is started, so that discussions are not rushed in

an emergency situation when there is increasing

respiratory failure [3]. Respiratory support, using non-

invasive ventilation (NIV), may be appropriate in MND

and DMD and has been shown to reduce symptoms, at

least initially, and to improve quality of life in MND [26].

The discussion of the risks and benefits may be complex.

Many people find the mask difficult to tolerate, and

dependence and increasing disability are likely. It should

be made clear that the NIV does not alter the progression

of the disease. Life may be prolonged but with increasing

disability, and although the patient’s quality of life may

be maintained this continued treatment during disease

progression may cause distress and strain on the family.

Discussion of future steps (see Box 4) should therefore

be encouraged before starting NIV and in ongoing care

[27, 28] as part of the wider consideration of advance

care planning. This may at any stage lead to an Advance

Decision to Refuse Treatment (ADRT) (see Chapter 9),

perhaps covering circumstances in which the person

would want the NIV to be withdrawn, or to a statement

of wishes and values that will serve to inform future

decisions if cognitive or communication failure or pro-

gressive fatigue compromise their ability to participate in

decision-making.

Botulinum toxin injections can be helpful if spasticity is

localised to one muscle group.

Tissue Pressure Pain

If a patient is immobile and remains in one position for

some time they may experience discomfort and the

insidious onset of pain developing slowly over time.

Good positioning and regular help in movement is

essential but analgesics are often also required. The

WHO analgesic ladder, developed for cancer pain, is a

good guide to their use in neurological disease – see

Chapter 10. Opioids are often helpful and experience,

particularly in MND, has shown that they can be used

effectively and safely [25].

Neuropathic Pain

Some neurological disorders affect the sensory nervous

system causing a number of pain syndromes, including

allodynia and hyperalgesia. For instance, in MS parox-

ysmal pains, similar to trigeminal neuralgia, are common

and may require treatment with anticonvulsant drugs used

as analgesic adjuvants (see Chapter 10 (Pain and Its

Management) for more information).

Breathlessness and Respiratory Issues

Patients with neurological disease may develop problems

with respiration for several reasons see Box 3.

Box 3: Causes of breathlessness

• Swallowing problems – may lead to aspiration pneumonia.

• Posture – a person who is wheelchair or bed-bound may

have restricted chest movement and hypoventilation and

develop stasis pneumonia.

• Weakness of respiratory muscles – the diaphragm and

intercostal muscles, particularly in MND and Duchenne

muscular dystrophy (DMD). This may cause breathlessness

but may present as respiratory failure without much breath-

lessness, with symptoms of orthopnoea, poor sleep due to

arousals caused by hypoxia, dreams, morning headache due

to carbon dioxide retention, non-refreshing sleep, anorexia

and feeling unwell or other sleep issues, such as nocturia as

an arousal at night may be misconstrued as the need to go

to pass urine.

• Coexistent cardiac or lung pathology. Common co-mor-

bidities may be present such as chronic obstructive pulmo-

nary disease and cardiac failure. Additionally, patients with

DMD have an associated cardiomyopathy, and are at risk of

arrhythmias and muscle weakness if given diuretics which

reduce potassium levels.

Box 4: Areas to cover when considering

starting non-invasive ventilation

• Continued disease progression in spite of NIV and that

while it may prolong life it will not prevent death

• Likelihood of increasing ventilator dependence in the future

• Ceilings of care and escalation to tracheostomy ventilation,

if it is available or if it is raised by patient or family

• Consideration of future withdrawal of ventilation if it is no

longer wanted, no longer tolerated, or no longer working,

or if it becomes incompatible with other goals.

These may be hard topics to raise and require good commu-

nication skills, but patients and families often find it reassur-

ing and helps them plan

https://t.me/med1917

210 Chapter 14

considered. Sleep apnoea may also be seen in this patient

group, and again CPAP may be helpful.

Nutrition and Dysphagia

Swallowing difficulties are very common. There may be

involvement of muscles in the chewing, handling of the

bolus of food in the mouth, moving the bolus to the

pharynx or in swallowing itself. Other problems may con-

tribute to nutritional failure, such as upper limb weakness

and respiratory failure, and assessment by the occupational

therapist may help to overcome these problems. Careful

assessment by a speech and language therapist and dieti-

tian is essential and videofluoroscopic examination of

swallowing may be helpful.

Management

Other causes of dysphagia, such as oral and oesophageal

candidiasis, should be excluded and treated appropriately.

Following assessment, dietary advice from the speech and

language therapist and dietitian is helpful – often a cus-

tard-consistency is easiest to swallow and high calorific

value foods should be encouraged. As dysphagia worsens,

education of carers – both family and professional – is

helpful to ensure food with the best consistency for the

person is presented in the most appropriate way.

If there is increasing difficulty, weight loss, and

significant risk of aspiration, alternative methods of nutri-

tion may be considered. Nasogastric feeding is rarely used,

except for short-term use in a crisis situation. Percutaneous

endoscopic gastrostomy (PEG) or percutaneous radiolog-

ical gastrostomy (PRG) or per-oral image guided gastros-

tomy (PIG) may be considered – see Chapter 11. This can

be a very difficult decision for patients and their families

and should be discussed early in the disease progression,

rather than in an emergency situation. Moreover, the prac-

ticalities of a gastrostomy may be more pronounced as the

disease progresses, due to immobility or, in MND,

respiratory muscle weakness, making the procedure more

hazardous. A period of discussion, over several weeks, may

allow the patient to discuss the issues and it should always

be stressed that the gastrostomy does not, of itself, preclude

oral feeding, which may continue, at risk, allowing the

taste and pleasure of eating.

Early discussion about the possible cessation of feeding

at the end-of-life, or if it is no longer tolerated, is important.

Discussion about treatment choices and burdens and bene-

fits should begin before a gastrostomy is formed and

feeding started. Continued feeding close to the end-of-life

can lead to an increased risk of reflux and aspiration.

Some patients may have invasive ventilation (IV), with a

tracheostomy. People with MND may receive a tracheos-

tomy when they present as an emergency with respiratory

failure, before the diagnosis has been made. Some people

may request elective IV, although this is rare in the UK,

whereas internationally the prevalence of IV is higher – in

Japan it is up to 38% [29]. Similar discussions should be

undertaken, particularly as IV leads to increased burden

and distress for the caregivers [30]. Withdrawal should

also be discussed.

Discussions about cessation of ventilatory support are

complex and the process of withdrawal is difficult for all

concerned. Although the withdrawal of a treatment that a

person no longer wishes, at their direct request or in a clear

advance care plan, is both ethical and legal, the procedure

may feel like assisting dying [31, 32]. Moreover, to prevent

distress at withdrawal, medication may be necessary

beforehand, or available at the time of withdrawal, to

ensure that the person does not become distressed. The

professionals involved may need support and there are

guidelines available [33] and the need for support of

everyone is important [31, 32].

•

Medication is helpful in alleviating the subjective expe-

rience and possible distress of breathlessness:

Benzodiazepines, such as Diazepam 2–5 mg TDS or sublin-

gual Lorazepam 500 micrograms to 1 mg for acute situations

Opioids, such as regular Morphine elixir 2.5–5 mg up to

every 4 hours

Careful titration of medication is necessary to ensure the

symptoms are effectively managed, without over-seda-

tion. Depending on the pattern of symptoms and the

response, administration may be either regularly or as

required.

•

Cough may be troublesome for patients with respiratory

muscle weakness, or where posture reduces the ability to

produce a good cough. Careful physiotherapy assessment

will help in ensuring the cough is as effective as possible.

The use of breath stacking or mechanical cough assist

devices may be considered [3].

Mucolytics (such as Carbocisteine 500mg TDS) and

nebulised saline may be helpful if the secretions are thick.

Small doses of opioids, such as morphine elixir 2.5–5 mg

every 4 hours, may be considered. Anticholinergic drugs

used to reduce the volume of secretions (see below) may

make them more tenacious and harder to clear.

•

Inspiratory stridor develops in about 30% of people

with MSA and can be distressing. Continuous positive air-

ways pressure (CPAP) can be helpful. Quick acting benzo-

diazepines, such as sublingual Lorazepam, can be helpful

in reliving the distress. Tracheostomy may also be

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Palliative Care for People with Progressive Neurological Disorders 211

The action of some anticholinergics drugs (including

tricyclics) is on the watery component. For some patients

the reduced volume comes at the expense of dry mouth or

thick sticky secretions that are difficult to remove. This

balance should be considered before starting, with

discussion with patients and families, careful titration of

the dose, and early review and discontinuation if the med-

ication is not tolerated (Box 5).

Constipation

Constipation is a common problem for people with neuro-

logical conditions due to many causes: immobility, poor

oral intake, medication such as opioids or anticholinergics,

damage to the nerve supply to the bowel and the reduced

abdominal muscular power needed to strain. There may

also be psychosocial aspects – such as embarrassment in

asking for the toilet or immobility making the transfer dif-

ficult (Box 6).

However, the gastrostomy may allow hydration and the

continuation of medication which may, in turn, allow a

family to care for the person at home. As with ventilatory

support an ADRT may be considered, specifying future cir-

cumstances under which continued feeding would be

refused – see Chapter 9.

Drooling

Drooling of saliva may occur if there is reduced swallowing

and weakness of facial muscles, causing poor lip control,

and neck weakness leading to the head falling forwards.

Drooling is usually due to reduced swallowing of the

normal production of saliva, rather than excess produc-

tion. Patients and their carers, family and professional,

may be distressed by this and it can greatly affect commu-

nication lead to isolation, due to embarrassment. It is a

common symptom – affecting 40% of MND and 65% of

PD patients [17, 34].

Box 5: Management of drooling

Careful attention to posture and explanation to the patient

and family is essential and may be sufficient. Medication may

be necessary:

• Good mouth care, ensuring the mouth and teeth are kept

clean

• Anticholinergic medications reduce saliva production:

Glycopyrronium bromide – can be given via a PEG as

200 micrograms TDS or by subcutaneous infusion using

a syringe driver, up to 1200 micrograms/24 hours

Hyoscine Hydrobromide 300 micrograms sublingually

TDS

Hyoscine Hydrobromide patches 1mg patch changed

every 72 hours

• Tricyclic antidepressants, such as amitriptyline, will have

the side effect of causing a dry mouth and can be helpful

particularly if there is also a depressive illness.

• Botulinum toxin injections into the salivary glands has

been shown to be very effective and may reduce symptoms

for several weeks or months.

• Local radiotherapy to the salivary glands can be considered,

but this is irreversible and the ensuing dryness can be dis-

tressing to some people.

• For some patients the saliva can become very sticky and

difficult to clear – pineapple chunks, papaya tablets, Car-

bocisteine, nebulised saline, or botulinum toxin injections

can be helpful.

Box 6: Management of constipation

This should address the particular cause of constipation if

possible:

• Increased roughage within the diet, including considera-

tion of supplement feeds

• Ensuring hydration and extra fluids – particularly in pa-

tients having enteral feeding by gastrostomy

• Encouraging and assisting mobility, and ensuring privacy

• Reduction of constipating medications if possible

• Oral aperients may be necessary

A combination of a stimulant laxative and softener/

osmotic laxative is helpful, particularly if constipat-

ing medications such as opioids cannot be withdrawn

Macrogols (Polyethyelene glycol) 1–3 sachets

daily

Senna 5-10 ml or 1–2 tablets BD

• Rectal measures may be necessary if sphincter control is

difficult

Regular suppositories or enemas may be helpful for

some patients

Insomnia

There are many causes of poor sleep – pain/discomfort,

anxiety, fear of not being able to attract attention due to

immobility, cough, orthopnoea/respiratory failure,

cramps, fear of incontinence, restless legs, and confusion.

https://t.me/med1917

212 Chapter 14

The speech and language therapist can assess and provide

advice on enhancement of communication. The provision

of communication aids can be helpful, from “low tech”

paper and pencils to computer systems and iPads, which can

include the ability to speak the words the person has typed

or entered into the system, to high tech “eye gaze” commu-

nication aids, when eye movements may be used to operate

a computer screen and allows communication even in the

most severely disabled person. The voice generated by the

communication aid can be similar to the person’s own voice,

if they have undertaken voice banking - before speech is lost

someone can record phrases and this can be used within a

computer system to generate a voice, similar to their own. It

is important to assess the cognitive abilities when consid-

ering communication aids, as if there is cognitive change it

may be very difficult to use any aid effectively.

It is extremely important to enable patients to communi-

cate their wishes and fears while speech is present, although

this may require encouragement as these are difficult issues

to discuss. This makes it easier to continue these discussions

later in the disease progression when communication aids

are needed. It is important to ensure that all involved in the

person’s care understand the importance of communica-

tion, as most very disabled patients may remain able to

express themselves and communicate with the right support.

Restlessness and Agitation

The causes of restlessness and agitation require careful

assessment as it could be due to infection, medication, hyp-

oxia, urinary retention, or constipation, as well as part of

the disease progression – for instance in PD [34, 35]. There

is often more than one cause. Ensuring the environment is

quiet and relaxed is important, as well as exclusion and

treatment of reversible causes. In PD, psychotic episodes are

common and up to 40% of patients may experience halluci-

nations [34]. The cause may be related to medication given

for PD, and titration of the doses is essential. Clozapine and

Quetiapine are widely used in PD for treatment of halluci-

nations. Risperidone or Olanzapine, often in combination

with benzodiazepines, may be helpful. Any of these medica-

tions should be used with care in PD and related disease as

it can cause increased stiffness and distress.

Cognitive Change

Cognitive change is now recognised in many progressive

neurological diseases. Up to 40% of people with MND have

evidence of frontal lobe dysfunction and 10–15% have

fronto-temporal dementia [36]. In PD about 30-40% of

patients develop dementia [35]. Dementia is rarer in MSA

but slight to moderate cognitive impairment may be seen in

These will vary with disease processes and in Parkinson’s

disease sleep disorders are very common, often with hallu-

cinations and confusion and may be related to medication

and the development of “off periods” [35, 36].

The management of poor sleep should be directed at the

underlying cause and may include simple measures, such

as a call button or alternative accessible system to allow the

patient to call for attention, or medication changes in PD.

Opioids can be helpful for immobile patients who develop

pain which affects sleep.

Weakness and Mobility

Patients with progressive neurological disease will face

many losses, particularly with mobility. Careful assessment

by a physiotherapist and occupational therapist, with

specialised knowledge of neurological disease, is essential.

The provision of equipment to aid daily living requires

sensitive communication and should be a gradual process of

discussion, rather than the sudden provision of equipment

that the person is not prepared to use and may not accept.

Careful explanation is necessary together with forward

planning, as equipment needs will change as the disease

progresses. Any aids should be adaptable to cope with these

anticipated changes and to be able to incorporate the use of

other equipment, such as NIV or communication aids [3].

Postural hypotension can be very disabling in MSA,

when any movement causes dizziness, syncope, nausea,

tremulousness, headache, and “coat hanger pain” (pain

across the neck and shoulders). Midodrine may be helpful.

Speech and Communication

Speech may be affected by muscle weakness of the mouth

and face, leading to slurring, as well as by respiratory

weakness, leading to a very quiet voice. However, commu-

nication may also be affected by changes in mood, arm

mobility and cognitive change and assessment of the

issues may be complex. Careful listening to the person is

important and allowing them to complete what they want

to say – whether this is by speech or by a communication

aid. Assisted communication is sometimes slower than

speech, but resist the temptation to talk across the person

while they are formulating responses. Treat their non-

speech communication as you would treat someone’s

speech and give it your full focus as it unfolds. Some peo-

ple find it very tiring to complete every word and sentence

and find it helpful if you can anticipate and suggest what

they want to say. Others, even if it is tiring, find this

off-putting and object as strongly to sentences being com-

pleted for them as most speaking people do. Ask people’s

preferences, and follow them.

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Palliative Care for People with Progressive Neurological Disorders 213

Depression and Anxiety

Any person with a progressive neurological disease, know-

ing that the progression will continue and death ensue may

have a low mood as part of their adjustment to their

situation. Many will develop a depressive illness, estimated

as 20-30% in MND and 40% in PD. It may be difficult to

make the diagnosis when the patient has severe communi-

cation issues and possible cognitive change [35, 36].

Listening to the patient and family and providing

counselling support is important. Antidepressant medica-

tion may be useful:

•

Amitriptyline may be considered, particularly if there is

drooling, pain, or poor sleep – at doses of 25—150 mg at

night.

•

Citalopram 10–20 mg OD has fewer side effects and

may be helpful for a patient with emotional lability (in

MND) or safer if there is coexistent cardiac disease (for

instance in DMD).

As the disease progresses anxiety is common, as fears

about extreme debility and dying develop. Listening,

information sharing, and correction of misperceptions

together with acknowledgement and appropriate reassur-

ances and action plans are vital. Behavioural or cognitive

therapies may be helpful and benzodiazepines, such as

Temazepam at night or Diazepam, 5–10 mg at night, may

be used.

Carer Support

The demands on families and close carers of people with

progressive neurological disease can be profound – physi-

cally, psychologically, financially, and emotionally. There

are often multiple losses faced by both the patient and

family with great changes of roles within family units. This

is particularly seen when speech and communication are

difficult and/or cognitive changes occur, adding increased

stresses of all concerned. They will face the similar issues

described above – of uncertainty, barriers to communica-

tion and personhood, inconsistent information, and

existential distress [39]. Families and caregivers may need

much support and explanation, in their own rights, as well

as enabling them to provide care to the patient.

There may be specific issues for some families, when

there is a clear genetic cause, such as in Huntingdon’s dis-

ease (HD) or in familial motor neurone disease (MND)

with a known mutation. It may be possible for family

members to have predictive testing which will enable them

to know if they are at risk of developing the disease later in

life. This predictive testing should only take place with

40% [37]. In PSP over 80% will develop dementia and many

will show cognitive change earlier [38]. In MS 40–50% show

cognitive change [39]. The changes may be subtle at first

and may not be recognised, although changes in personality

may have been noticed by the family and close carers.

Careful assessment is essential, as mild changes may not

be recognised. However, there may be effects on decision-

making and personality, which may affect care and planning

ahead. As there is likely to be progression, with the possible

reduction in the capacity in decision-making, advance care

planning may need to be discussed early in the disease pro-

gression, and sometimes soon after diagnosis. It may be

very difficult for patients and their families to cope with

looking at a difficult future but if they wish to have influence

on their care and decisions about their future care they

need to be supported in facing these issues (see Chapter 9).

Psychological, Social and Spiritual

Distress

People with progressive neurological disease may experi-

ence many emotional and psychological issues as they

deteriorate (see Box 7).

Box 7: Psychological and spiritual issues

• Fears about the disease – for instance, much is written

about the “distressing nature” of MND and other neurologi-

cal disease. For others social stigma may be an issue. Good

information may be helpful, for instance that produced by

some voluntary organisations with a disease-specific focus,

and patient and carer forums can be invaluable to some.

• Fears about the future and deterioration.

• Coping with the uncertainty and threat to themselves as

people – with possible loss of speech and cognition [40].

Careful listening and explanation by the MDT may be able

to help patients cope with these issues.

• Inconsistent information – this may come from various

teams and many different professionals and it is important

to aim to keep clear communication, often through one

main MDT which undertakes monitoring and assessment,

and through having common approaches to decisions that

are shared across networks of services.

• Existential distress due to the loss of function and auton-

omy and the fears of death. The aim of care should be to

enable patients and families to maintain their quality of life.

This includes supporting their adjustment to a changing

reality, and listening to their fears to help cope with these

deeper issues [40]. Both for those with faith and those with

none spiritual support can be very helpful in coping with

such questions as “why me?”

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214 Chapter 14

necessary to discuss future choices, including end-of-life,

to enable informed decisions about the intervention [3].

It is important to recognise that a patient is deteriorating

and for this to trigger discussions and preparations for the

end-of-life. This need may be identified before patients

and families would prefer to discuss it, and clinicians must

be skilled in their approach to the issue. Discussion should

not be forced on patients and families who do not want it,

but it is very important that the opportunity is offered. It

should be explained why the discussion is proposed and

what the risks might be if it is delayed, such as losing the

opportunity to influence their future care. Some people

with a preference not to consider the future nonetheless

decide to discuss it once they know and understand the

benefits of doing so. Others will continue to take an

approach to decision-making in the moment or by others.

Recognition of progression may be difficult when there

is a slow progression and fluctuating condition, such as in

PD. Several ways have been suggested for recognising the

later stages of neurological disease (see Box 8).

Key considerations in relation to the end-of-life phase

are found in Box 9 (see Chapter 20 for more detail).

careful genetic counselling, both before and after testing,

as there are many implications for someone with a positive

test, including coping with uncertainty, consideration of

family planning, discussing the test and diagnosis with the

wider family who may also be at risk, financial and insur-

ance consequences and planning for future care. These

issues may become more prominent as the underlying ge-

netic background of neurological diseases becomes clearer

over the coming decades.

Following the death of the person there may be many

varying feelings – including ambivalence as there is sad-

ness at the loss, yet relief that the challenges of caring have

ended, and the guilt that is often associated with that relief.

All involved need time to talk and the primary health care

team are well placed to provide the ongoing support and

care.

Professional Carers

Many of the challenges and stresses faced by families may be

experienced by the professional carers as well. They may feel

hopelessness and despondency at seeing the progression of

the disease, together with the issues of uncertainty over the

timelines of deterioration, loss of personhood of the patient

and the existential distress [40]. Involvement in decisions to

withdraw life sustaining treatment such as ventilatory

support is difficult for many staff, more so because it is

sometimes required of teams who rarely experience it. The

team approach, including the support and liaison with other

teams, is very helpful and discussion and sharing of the feel-

ings experienced in caring for the patient and their family is

essential [33]. Regular team meetings when the issues can

be shared are helpful and following the death a debriefing

meeting allows the ambivalent feelings of team members to

be expressed and shared, so that team members are not iso-

lated. Guidance and peer support from teams elsewhere

who have experienced similar challenges can help.

End-of-Life Care

People with progressive neurological death will face death,

varying from a period of 2–3 years in MND to perhaps

many decades in MS, when life may be shortened by only a

few years (see Table 1). The joint EAPC / EAN Consensus

Statement encourages early discussion about deterioration

and dying, particularly if communication or cognition

may be compromised later [6]. Moreover, there may be

particularly pertinent opportunities to discuss the issues of

dying when new interventions are suggested, such as gas-

trostomy or ventilatory support. At these times it is

Box 8: Recognition of the end-of life phase

• Triggers of consideration of end-of-life:

Swallowing problems

Recurring infection (particularly aspiration pneumonia)

Marked decline in physical status

First episode of aspiration pneumonia

Cognitive difficulties

Weight loss

Significant complex symptoms [2]

These have been shown to increase towards death

[41] and the terminal stage could be recognised in

72% of patients [42].

• The “Surprise Question” suggested by the Gold Standards

Framework – “Would you be surprised if this patient died

in the coming 6 to 12 months?” If the answer to this is “No,”

this would suggest that the patient could deteriorate and die

soon. The Proactive Identification Guidance also provides

triggers to consider the end-of-life phase, similar to those

outlined earlier (goldstandardsframework.org.uk/pig).

• The Supportive and Palliative Care Indicators Tool (SPICT

)

(see Chapter 1) suggests clinical indicators to help identify

people who are deteriorating and for neurological disease

these are: progressive deterioration in physical and/or cog-

nitive function, speech and swallowing problems, recurrent

aspiration pneumonia, respiratory failure (spict.org.uk/the-

spict/).

https://t.me/med1917

Palliative Care for People with Progressive Neurological Disorders 215

particularly when the case for a change in the law allowing

assisted dying is presented. Openness and listening to the

concerns of the person, and their family, is important so

that any issues can be addressed. For instance, discussion

of the very small actual risk of choking in MND, ensuring

that there are ways to facilitate communication, the provi-

sion of a DNACPR form and other measures to protect the

person from unwanted interventions or unwanted prolon-

gation of life, the provision of anticipatory medication for

any distress and support from the MDT may help to alle-

viate the fears and reduce the wish for a hastened death

(see Chapter 6 – Ethics in Palliative Care).

Conclusion

The care of patients with progressive neurological disease

requires:

•

Involvement of palliative care services earlier in the dis-

ease progression – to allow planning and discussion while

the person can communicate and has no cognitive change

and can communicate well

• Involvement of many disciplines – working together as a

team

• Co-ordinated care

•

Careful assessment and management of symptoms

•

Support for patients and their families – physical,

psycho-social, and spiritual

• Proactive care – and preparation for symptoms or prob-

lems that may occur

• Collaboration with voluntary agencies and charities

who can provide specialist advice and support

• Good team working and support

Disease Specific Organisations

Motor Neurone Disease

Association for England and Wales

mndassociation.org

MND Scotland mndscotland.org.uk

Multiple Sclerosis Society mssociety.org.uk

Multiple Systems Atrophy Trust msatrust.org.uk

Parkinson’s UK parkinsons.org.uk

Progressive Surpranuclear Palsy

Association

pspassociation.org.uk

Huntington’s Disease Association hda.org.uk

Muscular Dystrophy UK musculardystrophyuk.org

Hastened Death

People with progressive neurological disease may wish to

talk about hastened death – euthanasia and physician

assisted dying. These are not legal in the UK and clinicians

should be careful to act only in line with relevant law and

regulations and do nothing that is intended to hasten

death. In some jurisdictions they can be provided legally,

within certain criteria, which again should be carefully

observed. There are many fears of the mode of dying with

neurological disease – including pain, breathlessness,

“choking” and distress – and these are widely discussed,

Box 9: Key considerations in end-of-life

care

• Discussion of the place of care and place of death – these

need to be considered separately as some families may wish

to care for the patient at home, but may not be able to face

them dying at home [43].

• Involvement of specialist palliative care teams, if they are

not already involved.

• Provision of anticipatory medication to cope with a crisis or

episode of distress – such as morphine for pain, midazolam

for agitation/spasm/seizure, and glycopyrronium bromide

for chest secretions.

• Ensuring medication is continued if this is necessary – for

instance in PD it is important to continue dopaminergic

medication to reduce ridigity – as liquid medication, trans-

dermal patch or as apomorphine infusion.

• Consideration of reducing or stopping medication that may

no longer be appropriate – such as riluzole in MND.

• Discussion about the patient’s wishes, informed if necessary

by those close to them, about hydration and feeding, car-

diopulmonary resuscitation and ventilatory support, and

about carers’ ability and willingness to support these where

needed. This may inform decisions about withdrawal of the

intervention or withholding them at the end-of-life (see

Chapter 20).

• Ensuring extra support and care is offered, including equip-

ment such as a hospital bed or hoist, so that family can re-

main as family members and do not become full time car-

ers, unless this is their wish.

• Consideration of advance care planning – so that the per-

son’s wishes are known if they become unable to make them

known, due to changes in communication or cognition.

These discussions may need to be early in the disease pro-

gression, before communication and cognition are lost, and

sometimes before the person and family feel they should

discuss these issues. However, they may need to be dis-

cussed early if the person’s real wishes are to be respected.

See Chapter 9.

https://t.me/med1917

216 Chapter 14

neurodegenerative disorders: Ne-PAL, a pilot randomized

controlled study. BMJ Supportive & Palliative Care 7:

164–172.

16.

Gao, W., Wilson, R., Hepgul, N. et al. (2020). Effect of short-

term integrated palliative care on patient-reported outcomes

among patients severely affected with long-term neurological

conditions. A randomized controlled trial. JAMA open Network

3 (8): e2015061. doi:10.1001/jmamnetworkopen.2020.15061.

17.

Oliver, D. (1996). The quality and care of symptom control

– the effects on the terminal phase of ALS/MND. Journal of

the Neurological Sciences 139 (suppl): 134–136.

18.

Lee, M., Walker, R.W., Hildreth, T.J. et al. (2006). A survey of

pain in idiopathic Parkinson’s disease. Journal of Pain and

Symptom Management 32: 462–469.

19.

Kahraman, T., Ozdogar, A.T., Ertekin, O. et al. (2019).

Frequency, type and distribution of pain and related factors

in persons with multiple sclerosis. Multiple Sclerosis and

Related Disorders 28: 221–225.

20.

Rana, A.Q., Qureshi, A.R., Siddiqui, O. et al (2019). prevalence

of pain in atypical parkinsonism: a systematic review and

meta-analysis. Journal of Neurology 266: 2093–2102.

21. Sprenger, G.P., van der Zwaan, K.F., Roos, R.A.C. et al.

(2019). The prevalence and the burden of pain in patients

with Huntington disease: a systematic review and meta-

analysis. Pain 160: 773–783.

22.

Lager, C. and Kroksmark, A.K. (2015). Pain in adolescents

with spinal muscular atrophy and Duchenne and Becker

muscular dystrophy. European Journal of Paediatric

Neurology 19: 537–546.

23.

Regnard, C., Reynolds, J., Watson, B. et al. (2007).

Understanding distress in people with severe communication

difficulties: developing and assessing the Disability Distress

Assessment Tool (DisDAT). Journal of Intellectual Disability

Research 51: 277–292.

24.

Warden, V., Hurley, A.C., and Volicer, L. (2003). development

and psychometric assessment of the Pain Assessment in

Advanced Dementia (PAINAD) scale. Journal of the

American Medical Directors Association 4: 9–15.

25. Oliver, D. (1998). Opioid medication in the palliative care of

motor neurone disease. Palliative Medicine 12: 113–115.

26. Bourke, S.C., Tomlinson, M., Williams, T.L. et al. (2006).

Effects of non-invasive ventilation on survival and quality of

life in patients with amyotrophic lateral sclerosis: a randomised

controlled trial. Lancet Neurology 5: 140–147.

27. Heffernan, C., Jenkinson, C., Holmes, T. et al. (2006).

Management of respiration in MND/ALS patients: an

evidence based review. Amyotrophic Lateral Sclerosis and

Other Motor Neuron Disorders 7: 5–15.

28. Oliver, D.J. and Turner, M.R. (2010). Some difficult decisions

in ALS/MND. Amyotrophic Lateral Sclerosis 11: 339–343.

29. Turner, M.R., Faull, C., McDermott, C.J. et al. (2019).

Tracheostomy in motor neurone disease. Practical Neurology

19: 467–475.

30. Kaub-Wittemer, D., von Steinbuchel, N., Wasner, M. et al.

(2003). Quality of life and psychosocial issues in ventilated

References

1. Department of Health. (2005). National Service Framework

for Long-term Conditions.

2. End of Life Care Programme. (2010). End of Life Care in

Neurological Conditions: A Framework for Implementation.

3. National Institute for Health and Care Excellence. (2016).

NICE guidance on motor neurone disease. NICE. www.nice.

org.uk/NG42 (accessed 19 October 2022).

National Institute for Health and Care Excellence. (2017).

NICE guidance on Parkinson’s disease in adults. NICE. www.

nice.org.uk/NG71 (accessed 21 October 2022).

5. GBD 2016. (2019). Neurology Collaborators. Global,

regional, and national burden of neurological disorders,

1990-2016: a systematic analysis for the Global Burden of

Disease Study 2016. Lancet Neurology 18: 459–480.

Oliver, D.J., Borasio, G.D., Caraceni, A. et al. (2016). A

consensus review on the development of palliative care for

patients with chronic and progressive neurological disease.

European Journal of Neurology 23: 30–38.

Aridegbe, T., Kandler, R., Walters, S.J. et al. (2013). The

natural history of motor neuron disease: assessing the impact

of specialist care. Amyotrophic Lateral Sclerosis 14: 13–19.

Rooney, J., Byrne, S., Heverin, M. et al. (2015). A

multidisciplinary clinic approach improves survival in ALS: a

comparative study of ALS in Ireland and Northern Ireland.

Journal of Neurology, Neurosurgery, and Psychiatry 86:

496–503.

Creutzfeldt, C.J., Robinson, M.T., and Holloway, R.G. (2016).

Neurologists as palliative care providers. Communication

and practice approaches. Neurology 6: 40–48.

10. Bede, P., Oliver, D., Stodart, J. et al. (2011). Palliative care in

amyotrophic lateral sclerosis: a review of current international

guidelines and initiatives. Journal of Neurology, Neurosurgery,

and Psychiatry 82: 413–418.

11.

Gao, W., Crosby, V., Wilcock, A. et al. (2016). Psychometric

properties of a generic patient-centred palliative care

outcome measure of symptom burden for people with

progressive long term neurological conditions. PLOS ONE 11

(10): e0165379.

12. Higginson, I.J., Hart, S., Burman, R. et al. (2008). Randomised

controlled trial of a new palliative care service: compliance,

recruitment and completeness of follow-up. BMC Palliative

Care 7: 7. doi:10.1186/1472-684X-7-7.

13. Higginson, I.J., McCrone, P., Hart, S. et al. (2009). Is short-

term palliative care cost-effective in multiple sclerosis? A

randomized phase II trial. Journal of Pain and Symptom

Management 38: 816–826.

14. Solari, A., Giordano, G., Patti, F. et al. (2017). Randomised

controlled trial of a home based palliative approach for

people with severe muliple sclerosis. Multiple Sclerosis

Journal 24: 663–674.

15.

Veronese, S., Gallo, G., Valle, A. et al. (2017). Specialist

palliative care improves the quality of life in advanced

https://t.me/med1917

Palliative Care for People with Progressive Neurological Disorders 217

37. Eschlbock, S., Delazer, M., Krismer, F. et al. (2020). Cognition

in multiple systems atrophy: a single center cohort study.

Annals of Clinical and Translational Neurology 7: 219–228.

38.

Golbe, L.I. (2014 April). Progressive supranuclear palsy.

Seminars in Neurology 34: 151–159. doi:10.1055/s-0034-

1381736. Epub 2014 Jun 25.

39.

Benedict, R.H.B., Amato, M.P., DeLuca, J. et al. (2020). Cognitive

impairment in multiple sclerosis: clinical management,

MRI, and therapeutic avenues. Lancet Neurology 19: 860–871.

40.

Gofton, T.E., Chum, M., Schultz, V. et al. (2018). Challenges

facing palliative neurology practice: a qualitative analysis.

Journal of the Neurological Sciences 385: 225–231.

41.

Hussain, J., Adams, D., Allgar, V. et al. (2014). Triggers in

advanced neurological conditions: prediction and

management of the terminal phase. BMJ Supportive &

Palliative Care 4: 30–37.

42.

Hussain, J., Allgar, V., and Oliver, D. (2018). Palliative care

triggers in progressive neurological conditions: an evaluation

using a multi-centre retrospective case record review and

principal component analysis. Palliative Medicine 32:

716–725.

43.

Agar, M., Currow, D.C., Shelby-James, T.M. et al. (2008).

Preference for place of care and place of death in palliative

care: are these different questions? Palliative Medicine 22:

787–795.

patients with amyotrophic lateral sclerosis and their caregivers.

Journal of Pain and Symptom Management 24: 890–896.

31.

Phelps, K., Regen, E., Oliver, D. et al. (2015). Withdrawal of

ventilation at the patient’s request in MND: a retrospective

exploration of the ethical and legal issues that have arisen for

doctors in the UK. BMJ Supportive & Palliative Care.

doi:10.1136/bmjspcare-2014-000826.

32.

Faull, C., Rowe-Haynes, C., and Oliver, D. (2014). Issues for

palliative medicine doctors surrounding the withdrawal of

non-invasive ventilation at the request of a patient with

motor neurone disease: a scoping study. BMJ Supportive &

Palliative Care 4: 43–49.

33.

Association for Palliative Medicine. (2015). Withdrawal of

assisted ventilation at the request of a patient with motor

neurone disease: guidance for professionals. Association for

Palliative Medicine of Great Britain and Ireland.

34.

Lee, M.A., Prentice, W.M., Hildreth, A.J. et al. (2007).

Measuring Symptom Load in Idiopathic Parkinson’s Disease.

Parkinsonism & Related Disorders 13: 284–289.

35. Poewe, W. (2008). Non-motor symptoms in Parkinson’s

disease. European Journal of Neurology 15 (Suppl 1): 14–20.

36. Goldstein, L. (2006). Control of symptoms – cognitive

dysfunction. In: Palliative Care in Amyotrophic Lateral

Sclerosis from Diagnosis to Bereavement, 2e (ed. D. Oliver, G.D.

Borasio, and D. Walsh). Oxford: Oxford University Press.

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