Chapter 34

Palliative medicine

Richard D W Hain

With contributions by, Megumi Baba, Joanne Griffiths, Susie Lapwood, YiFan Liang, Mike Miller

Learning objectives

By the end of this chapter the reader should:

• Be aware of life-limiting conditions and their epidemiology

• Know about the principles of symptom control – pain, nausea and vomiting, dyspnoea, constipation, skin conditions and emergencies

• Be aware of the ethical issues in children with life-limiting conditions

• Know about the practical issues around the death of a child

• Be aware of bereavement and grief

Philosophy of palliative medicine

A child’s illness profoundly impacts on child and family, particularly when the illness might lead to death. The science of medicine is increasingly able to intervene to cure even serious illness. However, a significant number of children cannot be cured. They have a ‘life-limiting condition’ (LLC), an illness which leads to premature death and/or a prolonged period of chronic illness. If cure is the only solution medicine can offer, doctors will never meet the needs of children living with LLC. Cure is a powerful way to improve the lives of ill children; fortunately, it is not the only way.

Key point

Palliative care (Box 34.1) refers to the support healthcare can offer where cure is not possible. Palliative medicine refers to the contribution that doctors can make to that care.

Box 34.1

What is palliative care?

Palliative care:

• Is aimed at improving life quality, rather than duration.

• Considers all aspects of life, including psychological, emotional and spiritual as well as physical.

• Considers the needs of the family as well as the child.

• Intervenes only when the benefit of intervention outweighs its burden.

• Is multi-professional and multidisciplinary.

Palliative care is not:

• Withdrawal of care.

• Synonymous with euthanasia.

• What is left after medical treatment has failed.

Unlike most medical specialties, palliative care is not defined by organ system, aetiology or age group, but by a philosophy of care. That complicates definitions. There have been many attempts to define which medical conditions are included in LLC. The most widely used is the ACT/RCPCH system, which defines four categories of LLC based on the trajectory of the condition. Conditions in the different categories are:

• Category I – those for which death and cure are both possible outcomes (e.g. cancer).

• Category II – might be living a normal or near-normal life in the present moment despite having a condition that will inevitably lead to premature death (e.g. Duchenne muscular dystrophy).

• Category III – relentlessly progressive towards death without any such normal period.

• Category IV – heterogeneous and unpredictable because the underlying condition is not progressive; premature death results from the cumulative effects of the condition, rather than from the condition itself (e.g. cerebral palsy).

The exact proportions are not clear (see below), but some reports suggest that categories I and IV each account for roughly a third of all LLCs, with II and III together making up the remaining third.

The multidimensional nature of palliative care means that it is informed by research in a wide range of disciplines, from anthropology – for example, Bluebond-Langner’s seminal work on how children see dying – to bioethics, moral philosophy and theology. Over the last fifteen years, it is perhaps in the fields of opioid pharmacology and epidemiology that the impact of scientific research is most obvious to paediatricians.

One of the barriers to good symptom management has traditionally been the belief that morphine should be withheld from children wherever possible, and that codeine was safer because it was weaker. A series of studies of morphine in children has shown that there is no pharmacological basis for a reluctance to prescribe morphine in children. In contrast, recent clinical studies have shown that the metabolism of codeine is dangerously unpredictable. Studies have made it clear that conventional practice in respect of opioids in children perversely recommended an alternative to morphine that was both less effective and more dangerous.

Epidemiology is beginning to shape palliative care. The ACT/RCPCH categories are descriptions of types of condition, rather than a list of diagnoses. They are not precise enough for epidemiological purposes. That has meant that, until recently, it was impossible to develop services for children with LLCs based on evidence. That has recently changed as a result of studies. One assigned an ICD10 code to around 400 of the commonest LLCs presenting to hospice and palliative care teams in the UK. Another used an analysis of prospective ‘hospital episode’ data. The result was that for the first time we now know that 32 in every 10,000 children in England are living with a LLC and that its prevalence has increased by almost a third in the last decade.

Palliative care in children provides a good illustration of the need for clinical practice to be informed by science, even when cure is no longer possible. Pharmacology and epidemiology are fields of research whose findings have begun to transform the way we can care for children with LLCs.

Symptom control

Pain

Pain management:

• Is an essential component of palliative care.

• Requires assessment, communication, planning and a sound knowledge of pharmacology and physiology.

• Is often under-recognized in children with disability.

• Can dramatically improve quality of life for child and family if done well.

According to the International Association for the Study of Pain, pain is ‘an unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage.’ It is:

• Subjective (whatever the child says it is)

• Influenced by past pain experiences and concerns about personal well-being or that of others

• Influenced by context (different situations).

Key point

Total pain expresses the concept that pain always occurs in the context of emotional need, fears, past experiences and understanding of the pain as well as biological experience.

All children, including the extremely preterm, are able to feel pain. Pain in palliative care is usually neither wholly acute nor entirely chronic. It has elements of both, and may be complicated by the existential context of deterioration towards death.

Assessment of pain

Assessment of pain in children requires:

• Detailed history (also from the child, if possible)

• Observation of the child (Box 34.2), ideally in a variety of settings

• Examination

• Consideration of all possible contributing factors (including psychological, spiritual and social)

• Discussion with parents, especially in the non-verbal child

• Use of pain assessment tools appropriate for age and cognitive ability.

The two most commonly-used types of pain scale are ‘faces’-type tools and scales based on observation of behaviour patterns associated with pain in non-verbal children, such as the Paediatric Pain Profile. Faces-type tools are based on a Likert scale and illustrate varying pain intensity using drawings of children’s faces. Unlike the Paediatric Pain Profile, most ‘faces’-type scales were validated for assessment of acute pain in cognitively normal children, rather than in children with LLCs.

Management of pain

Consider and treat specific reversible causes

• Constipation

• Gastro-oesophageal reflux

• Orthopaedic, especially hip dislocation

Consider non-pharmacological measures

• Attention to reversible sources of fear and anxiety

• Counterirritants (hot or cold packs, acupuncture or TENS), distraction techniques

• Behavioural techniques (cognitive behavioural therapy, relaxation, visualization or art therapy)

Pharmacological approach: the pain ladder

Key point

The WHO pain ladder (Fig. 34.1) is the basis for rational management of palliative pain. It expresses the concept that increases in the intensity of pain should be matched by changes both in the type of analgesic, and the manner in which they are prescribed.

Fig. 34.1 WHO analgesia ladder showing use of analgesics according to severity of pain. Recently, the WHO ladder recommends only simple analgesics and opioids. Opioids are no longer divided into minor and major, as high dose of a weak opioid is pharmacologically equivalent to a low dose of a strong one. Simple analgesics are: paracetamol, NSAID, aspirin where appropriate. Opioids are: morphine, diamorphine, fentanyl, buprenorphine, methadone. Codeine is no longer recommended in most places owing to pharmacogenetic variation in its hepatic activation to morphine, which leads to inconsistent effectiveness. Tramadol is an opioid but has additional non-opioid analgesic properties that make it equivalent to morphine, but it is often poorly tolerated.

There are three steps on the WHO pain ladder. As pain intensity increases and the effect of prescribing on one step becomes inadequate, the prescriber should move to the next step. Each step is characterized by:

• A specific class of analgesic

• A specific approach to dosing (regular versus ‘as needed’)

• The need to consider adjuvants (Box 34.3) appropriate to the nature of pain

Box 34.3

Adjuvants to analgesics

These depend on characteristics of pain:

• Bone pain – NSAID, radiotherapy (if metastatic cause), bisphosphonates (especially in presence of osteopenia), steroids (if metastatic cause)

• Nerve pain – Anticonvulsants, antidepressants, NMDA antagonists (ketamine, methadone), baclofen, steroids (if cause is pressure, e.g. from tumour oedema)

• Muscle spasm – Baclofen, benzodiazepines, botulinum toxin

• Cerebral irritation – Phenobarbital, benzodiazepines

• Neurolytic and other interventions – Regional nerve blocks and spinal blocks

Key point

An adjuvant is a medication or other intervention that is not an analgesic but, used alongside analgesics, its actions can reduce pain in certain specific situations.

For prescription of major opioids (Box 34.4), there are three phases, namely: initiation, titration and maintenance. The opioids can be given as immediate release (e.g. oramorph, buccal diamorphine), continuous release (e.g. MST, transcutaneous patch, syringe driver). There should always be both regular (background) and ‘as needed’ doses. This is a specialist skill and should be undertaken in discussion with the local or regional palliative care team.

Box 34.4

Common fears and myths about morphine

• ‘It is the ‘death drug’.’ Explanation with family to increase understanding about benefits and side-effects.

• ‘It will stop my child breathing.’ Respiratory depression is extremely rare when opioids are used for pain. It is avoided by careful titration of dose.

• ‘It has all kinds of side effects.’ There are adverse effects, but fear of them is often disproportionate to the reality:

• Drowsiness: The child is likely to be drowsy for 3–5 days when first starting strong opioids or when doses are increased.

• Nausea and vomiting: This can occur when first starting, is less common than in adults and wears off.

• Constipation: Laxatives are necessary and can be titrated according to need.

• Other side effects should be monitored but are rare (e.g. pruritus, urinary retention, nightmares)

• ‘It is addictive.’ Explain to the family about issues relating to addiction and dependence. Physical dependence is not usually a primary concern in the palliative care setting, but opioids should always be weaned slowly if the pain resolves to avoid withdrawal.

• ‘Once you start morphine, there’s nothing to turn to later when the pain becomes really bad.’ Tolerance probably occurs if opioids are used for long periods. The remedy is to increase the dose of opioids. Families may find it beneficial to understand the principle of tolerance rather than assuming that escalating doses of analgesia imply disease progression.

• ‘People will break in and steal it.’ Discuss safe storage, particularly in the home setting.

Nausea and vomiting

• Nausea and vomiting are distressing symptoms that may cause more upset than pain.

• Full control of nausea and vomiting is more difficult than that of pain – negotiating acceptable goals with the family may be necessary.

• In children with cognitive impairment, feeds are a common cause – liaise with dietitians to manipulate volumes/rates/type of feed.

• Constipation is another common cause.

Key point

Mediators of nausea and vomiting act through receptors in the gastrointestinal tract, liver and brain (chemoreceptor trigger zone and vomiting centre). Rational management of nausea and vomiting relies on knowledge of receptors.

Nausea and vomiting due to:

• Gastrointestinal tract damage (chemotherapy, radiotherapy) is often mediated through 5-HT₃ receptors and blocked by 5-HT₃ antagonists (ondansetron, granisetron)

• Liver swelling/damage or toxins (infection, metabolic, uraemia, drugs) are often mediated through dopamine receptors and blocked by dopamine blockers (domperidone, metoclopramide, haloperidol). Because metoclopramide blocks dopamine receptors in the CNS as well as peripherally, it is both more effective than domperidone and more likely to cause adverse neurological effects.

• Vestibular problems (travel sickness, vertigo) and raised intracranial pressure is largely mediated through acetylcholine and histamine (H₁) and is blocked by anticholinergics, anti-H₁ (cyclizine acts at both receptors) medications.

Additional factors to consider are:

• Levomepromazine (phenothiazine) blocks most receptors and is good second-line treatment, or first-line if cause is multifactorial/not known

• Functional delay – can use prokinetics (dopamine antagonists, erythromycin)

• Efferent (motor) pathway is through vagus nerve (acetylcholine) so anticholinergics are often partially effective irrespective of cause

• Steroids can be valuable by reducing:

– Oedema (e.g. in raised intracranial pressure)

– Tissue damage

– Release of emetogenic mediators

but adverse (Cushingoid, etc.) effects after prolonged administration of steroids can limit their usefulness.

In bowel obstruction, consider:

• Anticholinergic to relieve spasm (hyoscine butylbromide (buscopan))

• Analgesic to relieve pain (strong opioid parenterally)

• Anti-secretory to reduce hypersecretion (octreotide)

Gastro-oesophageal reflux (GOR) is common but does not always cause discomfort. The risk is increased by prone position, decreased activity, medication and liquid feeds (all more likely among debilitated patients). A presumptive diagnosis and treatment of GOR may be appropriate in pain and discomfort related to feeding without an obvious cause. In reflux, consider:

• Antacid to relieve pain (ranitidine, proton blockers)

• Prokinetic to improve gastric emptying (domperidone, metoclopramide)

• Dopamine blocker to reduce reflux (domperidone, metoclopramide)

Question 34.1

Nausea and vomiting

The following are used in the treatment of nausea and vomiting:

F. Methotrimeprazine (Nozinan®)

For each of the scenarios below, choose the most appropriate first-line intervention. Select ONE answer for each. Note: Each answer may be used more than once.