Overview of paediatric palliative care

Key points.

• •Paediatric palliative care involves a holistic approach to life-limited children, encompassing symptom management, psychosocial, and bereavement care.
• •The number of children with palliative care needs is increasing worldwide.
• •Life-limited children are a highly complex group with healthcare needs that span primary, secondary, and tertiary care.
• •Interdisciplinary ethical decision-making and advance care planning may pose challenges to the routine and emergency care of life-limited children.
• •Complex symptoms may require multidisciplinary management involving paediatric palliative care teams in the perioperative period.

Learning objectives.

By reading this article, you should be able to:

• •Identify UK referral criteria for children with palliative care needs.
• •Describe the UK core care pathway for life-limited children.
• •Initiate basic symptom management of a life-limited infant, child, or young person.
• •Define advance care planning for life-limited children and contribute to the identified documents for these decisions in healthcare settings.

Paediatric palliative care is defined as an active and total approach to care, from the point of diagnosis or recognition, throughout the child's life, death, and beyond. It embraces physical, emotional, social, and spiritual elements, and focuses on the enhancement of the quality of life for the child or young person, and support for the family. It includes the management of distressing symptoms, and provision of short breaks and care through death and bereavement.¹ This involvement may begin antenatally, and continue throughout infancy, childhood, and adolescence, or referral may be prompted by a change in the focus of care. Engagement with services stops when the patient dies, or by appropriate transition of patients to adult services. Together for Short Lives, the UK charity for professionals and families of infants, children, and young people with life-limiting illnesses, categorises life-limiting and -threatening illness (Table 1). Most referrals to paediatric palliative care teams are in situations where these diagnostic criteria are fulfilled.

Table 1.

Life-limiting and life-threatening conditions affecting children and young people¹

Group	Illness trajectory	Examples
1	Life-threatening conditions for which curative treatments may be feasible, but can fail	Cancer and irreversible organ failure
2	Conditions in which premature death may occur in childhood despite intensive treatment aimed at prolonging life	Cystic fibrosis
3	Progressive, incurable conditions where treatment is exclusively palliative	Batten disease and muscular dystrophy
4	Irreversible but non-progressive conditions causing severe disability and susceptibility to health complications and likelihood of premature death	Severe cerebral palsy

In the UK, the prevalence of life-limiting conditions in children and young people increased in the decade up to 2010 from 25 to 32 per 10,000 population, with a higher prevalence in children from ethnic minority groups and the most deprived areas.⁵ In 2013, the prevalence of children in the 0–14 yr age group identified with a life-limiting condition from inpatient hospital records was close to 50%.⁶ This may be a result of improved data collection techniques, more representative estimates of prevalence, and the increased survival of inpatients with life-limiting conditions. Therefore, it is likely that children with life-limiting conditions are a significant proportion of the patients served by acute children's healthcare services. These children carry a substantial burden of morbidity, may have complex medical histories and special educational needs, and are managed by multi-professional teams in different care sectors. They may have specific regimens for supportive care, complex medications, evolving symptoms, and advance care plans. It is important that healthcare professionals, including specialists in anaesthesia, understand the specific needs of these children, and provide a sensitive, safe, and informed service to them.

This article will describe the key areas of paediatric palliative care with which all healthcare professionals should be familiar: (i) redirecting the goals of care, (ii) parallel planning, (iii) basic symptom management, (iv) advance care planning, and (v) bereavement care.

Together for Short Lives published a framework for an integrated care pathway for children and young people with life-limiting and life-threatening illnesses; this proposed five standards for care provision after events that occur during the course of a life-limited child's illness (Table 2).² There may be uncertainty of the child's prognosis, and therefore, of the time span over which these events occur. Therefore, decision-making about the appropriateness of interventions at different points in the pathway may vary according to the situation.

Table 2.

Standards for care, based on the Together for Short Lives core care pathway for children with life-limiting and life-threatening conditions²

Standard
1	Prognosis sharing and significant news	Disclosure of prognosis and provision of information
2	Transfer and liaison between hospital and community services	Liaison between hospital and community care settings
3	Multidisciplinary assessment of needs	Multidisciplinary and multi-agency assessment of care needs
4	A child and family care plan	Ongoing multi-agency care plan, including input from health, social care, and education sectors
5	An end-of-life care plan	Multi-agency care plan involving the child and family
6	A bereavement support plan	Ongoing support and appropriate referral to specialist services if required

Redirecting the goals of care refers to the concept of evaluating the risks and benefits of a proposed intervention as applied to the current situation. This acknowledgement may then alter the balance between disease-modifying interventions and symptom management interventions for a given clinical situation. Types of care include disease-modifying care (such as chemotherapy), intensive care interventions (such as invasive mechanical ventilation and inotropic support), supportive care (such as antibiotic therapy), and basic care (such as feeding). Basic care and supportive care are seldom withdrawn, but the risks and benefits of intensive care interventions and disease-modifying interventions may be re-evaluated at certain times during the care of a life-limited child. The reasons for consideration of withdrawing or withholding intensive care for children and young people with life-limiting illnesses are outlined in a Royal College of Paediatrics and Child Health document.⁷

Parallel planning is planning concurrently for end-of-life care and continuing life. This concept acknowledges that there is uncertainty of prognosis or timing of death, and therefore, allows practical and emotional support to be offered even if the child unexpectedly survives longer than anticipated (see Case study 1).

Case study 1 Child A is 5 yrs old with severe spastic quadriplegic cerebral palsy (a non-progressive motor disorder). He is wheelchair bound, has epilepsy, is fed by gastrostomy, and requires frequent suctioning of copious respiratory secretions. Child A has had several hospital admissions with respiratory illnesses since the neonatal period, and is admitted again with pneumonia. Despite intensive ward care, Child A's condition deteriorates. The outcome of his respiratory failure is uncertain, and it is appropriate to consider redirecting the goals of care and offer a choice to manage the symptoms alone rather than escalating ventilatory support. This offers an opportunity for the family to express their wishes for the preferred place of end-of-life care if Child A succumbs to this illness; changes to interventions can take place to accommodate this. These clinical decisions, made within legal and ethical decision-making frameworks, in partnership with the family and child, if appropriate, enable a specific plan for this illness episode to be formulated.

Case study 2 Child B is a 12-yr-old girl with a brainstem glioma that has progressed. Planned care involves palliative radiotherapy; Child B and her parents have expressed together the wish that cardiopulmonary resuscitation should not be attempted in the event of cardiorespiratory arrest at the end of life, as it would be deemed futile. She is to have a short general anaesthetic for her course of radiotherapy. The plan for ‘do not attempt resuscitation’ is suspended during radiotherapy treatment because if she were to have a cardiorespiratory arrest during general anaesthesia, this may not be disease related, and therefore, would be potentially reversible with appropriate intervention.

Basic symptom management

Symptoms in palliative care are multidimensional with physical, psychological, sociological, and spiritual aspects. This biopsychosocial model is not unique to palliative care, but the spiritual aspect of a symptom in palliative care includes the existential nature of the symptom experienced in the life-limited patient's situation. Existential suffering has been described as the morbid suffering relating to loss of hope, futility, remorse, and fear of death.⁸ This may be felt by the patient, carer, and health professional, leading to a complex dynamic that plays a vital role in the presentation of a symptom by a life-limited child or young person. It may result in symptoms not being managed optimally and which appear resistant to escalating pharmacological interventions (Table 3).

Table 3.

Principles for the management of common symptoms in paediatric palliative care3, 4

Symptom	Factors to consider	Management strategies
Pain	•Cause of the pain •Associated pathology •Psychological factors •Views of the patient/family	•WHO pain ladder •Routes of administration of opioids (enteral, parenteral, or trans-mucosal) •Agents directed at neuropathic pain (gabapentin, amitriptyline, ketamine, and clonidine) •Non-pharmacological strategies (transcutaneous electrical nerve stimulation, massage, and complementary therapies)
Agitation	•Medical cause of agitation (pain, hypoxia, urinary retention, constipation, or delirium) •Psychological cause (anxiety or depression) •Drug interactions	•Non-pharmacological strategies, such as quiet and calm environment, and addressing fears and concerns •Short- or long-acting benzodiazepines (midazolam, diazepam, and lorazepam) •Neuroleptic agents (haloperidol and levomepromazine)
Dyspnoea	•Consider psychological causes. •Consider medical causes, which may require referral for other specialist opinions if the child is not at the end of life. •Management directed at excessive secretions obstructing the airway •Management directed at the subjective sensation of distress when breathing	•Explanation to the patient and carer about the nature of the breathing difficulty •Management of accumulated secretions (with bronchodilators, nebulised saline, chest physiotherapy, gentle upper airways suctioning, and use of medications to reduce nasal secretions such as glycopyrronium bromide and hyoscine hydrobromide) •Management of distressing breathing (supplemental oxygen, fan therapy, low-dose opioids, and short-acting benzodiazepines such as buccal midazolam)
Seizures	•Cause of seizure, such as fever, electrolyte disturbances, or drug interactions •Consider whether the child is for active management of prolonged seizures as per national guidelines for status epilepticus, or if he/she is for exclusive palliation.	•Explanation of the nature of seizures to the child and family •Advice about positioning to minimise obstruction of the upper airway and supplemental oxygen available at home or school •Benzodiazepines, such as buccal midazolam or rectal diazepam •Rectal paraldehyde •Enteral loading doses of phenobarbital •Continuous i.v. or subcutaneous infusions of midazolam, phenobarbital, or levetiracetam
Nausea and vomiting	•Education about prevention of this symptom is preferable to reactionary management. It is important to be aware that the likelihood of complete resolution of symptoms may not be possible in some conditions, such as with end-stage brain tumours. •Reversible causes, such as pain and drug interactions •Associated causes, such as chemotherapy and neurological conditions •Antiemetic agents should be directed by the likely mechanism or mechanisms involved, such as dexamethasone and ondansetron for chemotherapy-induced nausea and vomiting. •Antiemetic agents should be chosen with an adverse-effect profile appropriate for the stage of palliation. Sedating agents, such as levomepromazine, may be appropriate at end of life, but may cause unacceptable sedation at earlier stages of the disease. •Route of administration may need to be altered if the severity of nausea and vomiting precludes the oral route. A continuous subcutaneous infusion can be delivered in the home or hospice if required. Once control of vomiting is achieved, the medication can be switched to the enteral route again. •Minimise strong odours, and keep meals small and appetising.	•Ondansetron acts at the chemoreceptor trigger zone (CTZ), medulla oblongata, and perhaps at the vagal level. It is more effective when combined with corticosteroids, but may cause headaches and flushing. •Cyclizine acts at the medulla oblongata and can cause sedation. •Levomepromazine has effects at all levels of the nausea and vomiting pathway, but can cause sedation and postural hypotension. •Domperidone, recommended under caution in drug formularies, such as the British National Formulary for Children (BNFC), acts at the vagal sympathetic level. •Metoclopramide, recommended under caution in drug formularies, such as the BNFC, acts at the vagal sympathetic level and may cause extrapyramidal adverse effects in children. •Dexamethasone may help to lower raised intracranial pressure when administered in short bursts. •Haloperidol acts at the CTZ and has anxiolytic benefits. •Chlopromazine acts at the CTZ and has sedation effects, but is contraindicated in epilepsy. •Prochloperazine acts at CTZ and vestibular centre, but has numerous adverse effects in children.
Constipation	•There may be more variability in bowel habit in children than in adults, so it is better to consider a reported change in bowel habit as a guide. •Examination of the perianal area should be kept to observation, and rectal examination avoided, as it is usually unnecessary. •Causes may include inactivity, neurological, metabolic, decreased gastrointestinal intake, fear of opening bowels because of pain, or drug related (e.g. opioid-induced constipation).	•Laxatives may be oral or enteral use, parenteral, or rectal preparations. They are predominantly softening agents or peristalsis-stimulating agents. •Oral softening agents are paraffin, surfactants (such as sodium docusate), bulk forming, osmotic, and saline types. •Rectal softening agents are lubricants (such as olive oil), surfactants, osmotic, and saline (such as sodium phosphate). •Peristalsis-stimulating agents are anthracene (such as senna) or polyphenolics (such as bisacodyl). •Opioid antagonists, such as methylnaltrexone bromide, are agents prescribed for opioid-induced constipation refractory to other laxatives. They are μ receptor antagonists that do not cross the blood/brain barrier, and therefore, do not reverse opioid analgesia. These agents are given by the parenteral route. They can have an immediate effect, and patients need to be made aware of this.

The most frequently reported symptoms in life-limited children with malignant disease are pain, fatigue, and drowsiness; the most frequently reported ‘psychological’ symptoms are irritability and sleep disturbances.⁹ Challenging symptoms in life-limited children with non-malignant disease, who are often unable to communicate verbally, include pain, dyspnoea, nausea and vomiting, dysomnia, constipation, seizures, and changes in alertness.¹⁰ The National Institute for Health and Care Excellence guidelines for end-of-life care for infants, children, and young people with life-limiting conditions make the following recommendations for symptom management:³

• (i)Recognise the multidimensional nature of a symptom and evaluate all aspects where possible.
• (ii)Assess the most appropriate available tool, such as patient or carer reporting, validated observational tools, or tools that may be validated in other care settings.
• (iii)Start a management strategy that aims to alleviate the cause of the symptom and palliate its effects.
• (iv)Tailor a management plan to suit the child, care setting, and the skills of the caregiving team.
• (v)Monitor the management plan and make appropriate changes as required.
• (vi)Involve a specialist paediatric palliative care team to support the child and other members of the multidisciplinary team.

Advance care planning

These are discussions between healthcare professionals and competent children or young people, together with their primary carers, about goals of care, treatment options, and future care. In a life-limited child's care, these discussions are often triggered or anticipated when clarity about the potential risks and benefits of certain interventions for that child is being considered.

The following are the aims of advance care planning:

• (i)Consider the risks and benefits of future care options.
• (ii)Educate families and professionals about the anticipated clinical course of the illness.
• (iii)Enable families to make their own plans within this context if they wish.
• (iv)Minimise anxiety about a life-limited child's future care.

Parents' perspectives of advance care planning suggest that they value these discussions even if they do not make definitive decisions. They prefer to approach advance care planning as a process rather than a single conversation, thus reserving the option to change their views or wishes as understanding develops over time. Parents may choose to defer definitive decisions until an urgent situation arises. Therefore, planning for anticipated emergency events may involve preparation for several potential scenarios (see Case study 2).¹¹

Documentation of advance care planning must be clear and accessible to the child, young person, family, and healthcare professionals. For children and young people aged <18 yrs, there is no legal pathway for advance care planning, but the documentation is a record of discussions that have taken place. Recommended Summary Plan for Emergency Care and Treatment is an example of the process of advance care planning for adults, children, and young people, proposed by the Resuscitation Council (UK).¹² Another example is the CYPACP document produced by the Child and Young Person's Advance Care Plan Collaborative.¹³ These allow professionals to record discussions and make personalised recommendations for a person's clinical care in an emergency, in which they are unable to make or express choices for themselves. These plans are disseminated to the patient or parent and healthcare professionals in all care settings, including ambulance services. This enables responding emergency services to be aware of recommendations and wishes that the patient has made previously, and helps them to make immediate decisions at the time. These are recommendations and not legal directives.

Bereavement care

The loss of a child is rare and unusual in the UK, and there is a wider impact on the child's siblings, extended family, school, and local community. The family may already anticipate the death of their child and are able to make memories, but parents have often commented that no preparation is adequate for what lies ahead. Theories about grief and loss include views on effective ways of coping with grief, and some models advocate coming to terms with loss, whereas others emphasise finding ways of developing a new relationship with the deceased in the bereaved person's life.¹⁴ It is helpful for professionals to be aware of bereavement theories without the expectation that bereaved families will fit into these models. Palliative care teams are helpful to support ward-based or community care teams in exploring these bereavement issues, and having challenging conversations about death and dying with the patient, families, and wider community from which they come. When a child is dying in a hospital, it is helpful to provide opportunities for the professional team looking after the child to discuss this challenging clinical situation. Referral to palliative care teams will enable these pre-bereavement briefings or ‘prebriefs’ to take place in the ward setting, and allows emotional challenges of loss for professionals to be addressed as the child is being managed.

Summary

Paediatric palliative care is an active and total approach to caring for the life-limited infant, child, and young person that involves primary, secondary, and specialised healthcare professionals delivering integrated care to highly complex patients. It recognises that such patients have a unique experience of healthcare, in which the expectation is that they will ultimately succumb to their condition. Therefore, the aims are to provide the best quality care in partnership with the individual's wishes and needs, and optimise their potential at any point during the course of their disease.

Declaration of interest

The authors declare that they have no conflicts of interest.

MCQs

The associated MCQs (to support CME/CPD activity) will be accessible at www.bjaed.org/cme/home by subscribers to BJA Education.

Biographies

Ella Aidoo MRCPCH DipPallMed (paeds) has completed higher specialist training in paediatric palliative care and is about to take up a consultant post. Her areas of special interest include pain management and teaching.

Dilini Rajapakse FRCPCH MSc DipPallMed (paeds) is a consultant in paediatric palliative care and chronic pain management at Great Ormond Street Hospital. Her main areas of interest are pain management in non-malignant disease, training, and education.

Matrix codes: 1D02, 2C06, 3D00

Appendix 1

A sample of web-based resources for help and support with childhood bereavement include Winston's Wish (www.winstonswish.org.uk), Child Bereavement UK (www.childbereavement.org.uk), The Child Death Helpline (www.childdeathhelpline.org.uk), The Compassionate Friends (www.tcf.org.uk), and Sands: stillbirth & neonatal death charity (www.sands.org.uk).