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Best practice
How and when to refer a child for specialist paediatric palliative care
  1. Emily Harrop1,
  2. Clare Edwards2
  1. 1Paediatric Palliative Care, Helen & Douglas House Hospices, Oxford, UK
  2. 2Clinical Services, Helen & Douglas House Hospices, Oxford, UK
  1. Correspondence to Dr Emily Harrop, Consultant in Paediatric Palliative Care, Helen & Douglas House Hospices, 14A Magdalen Rd, Oxford OX4 1RW, UK; emilyharrop{at}yahoo.com, eharrop{at}helenanddouglas.org.uk

Abstract

Specialist paediatric palliative care is a relatively new area of paediatrics, and the interface with other disciplines can occasionally pose challenges for referrers due to lack of information about the diverse services available. Although services vary on a regional basis, there are common principles which may be used to guide and support referrals. Children may be referred to palliative care services via a number of routes from community-based primary care to regional tertiary centres. Identifying those most likely to benefit from the finite resources available can be a challenge, and healthcare professional's negative attitudes to palliative care have been further identified as a potentially modifiable barrier. This article aims to clarify the role of specialist paediatric palliative care, identify who should be eligible for such care, describe the services available (including those from children's hospices) and provide a tool for assessing some of the most challenging referrals. Many of the documents referenced can be downloaded from the Together for Short Lives website, and in many cases, there is no charge.

  • Palliative Care
  • Paediatric Practice
  • Pain
  • Neurodisability
  • Oncology

http://dx.doi.org/10.1136/archdischild-2012-303325

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    Introduction

    Palliative care for adults is a well-established discipline, with evidence that early engagement may both enhance and even prolong life when facing a life-limiting illness.1 While growing up alongside the adult models, paediatric palliative care is generally delivered over a longer time frame and for a wider range of conditions.2 The 1997 publication ‘A Guide to the Delivery of Children's Palliative Care Services’3 was one of the first documents to define palliative care for children and to suggest which categories of patients were most likely to benefit. This was written by the RCPCH (Royal College of Paediatrics and Child Health) in conjunction with the Association for Children with Life-Threatening or Terminal conditions and their families (ACT). ACT has since merged with Children's Hospices UK, to form the new charity Together for Short Lives.

    Palliative care for children and young people with life-limiting conditions was defined as: ‘An active and total approach to care, embracing physical, emotional, social and spiritual elements which focuses on enhancements of the quality of life for the child and support for the family and includes the management of distressing symptoms, provision of respite and care through death and bereavement’.3

    The statement above was further echoed by the WHO definition of paediatric palliative care in 1998, which concludes that such care ‘begins when illness is diagnosed and continues regardless of whether a child receives treatment targeted at the disease’.4 The International perspective was formalised in March 2006, when healthcare professionals from Europe, Canada, Lebanon and the USA met to define eligibility criteria and standards for paediatric palliative care in a document known as the IMPaCCT statement.5 This document used the same categories of patients previously defined by ACT and RCPCH.3

    How does this interface with other disciplines within paediatrics, or even the wider medical community? Specialist palliative care services for children may be delivered in a variety of settings. Consultant-led teams may be found within some children's hospices, as well as in tertiary children's hospitals, and occasionally within community-based services. Hospices and community services offering a specialist service will often ‘in-reach’ to local hospitals, as well as supporting palliative care in hospice, in school and at home. Core palliative care skills exist in most local community teams, among children's community nurses and general paediatricians/general practitioners (see figure 1).

    Figure 1
    Figure 1

    Pyramid of paediatric palliative care services.

    Who to refer

    Specialist palliative care is provided to children with life-threatening or life-shortening conditions who have an extremely wide range of diagnoses (in excess of 300), and there is an overlap with those with severe disabilities and complex needs. However, a significant proportion of children with palliative care needs (up to 15%) do not have a definitive underlying diagnosis.6 Unlike adult practice, oncology makes less than half of the work load for most specialist paediatric palliative care services, with inherited metabolic disease, neuromuscular diseases and acquired brain injury making up a significant proportion.

    Identifying children most likely to benefit from specialist paediatric palliative care, in a timely fashion, can pose a significant challenge. Referrals typically occur late in the illness trajectory with many children who would benefit not referred at all. Recent research from a tertiary hospital in the UK, with a well-established paediatric palliative care team, has shown that other healthcare professional's association of palliative care only with death and dying may be a modifiable factor relevant to late and non-referral.7 Various approaches to identifying appropriate patient populations have been explored on a worldwide basis. Examples include criteria based principally on lists of individual diagnoses (USA)8 or selection based on the existence of an advance directive, in the context of life-limiting/life-threatening diagnosis (Western Australia).9

    A more comprehensive European instrument known as the Paediatric Palliative Screening Scale (PaPaS scale) is currently in the final stages of development. This aims to support healthcare professionals in more accurately identifying children with palliative care needs. It is based on five domains: trajectory of disease and impact on daily activities; expected outcome of disease-directed treatment and burden of treatment; symptom burden; preferences of patient, parents or healthcare professional; and estimated life expectancy. Early validation of this tool has yielded promising results, and it may indeed become a lever to supporting timely referrals.10

    The process of referral

    Most paediatric palliative care services will accept initial referrals from any healthcare professional supporting the child, or in many cases from the family themselves. The first contact is often followed by a process of information gathering. In many cases, a clinician will therefore be asked to provide a medical summary including some prognostic information. They are likely to be asked whether they would be ‘surprised if the child survived beyond their 18th birthday’. The ‘surprise’ question originates from adult palliative care and has been used successfully to aid practitioners in recognising which patients are reaching the end of life, and therefore in need of enhanced support.11

    The distinction is made because dedicated paediatric palliative care services are usually focused on supporting children and young people with either life-limiting or life-threatening conditions that are considered not likely to adulthood. This support may start antenatally and in some cases continue beyond their 18th birthday when aiding transition to adult services. Care follows the child's illness trajectory, often from diagnosis to end-of-life care and through bereavement. As previously mentioned, the prognosis and trajectory can be difficult to predict, and a number of patterns have been recognised, whereby palliative care and other healthcare services, aimed at prolonging life, may be delivered alongside each other, in varying proportion (see figure 2).

    Figure 2
    Figure 2

    Disease trajectories.

    Referral criteria based on the presumption of death in childhood may feel harsh to many healthcare professionals working in paediatrics. We all recognise the burden of care faced by the families of children with relatively stable diseases involving complex neurodisability. In practice, the resources of palliative care teams are stretched and need to be focused on the children and families most likely to benefit from all their elements, including complex symptom management and end-of-life care. Children felt not to meet these criteria would usually be signposted to a more appropriate healthcare team, or a more appropriate respite provider.

    As described in figure 3, four categories of patients requiring palliative care have been defined by ACT and RCPCH. In some cases, the child's diagnosis alone makes them likely to meet referral criteria for palliative care (particularly Category 3). In other cases, a definable stage of the illness trajectory will provide a trigger for appropriate referral (Category 1). Children with static neurological disease (Category 4) are by far the hardest to assess.12 ,13

    Figure 3
    Figure 3

    Categories of patients requiring palliative care.

    Assessing referrals where prognosis is challenging

    Although children with static neurological disease make up a significant proposition of the paediatric palliative care population, they have historically been the most likely to have their referral declined. This is partly because it is recognised that only around 18% of children with cerebral palsy die in childhood.14 In recognition of the difficulty involved in referring and assessing children with static neurological conditions, a tool has recently been developed by the multidisciplinary team at Helen & Douglas House Hospices, Oxford (figure 4).

    Figure 4
    Figure 4

    Evidence-based guide to vulnerability factors for a child with a static neurological condition.

    This was produced by examining the referral documents currently in use by a number of children hospices and evidencing practice more rigorously by a comprehensive literature review on the prognostic indicators available for this patient group.14–21 The resulting tool is based on a ‘traffic lights’ system rather than numeric values, in recognition of the low level of certainty involved. It is also available online free of charge, via the Together for Short Lives website. The tool comprises one side of A4 paper and is relatively easy to use. To date it has led to a reduction in declined referrals within our own service from 79% to 25% for this category of patient over a 2-year period.12

    Examples of services which may be available from providers of Paediatric Palliative Care

    1. Medically supported respite for life-limited or life-threatened children. This is usually offered for a defined number of nights per year, depending on the review of a multidisciplinary panel, and may be delivered in a children's hospice or in the child's own home (by a community team). In the case of a children's hospice, children will often initially stay in-house with their family, who would be accommodated in a family flat, before eventually being able to come for respite on their own. Children are seen by a doctor or senior nurse with palliative care experience during their respite in most services and are usually able to have a review of their palliative care needs. Other important interventions such as advanced care planning and the writing of symptom management plans may eventually be supported, as the team get to know the child and family.

    2. Acute admissions for the management of poorly controlled symptoms. Children may come in to an inpatient setting (hospice or hospital) at short notice for review of difficult symptoms, and sometimes to be initiated on specialist treatment such as subcutaneous syringe drivers, buccal medication, second line opioids or patient-controlled analgesia. They are often able to return to the community (if preferred) with support from local services working with the palliative care team.

    3. Stepped discharge from hospital. Children already known to a hospice or specialist community palliative service may be able to be discharged earlier from hospital following admissions for elective surgery or intercurrent illnesses. This allows a period of convalescence in a less medically intensive setting (or even in their own home) and may allow parents enhanced support in learning new techniques, such as gastrostomy feeding or tracheostomy care. Discharge from hospital at a time of increased vulnerability may also be a time to consider a new referral to palliative care if the child is not already known to services.

    4. End-of-life care. This may involve a new emergency referral or relate to the ongoing care of a known patient. Children should be supported in the family's choice of setting. It is recognised that this may be home, hospice or hospital. Ideally, paediatric palliative care teams will be able to support the child's end-of-life care needs wherever the family choose to be, working closely with the acute medical teams involved.

    5. Compassionate withdrawal of life-sustaining treatment (eg, ventilation). Specialist paediatric palliative care teams are often able to support the out-of-hospital withdrawal of life-sustaining treatment, allowing families to make an active choice at the time that further treatment is felt to be futile or against a child's best interests. ACT produced a guide to support extubation within a children's hospice framework in 2011 after consulting widely with teams from palliative care, neonatal intensive care unit (NICU), paediatric intensive care unit (PICU) and ethics.22 Much of the information in the guide is also useful in other non-hospital settings, such as the family's own home. Families are able to receive support for parents and siblings from many different providers of palliative care, and most hospices also have access to a ‘cold room’ facility which allows the child to remain at the hospice for a number of days after death with continued access possible for family and friends. In the case of home extubation, a similar service following death may be made possible with the use of portable ‘cooling’ mattresses or cots, particularly in the case of neonates.

    6. Specific support for sibling. Most children's hospices and some community palliative care teams run programmes of activities for the siblings of children with life-limiting illnesses and/or bereaved siblings. This may involve weekend activities or even siblings’ camps, aimed at normalising the experience of having a chronically ill brother or sister.

    7. Specific child bereavement services. Emotional support during palliative care and through bereavement is available within most specialist paediatric palliative care services. This usually involves individual support sessions, and in some settings, there may also be dedicated remembrance days.

    Summary

    • Referrals for paediatric palliative care should ideally be made early enough to allow the hospice or other specialist palliative care team to form a relationship with the child and family.7

    • The most appropriate referrals are for children not expected to live to adulthood, but it is acknowledged that prognosticating can be an imprecise science.

    • For those children who exceed expectation, support during transition to adult services can be supported by providers of specialist paediatric palliative care.

    • Historically, there have been different approaches to identifying populations most likely to benefit from paediatric palliative care; a newer comprehensive scale (PaPaS Scale) may improve the situation.10

    • A new tool is available to support the assessment of children with static neurological conditions, with respect to the eligibility for specialist paediatric palliative care.12

    • A large amount of good quality information is available on line; in most cases, this is free of charge. For example, Together for Short Lives has produced a number of helpful guides to providing palliative care for children covering the stages of the process and specifically relating to the care of neonates and adolescents facing transition.22–27

    • Healthcare professionals prescribing in paediatric palliative care may benefit from the Association of Paediatric Palliative Medicine Master Formulary,26 which contains prescribing information specific to children's palliative care, supported by the best evidence available. A third edition is currently being produced for publication in 2014.

    • Research has shown that care coordination can be one of the most daunting and often unsuccessful elements of a family's palliative care experience—dealing with a wide range of care providers who may approach the same issue differently or treat the child in an unfamiliar manner can provide undue stress in circumstances that are already potentially traumatic and upsetting.28

    • Getting to know your local paediatric palliative care team (whether in a hospice, community setting or tertiary hospital) is likely to help with the delivery of joined-up care for life-limited children within individual paediatric disciplines. In the UK, the Together for Short Lives website is likely to be able to direct you to a service within your local area.

    Test your knowledge

    One or all answers may be correct

    1. Specialist paediatric palliative care teams usually accept referrals:

      1. From birth to 18 years

      2. Only from medically qualified referrers

      3. Based only on the dependency of the child

      4. Only after lengthy consideration

      5. In a way that aims to be equitable and transparent

    2. The children who are the most difficult to assess fairly and accurately are:

      1. Those with terminal cancer

      2. Those with progressive neurological diseases

      3. Those with metabolic diseases

      4. Those with neuromuscular progressive illness

      5. Those with static neurological diseases

    3. Children's hospices receive most of their funding from:

      1. Generous subsidy from the government

      2. The NHS nationally

      3. Local CCGs

      4. Local NHS trusts

      5. Around 85% by charitable fundraising

      6. Father Christmas/Easter Bunny/Tooth Fairy

    4. Specialist palliative care for children may include:

      1. End-of-life care

      2. Compassionate extubation out of hospital

      3. Stepped discharges from acute hospitals

      4. Symptom management alongside other long-term therapy

      5. Specialised respite care

      6. Support for siblings

      7. Support for the family during life and following bereavement

    5. Palliative care for children can be delivered to a high standard:

      1. In a children's hospice

      2. In a child's home

      3. In a hospital ward

      4. In a child's school

      5. While in the care of grandparents or other family members

    Answers to the quiz are on page 208.

    Answers from the quiz and explanatory notes

    • E is true

    • Most services will accept referrals antenatally, up until adolescence. Referrals can usually be made by anyone working with the child, and often self-referrals by families are also considered. The physical dependency of a child does not always correlate with their prognosis (ie, may be highly dependent but stable). Most services can accept fast tracked emergency referrals, particularly for episodes of care such as withdrawal of ventilation outside of hospital. Most hospice referral panels now have wide multiprofessional representation and work with assessment tools and guidelines, in order to be equitable and transparent.

    • E is true

    • Children with all kinds of progressive disease are usually easier to predict in terms of their prognosis. Children with oncological disease, for whom curative treatment is no longer likely to succeed, are easy to accept in parallel with any remaining ongoing active treatment. Children in ACT Category 4, with conditions such as cerebral palsy, are historically the most likely to be referred inappropriately. This article describes an evidence-based tool to support those referring children with static brain injury to children's palliative care services.

    • E is true

    • Children's hospices receive much less funding from healthcare services than do adult hospices and are much more reliant on charitable donations. The palliative care funding review is unlikely to make a difference overnight, but may introduce ‘tariffs’ for certain elements of care, which could then be centrally commissioned. Please support your local children's hospice. There are currently 49 in the UK.

    • All are true

    • Children's palliative care teams vary in how they go about offering care, but all would seek to offer care to the whole family, including bereavement support. This may be through patient support or outreach. Different elements of the service will be provided by doctors of differing levels of specialist training, working closely with well-qualified nurses.

    • All are true

    • Choice of place of care is paramount. A child who has known an oncology ward and its staff for many years may not want to be transferred anywhere else for end-of-life care, but a hospice in reach may be possible. Some children who have lived most of their recent life in specialist residential school may be least stressed by remaining there (with outreach support in place). Children's community services can work with specialist palliative care teams to plan for care in family homes. Use of advance care plans and symptom management plans allows the care to move with the child and family.

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    Footnotes

    • Contributors CE and I worked on this project jointly. She provided useful insight as a Director of a clinical service (from a Nursing background).

    • Competing interests None.

    • Provenance and peer review Commissioned; externally peer reviewed.