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Developments in paediatric palliative care
OCCASIONAL REVIEW
Developments in paediatric palliative care
Child Health (RCPCH) define four categories of life limiting conditions (LLC) in children (Figure 1) and describe patterns of disease trajectories within each group. A wide range of LLC can be encompassed in this practical classification of young people who are likely to require PC. Over the past decade this classification has provided a template for service development and a means of classifying data on expected death in childhood. It has also led to recognition of the considerable overlap between children with LLC and those with complex healthcare needs and disability. As highlighted by Professor Sir Alan Craft in his independent review of 2007, PC may form a thread throughout the lives of many children with complex needs. PPC is concerned with symptom control and maintaining quality of life, not only during the end of life phase, but from the time of diagnosis of an LLC. ‘Parallel planning’ is an approach through which professionals can introduce discussions about PC and end of life care planning alongside the continuation of other aspects of medical care and, for some, potentially curative treatment. This approach may allow families to maintain hope for the future but also permit early introduction of support from PC services.
Charlotte Mellor Emma Heckford Jo Frost
Abstract This article tracks the development of paediatric palliative care from the appreciation of children’s entitlement to high quality palliative care in the 1970s through to the formal recognition of paediatric palliative medicine as a subspecialty by the RCPCH in 2009. It summarizes recommendations from recent key documents with respect to the development of equitable and sustainable paediatric palliative care services and it also outlines opportunities for training in this evolving specialty. We then describe best practice in three areas of paediatric palliative care that are topical at present; advance care planning, transitional care and extubation within a palliative care framework. The principles of practice in each of these areas can be applied to much of paediatrics and it remains the case that much of palliative medicine in children can and should be provided by generalists. However, it is hoped that increased awareness of how paediatric palliative care services are evolving and the specialist care that is available, will enable enhanced collaboration with specialist services when required.
The evolution of PPC Adult hospices have existed since the 11th century but their popularity has varied over the ages. They experienced a sustained revival in the mid 19th century when there was increasing
Keywords advance care planning; child; extubation; palliative care; service development; training; transition
Normality
Group II Group I
Introduction In the United Kingdom there are an estimated 23,500 children and young people not expected to live to reach adulthood. Palliative care (PC) is now widely recognized as an important component of paediatrics. The underlying philosophy of PC overlaps in many ways with the approach taken by all professionals working in paediatrics. Paediatric palliative care (PPC) was defined in 2007 as ‘an active and total approach to the care of children and young people with life-limiting conditions, embracing physical, emotional, social and spiritual elements through to death and beyond.’ The Association for Children’s Palliative Care (ACT) and the Royal College of Paediatrics and
Group III
0
10
20
Time (years) Group I – Life-threatening conditions for which treatment is possible but may fail, e.g. cancer Group II – Conditions where there may be long periods of intensive treatment aimed at prolonging life, but premature death is still possible, e.g. cystic fibrosis
Charlotte Mellor MBChB MRCPCH Dip Pall Med is SpR Paediatrics at the Bristol Royal Hospital for Children, Bristol, UK. Conflict of interest: none.
Group III – Progressive conditions without curative treatment options where treatment is exclusively palliative and may extend over many years, e.g. Batten disease
Emma Heckford MBBS MRCPCH Dip Pall Med is SpR Paediatric Palliative Medicine at the University Hospital for Wales, Cardiff, UK. Conflict of interest: none.
Group IV – Conditions with severe neurological disability, which may cause susceptibility to health complications. Patients may deteriorate unpredictably but conditions are not considered to be progressive e.g. severe cerebral palsy
Jo Frost MBChB MRCPCH Dip Pall Med is Associate Specialist Community Paediatrics at the Children’s Hospice South West, Charlton Farm Children’s Hospice, Wraxall, North Somerset, UK. Conflict of interest: none.
PAEDIATRICS AND CHILD HEALTH 22:3
Group IV
Death
Figure 1 Disease trajectories followed by children with life-limiting illnesses in ACT/RCPCH categories IeIV (reproduced with permission from Dr R Hain).
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OCCASIONAL REVIEW
recognition of the needs of terminally ill adults. Cicely Saunders, who has become widely regarded as the founder of modern palliative medicine in adults, founded St. Christopher’s Hospice in London in 1967. Recognition of the need to provide PC for children occurred relatively recently and was first alluded to in articles by Chapman and Goodall in the late 1970s. The growing awareness around that time, of the existence and achievements of adult PC was clearly influential and contributed to paediatricians’ recognition of the need to extend a palliative approach to children with lifelimiting conditions. Helen House, the world’s first children’s hospice opened in Oxfordshire in 1982. Since the 1970s, various models of PPC service provision have developed. Individual services have evolved in different parts of the UK in response to local need, local resources, existing service configuration and the vision of motivated professionals in that area. More recently, there has been considerable effort, at regional and national levels, to promote the development of equitable and sustainable services across the UK. The government have identified community PPC provision, short breaks and regional PPC networks as key priorities and are re-examining the way in which PC provision is funded. ACT and Children’s Hospices UK (CHUK), now a joint organisation known as ‘Together for short lives’ have been instrumental in defining PPC standards, identifying a workforce development strategy, outlining key PC pathways for children and providing a forum for sharing of key resources. Regional PPC networks present excellent opportunities for training and for collaborative working between PC providers from all sectors. They also facilitate consolidation of data about local population need and service provision. Since 2009 paediatric palliative medicine has been recognized as a paediatric subspecialty by the RCPCH. Although it shares many common goals with adult PC, there are a number of important distinctions to be made. Children with PC needs have a very diverse range of conditions, many of which are rare or even undiagnosed. Although categories of disease trajectory have been identified for children with LLC (Figure 1), predicting the trajectory for any one particular child remains challenging. It is common for children, particularly those with neurological conditions, to ‘rehearse’ their death on a number of occasions before they die. In PPC there are also specific issues to be considered with regard to child development and family dynamics. Paediatric palliative medicine is recognized as a distinct paediatric subspecialty because, whilst generic PPC can be provided by many paediatric practitioners, the provision of specialist PPC requires specific specialist skills (see Box 1).
Specialist skills required of PPC practitioners C C
C
C
C C
Box 1
Formal recognition by RCPCH of children’s PC as a subspecialty. Published in 2008, ‘Better Care, Better Lives: Improving outcomes and experiences for children, young people and their families living with life limiting and life threatening conditions,’ was the first national strategy for PPC. It set out a vision for equitable access to high quality family-centred sustainable care and support, with services provided in a setting of the child and family’s choice. In July 2010 the Department of Health announced the availability of £30 million to fund PPC projects. This was hugely welcomed and led to a surge in innovative projects which have resulted in a wealth of new evidence on which to base service development and also many valuable new resources to support practice. The details and outcomes of these projects are available at www.act30millionstars.org.uk. The ‘Palliative Care Funding Review’ of 2011 assesses the current funding mechanisms for dedicated PC for adults and children in England. The review proposes a per patient NHS tariff to meet the needs of the patient regardless of care provider. The proposed tariff aims to be fair to all providers (with the tariff following the patient across hospital, hospice or home); to support choice in location of care, with the aim of avoiding end of life care and death in hospital, and to encourage development of community based PC services. The key recommendations of this document are summarized in Box 2. Implementation of these recommendations within PPC raises a number of challenges and questions. There are unanswered questions about how the proposed classification system for phase of illness and problem severity would translate into practice and who would be appropriately skilled and qualified to provide assessments. It is also not yet clear what a minimum national dataset for children should contain. The need for the development of 24/7 community PC services to allow genuine, equitable choice of place of care to children and families has already been recognized but the challenges around putting such a service in place remain. Pilot sites for the proposed paediatric tariff are to be identified.
Recent key documents related to the provision and funding of PPC services The Craft and Killen palliative care services review (2007) highlighted concerns regarding the sustainability of services and reported on families’ requests for more community services available to offer help and advice 24 h a day 7 days a week, including at the end of life. Recommendations included: Improved planning and commissioning of services at a regional level supported by regional and local PC networks. Increased focus on community services to help manage and support children with PC needs to stay at home.
PAEDIATRICS AND CHILD HEALTH 22:3
Ability to manage complex pain and other symptoms Ability to understand the range and characteristics of LLC in children Understanding of how children may die and experience of the dying process Skills for planning and facilitating family choices about care (including care outside the acute care setting) Advanced communication skills Knowledge and experience of managing complex ethical dilemmas
Training in paediatric palliative medicine (PPM) In line with the ad hoc development of services, PPM doctors have historically come from a range of backgrounds including
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OCCASIONAL REVIEW
Following the appointment of the first UK Consultant in Paediatric Palliative Medicine in 1990 the creation of new posts has been slow. Currently in the UK, there are 10 tertiary PPM consultants and approximately 50 PWSIs. The challenges in developing new consultant posts mirror those in service development. In view of the small numbers of children over large geographical areas, in reality only a small number of tertiary specialists will be needed nationwide to provide regional leadership, whilst many paediatricians with a special interest working locally are needed to facilitate the subspecialty’s vision of providing a genuine choice of place of care for children and their families (Figure 2).
Key recommendations of The Palliative Care Funding Review 2011 C
C
C
C
C
C
C
C
A per-patient funding model using a classification system based on age, phase of illness and a measure of complexity. The tariff would be set nationally, but commissioned and delivered locally. Palliative care services for children would be commissioned on a population basis of up to 1.5 million. An end of life locality register should be maintained by every commissioning locality group to support the capture, storage and sharing of electronic patient records. When a patient is placed on the end of life locality register the NHS will meet both their health and social care needs. A palliative care needs assessment should be undertaken by an appropriately skilled practitioner. This informs palliative care provision, including coordination of care; reassessments should be undertaken in response to a change in the level of need of the patient. A lead palliative care provider in every Clinical Commissioning Group should coordinate all palliative care services, including those not funded by the NHS. Outcome measures, supported by the newly created dataset for the tariff and the NHS outcomes framework should be developed. There should be a standard contract for commissioning NHS palliative care services. Services, to provide 24/7 access to community care across the country. Palliative care education and training for NHS professionals should be funded by the NHS. All other professionals should be trained to standards which should be outlined in the standard contract for commissioning NHS palliative care services and should be funded by the provider organization.
Current hot topics in PPC Advance care planning Supporting families of children and young people with LLC in planning for their care towards the end of life is an important responsibility of professionals working with this patient group. An advance care plan (ACP) aims to document the wishes of a child or young person and their family about the care they wish to receive towards and at the end of life; it may contain information about resuscitation choices but such decisions form one part of a holistic plan of care. Many children living with LLC in the UK have complex healthcare needs often met by professionals working in a number of settings across primary, secondary and tertiary care. When one also considers social, educational and spiritual needs the number of professionals and potential places of care increases further; such professionals might be working in social care or education settings and in the statutory or voluntary sectors. An ACP acts as a communication tool, encouraging discussion between families and professionals as well as inter-professional working. It acts to ensure that all who care for a child or young person might be aware of their and their family’s wishes in the event of a sudden and or significant deterioration in their health and serves to ensure that families are not having similar, potentially distressing discussions about end of life care with many different professionals. It is hoped that an ACP might serve not only to avoid inappropriate or unwanted interventions, such as cardiopulmonary resuscitation but, importantly, ensure that PC might be accessed in the way a family wishes it to be. ACP templates have been developed by a number of groups around the UK. The Wishes Document from Bristol and The West Midlands Children and Young People’s Palliative Care Toolkit’s advance care plan are both available to download from www.act. org.uk. Whilst these are helpful references professionals should recognize that successful implementation of an ACP for an individual is likely to require multi-agency support and so local recognition and approval of a template by all appropriate local professionals will be key to its successful implementation. Proposed content of an ACP can be found in Box 3. Pace of completion of the plan should be dictated by the patient and family and they may choose to leave some sections unfilled. For children and young people ACPs carry the same legal weight as any other plan for care documented in healthcare records. ACPs for children and young people should not be confused with advanced directives, which allow adults with capacity (18 years and over) to make advance decisions to refuse specific treatments. Such advance decisions, if valid, are legally
Box 2
community paediatrics, general paediatrics, paediatric oncology, general practice and adult palliative medicine. These professionals have worked in a number of settings including hospitals, hospices and the community. Over the past 5 years significant steps have been taken to standardize the training of these doctors with the development of a recognized set of competencies. A curriculum for PPM was created in 2008 by the education and training subgroup of the British Society for Paediatric Palliative Medicine, which has subsequently become the Association for Paediatric Palliative Medicine (APPM). This document lays out the competencies expected of general practitioners (GPs) or paediatricians who wish to develop a special interest (PWSI) in PPM and of those who are completing one of the two grid training posts with the intention of becoming consultants in PPM. There are currently grid posts in Cardiff and Great Ormond Street; creation of a third is proposed. It is planned that, in the near future, guidance will be available from APPM about the training requirements for doctors who wish to develop an interest in PPM. This will enable a clearer understanding of what is expected of these doctors and also more formal recognition of their status.
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OCCASIONAL REVIEW
2006 Secretary of State for Health committed £27 million over 3 years to support Children’s hospices and commissioned an independant children’s palliative care services review.
2007 Craft and Killeen palliative care services review. 2008 “Better Care, Better Lives: improving outcomes and experiences for children, young people and their families living with life limiting and life threatening conditions”: The first ever National Strategy for Children’s Palliative Care.
1997
2009
ACT “A guide to the development of Children’s palliative care services” first published definitions of children’s palliative care and the numbers and needs of LLC.
Recognition of Paediatric Palliative Medicine as a sub-specialty by RCPCH. 2010 Department of health £30 million funding for palliative care projects.
2003 2011
Big Lottery funding grants funded development of specialist community nursing teams and hospice services.
The Palliative care funding review A review of funding of the current funding mechanisms for dedicated palliative care for adults and children in England.
Figure 2 Key policy documents, funding arrangements and commissioning arrangements influencing the development of PPC services.
binding and doctors who initiate contravening treatments could face legal action. Young patients and their families with ACPs should be aware that healthcare professionals remain obliged to instigate the treatment they consider to be in the best interests of the young person at any point and, although unusual, such action may not be consistent with the ACP. In addition, patients and families are able to change their minds and consider options different to those described in their ACP at any point. The question of when to start the planning process is often challenging. The lives of young people may be limited by any one of a large number of conditions, following different disease trajectories as described above. Although confidence and accuracy in predicting death increases as death approaches; predicting the timing of end of life remains challenging for
professionals. This uncertainty may deter professionals from starting the planning process. However, if one chooses to delay advance care planning until a critical event occurs this may lead to discussions taking place with a time pressure, with likely heightened emotional distress and possibly with a team of professionals who the family does not know well. It would seem better for a number of conversations to take place as part of a longitudinal process with the concept of advance care planning being introduced soon after diagnosis of an LLC instead of in response to individual crises. Introducing advance care planning and the concept of PC early on may also serve to ensure a sense of openness, attention to the child’s quality of life and greater opportunity for families to engage in making choices. Transition Transition has been described as ‘a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic physical and mental conditions as they move from child-centred to adult-oriented healthcare systems.’ As increasing numbers of children with LLC are surviving to adulthood we must plan how best to meet their ongoing PC needs. Adolescence is a time of change and uncertainty for any young person. For some with LLC adolescence may coincide cruelly with deterioration in their health and an increase in their dependence on family members. Facilitating independence in living arrangements, employment and healthcare are all important aspects of transition planning. Effective transition relies on forward planning, co-ordination, and excellent communication. It is recognized that transition for
Proposed content of an ACP C C C C C
C
Patient & family’s demographic information Background diagnosis & brief summary of health needs Details of involved professionals Patient and family’s wishes during life Plans for care if the child becomes more unwell (including preferred place of care and treatment options) Wishes for care around the time of and after death (including place of care, interest in organ and tissue donation, spiritual and cultural wishes)
Box 3
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OCCASIONAL REVIEW
children who solely have health needs should ideally start to be implemented 1 year prior to an anticipated transfer date. For young people with complex needs effective inter-disciplinary and multidisciplinary working is vital to facilitate a successful transfer package and this work may need to take place over a longer timeframe. Barriers to transition are described in Box 4. Service-based restrictions may be a particular challenge for those with PC needs. For children with progressive conditions where curative treatment is not available or those with severe neurodisability (ACT/RCPCH categories 3 and 4) there is often a question mark over which medical professional should take on their care in adulthood. Many GPs feel uncomfortable dealing with dying children. Throughout childhood, the GP will more often than not take a step back allowing a general or community paediatrician fulfil the role that a primary healthcare provider might for adults. On reaching adulthood, the PC needs for such young people may lead them to fall between two stools e too specialist to be met by primary care yet not within the remit of specialist adult palliative medicine services. The need to meet the requirements of these young adults has been recognized by ACT who, through the publication of their transition care pathway identify key stages to be negotiated to implement a successful transition. They describe three phases of transition; recognizing the need to move on, moving on and recognition of the end of life. The core principles that they identify: involvement of the young person in decision making, flexibility and choice, continuity of support, anticipation of needs and the execution of individually tailored plans are central to the process of transition being a success for these young people.
Barriers to effective transition
C C C C
C
Box 4
collaboration with expert professionals and offers guidance on decision making, working with the family to develop an end of life plan for their child, the practicalities of withdrawing ventilation and care at the time of death. Examples of the guidance contained in this document are shown in Box 5. The ACT guidance can be used to support the process of palliative extubation in any setting, including acute care. It is hoped that it’s existence will help prompt professionals to offer
Care pathway guidance: ‘A Care Pathway to Support Extubation within a Children’s Palliative Care Framework’ 1. The journey towards a decision to withdraw ventilation The ethical and legal considerations Offering choices and making plans: Location of death Who will be involved (core staff, family, friends) Wishes, goals, ceremonies and memory building Resuscitation decisions Symptom control Discussions about organ donation Discussions about post mortem Emotional support
Extubation in a palliative care framework One of the key goals of PPC is to facilitate the choices of children and families. In the past it has been assumed that certain levels of care precluded transfer from a paediatric/neonatal intensive care unit or hospital. Recently, more emphasis has been placed on the choices that can be offered even when a high level of invasive care has been ongoing until that time. In some areas and on some occasions it is now possible to facilitate transfer out of the acute setting to either the child’s home or a hospice and for withdrawal of intensive care to take place there. This opportunity to spend time with their child outside a clinical environment is much valued by families. However, the availability of this service is patchy and practice is variable as, until now, it has been dependent on local resources and the willingness and availability of individuals in that area. There are clearly a number of challenges that face professionals looking after children for whom further invasive care is likely to be futile, not least guiding the family through discussions about how their child’s best interests might be met by discontinuing artificial ventilation and how and where this might be possible. It is then of utmost importance that extubation is carried out sensitively and without avoidable complications, with considerable attention to emotional and psychological support for the whole family. In an effort to support individuals who are increasingly being called upon to provide this service, in 2011 ACT published ‘A Care Pathway to Support Extubation within a Children’s Palliative Care Framework’ (see Box 5). It was developed in
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Poor communication Poor planning Service-based restrictions e e.g. a lack of equivalent services Lack of confidence in colleagues Lack of experience in the adult sector of conditions with which children previously did not survive into adulthood Patient and family preference to avoid transition to new services
C
Parallel planning for ongoing care if child does not die after extubation 2. The practicalities of withdrawing ventilation Developing an extubation plan Reviewing plan with family after transfer out of acute setting Extubation checklist Planning for imminent death and also longer term survival 3. Care at the time of death Enabling end of life wishes Ceremonies and memory building Practical information about choices and procedures after death Bereavement support
Association for Children’s Palliative Care, 2011 Box 5
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OCCASIONAL REVIEW
FURTHER READING A care pathway to support extubation within a Children’s Palliative Care Framework, ACT. 1st Edn., 2011. A guide to the development of children’s palliative care services, ACT. 3rd Edn., 2009. Better care, better lives: improving outcomes and experiences for children, young people and their families living with life limiting and life threatening conditions. Department of Health, 2008. Blum R, et al. Transition from child-centered to adult healthcare systems for adolescents with chronic conditions. J Adolesc Health 1993; 14: 570e6. Brook L, Hain R. Predicting death in children. Arch Dis Child 2008; 93: 1067e70. Craft A, Killen S. Palliative care services for children and young people in England. An independent review for the Secretary of State for Health. Department of Health, 2007. Fraser J, et al. Advanced care planning for children with life-limiting conditions e the Wishes Document. Arch Dis Child 2010; 95: 79e82. Hain R, et al. Curriculum in paediatric palliative medicine. British Society of Paediatric Palliative Medicine, www.act.org.uk; 2008. Hughes-Hallett T, et al. The Palliative care funding review: funding the right care for everyone; an independent review for the Secretary of State for Health. Department of Health, 2011. The Transition Care Pathway, ACT. 1st Edn., 2007. Wolff T, Browne J. Organizing end of life care: parallel planning. Paediatr Child Health 2011; 21: 378e84. World Health Organisation. WHO definition of Palliative Care 1998, http://www.who.int/cancer/palliative/definition/en/; October 2011. www.act30millionstars.org.uk.
choices and to know how they can facilitate these choices for families. It is also hoped that improving parents’ memories of the end of their child’s life will offer them some comfort in bereavement.
Summary There is growing recognition of the importance of high quality PC for children and young people as greater numbers of young people survive conditions that would previously have led to their demise in early childhood. Doing this work well and according to the wishes of children and their families requires improved commissioning of local and regional services, joined up working across education, health and social care and across statutory and voluntary sectors. These challenges and how best to meet them are described in a number of key documents described in this article. The medical profession have also recognized the importance of good PC for children by formalizing training for PPC doctors. Whilst it is important that some professionals have the opportunity to develop specialist paediatric palliative medicine skills and that numbers of paediatric palliative medicine consultants increase it remains true that all doctors working with children have a duty to develop a generic skill base that enables them to contribute effectively to the work of teams of professionals caring for life-limited children and their families.
Funding None required
PAEDIATRICS AND CHILD HEALTH 22:3
A
120
Ó 2011 Elsevier Ltd. All rights reserved.
Developments in paediatric palliative care
Child Health (RCPCH) define four categories of life limiting conditions (LLC) in children (Figure 1) and describe patterns of disease trajectories within each group. A wide range of LLC can be encompassed in this practical classification of young people who are likely to require PC. Over the past decade this classification has provided a template for service development and a means of classifying data on expected death in childhood. It has also led to recognition of the considerable overlap between children with LLC and those with complex healthcare needs and disability. As highlighted by Professor Sir Alan Craft in his independent review of 2007, PC may form a thread throughout the lives of many children with complex needs. PPC is concerned with symptom control and maintaining quality of life, not only during the end of life phase, but from the time of diagnosis of an LLC. ‘Parallel planning’ is an approach through which professionals can introduce discussions about PC and end of life care planning alongside the continuation of other aspects of medical care and, for some, potentially curative treatment. This approach may allow families to maintain hope for the future but also permit early introduction of support from PC services.
Charlotte Mellor Emma Heckford Jo Frost
Abstract This article tracks the development of paediatric palliative care from the appreciation of children’s entitlement to high quality palliative care in the 1970s through to the formal recognition of paediatric palliative medicine as a subspecialty by the RCPCH in 2009. It summarizes recommendations from recent key documents with respect to the development of equitable and sustainable paediatric palliative care services and it also outlines opportunities for training in this evolving specialty. We then describe best practice in three areas of paediatric palliative care that are topical at present; advance care planning, transitional care and extubation within a palliative care framework. The principles of practice in each of these areas can be applied to much of paediatrics and it remains the case that much of palliative medicine in children can and should be provided by generalists. However, it is hoped that increased awareness of how paediatric palliative care services are evolving and the specialist care that is available, will enable enhanced collaboration with specialist services when required.
The evolution of PPC Adult hospices have existed since the 11th century but their popularity has varied over the ages. They experienced a sustained revival in the mid 19th century when there was increasing
Keywords advance care planning; child; extubation; palliative care; service development; training; transition
Normality
Group II Group I
Introduction In the United Kingdom there are an estimated 23,500 children and young people not expected to live to reach adulthood. Palliative care (PC) is now widely recognized as an important component of paediatrics. The underlying philosophy of PC overlaps in many ways with the approach taken by all professionals working in paediatrics. Paediatric palliative care (PPC) was defined in 2007 as ‘an active and total approach to the care of children and young people with life-limiting conditions, embracing physical, emotional, social and spiritual elements through to death and beyond.’ The Association for Children’s Palliative Care (ACT) and the Royal College of Paediatrics and
Group III
0
10
20
Time (years) Group I – Life-threatening conditions for which treatment is possible but may fail, e.g. cancer Group II – Conditions where there may be long periods of intensive treatment aimed at prolonging life, but premature death is still possible, e.g. cystic fibrosis
Charlotte Mellor MBChB MRCPCH Dip Pall Med is SpR Paediatrics at the Bristol Royal Hospital for Children, Bristol, UK. Conflict of interest: none.
Group III – Progressive conditions without curative treatment options where treatment is exclusively palliative and may extend over many years, e.g. Batten disease
Emma Heckford MBBS MRCPCH Dip Pall Med is SpR Paediatric Palliative Medicine at the University Hospital for Wales, Cardiff, UK. Conflict of interest: none.
Group IV – Conditions with severe neurological disability, which may cause susceptibility to health complications. Patients may deteriorate unpredictably but conditions are not considered to be progressive e.g. severe cerebral palsy
Jo Frost MBChB MRCPCH Dip Pall Med is Associate Specialist Community Paediatrics at the Children’s Hospice South West, Charlton Farm Children’s Hospice, Wraxall, North Somerset, UK. Conflict of interest: none.
PAEDIATRICS AND CHILD HEALTH 22:3
Group IV
Death
Figure 1 Disease trajectories followed by children with life-limiting illnesses in ACT/RCPCH categories IeIV (reproduced with permission from Dr R Hain).
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Ó 2011 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
recognition of the needs of terminally ill adults. Cicely Saunders, who has become widely regarded as the founder of modern palliative medicine in adults, founded St. Christopher’s Hospice in London in 1967. Recognition of the need to provide PC for children occurred relatively recently and was first alluded to in articles by Chapman and Goodall in the late 1970s. The growing awareness around that time, of the existence and achievements of adult PC was clearly influential and contributed to paediatricians’ recognition of the need to extend a palliative approach to children with lifelimiting conditions. Helen House, the world’s first children’s hospice opened in Oxfordshire in 1982. Since the 1970s, various models of PPC service provision have developed. Individual services have evolved in different parts of the UK in response to local need, local resources, existing service configuration and the vision of motivated professionals in that area. More recently, there has been considerable effort, at regional and national levels, to promote the development of equitable and sustainable services across the UK. The government have identified community PPC provision, short breaks and regional PPC networks as key priorities and are re-examining the way in which PC provision is funded. ACT and Children’s Hospices UK (CHUK), now a joint organisation known as ‘Together for short lives’ have been instrumental in defining PPC standards, identifying a workforce development strategy, outlining key PC pathways for children and providing a forum for sharing of key resources. Regional PPC networks present excellent opportunities for training and for collaborative working between PC providers from all sectors. They also facilitate consolidation of data about local population need and service provision. Since 2009 paediatric palliative medicine has been recognized as a paediatric subspecialty by the RCPCH. Although it shares many common goals with adult PC, there are a number of important distinctions to be made. Children with PC needs have a very diverse range of conditions, many of which are rare or even undiagnosed. Although categories of disease trajectory have been identified for children with LLC (Figure 1), predicting the trajectory for any one particular child remains challenging. It is common for children, particularly those with neurological conditions, to ‘rehearse’ their death on a number of occasions before they die. In PPC there are also specific issues to be considered with regard to child development and family dynamics. Paediatric palliative medicine is recognized as a distinct paediatric subspecialty because, whilst generic PPC can be provided by many paediatric practitioners, the provision of specialist PPC requires specific specialist skills (see Box 1).
Specialist skills required of PPC practitioners C C
C
C
C C
Box 1
Formal recognition by RCPCH of children’s PC as a subspecialty. Published in 2008, ‘Better Care, Better Lives: Improving outcomes and experiences for children, young people and their families living with life limiting and life threatening conditions,’ was the first national strategy for PPC. It set out a vision for equitable access to high quality family-centred sustainable care and support, with services provided in a setting of the child and family’s choice. In July 2010 the Department of Health announced the availability of £30 million to fund PPC projects. This was hugely welcomed and led to a surge in innovative projects which have resulted in a wealth of new evidence on which to base service development and also many valuable new resources to support practice. The details and outcomes of these projects are available at www.act30millionstars.org.uk. The ‘Palliative Care Funding Review’ of 2011 assesses the current funding mechanisms for dedicated PC for adults and children in England. The review proposes a per patient NHS tariff to meet the needs of the patient regardless of care provider. The proposed tariff aims to be fair to all providers (with the tariff following the patient across hospital, hospice or home); to support choice in location of care, with the aim of avoiding end of life care and death in hospital, and to encourage development of community based PC services. The key recommendations of this document are summarized in Box 2. Implementation of these recommendations within PPC raises a number of challenges and questions. There are unanswered questions about how the proposed classification system for phase of illness and problem severity would translate into practice and who would be appropriately skilled and qualified to provide assessments. It is also not yet clear what a minimum national dataset for children should contain. The need for the development of 24/7 community PC services to allow genuine, equitable choice of place of care to children and families has already been recognized but the challenges around putting such a service in place remain. Pilot sites for the proposed paediatric tariff are to be identified.
Recent key documents related to the provision and funding of PPC services The Craft and Killen palliative care services review (2007) highlighted concerns regarding the sustainability of services and reported on families’ requests for more community services available to offer help and advice 24 h a day 7 days a week, including at the end of life. Recommendations included: Improved planning and commissioning of services at a regional level supported by regional and local PC networks. Increased focus on community services to help manage and support children with PC needs to stay at home.
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Ability to manage complex pain and other symptoms Ability to understand the range and characteristics of LLC in children Understanding of how children may die and experience of the dying process Skills for planning and facilitating family choices about care (including care outside the acute care setting) Advanced communication skills Knowledge and experience of managing complex ethical dilemmas
Training in paediatric palliative medicine (PPM) In line with the ad hoc development of services, PPM doctors have historically come from a range of backgrounds including
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Following the appointment of the first UK Consultant in Paediatric Palliative Medicine in 1990 the creation of new posts has been slow. Currently in the UK, there are 10 tertiary PPM consultants and approximately 50 PWSIs. The challenges in developing new consultant posts mirror those in service development. In view of the small numbers of children over large geographical areas, in reality only a small number of tertiary specialists will be needed nationwide to provide regional leadership, whilst many paediatricians with a special interest working locally are needed to facilitate the subspecialty’s vision of providing a genuine choice of place of care for children and their families (Figure 2).
Key recommendations of The Palliative Care Funding Review 2011 C
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A per-patient funding model using a classification system based on age, phase of illness and a measure of complexity. The tariff would be set nationally, but commissioned and delivered locally. Palliative care services for children would be commissioned on a population basis of up to 1.5 million. An end of life locality register should be maintained by every commissioning locality group to support the capture, storage and sharing of electronic patient records. When a patient is placed on the end of life locality register the NHS will meet both their health and social care needs. A palliative care needs assessment should be undertaken by an appropriately skilled practitioner. This informs palliative care provision, including coordination of care; reassessments should be undertaken in response to a change in the level of need of the patient. A lead palliative care provider in every Clinical Commissioning Group should coordinate all palliative care services, including those not funded by the NHS. Outcome measures, supported by the newly created dataset for the tariff and the NHS outcomes framework should be developed. There should be a standard contract for commissioning NHS palliative care services. Services, to provide 24/7 access to community care across the country. Palliative care education and training for NHS professionals should be funded by the NHS. All other professionals should be trained to standards which should be outlined in the standard contract for commissioning NHS palliative care services and should be funded by the provider organization.
Current hot topics in PPC Advance care planning Supporting families of children and young people with LLC in planning for their care towards the end of life is an important responsibility of professionals working with this patient group. An advance care plan (ACP) aims to document the wishes of a child or young person and their family about the care they wish to receive towards and at the end of life; it may contain information about resuscitation choices but such decisions form one part of a holistic plan of care. Many children living with LLC in the UK have complex healthcare needs often met by professionals working in a number of settings across primary, secondary and tertiary care. When one also considers social, educational and spiritual needs the number of professionals and potential places of care increases further; such professionals might be working in social care or education settings and in the statutory or voluntary sectors. An ACP acts as a communication tool, encouraging discussion between families and professionals as well as inter-professional working. It acts to ensure that all who care for a child or young person might be aware of their and their family’s wishes in the event of a sudden and or significant deterioration in their health and serves to ensure that families are not having similar, potentially distressing discussions about end of life care with many different professionals. It is hoped that an ACP might serve not only to avoid inappropriate or unwanted interventions, such as cardiopulmonary resuscitation but, importantly, ensure that PC might be accessed in the way a family wishes it to be. ACP templates have been developed by a number of groups around the UK. The Wishes Document from Bristol and The West Midlands Children and Young People’s Palliative Care Toolkit’s advance care plan are both available to download from www.act. org.uk. Whilst these are helpful references professionals should recognize that successful implementation of an ACP for an individual is likely to require multi-agency support and so local recognition and approval of a template by all appropriate local professionals will be key to its successful implementation. Proposed content of an ACP can be found in Box 3. Pace of completion of the plan should be dictated by the patient and family and they may choose to leave some sections unfilled. For children and young people ACPs carry the same legal weight as any other plan for care documented in healthcare records. ACPs for children and young people should not be confused with advanced directives, which allow adults with capacity (18 years and over) to make advance decisions to refuse specific treatments. Such advance decisions, if valid, are legally
Box 2
community paediatrics, general paediatrics, paediatric oncology, general practice and adult palliative medicine. These professionals have worked in a number of settings including hospitals, hospices and the community. Over the past 5 years significant steps have been taken to standardize the training of these doctors with the development of a recognized set of competencies. A curriculum for PPM was created in 2008 by the education and training subgroup of the British Society for Paediatric Palliative Medicine, which has subsequently become the Association for Paediatric Palliative Medicine (APPM). This document lays out the competencies expected of general practitioners (GPs) or paediatricians who wish to develop a special interest (PWSI) in PPM and of those who are completing one of the two grid training posts with the intention of becoming consultants in PPM. There are currently grid posts in Cardiff and Great Ormond Street; creation of a third is proposed. It is planned that, in the near future, guidance will be available from APPM about the training requirements for doctors who wish to develop an interest in PPM. This will enable a clearer understanding of what is expected of these doctors and also more formal recognition of their status.
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2006 Secretary of State for Health committed £27 million over 3 years to support Children’s hospices and commissioned an independant children’s palliative care services review.
2007 Craft and Killeen palliative care services review. 2008 “Better Care, Better Lives: improving outcomes and experiences for children, young people and their families living with life limiting and life threatening conditions”: The first ever National Strategy for Children’s Palliative Care.
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ACT “A guide to the development of Children’s palliative care services” first published definitions of children’s palliative care and the numbers and needs of LLC.
Recognition of Paediatric Palliative Medicine as a sub-specialty by RCPCH. 2010 Department of health £30 million funding for palliative care projects.
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Big Lottery funding grants funded development of specialist community nursing teams and hospice services.
The Palliative care funding review A review of funding of the current funding mechanisms for dedicated palliative care for adults and children in England.
Figure 2 Key policy documents, funding arrangements and commissioning arrangements influencing the development of PPC services.
binding and doctors who initiate contravening treatments could face legal action. Young patients and their families with ACPs should be aware that healthcare professionals remain obliged to instigate the treatment they consider to be in the best interests of the young person at any point and, although unusual, such action may not be consistent with the ACP. In addition, patients and families are able to change their minds and consider options different to those described in their ACP at any point. The question of when to start the planning process is often challenging. The lives of young people may be limited by any one of a large number of conditions, following different disease trajectories as described above. Although confidence and accuracy in predicting death increases as death approaches; predicting the timing of end of life remains challenging for
professionals. This uncertainty may deter professionals from starting the planning process. However, if one chooses to delay advance care planning until a critical event occurs this may lead to discussions taking place with a time pressure, with likely heightened emotional distress and possibly with a team of professionals who the family does not know well. It would seem better for a number of conversations to take place as part of a longitudinal process with the concept of advance care planning being introduced soon after diagnosis of an LLC instead of in response to individual crises. Introducing advance care planning and the concept of PC early on may also serve to ensure a sense of openness, attention to the child’s quality of life and greater opportunity for families to engage in making choices. Transition Transition has been described as ‘a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic physical and mental conditions as they move from child-centred to adult-oriented healthcare systems.’ As increasing numbers of children with LLC are surviving to adulthood we must plan how best to meet their ongoing PC needs. Adolescence is a time of change and uncertainty for any young person. For some with LLC adolescence may coincide cruelly with deterioration in their health and an increase in their dependence on family members. Facilitating independence in living arrangements, employment and healthcare are all important aspects of transition planning. Effective transition relies on forward planning, co-ordination, and excellent communication. It is recognized that transition for
Proposed content of an ACP C C C C C
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Patient & family’s demographic information Background diagnosis & brief summary of health needs Details of involved professionals Patient and family’s wishes during life Plans for care if the child becomes more unwell (including preferred place of care and treatment options) Wishes for care around the time of and after death (including place of care, interest in organ and tissue donation, spiritual and cultural wishes)
Box 3
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children who solely have health needs should ideally start to be implemented 1 year prior to an anticipated transfer date. For young people with complex needs effective inter-disciplinary and multidisciplinary working is vital to facilitate a successful transfer package and this work may need to take place over a longer timeframe. Barriers to transition are described in Box 4. Service-based restrictions may be a particular challenge for those with PC needs. For children with progressive conditions where curative treatment is not available or those with severe neurodisability (ACT/RCPCH categories 3 and 4) there is often a question mark over which medical professional should take on their care in adulthood. Many GPs feel uncomfortable dealing with dying children. Throughout childhood, the GP will more often than not take a step back allowing a general or community paediatrician fulfil the role that a primary healthcare provider might for adults. On reaching adulthood, the PC needs for such young people may lead them to fall between two stools e too specialist to be met by primary care yet not within the remit of specialist adult palliative medicine services. The need to meet the requirements of these young adults has been recognized by ACT who, through the publication of their transition care pathway identify key stages to be negotiated to implement a successful transition. They describe three phases of transition; recognizing the need to move on, moving on and recognition of the end of life. The core principles that they identify: involvement of the young person in decision making, flexibility and choice, continuity of support, anticipation of needs and the execution of individually tailored plans are central to the process of transition being a success for these young people.
Barriers to effective transition
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collaboration with expert professionals and offers guidance on decision making, working with the family to develop an end of life plan for their child, the practicalities of withdrawing ventilation and care at the time of death. Examples of the guidance contained in this document are shown in Box 5. The ACT guidance can be used to support the process of palliative extubation in any setting, including acute care. It is hoped that it’s existence will help prompt professionals to offer
Care pathway guidance: ‘A Care Pathway to Support Extubation within a Children’s Palliative Care Framework’ 1. The journey towards a decision to withdraw ventilation The ethical and legal considerations Offering choices and making plans: Location of death Who will be involved (core staff, family, friends) Wishes, goals, ceremonies and memory building Resuscitation decisions Symptom control Discussions about organ donation Discussions about post mortem Emotional support
Extubation in a palliative care framework One of the key goals of PPC is to facilitate the choices of children and families. In the past it has been assumed that certain levels of care precluded transfer from a paediatric/neonatal intensive care unit or hospital. Recently, more emphasis has been placed on the choices that can be offered even when a high level of invasive care has been ongoing until that time. In some areas and on some occasions it is now possible to facilitate transfer out of the acute setting to either the child’s home or a hospice and for withdrawal of intensive care to take place there. This opportunity to spend time with their child outside a clinical environment is much valued by families. However, the availability of this service is patchy and practice is variable as, until now, it has been dependent on local resources and the willingness and availability of individuals in that area. There are clearly a number of challenges that face professionals looking after children for whom further invasive care is likely to be futile, not least guiding the family through discussions about how their child’s best interests might be met by discontinuing artificial ventilation and how and where this might be possible. It is then of utmost importance that extubation is carried out sensitively and without avoidable complications, with considerable attention to emotional and psychological support for the whole family. In an effort to support individuals who are increasingly being called upon to provide this service, in 2011 ACT published ‘A Care Pathway to Support Extubation within a Children’s Palliative Care Framework’ (see Box 5). It was developed in
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Poor communication Poor planning Service-based restrictions e e.g. a lack of equivalent services Lack of confidence in colleagues Lack of experience in the adult sector of conditions with which children previously did not survive into adulthood Patient and family preference to avoid transition to new services
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Parallel planning for ongoing care if child does not die after extubation 2. The practicalities of withdrawing ventilation Developing an extubation plan Reviewing plan with family after transfer out of acute setting Extubation checklist Planning for imminent death and also longer term survival 3. Care at the time of death Enabling end of life wishes Ceremonies and memory building Practical information about choices and procedures after death Bereavement support
Association for Children’s Palliative Care, 2011 Box 5
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FURTHER READING A care pathway to support extubation within a Children’s Palliative Care Framework, ACT. 1st Edn., 2011. A guide to the development of children’s palliative care services, ACT. 3rd Edn., 2009. Better care, better lives: improving outcomes and experiences for children, young people and their families living with life limiting and life threatening conditions. Department of Health, 2008. Blum R, et al. Transition from child-centered to adult healthcare systems for adolescents with chronic conditions. J Adolesc Health 1993; 14: 570e6. Brook L, Hain R. Predicting death in children. Arch Dis Child 2008; 93: 1067e70. Craft A, Killen S. Palliative care services for children and young people in England. An independent review for the Secretary of State for Health. Department of Health, 2007. Fraser J, et al. Advanced care planning for children with life-limiting conditions e the Wishes Document. Arch Dis Child 2010; 95: 79e82. Hain R, et al. Curriculum in paediatric palliative medicine. British Society of Paediatric Palliative Medicine, www.act.org.uk; 2008. Hughes-Hallett T, et al. The Palliative care funding review: funding the right care for everyone; an independent review for the Secretary of State for Health. Department of Health, 2011. The Transition Care Pathway, ACT. 1st Edn., 2007. Wolff T, Browne J. Organizing end of life care: parallel planning. Paediatr Child Health 2011; 21: 378e84. World Health Organisation. WHO definition of Palliative Care 1998, http://www.who.int/cancer/palliative/definition/en/; October 2011. www.act30millionstars.org.uk.
choices and to know how they can facilitate these choices for families. It is also hoped that improving parents’ memories of the end of their child’s life will offer them some comfort in bereavement.
Summary There is growing recognition of the importance of high quality PC for children and young people as greater numbers of young people survive conditions that would previously have led to their demise in early childhood. Doing this work well and according to the wishes of children and their families requires improved commissioning of local and regional services, joined up working across education, health and social care and across statutory and voluntary sectors. These challenges and how best to meet them are described in a number of key documents described in this article. The medical profession have also recognized the importance of good PC for children by formalizing training for PPC doctors. Whilst it is important that some professionals have the opportunity to develop specialist paediatric palliative medicine skills and that numbers of paediatric palliative medicine consultants increase it remains true that all doctors working with children have a duty to develop a generic skill base that enables them to contribute effectively to the work of teams of professionals caring for life-limited children and their families.
Funding None required
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