Setting the context

In the past, much of the clinical experience and published research in children’s palliative care has been derived from practice in children’s cancer. Although terminal care at home for children with cancer has long been advocated,2 3 this debate has only recently begun for children with other life-limiting conditions.

Currently, most children with non-malignant life-limiting conditions die in hospital. In May 2008, the Confidential Enquiry into Child and Maternal Health (CEMACH) published its study “Why Children Die”. The researchers investigated 1000 children, aged 1–19 years, who died during 2006 in three regions of England, all of Wales and Northern Ireland. They found that 77% of children who died had a pre-existing disability or medical condition. Among this group, 73% died in hospital, 19% at home and only 2.6% in hospices. Of the 126 cases that were reviewed in detail, 48% had a congenital condition or longterm illness that would have been associated with a shortened life expectancy, and only two of these 126 children died in a hospice. The authors concluded that the low proportion of deaths in hospices suggests that end-of-life planning might be improved in children with life-limiting conditions.4 A study in south-west England over a 5-year period looking specifically at children with non-malignant life-limiting conditions found that 54% of children died on a hospital ward or intensive care unit, 24% died at home and 10% in a hospice. Other places of death included the accident and emergency department, foster care and overnight respite care.5

The preferred location of death should not be assumed, as it will vary according to the individual wishes and needs of the child and their family. Each location has its relative merits. Many families prefer to care for their dying children at home, where they can be in their own surroundings. However, caring for a dying child can be an exhausting process, and the additional strains on family members can take its toll. A death at home may place an extra burden of responsibility on the primary care team, although children’s community nursing teams and specialist palliative care services are increasingly able to provide advice and support. Many families of children with chronic life-limiting conditions develop close ties with their hospital teams and may choose to remain in hospital while caring for their dying child. While this has the advantage of being in an environment familiar to the child and their family, many hospital settings are focused on active treatment rather than palliation, and ward staff will need to be flexible and responsive to the specific needs of a dying child and their family.6 Children on an intensive therapy unit (ITU) who have ventilation withdrawn, will require intensive care expertise, although advanced planning may still allow the child to die in the location of their choice. Integrating palliative care teams into the paediatric intensive care unit (PICU) setting facilitates this, and may result in a reduction in the number of children receiving end-of-life care on PICU, while increasing numbers are either transferred home or to a hospice.7 8

National policy and guidance

Children’s palliative care is currently in the mainstream of national service planning and there is an expectation that healthcare professionals will help families develop plans for children with life-limiting conditions. Some key recommendations are described below.

• The National Service Framework for Children, Young People and Maternity Services, 2004 (NSF).9 Standard 8 of the NSF states that high quality palliative care services should be available for all children who need them, should take into account the need of families and siblings, and recommends that arrangements should be made to avoid unnecessary emergency admission to hospital.

• ACT (Association for Children with Life-threatening and Terminal Conditions and their Families). In December 2004, the Department of Health (England) commissioned ACT to produce an integrated multi-agency care pathway to complement the NSF guidance. The resulting pathway outlined five sentinel standards that should be developed as a minimum for families of children with life-limiting conditions, as well as provide a benchmark against which commissioners may assess local services. In relation to end-of-life planning, standard 5 states that “Every child and family should be helped to decide on an endof-life plan and should be provided with care and support to achieve this as closely as possible”. Importantly, the ACT care pathway advises that bereavement care for the whole family, including siblings, should be an ongoing process from the point of diagnosis. Clear documentation is vital and parent-held records should be used to facilitate this.10

• Commissioning Children and Young People’s Palliative Care Services: A Practical Guide for NHS Commissioners, 2005. This document states that children and young people should have a written care plan and should have the choice to be at home as much as possible.11

• Better Care Better Lives – Improving the Outcomes and Experiences for Children, Young People and their Families living with Life-limiting and Lifethreatening Conditions, 2008.12 This document outlines key strategic service development goals towards ensuring better end-of-life care. Families should have access to multidisciplinary community team working 7 days a week, as well as to 24 h specialist support and advice. Commissioners have a duty to specify services, and providers have a duty to develop choice of provision according to identified needs.

• Decisions Relating to Cardiopulmonary Resuscitation – A Joint Statement from the British Medical Association, the Resuscitation Council (UK) and the Royal College of Nursing, 200713 These guidelines identify the key ethical and legal issues that should inform all cardiopulmonary resuscitation (CPR) decisions. In particular, they emphasise that advanced care planning, including making decisions about CPR, is an important part of good clinical care for those at risk of cardiorespiratory arrest. Inappropriate CPR may subject people to an undignified death, and may prolong suffering in some cases. It is important to identify those patients with capacity who state clearly that they do not want CPR to be attempted. Patients over 16 years of age are presumed to have capacity to make decisions for themselves unless the contrary is proven.

Although national guidance is clear, anecdotal evidence suggests that there is still scope for improvement. An audit of patients with life-limiting conditions on PICU found that only 20% had a documented end-of-life plan, and that an equally small percentage met any of the five sentinel ACT standards (W Booth, personal communication , October 2008). Similarly, a review in a children’s hospice found that only 53% of children with a resuscitation order had an emergency management plan clearly documented in their case notes (N Harris, Children’s Hospice South West, personal communication, January 2009). Many babies die on neonatal units but only a minority of neonatal intensive care units have neonatal end-of-life guidelines (Sargant and Finlay, unpublished data).

Why is end-of-life planning so often poorly achieved?

Two reasons are often cited by health professionals as to why end-of-life planning is so often poorly achieved: first, the difficulty predicting the end of life, and second the difficulty of “raising the issue”. We will address each of these in turn.

Predicting the end of life

Predicting the end of life in a child with a life-limiting illness is an inexact science.14 Many children with a life-limiting condition will have long periods of stability interspersed with episodes of serious illness. Such episodes often result in an intensive care admission and it may be difficult to predict which intensive care admission will eventually coincide with death. Other children with degenerative neurological or neuromuscular conditions may follow a more predictable path of gradual deterioration. Increased frequency of admission to hospital or PICU, episodes of acute illness without recovery to baseline health, and physiological changes such as decreased oral intake and decreased periods of wakefulness, may all herald the beginning of the dying process.13 However, all too frequently, the patient is “unexpectedly” in the last phase of their life and paediatricians or other professionals have not found the opportunity to initiate conversations about death. Clinical uncertainty about the timing of death results in poor preparation for death and health professionals should recognise that the anticipation of the precise timing of death is less important than anticipating the fact of death itself. It is the acknowledgement of the latter that allows families and children to make choices and plan their lives.

The difficulty of “raising the issue”

Health professionals may worry that discussing end-of-life plans may shift the focus of attention to a child’s death at time when clinical management is concentrated on prolonging life. However, evidence suggests parents appreciate open and honest discussion, and may feel resentful and angry when sensitive topics are avoided. Talking to children about their life-limiting condition and deciding on appropriate timing can be difficult, but evidence suggests that when parents have discussed such matters with their children regret has not been expressed by either party.15 Much of the available literature on what works in terms of constructive information sharing comes from experience in adult oncology patients. Consistent themes emerge in the art of communication at disclosure of a diagnosis with a poor or uncertain prognosis. Positive attributes in terms of good communication have been identified as “playing it straight, staying the course, giving time, making it clear and pacing information”.16 Paediatric consultants report that discussions tend to “go well” when there is good planning, honesty, mutual respect and the “right environment” with time and privacy.17 Other qualities identified as being valued by both children and their parents in improving communication include ability at relationship building, demonstration of effort, competence and availability for discussions. Recognised negative characteristics include arrogance, insensitivity in breaking bad news and the withholding of information.18

End-Of-Life planning in children with life-limiting conditions should start at diagnosis

In paediatric oncology, there is a growing consensus that the needs of the child and family are best met if the concept of palliative care is introduced at the point of diagnosis. Many have advocated a similar approach in children with nonmalignant life-limiting conditions,20, 21 whose life expectancy may be extremely variable, and in whom it is recognised that early discussions may not preclude either curative or life-prolonging therapies at a future date.22

Early discussions with the child, family and close friends may also help to ensure attention to quality of life at an early stage, openness with siblings, emotional and psychological adjustment before the child’s death and a sense of “more personal control of events and choices” by the family.19 23 The Wishes Document has been developed to help encourage discussion about such choices with children and their families. The challenge within such discussions is to balance unrealistic expectations on the part of the family while at the same time ensuring that the best interests of the child are always met.

The wishes document (see online supplement)

In 2007, the West of England Children’s Palliative Care Partnership “End of Life” group set out to develop documentation to facilitate end-of-life planning in children with life-limiting conditions. The group was multiprofessional and included a director of children’s palliative care research, consultant community paediatricians, a paediatric intensive care consultant, an ITU matron, an adult palliative care consultant, a clinical director from a children’s hospice, a nurse from a children’s hospice, a clinical psychologist and a chaplain. The group aimed to produce a document that was hand held, incorporated the views of the child, family and close friends, and was introduced to the family soon after diagnosis rather than at the time of impending death.

The basis for the Wishes Document was originally conceived by the Lifetime Team.24 The lifetime framework was a simple 3×3 framework devised to aid proactive planning, capturing the views of the child, their parents and others on care provided during life, around the time of death and after death. The working group expanded this framework to include wishes during life, plans for when a child becomes more unwell, plans for care during an acute life-threatening event and wishes after death. Although it is intended that the whole Wishes Document be shared with the family at the outset, the various sections of the document lend themselves to completion in a flexible manner. For example, some families may complete the “Wishes during life” and “Plans for when child becomes more unwell” sections first, completing the other sections at a later date. Plans may evolve over time and may be updated according to the child’s and family’s wishes or the child’s clinical condition.

Wishes during life (wishes document, page 2)

Children, young people and their families have aspirations, but these need to be actively sought, and then acted upon. The moment of relative health may be brief, and parents need to be given encouragement to “take the opportunity”.

Plans for when child becomes more unwell (wishes document, page 3)

Children with a life-limiting condition may become more unwell slowly over a period of time and this page of the document allows views on antibiotics, feeding, respiratory support and seizure management to be recorded.

Plans for care during an acute life-threatening event (wishes document, page 4)

Children with life-limiting conditions may have both predictable and unpredictable crises of acute illness. The patient with advanced neuromuscular disease may develop respiratory failure in the context of a trivial lower respiratory tract infection. Action plans need to be thought through, the place of domiciliary non-invasive ventilation carefully considered with appropriate specialists, and escalation to full paediatric intensive care discussed. The child with severe cerebral palsy may have life-threatening seizures and again, action plans need to be documented, place of care discussed, and treatment options considered. The merits of a tick box list versus spaces for free text were discussed at length by the working group and it was decided to use a tick box approach to outline wishes for the acute life-threatening event situation where clear emergency plans are required specifically around ventilation, resuscitation and inotropic support.

Wishes for after death (wishes document, page 5)

Children, young people and their families may have very clear views on funeral arrangements, and what should happen to their bodies and possessions after they have died. The subject of organ donation is also challenging but families who have had the opportunity to donate their child’s organs often subsequently state that this has given them some comfort in the midst of their grief.

The final page (page 6) of the Wishes Document gives further details of useful resources and supporting information.

The Wishes Document may be introduced to the family by any member of the healthcare team, and often the child’s community nurse, who has developed a close relationship with the family over many years, is best placed to do this. Alternatively, the child’s paediatrician, or on occasions the family general practitioner, may be more appropriate. It should be a professional who knows the child well, has some familiarity with their condition and most importantly has the confidence and trust of the family to talk frankly about potentially distressing issues. As the child becomes older, their own views should be sought, and they should be encouraged to participate in discussion and decision-making at a level appropriate to their age and capacity. In all cases plans should be completed with honesty and compassion, taking into account physical and psychosocial care needs in addition to the cultural and spiritual needs of the child and their family.

It is not intended that the Wishes Document is completed at one sitting. Staff should be led by the needs of the family and the document should be revised as the child’s situation changes. The needs and wishes of the parents of a child with, for example, Edward’s syndrome, may be very different from those of the parents of a boy with Duchenne muscular dystrophy. The document has no legal status and it belongs to the family. It will generally be in a hand-held form that can be presented to involved professionals and the family may give their permission for it to be copied for notes for the hospital ward, emergency department, PICU, hospice, general practitioner surgery, respite care and the ambulance service. Such advanced care planning documents are vital to ensure that, when a child dies at home or in a hospice, there is not an inappropriate rapid response escalation as part of the investigation of a sudden unexpected death in childhood.25

Summary

The number of children with severe lifelimiting conditions is increasing, and at present most of these patients die in hospital, often on PICU. We advocate that end-of-life planning should take place with children and their families at an opportune time following diagnosis, and not just prior to death. This approach should not eliminate hope but rather allow a family to refocus their aspirations in a realistic fashion.

All paediatricians should be aware of national policy in respect to palliative care, and be mindful of their responsibility towards ensuring that every child with a life-limiting illness has an end-of-life plan. The Wishes Document has been adopted by the Association for Children’s Palliative Care and is offered as a guide to clinical teams who may hold such discussions with children and their families.