Palliative care for children is about quality of living ensuring the child and family live fully in the knowledge that early death is expected (Association for children with a life-threatening illness (ACT) 2004, Levetown and the Committee of Bioethics 2008). Over recent years, the care of children and young people with life-limiting illnesses has been the subject of growing interest (Hain & Wallace 2008) and as a result paediatric palliative care has evolved as a small but distinct area of practice across the UK and internationally (McNeilly et al 2004). Government strategy and policy within the UK have children with palliative care needs and their families central to their agenda (Department of Health, 2005, Department of Health, 2007 and Department of Health, 2008). Knowing that a child is life-limited poses a particular challenge for parents and professionals (Armstrong-Dailey 1990).

The subsequent death of the child causes a devastating loss to families and communities, and in turn leads to one of the most profound and long-lasting of griefs. The death of the child defies the natural expected order of life events (Sourkes et al 2005).

Although it has been argued that the principles and ethics underpinning palliative care delivery are universal across age spans, the caring for children within this and other specialist areas of health care bring unique and different challenges, issues and dilemmas (Sourkes et al 2005). Whilst some of the principles of caring for the adult patient may be useful it is essential to recognise and acknowledge the very unique needs of the child requiring a palliative approach and their family (ACT 2003, Price et al 2005). One of the most distinctive differences within children’s palliative care is the broad diagnostic diversity which results in the challenging uncertainty around prognosis experienced by children and families. Many of the disorders experienced by children are rare (Watterson & Hain 2003). Others may be familial and hence genetic counselling is crucial. In addition due to the degenerative nature of many of the life-limiting illnesses of childhood, palliative care services are traditionally involved with the child and family from an early stage, often over a long period of time (Hynson & Sawyer 2001).

The need for care can be unrelenting and can lead to social deprivation and extreme stress for the family (Corkin et al 2006). Burn-out in staff regularly caring for these children and families has also been noted (Costello & Trinder-Brook 2000).

Palliative care for children is about quality of living for both the child whose life is limited and their family (ACT 2009, Liben et al 2008). The development of this specialty can be attributed to the advent of the children’s hospice movement and the further development of paediatric oncology outreach services. Born out of a desire to improve care for dying people the hospice movement evolved as it had become accepted that the modern medical establishment was not fully addressing all the needs of dying people and their families. The need for expansion of services was soon identified from simply providing care for adult cancer patients and in 1982 the first children’s hospice was opened in Oxford. Helen House developed from the special friendship between Sister Frances Dominica and a child called Helen, who had a life-limiting illness. Through this relationship, Sister Frances recognised the need of respite care and practical support for other families in similar situations. Her vision was for a haven where this practical support and respite care could be delivered. Helen House provided a ‘home from home’ where families could share the caring, providing them an environment where practical help, friendship and quality time were offered. This was to ‘blaze a trail in the provision of hospice care for children and young people’ (Worswick 2002 p 160). Helen House remains an exemplar for the development of children’s hospice services world wide and the number of children hospices grew in conjunction with the development of ‘hospice-at-home teams’. The growth generated an appraisal into the care delivered to children with life-limiting illnesses and their families. In 1992 the ACT (Association for Children with Life-Threatening or Terminal Conditions and their families), since renamed the Association for Children’s Palliative Care, was set up to influence and promote excellence and equity in care provision and support for children and young people with life-threatening or life-limiting conditions and their families.

ACT work closely with other agencies, for example Children’s Hospices UK (Formerly ACH), to promote excellence for all children requiring palliative care and their families (Price et al 2005). Although this philosophy of care has developed largely from within the hospice movement it has since permeated to a variety of additional settings – namely hospital and home. This being so, it is now referred to as paediatric palliative care or palliative care for children and the specialty has greatly developed in recent years through policy development and international networks (Hain & Wallace 2008). Despite the expansion and development of services in recent years, providing palliative care tailored to a child’s individual needs has not been without its challenges. These challenges include difficulty recognising which children require palliative care, the variation in availability of services depending on geographical location, a lack of understanding by policy makers about what constitutes palliative care and the limited evidence base underpinning practice (Price et al 2005).

Although many of the terms used within the specialty are used interchangeably one of the most widely accepted definitions in the UK is from ACT (2009 p 9):

Palliative care is not purely about dying but is about quality of living and is provided to children with life-threatening life-limiting condition. The above definition highlights the different attributes that contribute to the philosophy of palliative care for children. The ACT Charter outlines the needs of children and families and can be viewed on the companion PowerPoint presentation.

Palliative care for children requires an integrated approach and it has become widely accepted that a mixed model of care is essential. Within this model the principles of palliative care and cure-focused care are delivered concurrently, ensuring consistency throughout the child’s illness (Michelson & Steinhom 2007).

At least 15,000 children require palliative care in the UK (ACT/RCPCH 2003) and a wide range of conditions renders their need for this type of care. Four broad groups of children have been identified as likely to require this unique type of care whose needs can change over time (ACT/RCPH 2003). Within the four groups of children many have complex chronic conditions and all the children have the possibility of an early death (Himelstein 2006). These groups are listed on the companion PowerPoint.

The diagnosis of a life-limiting/life-threatening illness rocks the most stable of families (Chad 2008). It is the start of a journey that can resemble a roller-coaster ride: the ups and downs along the uncertain road of the illness trajectory and can lead to a long-standing relationship with the healthcare team (Nuutila & Salantera 2006). The way the news is broken can stay with a family for many years and a variety of models have been established to assist healthcare professionals in this complex and challenging task (Price et al 2006). In addition other factors affect the psychological impact of facing a life-limiting illness. These include the age and cognitive development of the child, the duration and type of treatment (if available), the prognosis, the degree of disruption to normal routine and education, the degree to which body image may be affected and the potential separation from siblings and main caregivers.

The overall reaction to the diagnosis of a child’s life-limiting condition or the birth of a baby with a life-limiting condition is similar to that of bereavement as parents are grieving the loss of their ‘well’ child or the ‘healthy baby’ they wished for (Davies 2004). The child may also be grieving for the ‘normal’ life and the future which they may feel they have lost (Maunder 2004). Soricelli & Utech (1985) identify that grief symptoms are experienced from diagnosis and throughout the illness trajectory of a child with a life-limiting condition and highlighted four distinct phases of bereavement – firstly bereavement at the time of diagnosis, secondly bereavement during integration, thirdly renewed bereavement if curative options have been available and are exhausted and death is approaching, with the fourth phase being post-death mourning.

Kubler-Ross (1970) identified five reactions to dying; these can equally be applied to a diagnosis of a life-limiting or life-threatening illness. These are denial, anger, bargaining, depression and acceptance. The child and the family may experience a multitude of different feelings at different times and there is no set way for how the individual may feel at any particular time. Regardless of the type of feelings being experienced at any particular time, the child and family require cohesively planned care and support from an experienced team who are responsive to individual need. The family needs information and support at the time of diagnosis and afterwards (RCN 1999, Price et al 2006). Planning palliative care for children must therefore be tailored to the individual and changing needs of that child and their families (Beardsmore & Fitzmaurice 2002) and include a wide array of agencies and services within the NHS and outside to ensure a holistic quality care is provided (ACT 2003).

The need for an interdisciplinary team approach to paediatric palliative care is essential in the provision of a quality seamless service to children and their families (McNeilly & Price 2007). Providing palliative care for children requires thorough planning, effective communication as well as coordination and cohesiveness within the interdisciplinary team (Hynson et al 2003).

Clear, concise communication is a fundamental component of successful team working with each team member having a role to play. Members of the interdisciplinary team must share identical goals of care and respect the individual roles of team members and also family needs and values. A cohesive package is required with clarity of roles in order that conflicting information is avoided. Team variance can create an environment where confusion is present. The healthcare professional must function effectively and efficiently on an individual basis and as part of the team. The palliative care package for the child and family should have an identified key worker (ACT/RCPCH 2003). This worker will coordinate the care and ensures a seamless package is provided, that clarity of roles is established and good communication is maintained (ACT 2003). The key worker could be a CCN, palliative care nurse, hospice or paediatric oncology outreach nurse specialist (POONS). Vickers et al (2007) purport that the POONS is in an ideal position to act as the key worker for a child with advanced cancer. The team will be made up of different professionals, depending on the individual family and their circumstances. Examples of members of the interdisciplinary team are listed on the companion CD-Rom.

The importance of good team working cannot be understated this is supported by ACT (2003) who clearly stipulate that the assessment, planning, implementing and evaluation of plans of care including symptom control for children with a life-limiting illness needs to be interdisciplinary.

Symptom management has been identified as a major component of palliative care (Michelson & Steinhorn 2007). Parents have identified that children suffer multiple symptoms, particularly at the end-of-life (Wolfe et al 2000). It is essential to recognise that the symptoms experienced by children are rarely simply physical events. Symptoms should be regarded as more often a complex experience with physical, psychological, social and emotional elements (Brady 1996).

Reliable, valid and frequent assessment is central to successful symptom management (Brown 2007). Assessment regardless of the symptom is an ongoing process, which permits choice and flexibility when circumstances for the child and family change (Anghelescu et al 2006, DoH 2000). The child’s stage of development must be central to assessment and management of symptoms. A variety of assessment tools can be used in the extracting and assimilating of information with children. Many assessment tools offer a combination approach using verbal and non-verbal indicators (see Chapter 17). Paediatric Pain Profile (Hunt 2003) is an example of an assessment tool for use with pre-verbal, unconscious and non-verbal children. Although a range of assessment tools exist, their under-use in practice with children with life-limiting conditions has been highlighted (McCluggage & Elborn 2006).

Adequate symptom control is viewed as the ultimate aim of palliative care regardless of the setting in which care is provided. Negotiation with and open lines of communication between the child, family and nurse are essential in achieving optimum symptom control. Symptoms experienced vary depending on the child and specific diagnosis and families have much to offer in the assessment and evaluation of symptom management. Symptom management plans should in addition be flexible, evidence based and include combinations of both pharmacological and non-pharmacological interventions (Anghelescu et al 2006). Education of parents and preparation regarding symptoms is crucial (Beardsmore & Fitzmaurice 2002) in order that they know what to expect. The nurse must consider that symptoms that may not be disturbing to the child can be very distressing to parents.

Scenario

Carol, an 8-year-old girl, was at the end-of-life stage – she was unconscious, settled and comfortable. Her parents were by her hospital bed and very involved with her care. A few days before her death, Carol developed noisy respirations. This caused her parents great distress and they found it extremely frightening. This symptom was managed by administering a hyoscine patch, which was placed behind Carol’s ear. Explanations and reassurance was provided for the parents. The noise was reduced and parental anxiety was eased.

This scenario illustrates the importance of involving the parents, consideration of parental need and recognising that they suffer alongside their child. Many symptoms are managed pharmacologically which can be challenging given that many of the drugs used effectively for symptom management with adults are not licensed for use in children (McCulloch et al 2008). A number of routes of administration can be used and the chosen route depends on the age, condition of the child and any access devices that the child may have in place.

The oral route is usually first choice for administration of medication in child – many drugs are available in elixir form. The child and family should be involved in decisions about the preferred format. Intramuscular injections should be avoided in children where possible. Many children at this stage in illness may have a central line in position, which provides a useful alternative route for administration. Some children may have a nasogastric tube in place and this can be utilised successfully for administration of medication. Infusions can be given subcutaneously via syringe drivers (McNeilly et al 2004) or via an existing central venous catheter. In recent years the transdermal route has become another option for the delivery of some drugs. Rectal preparations are available for many drugs; they should be avoided in children with low platelet counts and repeated use can cause soreness in children.

The focus of complementary therapies in palliative care is on symptom control. Buckle (2003) discusses the value of aromatherapy and massage in children’s palliative care. These techniques can aid in promoting communication with children, reducing anxiety, enhancing relationships and enhancing quality of life in terms of symptom management. Other types of complementary therapies include reflexology, guided imagery and hypnotherapy. These therapies can be used in conjunction with conventional medicine. As with all interventions the child’s welfare must be paramount and underpin all care delivered.

Commonly occurring symptoms experienced by children and interventions are identified in Table 46.1. Further details of symptoms and their management are listed in Rainbow Children’s Hospice Guidelines (see below). A study by
Pritchard et al (2008) examining parents’ perceptions of symptoms experienced by children with advanced cancer included changes in their child’s behaviour and appearance. Healthcare professionals should prepare and support parents regarding these two distressing symptoms. ACT has a discussion forum which brings together those working within paediatric palliative care both nationally and internationally – this is a very good forum for sharing practice experiences about symptom control and other issues. Information on this is available at http://www.act.org.uk (click on ‘networking’ and then ‘Paedpalcare’).

Palliative care to children and families is developed around an ethos of holistic care delivery – that is, care that addresses physical, psychological, social and spiritual needs of the child and family (Goldman et al 2006a). Seldom are the issues and symptoms experienced by children and families simply in one category, much more often they are complex and multi-faceted. The child with palliative care needs must be viewed within the context of a family system (McNeilly et al 2006). The philosophy of palliative care for children is thus premised on a holistic, individualised approach, which centres round the specific needs of each individual child and family during their limited life trajectory (Price et al 2005). The care should be assessed, planned, implemented and evaluated using a collaborative partnership approach and should address the biological, psychological, social and spiritual needs of the child and family (see Chapter 6 and Chapter 7).

The collaborative partnership approach should be based on a trusting, therapeutic relationship with families (Monterosso & Kristjanson 2008). Families should be welcomed as partners in all stages of the nursing process, parents should feel they have a choice and be in control of the situation (Vickers & Carlisle 2000).