2 - The child s journey - Transition from health to ill-health
Editors: Goldman, Ann; Hain, Richard; Liben, Stephen
Title: Oxford Textbook of Palliative Care for Children, 1st Edition
Copyright 2006 Oxford University Press, 2006 (Chapter 34: Danai Papadatou)
> Table of Contents > Section 1 - Foundations of care > 2 - The child's journey: Transition from health to ill-health
2
The child's journey: Transition from health to ill-health
Jenny L Hynson
Introduction
A distinctive feature of paediatric palliative care is the diagnostic diversity encountered within the patient population. Children die from a range of conditions [1] and unlike the patient population encountered in the palliative care of adults, it has been estimated that less than half of children with palliative care needs have a malignant condition [2]. The remainder have a range of conditions including neurodegenerative diseases, congenital anomalies, and chromosomal disorders [3]. The ways in which children move from health to ill-health are myriad but may be broadly categorised into distinct illness trajectories, each with particular implications for children and their families, the health professionals caring for them and the broader community. The transition may be abrupt or gradual and it is not always possible to identify a point at which the focus of treatment becomes exclusively palliative.
The idea that palliative care begins when curative treatment fails is outdated. The World Health Organization [4] and the American Academy of Pediatrics (AAP) [5] both now advocate an integrated model of palliative care that is applicable early in the course of illness, in conjunction with other therapies that are intended to prolong life'. [4] Perhaps more than their colleagues in adult medicine, paediatricians have been forced to think of an integrated approach to palliative care because of the diagnostic diversity they encounter, the often protracted nature of the many illness trajectories, and the developmental needs of children and their families. The AAP defines an integrated model of palliative care as one in which the components of palliative care are offered at diagnosis and continued throughout the course of the illness, whether the outcome ends in cure or death [5, 6].
A mixed model of care in which facets of palliative care and cure-oriented or life-prolonging treatment are married together is a helpful way of approaching the management of many children with life-threatening or life-limiting illnesses [7, 8]. In this way, a child might participate in a Phase One trial or be placed on the list for organ transplantation while receiving optimum symptom management and support in living with uncertainty and the possibility or probability of death. The family is assisted to hope for the best but prepare for the worst'. One of the major challenges is balancing hope with reality but it has been demonstrated that it is possible for families to balance dual goals simultaneously [9]. Indeed, it is important for children and families to know what options are available to them so that they can make informed decisions. They need reassurance that palliative care is an active approach and that care does not end when cure is no longer possible.
Transition from health to ill-health: Illness trajectories
In the palliative care setting, children can be understood to move from health to ill-health in four distinct ways:
The child has a potentially curable illness but treatment fails.
Intensive treatment can be expected to prolong and enhance life but the child is likely to die prematurely.
The child is diagnosed with a progressive condition for which no curative treatment exists.
The child has a non-progressive condition but is vulnerable to early death as a result of general debility and complications such as respiratory infection etc. [10].
An understanding of the various ways in which children move from health to ill-health provides a foundation on which
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to build a flexible and responsive service system for children with palliative care needs and their families. It is important to remember however that the existence of multiple other variables means that the circumstances for each individual child and family are unique. These include the particular features of the child's condition (e.g. histology, genetic determinants, extent of disease, response to treatment etc.), the age and developmental stage of the child, the structure of the family, relationships within the family, educational background, social supports, geographic location, the health professionals involved and the service systems available. This means that for reasons that are at times unclear, two children with the same diagnosis can experience quite different outcomes.
Children and their families may make multiple transitions during the course of the same illness. The goals of care should be regularly discussed and renegotiated so that they reflect the child's changing circumstances. Take for example a 2-year old boy diagnosed with Duchenne muscular dystrophy after a delay in his gross motor development is noted. Although he is otherwise quite well, a major transition has occurred. He is no longer a well child who has taken a little longer to learn to walk. He is a boy with a life-limiting illness for which no cure exists. His parents know that early intervention will optimise his quality of life and they focus on this, hopeful that in the next few years a cure will be found. Over time, he makes the transition from being able to being disabled but he attends and enjoys school and is socially active. During adolescence, his lung function deteriorates and hospitalisations become more frequent. He becomes more and more dependent on his family for basic needs and is no longer able to attend school. During these years he becomes increasingly aware of his lack of independence and is concerned about body image and what his peers think of him. His family's hopes for a cure fade. Non-invasive ventilation is commenced but the burdens of intervention begin to outweigh the benefits and a decision is made not to progress to more invasive forms of ventilation. The transition to the terminal phase of the illness is made and therapy aimed at minimising respiratory distress initiated. Yet another transition is made when the young person dies and the family is bereaved.
Conditions for which potentially curative treatment fails
Case
Frederic, aged ten, was found to have a tumour of his left frontal lobe following a generalized seizure. A diagnosis of glioblas-toma multiforme was made and the family was informed that the prognosis was poor although effective surgical resection offered hope of survival. The lesion was surgically removed with good clearance. Frederic received chemotherapy and radiotherapy but the tumour recurred a few months later and was again resected. Despite further chemotherapy, a small nodular recurrence was noted 6 months later. The family elected to participate in an investigational Phase One trial but the tumour continued to progress and the child's condition deteriorated until his death 11 months after diagnosis.
For most children with malignant conditions, cure is a probability or possibility at the time the diagnosis is made. In this situation, children and their families are dealing with a life-threatening illness and move into the treatment phase with a prospect of cure. Whatever the ultimate outcome, malignancy is a dangerous condition with the ever present possibility of death as a consequence of infection or other complications. Children and families need support in dealing with this uncertainty. This has been referred to as upstream palliative care in which the seriousness of the condition is revisited and discussed at regular intervals during the child's illness [1]. Many clinicians are concerned about raising such issues when the child is relatively well but for most families such thoughts are never far away and many are grateful for an opportunity to address them. Knowing that care will continue if cure is not achieved may be very reassuring [1]. Unhappily for some, curative treatment fails and there is a clear transition to palliative treatment which may or may not include chemotherapy or radiotherapy. For others, the transition is less clear with ongoing use of experimental therapies (Figure 2.1). The transition to palliative care may come after
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years of treatment. The child may have survived a number of life-threatening complications and there may be a sense that he or she could pull through again.
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Fig.2.1 Example of an illness trajectory for a potentially curable malignancy:early treatment causes the child's condition to deteriorate temporarily. Subsequent recovery and remission is followed by relapse. Further treatment is partially successful but the child's condition deteriorates and the child dies. (Adapted from Committee on Palliative and End-of-Life Care for Children and Their Families, Institute of Medicine (2002). In M.J. Field, R.E. Behrman, eds. When Children Die:Improving Palliative and End-of-Life Care for Children and Their Families. 2003 by the National Academy of Sciences. Courtesy of the National Academies Press, Washington, D.C. Reprinted with Permission.) |
Conditions in which intensive treatment can be expected to prolong and enhance life but premature death is likely
Case
Kylie was diagnosed with cystic fibrosis at the age of three but remained well until her tenth birthday. At that time, she began to suffer recurrent chest infections which increased in frequency and severity over the next 2 years. There was a substantial decline in her lung function over that time. Kylie spent long periods in hospital receiving intravenous antibiotics and no longer wanted to see her school friends. The option of lung transplantation was explored and her parents decided to pursue this. They asked that their daughter not be told about her prognosis. Appropriate investigations were carried out and Kylie was placed on the list of patients awaiting transplantation. She suffered recurrent episodes of severe respiratory deterioration requiring intensive care and her parents were told to prepare for her death on a number of occasions. Kylie recovered each time but became increasingly withdrawn and angry. She started refusing medications and physiotherapy. One night she confided to the nurse caring for her that she was frightened of dying but did not want to upset her parents by talking about this. The next day, the social worker met the family and helped Kylie express her fears and wishes. Her parents asked what she wanted to do and she explained that she was tired and did not want to return to intensive care. She asked to see her friends. Kylie's requests helped guide her parents and doctors in their decision-making. She died in the ward two days later.
Cystic fibrosis is a condition in which early recognition and intensive treatment significantly prolongs and enhances life (Figure 2.2). Premature death occurs, however, not infrequently during childhood or adolescence. Respiratory failure in cystic fibrosis is characterised by periods of severe deterioration which the child very often survives with the help of intravenous antibiotics and intensive chest physiotherapy. Families, health professionals, and patients are very reluctant to forego these interventions as they are perceived to pose relatively little burden with the potential benefit of a period of survival. In this way, the palliative care of a child with cystic fibrosis is quite different to that of a child with terminal cancer. There may be no clear transition to palliative care but rather a mixed approach to management as outlined earlier. Indeed, the care of children and young people with cystic fibrosis who are at the end of life has been described as an amalgam of three types of care: therapeutic (e.g. intravenous antibiotics), palliative (e.g. opioids for dyspnoea), and preventive (e.g. the continuation of enzyme replacement to prevent malabsorption) [11]. Children and young people with cystic fibrosis frequently die in the hospital setting [11]. Outdated concepts of palliative care as mutually exclusive from active therapeutic endeavours may mean important aspects of care are not adequately addressed. If health professionals wait until they are certain that death is imminent before initiating conversations with families about death and dying, this important intervention may never occur [12].
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Fig.2.2 Example of an illness trajectory for cystic fibrosis: disease-modifying treatment maintains the child's condition for a number of years. The advanced stages of disease are characterised by severe exacerbations. (Adapted from Committee on Palliative and End-of-Life Care for Children and Their Families, Institute of Medicine (2002). Reprinted with permission from M.J. Field, R.E. Behrman, eds. When Children Die:Improving Palliative and End-of-Life Care for Children and Their Families. (2003) by the National Academy of Sciences. Washington DC:National Academy Press.) |
Parents and health professionals are forced to make an impossible choice when aspects of support, such as interventions directed to cure and those directed to comfort, are presented as a mutually exclusive dichotomy. [13]
One of the features of the terminal phase of cystic fibrosis is uncertainty. Episodes of acute deterioration may see the patient, their family, and the staff caring for them prepare for imminent death on numerous occasions before it actually occurs. While lung transplantation now offers hope for some sufferers of cystic fibrosis, there are numerous barriers to the success of this intervention such as strict criteria for being placed on the list, limited organ availability, and the rigours of surgery and the post-operative phase [12, 14].
Progressive conditions where treatment is exclusively palliative from the time of diagnosis
Case
Max, aged 4 years, fell from his bed and complained of a sore knee. There was no swelling and Max remained ambulant so his parents managed the situation expectantly. Complaints of knee pain persisted and after one week, the local doctor was consulted. Examination and imaging revealed no abnormality of the knee and the family was reassured. Weeks later, following the development of pallor and weight loss, Max was diagnosed with advanced metastatic Ewing's sarcoma of the hip. There was no possibility of cure and he died at home 3 months later.
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Fig.2.3 Example of an illness trajectory for incurable cancer: palliative chemotherapy and/or radiotherapy improve the child's condition but there is a subsequent deterioration. (Adapted from Committee on Palliative and End-of-Life Care for Children and Their Families, Institute of Medicine (2002). Reprinted with permission from M.J. Field, R.E. Behrman, eds. When Children Die:Improving Palliative and End-of-Life Care for Children and Their Families. (2003) by the National Academy of Sciences. Washington DC:National Academy Press.) |
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Fig.2.4 Example of an illness trajectory for incurable neurodegenerative disease:the child is relatively well at diagnosis but slowly deteriorates. There is a stepwise progression towards death. (Adapted from Committee on Palliative and End-of-Life Care for Children and Their Families, Institute of Medicine (2002). Reprinted with permission from M.J. Field, R.E. Behrman, eds. When Children Die: Improving Palliative and End-of-Life Care for Children and Their Families. (2003) by the National Academy of Sciences. Washington DC: National Academy Press.) |
A child may be diagnosed with a condition which will be fatal within a short period of time and for which no curative treatment is available (Figure 2.3). Examples of such conditions include central nervous system tumours considered inoperable at the time of diagnosis or certain forms of solid tumour with widespread metastatic disease noted at the time of presentation. Chemotherapy or radiotherapy may be utilised as a means of controlling symptoms and increasing longevity but the child will die within months. In this way, treatment is palliative from the time of diagnosis. These children and their families must assimilate such devastating news in a short time.
The illness trajectory associated with incurable illness is not always so short. More often in children, the prognosis is one of slow deterioration over many years (Figure 2.4). Children in this group account for a substantial number of those requiring palliative care. Many have neurodegenerative conditions. Although individually rare, degenerative conditions of the central nervous system comprise a significant proportion of the patient population in paediatric palliative care. Indeed, the experience of paediatric hospices in the United Kingdom has been that children with neurodegenerative disorders comprise 41% of their patient group [15]. Patterns of deterioration and symptomatology vary according to the disease process. Care is generally provided by parents in a community setting and children may have little contact with tertiary centres. While something is known of the needs of families caring for children with malignant conditions, relatively little attention has been paid to understanding the palliative care needs of children with neurodegenerative conditions.
Case
John, aged 2 years, was still unable to walk. He was otherwise a bright and engaging child whose other developmental skills were age-appropriate. A neurologist diagnosed Duchenne muscular dystrophy and over two sessions with John's parents, described what was likely to evolve over the following years. John would learn to walk and later lose that skill such that he would be wheelchair-bound by about the age of 8 years. For a time, he would be able to attend and actively participate in school and other activities. Weakness of the upper limbs would follow and John would become increasingly dependent during his teenage years. Later, respiratory failure would occur and John would probably die in late adolescence or early adulthood.
In some circumstances children present early in life with symptoms which may not necessarily lead parents to suspect a life-threatening condition. Indeed the child may be quite well apart from developmental delay, regression, or unsteadiness. In this context, parents must learn that their child has a condition which will cause progressive neurological deterioration and ultimately death. For some, a relatively accurate prognosis may be given and information provided about the nature of the condition and the anticipated rate of decline. For others, the condition is so rare, that little information is available and the prognosis cannot be predicted with certainty. Circumstances occasionally arise in which, despite exhaustive tests, only a broad diagnosis can be made. The clinical course will guide prognosis. Children may be very well in infancy and early childhood with gradual and variable deterioration. The
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subsequent clinical course is often protracted with long periods of severe disability necessitating intensive nursing care. This has important implications for parents who generally assume the role of direct caregiver. Children in the later stages of neurodegenerative disease experience problems with communication, mobility, and feeding. Respiratory difficulties occur in many children secondary to reduced mobility, weakness, aspiration, and spinal deformities. Excessive secretions may also be problematic and for some patients, suctioning is required around the clock [16]. Central nervous system involvement in these conditions may result in seizures and irritability as well as an inexorable decline in intellect and communication making the assessment of symptoms very difficult.
Many children experience recurrent episodes of severe, acute deterioration which appear life-threatening. The family may be told that death is near and relatives are called to say farewell. The child then recovers. This phenomenon has been termed Lazarus Syndrome [17]. Families describe the emotions that accompany these episodes as intense and exhausting. The child could die at any time but might also live for months or even years a situation which has been referred to as certain death at an unknown time [18]. Some parents harbor a secret wish that it would all be over but feel afraid or guilty to share this with even their closest relatives and friends.
Although relatively little attention has been given in the literature to the palliative care needs of children with neurodegenerative conditions, something has been written about respiratory care in the advanced stages of conditions like Duchenne muscular dystrophy and spinal muscular atrophy. Parents are now faced with difficult decisions in these situations particularly in relation to the question of ventilatory support [19] (see also Chapter 27, Respiratory symptoms). This raises the complex interaction between children with life-limiting illnesses and medical development. As the child makes various transitions over time so too does the health care system and its capacity to offer new therapies and modes of support. In the past, boys with Duchenne muscular dystrophy and their families expected death to occur in late adolescence or early adulthood. More recently, various forms of ventilatory support have become available. Non-invasive forms of ventilation used overnight may have an important role in managing the daytime symptoms of respiratory failure thereby enhancing quality of life. More invasive or continuous forms of ventilatory support do not offer a cure for the underlying condition, but offer the prospect of increased longevity [20]. In this way, what was once an anticipated and expected transition to the terminal phase of a progressive illness has become blurred by advances in technology.
Non-progressive conditions which result in an increased susceptibility to complications and premature death
Case
Lisa was diagnosed in the first year of life with infantile spasms, spastic quadriplegia, and developmental delay. At age 7, her oral intake was inadequate and a gastrostomy was placed percutaneously. At age 12, she was unable to walk and her ability to communicate was minimal. Each winter and autumn, she would be admitted frequently (at least monthly) to hospital with respiratory infections and protracted seizures. There had been numerous occasions when her parents had been asked to prepare for her imminent death. Each time, Lisa had recovered. Her father felt her quality of life was extremely poor and believed care should be limited to pain and symptom control. Her mother had seen Lisa recover from life-threatening complications and did not want to give up . She believed care should include intravenous antibiotics, intubation, and ventilation.
Children with severe spastic quadriplegia are vulnerable to premature death as a result of respiratory infection or intractable seizures. Although their condition is not progressive they share many features with children who have neurodegenerative disorders including the uncertainty surrounding their prognosis. This case highlights the difficulties encountered in circumstances where the prognosis in terms of time is unclear and families are reluctant to forego interventions which have been successful in the past.
While a particular condition may be non-progressive, changing developmental needs mean that a child's quality of life may be viewed quite differently at various points in time. Similarly, the burdens and benefits of any particular intervention are measured differently according to the child's stage of development and by different people involved. Consequently, the goals of care need to be re-evaluated and renegotiated over time.
Special circumstances
Perinatal diagnosis of a fatal condition
In developed countries, birth defects constitute one of the commonest causes of neonatal death and infant mortality [1, 21]. Antenatal screening and advances in diagnostic imaging and genetic testing mean that parents may be aware of a lethal congenital condition affecting their child from quite early in the pregnancy. Some families will elect to terminate the pregnancy but others will continue albeit knowing their child will die soon after birth. In the past, such abnormalities would have led to unexpected neonatal death and there has been a growing literature on the impact of this on parents, in
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particular on mothers. Now, parents may know that their child will die for months before this actually occurs. The sophistication of the technology which allows prenatal diagnosis has not necessarily been matched by understanding regarding the optimal way of managing such a pregnancy. While there is a paucity of medical literature to guide practice in this area, some writers have advocated a model of care for these families known as perinatal hospice [22]. In this way, the fundamental principles of palliative care are applied to families who find themselves in this situation. These principles include regarding the family as the unit of care, ensuring pain and other symptoms are controlled, considering the emotional, social and spiritual elements of care, and providing support from diagnosis through death and into bereavement. Parents who elect to terminate a pregnancy after receiving a diagnosis of fetal abnormality can also be expected to benefit from ongoing care in the period leading up to and following this difficult decision.
Case
A male fetus is diagnosed with megalencephaly, aqueduct stenosis, and severe hydrocephalus at 18 weeks gestation. There is evidence of only residual cortex and the prognosis is extremely poor. The family elect to continue the pregnancy and are informed that the infant will probably die in the first few days of life. Provision is made for the child to be cared for on the neonatal ward and the parents are assisted to make decisions regarding the nature of the care provided. The infant is delivered by elective Cesarean section at 37 weeks gestation and survives the first few days of life. His head circumference begins to enlarge rapidly and the insertion of a ventriculoperitoneal shunt is considered as a way of managing what is emerging as a major practical problem. The child begins experiencing episodic apnoea and the insertion of a shunt is no longer considered appropriate. The family members suddenly decide that they wish to care for him at home but no supports have been arranged. In the absence of other services and in their own time, a nurse and a doctor from the hospital visit the family at home and support them through apnoeic episodes until two days later, the infant dies. The parents feel positive about having been able to care for their child at home and are supported by family and friends during bereavement.
Effective palliative care involves anticipating potential symptoms and other eventualities and planning for these in advance. In this case, the infant survived longer than was anticipated so community supports were not established. Although the family's wishes were ultimately accommodated, there was a delay and hospital staff were required to visit the child at home some distance away. Families may feel committed to a certain plan of care early in their child's illness but often change their minds suddenly or as circumstances change. Even a short period of time at home may help parents feel that they have brought their baby home and may allow them to create important memories and spend time with family and friends. The service system requires flexibility and preparation so it can respond quickly to requests such as the one described above. This case also provides an important reminder that prognostication is an inexact science and one should prepare for both the expected and the unexpected.
Familial conditions
Case
Inisha was diagnosed with renal medullary cystic disease at age five. Her younger sister was also found to have the disease. Management aimed at slowing the progression of renal insufficiency was initiated for both the girls and dialysis commenced some years later. Inisha underwent renal transplantation but suffered severe rejection. A second transplant was also complicated by rejection and the family asked that Inisha be discharged home from the intensive care unit after it became clear she was dying. She spent 12 hours at home where her sister was well-informed about and very involved in Inisha's care. A palliative care team was involved at short notice and she died peacefully. Both her doctor and family were concerned that the situation be managed carefully to enable Inisha's sister to continue her treatment and cope with the heightened awareness of the life-threatening nature of her condition.
Some families find themselves in the devastating situation of having more than one child affected by a genetically determined life-limiting condition. Little has been written on the experience of such families but their circumstance requires special mention as there are significant implications for both parents and siblings. The guilt commonly experienced by parents who have children with genetically determined conditions is compounded as are the physical, emotional, and financial burdens associated with caring for a sick and dying child. Younger siblings witness the progression of an illness that they know they have and future challenges are played out on a daily basis. Such a complex scenario requires careful management with particular attention to the principles of honesty and trust. Many familial conditions begin with subtle signs such as developmental delay, regression, or unsteadiness. Where genetic testing is unavailable, families describe watching healthy siblings intently for early signs of illness. Those able to access prenatal diagnosis face the prospect of terminating a pregnancy and the subsequent grief and guilt associated with such a difficult decision.
Organ transplantation
Organ transplantation has become an option for some children with life-threatening conditions. These include various cardiac anomalies and cystic fibrosis. While this intervention offers hope to children who would in the past have certainly
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died, there are no guarantees. Criteria for acceptance onto the transplant list may be narrow, donor organs may be difficult to obtain and survival rates post-transplant may be poor. Families and those caring for them are faced with the difficult task of balancing the hope of cure with the probability or possibility of death. In this way, they are asked to hope for the best and prepare for the worst'. Although the transition may not be clear, elements of palliative care can sit comfortably along side active efforts to pursue cure including listing a child for transplant. Children and their families can be supported through the uncertainty that they face and the child's symptoms can be carefully managed. A mixed model of care, as described earlier, is an appropriate approach to this sort of a situation [7, 8].
The effects of transition and the meaning of illness
Ask not what disease the person has, but rather what person the disease has. (attributed to) Sir William Osler. [23]
The experience of illness involves numerous transitions for children and their families: from healthy to sick, from person to patient, from home to hospital, from parent to nurse . There are also numerous points of transition along the illness trajectory from a worrying symptom complex to a diagnosis, from the diagnosis of a potentially curable illness to an incurable illness, from life to death. For children with neurodegenerative conditions, these transitions may be less clear and less predictable, their families live with chronic uncertainty never knowing which episode of deterioration will be the last.
The child
The transition from health to ill-health has important implications for the child, their parents, the wider family, and the community. For the child, the transition to ill-health is experienced differently at various stages of development. In addition to physical symptoms, the infant or toddler may experience separation from key attachment figures and the home environment as well as the disruption of routines. Parents of sick toddlers may also be more protective at a time when the child is needing to develop a sense of autonomy and self [24]. They often have difficulty setting and enforcing limits.
Preschoolers have distinct characteristics which impact upon their experience of illness. Their tendency to think magically may mean they view illness and procedures as punishment for wrongdoing. The development of peer relationships may be impaired by illness and the capacity to master new skills may also be compromised by pain and disability.
School-aged children develop increasing independence and become more reliant on peers for support. There may be a strong desire to be like other children. School represents a significant part of the child's life and is more than a place of learning.
Cognitive ability influences the way in which children conceptualise illness, but their understanding changes over time as they develop and acquire information and experience. Children are known to be very proactive in seeking information about their condition. In addition to the information provided by parents and health professionals, they learn from other children with similar illnesses on the ward and in the outpatient clinic and they watch the progression of disease in their peers. In this way, a 6-year-old child with a chronic illness may know more about illness and death than a healthy 9-year old. Sick and dying children have been found to have a more sophisticated understanding of their illness and prognosis than previously thought and those dying of malignant conditions have been shown to progress through five consecutive stages of understanding [25].
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The final stage is characterized by a preoccupation with death in conversation and play, oppositional behaviour in relation to procedures and medication, and a reluctance to discuss events in the future.
The transition from health to ill-health occurs at the time of another important transition for the adolescent: the transition from child to adult. Serious illness has the potential to disrupt or delay some of the major developmental tasks of adolescence [26]. As well as a growing sense of autonomy, adolescents develop a greater capacity for understanding likely outcomes and are therefore able to participate more fully in decision-making. They gain a sense of future in terms of long-term relationships, child bearing, and employment and therefore come to know what it is they will lose. The experience of this loss of the future is highlighted in the following vignette.
Case
Jennifer was a 13-year old with cystic fibrosis admitted for a severe exacerbation of her illness. In discussing the uncertainty over what could happen over the next while, she expressed her wishes to live long enough to achieve some of her life goals including becoming a marine biologist and having children of her own. When asked if there was anything she might like to do sooner just in case things didn't go as well as everyone hoped, Jennifer expressed her wish to hold and feed a baby. A staff member with
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a young baby agreed to help and Jennifer spent some time cuddling and feeding the infant and pictures were taken for her family. (Personal Communication: Dr. Gerri Frager, Medical Director, Pediatric Palliative Care Service, IWK Health Centre, Halifax, Nova Scotia, Canada with gratitude to the family of Jennifer Rozee.)
Illness increases the adolescent's sense of dependence at a time when they are striving to achieve independence. Consequently, they may feel a loss of control at a time when they are learning to exert control over some elements of their lives. The physical effects of illness and treatment (alopecia, amputation, weight loss or gain) occur at a time of acute awareness of body image and may be a source of great distress to the adolescent. Peer relationships may also be disrupted at a time when they are needed most and opportunities for sexual expression and experimentation may be curtailed. Rates of depression have been found to be higher in adolescents with life-threatening illness [27].
The adolescent (see Chapter 9, Adolescents and young adults) faces particular challenges when confronted with terminal illness and their needs are quite distinct from those of children and adults [28]. One of the added complexities faced by this group is the need to make the transition from paediatric to adult services. Advances in the management of conditions like cystic fibrosis mean children are now surviving into adulthood with life-limiting conditions not frequently encountered in the adult sector. Until recently, sufficient expertise has not been available within that service system and this has created a barrier to this important transition [28].
The parents
The diagnosis of fatal illness in a child strikes at the very core of what it is to be a parent. The role of parent as nurturer and protector is fundamentally challenged by the development of a condition over which they have no control.
For parents, the child's transition to ill-health may be viewed as a loss the loss of the healthy child and all the hopes, dreams, and aspirations that go with it. In this way, much of what is experienced by families at this time may be seen as grief with all the reactions and experiences associated with this phenomenon. Families may experience shock and disbelief, confusion, anger, sadness, fear, and despair [17, 29]. Some describe feeling numb or overwhelmed. When children become ill, parents very often look for some act or omission that may have caused their child's illness. They may believe that they or someone else should have recognised the problem earlier [30]. Self blame or blaming others may be a major part of the reaction. In circumstances where the condition is inherited, parents may feel directly responsible for passing the condition on to their child. Many parents also describe fearing for the health of their other children.
As is the case with the grief that follows bereavement, the reactions of shock, denial, anger, and sadness do not occur in a predictable order. Many reactions may be present simultaneously and some that were apparent initially may fade and reappear at a later time. This is particularly true of children who relapse or suffer a marked deterioration in their condition. Reactions experienced at the time of initial diagnosis are experienced time and time again [31]. Families cope in different ways depending on the nature of the illness, their previous experience with trauma, their coping strategies, culture, social supports, and family dynamics [32].
Families need time to adjust to new information and a sudden shift in thinking should not be expected. The existence of significant discrepancies between physicians and parents in regard to prognosis has been documented [33]. In one study, the parents understanding of the child's prognosis lagged significantly behind the physician's understanding by more than 3 months [9]. Importantly, in cases where both the physician and the parent recognised the child had little chance of cure early, elements of palliative care were more likely to be integrated into the child's care.
Parents of children with life-threatening illnesses have been described as having a variety of needs which may be broadly divided into two categories: conscious and unconscious (see Table 2.1) [29]. Unconscious needs are often more significant but are less likely to be articulated by parents. They are therefore much more difficult for staff to identify. This has significant implications for practice. Health professionals need to be aware that many of the things that concern, and in some cases preoccupy, parents remain unspoken.
Table 2.1 Parents of children with life-threatening illnesses have a variety of needs: conscious and unconscious [29]. | ||||
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There are many other losses incurred as a consequence of illness loss of a way of life, loss of income if one or both parents cease work, loss of friends, loss of privacy, and loss of control. Most importantly for some families, there is a loss of naivety that comes with the realisation that this is not something that happens to others. That life is not just. There is a loss of the certainty which makes daily life possible. For the child there is a loss of confidence in his or her own body. Things that were once taken for granted can no longer be. The desire for normalcy may be intense but illness brings with it the potential for significant disruption to family life and interactions. Special occasions, holidays, and daily activities are constantly threatened by the unpredictability of the child's condition.
The transition to ill-health brings significant changes to the life circumstances of the family. In one study, 73% of parents of children with a non-malignant life-threatening illness described significant effects on their employment status with at least one parent leaving work to care for the child [34]. Reduced income and increased expenditure on travel, medications, and equipment conspire to compromise the family's financial status [30]. The physical effort involved in caring for a sick and disabled child is immense and many need to learn new technical skills such as suctioning, managing nasogastric feeds, maintaining central venous lines, and even operating a ventilator. For parents who take on such nursing tasks there may be a difficulty in reconciling to the role of parent as nurturer and protector and the role of nurse [35]. Many families find that they are unable to maintain regular contact with family and friends and this places them at risk of social isolation. Mothers and fathers appear to cope very differently. Mothers are more likely to be emotionally expressive. Fathers tended to withdraw and focus on practical issues and often feel a sense of helplessness [34]. The risk of mental health problems is increased among women and members of less cohesive families [34].
Families also make important transitions between care settings. Much of the early care may occur in a tertiary paediatric centre. This involves settling into a new context, meeting new people, and learning about the disease, its treatment, and the service system. Many lose confidence and feel a loss of control. Some find the acquisition of information slow and demanding. Much of it comes from other parents [36]. Later in the disease when home care becomes a priority for the child, and therefore the family, there is a shift back to what may now seem a less secure environment. Families leave a setting and group of individuals with whom they have become very familiar and secure [35]. They may feel that the health professionals caring for them are the only ones who can understand their predicament. When a family goes home, community-based staff need to be introduced and a whole new service system negotiated. Some children also move to a hospice setting thus adding another range of staff and another context to understand.
Chronic illness is always associated with an element of uncertainty and it is this aspect that many families find most difficult to cope with [18]. There may be periods of relative calm interspersed with exacerbations of illness and intense anxiety. There is always a sense that stability cannot and will not be sustained. During periods of calm, parents may be able to contain anxiety regarding prognosis but they are always waiting for the next decline. Deterioration in the child's condition upsets the delicate equilibrium and sends the family into uncertainty again. Some have likened this to living on the side of an active volcano [37]. For many children the illness trajectory is not a smooth downward trend but rather a stepwise progression towards death. Families have been found to expend much energy in maintaining the child in the best possible physical condition in an effort to avoid the next step down [18]. They grieve anew each time their child's condition deteriorates. Such anticipatory grief may mean that exhausted parents concerned about the suffering of their child are wishing it would all end. Death may come as something of a relief.
The transition to bereaved parent is associated with multiple losses loss of the living child, loss of the future, loss of identity. Parents may however, experience other less obvious losses. They may lose a way of life and contact with significant individuals upon whom they have relied heavily for support. For some, the care of the child has required turning, suctioning, feeding, and the administration of medication around the clock. For others, there will have been long hospital stays and frequent outpatient visits. Death brings a sudden end to these tasks and leaves a vacuum many find hard to fill in the short term. Contact is lost with staff members and other parents who have seen the family through the intense highs and lows of chronic illness. Social isolation is a real risk.
Much can be said about the devastating impact that life-threatening illness has upon a family but for some there are positive aspects. Parents may find courage and skills that they had not previously recognised and many go on to support others in similar circumstances.
Case
Steven, aged 15 years, sustained serious head injuries in a motor vehicle accident. He was left severely disabled. During his multiple hospital admissions, Steven experienced attitudes and practices which he believed were unhelpful and should be addressed. With the help of his doctor, he and his mother became involved in educating medical staff about how patients experience interactions with their doctors. His mother commented that she would never have imagined herself in such a role prior to the accident and had discovered a previously unrecognized strength of personality.
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Some children and families are able to make meaning out of their situation and view it as an opportunity for personal growth [18]. They talk of meeting people, learning things, and achieving goals that they could not have achieved if their child had not become ill. For others there is a sense that they have been able to appreciate their child's life in a way they may not have done if they had not been faced with fatal illness. They talk of saying things and doing things which families without such pressures do not. While much has been written about the negative impact of chronic illness on family relationships, some families find their relationships strengthened by the adversity they face [30]. Parents have described having a greater appreciation of life and a better understanding of what is really important as a consequence of their child's illness and death. For some there is a sense that they have survived the very worst that life can offer [38]. Grief is a normal human reaction and although the death of a child represents a profound loss, the achievements of surviving family members may effect positive social changes through advocacy and the fruits of individual careers [39].
The siblings
The siblings of an ill child also experience loss and grief. Their sick brother or sister and their parents may become physically and emotionally unavailable to them for long periods. Life as they knew it is changed forever. Siblings who are not adequately informed and included may misunderstand the nature of the illness and its treatment [40]. This may cause them to fear for their own health and the health of others. Siblings frequently experience anxiety and social isolation [41]. They may feel different or even embarrassed as a result of their brother's or sister's illness. They may also feel jealous of the attention the sick child receives and then feel guilty about having such negative feelings. Very often they are torn between friends and family. Siblings often feel unable to share their concerns with already burdened parents and may express their distress indirectly. Developmental regression, academic failure, and social withdrawal may be signs of difficulty [31, 42].
The health professional
Supporting a child through a lengthy illness can be physically and emotionally exhausting for parents as they lurch from one crisis to another, never knowing which deterioration will be the last. Chronic illness can also take its toll on health professionals and burnout is a possibility. Families sometimes fear abandonment and the steadfast and supportive presence of a familiar team can provide a sense of security in such uncertain times. The fear of abandonment is very real. The death of a child is a relatively unusual event and the modern paediatrician is more familiar with cure and prevention than with death and dying. While advances in medicine have led to happier outcomes for the majority of children, there remains a group for whom cure is impossible. The relative infrequency with which death occurs in childhood has implications for those caring for this group of children. Health professionals may feel a sense of failure and impotence. A lack of exposure to dying children may also leave them feeling ill-equipped to support a child and family through this phase of their care. Particular challenges can arise when children and families hit out at members of the team caring for them. It is important to understand that episodes of aggression and anger are usually not personal. Indeed, the capacity of the family to ventilate negative emotions may reflect the safety of the therapeutic environment in which they find themselves. Doctors, nurses, and allied health professionals may also become very attached to the child and family and experience their own grief. All of these responses are normal but in the absence of adequate self-awareness and support, health professionals may, over time, become burnt out .
Burnout is the progressive loss of idealism, energy and purpose experienced by people in the helping professions as a result of the conditions of their work [43]. This may manifest as excessive cynicism, a loss of interest in work, and a sense of going through the motions [44]. Other features include fatigue, difficulty concentrating, depression, anxiety, insomnia, irritability and the inappropriate use of drugs or alcohol.
The consequences for families are significant as staff affected in this way may:
avoid families or blame them for difficult situations
be unable to help families define treatment goals and make optimal decisions
experience physical signs of stress when seeing families.
The quality of care may be compromised and families may become disenchanted with the health professional and seek help elsewhere, sometimes from inappropriate sources.
Supporting children and families through transition
A key function of the health professional is to offer ongoing support through all the phases of the child's illness and all the transitions that the family makes. Reliability and dependability are highly valued by families experiencing such immense change.
Information
Children are endlessly curious and filled with wonder about things great and small. How remarkable it would be if their wonder and curiosity were to halt abruptly only when they were terminally ill.[45]
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When faced with a crisis, an almost intuitive human response is to try and better understand one's situation. Children seek information just as adults do but they may be protected from bad news by those around them. Through the work of Bluebond-Langner and others it has become clear that even quite young children very often know a great deal about their illness and its prognosis even when parents and staff go to great lengths to conceal such information [25, 46, 47]. They may not reveal what they know however, for fear of upsetting their parents. The anxiety generated by misinformation is potentially more harmful than any arising from the truth. Children may have all sorts of fantasies and worries, many of which an adult might not expect. Information presented in a developmentally appropriate way and at a pace determined by the child has the potential to reduce misunderstandings and feelings of isolation.
The amount of information desired by parents varies, but in general, they appreciate honest, accurate information presented in a sympathetic way. Information allows them to understand their situation better but it needs to be presented carefully and at a pace determined by them so as not to overwhelm a shocked and potentially disbelieving family. Repeating information on a number of occasions, writing it down, and even recording it can be helpful as can having a family member or friend present. Opportunities to ask questions and explore certain aspects in more detail are invaluable. Time invested in generating a trusting relationship early in the illness creates a solid foundation on which to face the challenges of palliative care later. Many parents will access a range of resources to better understand their child's condition. These might include books, journals, and information available through the internet. Some of this will be accurate and helpful, some will be confronting, and some will be inaccurate. Families may need help in sorting through what can be a vast array of information.
Parents often talk of losing control of their lives. Providing families with information, including them in case conferences, and viewing them as key partners in the child's care help to increase their sense of control [48].
It is important to be aware that what parents are able to articulate as their needs represents only a small proportion of what is really concerning them. Awareness of some of the unconscious needs described earlier allows health professionals to proactively address them with families where appropriate. Framing questions in ways that normalise negative feelings can help put parents at ease. For example, Some parents have told me they worry a lot about what will happen close to the time of death but feel they shouldn't be thinking of such things. Have you had any of those sorts of worries and is there anything you would like to ask me?'
Parents might also seek guidance as to how best to support their child and how they should inform siblings and other members of the family.
For many parents, key information and support comes from other parents who have lived through what they are currently experiencing. Likewise, older children may find sharing experiences with peers comforting and helpful. Health professionals involved in supporting families should make themselves aware of the various support groups and agencies in their local community so that they can make this information available to families.
Consider the impact on siblings and broader family
In a hospital setting, health professionals are very aware of the needs of the unwell child and their parents. It is easy to forget the other, less visible, members of the family who may be equally affected by the development of serious illness in the child. Siblings and grandparents often benefit from an opportunity to ask questions of medical and nursing staff and this simple intervention may avoid misunderstandings at home.
Listen
As families struggle to make sense of a world gone awry, they may wish to spend time sharing their concerns, fears, hopes, and expectations. Doctors, nurses, and social workers often feel families expect them to be wise and to fix the problem . Health professionals may also expect this of themselves. Parents would obviously wish that someone could cure their child but most know the reality and what they seek is support. Health professionals may feel that they have nothing to offer if they can't cure an illness or solve a difficult situation. On the contrary, by listening to the concerns of parents, providing guidance, affirming their skills and resources, and staying with them through it all, health professionals can make a major difference to how a family copes.
Home care
Minimizing time spent in hospital reduces the stress placed on a family at the time of transition. Many describe the overall experience of caring for their child at home as a positive one [49, 50]. In the familiar and private surrounds of home, children and parents feel more in control and are subject to fewer interruptions and intrusions [35, 51]. Parents are better able to attend to the needs of siblings, and friends and family may be more accessible. Encouraging and facilitating family outings, holidays, and ongoing attendance at school (e.g. by sensitively timing admissions and treatments) can also be helpful. One of the central
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tenets of palliative care is affirming life encouraging life to go on. While home care is desirable for many families, it is important to remember that this is not true for all. Some children and families feel safer in a hospital environment.
School
The school environment represents a significant part of a child's life. Interventions aimed at supporting the child's return to school can significantly enhance the child's ability to participate in school activities and maintain social relationships. Helpful interventions include renegotiating the goals of school attendance, working with and informing the child's teacher (with the family's permission), and providing a clear plan for emergencies. Children unable to attend school might benefit from ongoing contact with peers through email, home visits, and telephone conversations. School attendance is also very important for siblings. It provides a point of relative constancy and predictability in a world disrupted by change.
Spirituality
There is more to spirituality than religion. Indeed, it has been said that all human beings are essentially spiritual [52]. Spirituality has been described as relating to the search for existential meaning within any life experience'[53]. Assisting a family to find meaning in what might seem a tragic and unjust situation may enhance their capacity to manage the many transitions they face. This might involve providing access to pastoral care workers, exploring the spiritual dimensions of patients, and encouraging families to seek out spiritual leaders and resources. Health professionals who care for dying children and their families may also need spiritual support.
Planning and coordination
Families very often move between care settings such as home, hospital, and hospice. In each setting, families interface with a number of individuals from a range of disciplines and there is the potential for intrusion, confusion, and unnecessary replication of services. The transition between places of care and the coordination of the individuals and many agencies involved may be facilitated by the early inclusion of community-based care providers and the appointment of a key worker. This individual might be a general practitioner, a paediatrician, a nurse, or a social worker [54]. Medical care management is important in its own right as children with long illness trajectories often have a range of subspecialists involved in their care. It is important that one of these individuals take overall responsibility for the care of the child to ensure that families are not exposed to a range of conflicting and confusing opinions. Regular communication between those involved is essential.
A proactive approach to care planning may avoid families lurching from one crisis to another [13, 55]. Anticipating the development or exacerbation of symptoms allows decisions and plans for treatment to be made ahead of time. Families feel more secure if they know who to call or where to go. Discussions with families about these issues necessarily includes informing them about what is likely to happen and helping them make decisions regarding the extent and nature of the care to be provided.
Care into bereavement
The time at which the child dies represents a major point of transition for the family. Depending on their circumstances, families may feel anything from relief to overwhelming sadness. Much of their life has been invested in the care of the child and the time of death can mean the end of a way of life. Families who have spent long periods in the hospital leave behind staff members and other families who have formed an important source of social contact and support. The various tasks involved in caring for a sick and dependent child suddenly cease. Some parents find themselves socially isolated and unoccupied. Continuity of care at this time can be very important and is achieved by offering the family a follow-up appointment with the doctor and an opportunity to visit the staff on the ward. Recognition of anniversaries is also important.
Research
Families seek and benefit from accurate information regarding their child's prognosis. They describe uncertainty as one of the most difficult aspects they have to cope with and yet, in most circumstances, prognostication remains an inexact and inadequate science. To date, efforts to enhance our capacity to predict with certainty a particular child's outcome, have met with meager rewards. Data collection, research, and collective thinking are required to better understand the various illness trajectories and clinical indicators of physical decline.
Conclusion
Children with life-threatening or life-limiting conditions make the transition from health to ill-health in a variety of ways. This transition may be sudden or gradual and although the various illness trajectories can be broadly categorised, other variables mean that the individual circumstances for each child and family are unique. Furthermore, children and families make multiple transitions during the same illness: from a child with a symptom complex to a child with a diagnosis; from a child with a potentially curable illness to a child
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with an incurable and fatal illness; from life to death. The impact of illness is such that children must make the transition from person to patient, home to hospital, and able to disabled. Their body image changes too and this may be of special concern to older children and adolescents. Parents are also forced to make transitions. Fatal illness strikes at the very core of what it is to be a parent. The protector and nurturer must witness the ravages of a disease they cannot control. In order to fulfil their child's wish to be at home, parents may need to take on what they see as nursing tasks which may conflict directly with their role as a parent. Their social, financial, and employment status may change significantly. Parents are thrust into a new world in which they must meet new people and learn about the disease, its treatment, and the service system. Much of what is experienced by these children and their families can be viewed as grief in response to a series of losses. For some however, there are significant gains. They uncover personal strengths they did not know they had and are able to appreciate their child's life at a level others do not. The health professional caring for families in these circumstances also has an opportunity to make an important transition as the following account describes.
In my office adjacent to the medical intensive care unit, I have a growing file of letters from relatives of patients we have treated, thanking us for our care. But the majority of these letters are not from families of patients who survived. Rather, most come from people who have lost a loved one, from the bereaved survivors of patients who died in our intensive care unit (ICU). Yet they are deeply grateful for what we did. At first, I found these letters ironic and odd. I expected and basked in appreciation for lives saved. But the ones about lives we could not save those I had trouble understanding. I read the letters over and over, wondering what the writers meant to me Saving deaths, I have come to realize, is as important and rewarding as saving lives. [56]
The face of paediatrics is changing. Many previously fatal conditions of childhood can now be either prevented or cured. However, advances in technology also mean that children who once died early of congenital anomalies and other conditions now live for longer periods in high states of dependency. Much of the early thinking in paediatric palliative care has focused on children with malignancies but the patient group encountered in paediatric palliative care is far more diverse than this. This diagnostic diversity and the prognostic uncertainty encountered in paediatric palliative care means that an integrated approach is required.
There is more to palliative care than terminal care. Many of the principles and fundamental tenets of palliative care can be applied early in the course of chronic conditions to help families live better with a fatal disease. Opportunities for professional development and liaison with experienced palliative care providers can assist paediatric health professionals who deal with children and parents at various points in the illness trajectory so that the experience of care is improved for all concerned. The early establishment of effective communication, trust, and a supportive therapeutic environment inclusive of community providers facilitates effective palliative care later on. A key function of the health professional is to offer ongoing support through all the phases of the child's illness and all the transitions that the family makes. Reliability and dependability are highly valued by families experiencing such immense change.
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