The Who, How, What, When, and Where of Pediatric Palliative Care

Smaller numbers of children die compared with adults. In the United States, approximately 55,000 children 19 years of age or younger die per year, compared with 2.3 million adults. Although precise statistics are not known, many children in developed (or richer) countries die in hospitals. For a medium-sized pediatric hospital of 150 to 200 beds, about 100 to 150 children may be expected to die per year. This means that health care workers in a pediatric hospital can expect two deaths per week, yet it has only recently been recognized that the needs of these dying children and their families require specialized knowledge and increased attention. Medical advances have resulted in the current high cure rate of approximately 85% for pediatric cancers, but the fact remains that 15% of such children still die. The relative paucity of pediatric deaths compared with adult deaths, coupled with the cultural taboos surrounding death in general, combine to make the experience of a child’s death a rarity for most people, including health care professionals. A hundred years ago, most families would have had at least one child die, and most physicians would have seen children die as part of their regular practice. Because most pediatric deaths in richer nations now occur in tertiary care hospitals, there are few professionals with experience and training in meeting the needs of the children who do die. The wider cultural milieu in which we live where all deaths are seen as a tragedy that could be avoided “if we just tried harder and had more technology” is amplified in pediatrics where the goal (implicitly or explicitly) is to have all children survive until adulthood This understandable goal often presents, however, as an expectation (“he/she can’t die!”) both by health care workers and by parents. If the unspoken goal is only cure, then death will always be seen as a failure. Much of the work in pediatric palliative care is the reframing of death from a failure to an inevitable part of life, including for some children.

Children die of a larger number of causes than do adults. Adult palliative care services have historically been oncology based, although it is recognized that other illnesses that lead to death in adults would also benefit from the principles of palliative care. The most common causes of death in children are age dependent :

• •
  

  Infants birth to 1 year: Congenital anomalies, prematurity, and sudden infant death syndrome

  
  
• •
  

  Children age 1 to 14 years: Accidental and nonaccidental trauma, cancer, congenital abnormalities, and “other”

  
  
• •
  

  Older children and adolescents age 15 to 19 years: Trauma (including suicide), cancer, and congenital abnormalities such as cardiac disease

Unlike the adult population in which cancer and cardiovascular disease make up a large percentage of the major causes of death, there exists a wide range of often rare diseases that lead to death in children. Although cancer is a common cause of death for adults, it is responsible for only 4% of deaths in childhood.

Large numbers of rare and often poorly understood disorders result in death in children, including rare inborn errors of metabolism and genetic syndromes. For many of these causes of death, little is known about the natural history of the disease and its optimal management, including how to best provide symptom control. For these children, the best way to manage the illness is to apply general principles of pediatric care together with the general principles of palliative care. Another way of answering the question “What is pediatric palliative care?” is to answer that it takes the best practice from general pediatric medicine and combines it with the specialized knowledge of adult palliative care ( Case Study 34-1 ).

Many children who might benefit from palliative care live with illnesses that have an unpredictable time course, which varies from a short-term illness that quickly leads to death to complex chronic diseases that may evolve over years. For complex chronic diseases, the time course may not only be long, spanning years, but may also be unpredictably intermittent in the need for acute and palliative care. For example, a child with severe cerebral palsy and a seizure disorder may be stable for years and then present suddenly with life-threatening aspiration pneumonia that may or may not become a fatal episode. At such times of intermittent crises, it is helpful if a management plan has been prepared in advance, including thinking through the kinds of interventions that may or may not be desired for that particular child (e.g., decisions about whether to provide mechanical ventilation). The life-threatening crisis may evolve into an end-of-life situation, or the crisis may resolve and the child may once again require high levels of supportive and rehabilitative care, including access to supportive services for the handicapped (e.g., seating programs, antispasticity programs, multispecialist care). In this way, pediatric palliative care is often not a smooth transition from health to ill health, but rather an intermittently required service that may need to move in and out of a child’s care plan as needs evolve. It is always helpful to plan ahead by initiating early discussions about how palliative care may be helpful if and when a life-threatening crisis occurs rather than waiting for the crisis to manifest. Evaluations of the child and family that occur between crises have the advantage of assessing the child when he or she is at the best level of function and offer the opportunity for families to reflect on discussions and take the time needed to come to important decisions about care planning.

Even in places where pediatric home palliative care is available, many children who die do so in hospitals. Most children who are able to make a choice prefer to die at home, if possible. There are several possible explanations for why many children still die in hospitals despite their preference to be at home. These include unpredictability—it is often not possible to know when a critical illness is actually a fatal event as opposed to one from which the child may recover; the lack of availability of home pediatric palliative care services; and the need of many parents to feel that everything possible was done to help avoid the death of their child, including the use of medical technologies that are possible only in a hospital. Pediatric and neonatal intensive care units are common locations of pediatric deaths; because these deaths are a frequent occurrence, many units have their own individualized palliative care and bereavement programs. In some centers, hospitals have set up palliative care rooms that allow families to remain together in a more homelike environment than is typically found in a hospital setting. Unlike adult palliative care, there are rarely entire wards in pediatric hospitals dedicated to palliative care. For the most part, pediatric palliative care programs have developed as consultant services (as opposed to primary care services) to help support the primary caregivers in other pediatric specialties. For example, in-hospital palliative care programs may follow children on different speciality wards as care is concurrently delivered with another pediatric subspecialty, such as respirology for patients with cystic fibrosis and neurology for children with severe cerebral palsy. Not surprisingly, some evidence indicates that instituting the supports necessary to provide services in the home results in larger numbers of children who then do die at home instead of at the hospital. In contrast to the United Kingdom, where large numbers of specialized pediatric hospice centers are already in operation, few adult hospices in North America will admit a child for terminal care. For a dying child and his or her family, the ideal situation is to be able to offer them as many options as possible, including the opportunity for care to alternate among hospital, home, and hospice as needs and situations change.

Communication with Children and Families

Communication is the cornerstone on which pediatric palliative care skills and competencies are based. Developing the ability to communicate effectively and compassionately with others in highly emotional situations is learnable with a combination of specific cognitive skills coupled with experientially based learning (in the moment, on the job). Such reflection in action and on action is a process that requires skilled mentors and periods of time in actual clinical practice. Communication (or, in other words, connection with others) is both the biggest challenge in this work as well as its greatest reward. Knowing you have made a positive difference in this work comes from a deep knowing that you have in some way made a caring, compassionate connection with others, regardless of the final outcome of the illness.

Addressing the meaning of hope is a common and challenging task for children, parents, and professionals. The difficult question is sometimes asked directly (“How can we maintain hope in the face of our child’s death?”) or indirectly (“What do we do now?,” “How can we go on?,” “What is the point of anything?”). These questions speak to the need to maintain hope and the struggle to find meaning in the tragic loss that is the death of a child. In the face of such questions (which may be unspoken for some), the role and challenge for the pediatric palliative care professional is to assist in reframing what hope can be when a cure is no longer possible. For example, it can be comforting to know that other families have found a way to maintain hope by shifting from hope for cure to hope for a meaningful life or hope for a peaceful death. A starting point for the health care professional is to recognize his or her own need to maintain hope and to find meaning in the work that he or she does. Hoping is understood to be distinct from wishing. Hope is the understanding that things will somehow be all right, no matter what the outcome. Wishing may be another way to deny reality and is the insistence that, despite the facts, the outcome will somehow “magically” be different. A practical approach to hope is as follows:

• 1
  

  Be mindful that at times loss of hope may be part of the process. Accepting that hope can at times be lost may relieve the sense of failure that can add to suffering.

  
  
• 2
  

  Understand that “being with” people and things the way they are in this moment is sometimes not only all there is “to do” but is sometimes paradoxically everything that is needed. “Don’t just do something, sit there” is a call to “being with” that holds the potential for reducing suffering.

  
  
• 3
  

  Facilitate the process that leads to a shift from hoping for cure, to hoping for quality of life, to hoping that death will be as comfortable as possible or to finding meaning.

  
  
• 4
  

  Create the conditions that allow for the possibility of maintaining hope and finding meaning by optimizing physical comfort and providing care in the best possible setting (e.g., in the home when it is desired and possible).

Understanding children’s developmental concepts of death is important in helping guide the content and timing of communication with ill children and their siblings. It is now understood that even very young terminally ill children are often aware that they are dying, despite efforts their parents may have made to keep that information from them. The following is an approximate guide to how children understand death at different ages:

• •
  

  Very young children, younger than 3 years: These children are mostly preverbal, with their needs focused on immediate physical gratification and emotional security. Their major concerns are often physical comfort and to not be separated from their parents.

  
  
• •
  

  Early childhood, age 3 to 6 years: These children lack appreciation of core concepts of death. At this stage, many fears result from misunderstanding the core concepts of death and may be alleviated by explanation and discussion. These concepts include the following:

  
  
  
  
  • 
    

    Irreversibility : Death is permanent, and the dead cannot be made alive again.

    
    
  • 
    

    Universality : Death happens to everyone, not just old people or those who become sick.

    
    
  • 
    

    Nonfunctionality : Being dead means that you do not have to breathe, or eat, or maintain other body functions.

    
    
  • 
    

    Causation : “Bad” people are not the only ones who die, and wishing death on someone cannot magically cause his or her death. It is important for siblings to understand this because they may have wished their brother or sister “dead” at some time in the past and now wonder whether that wish is coming true.

  
  
  
  
• •
  

  Middle childhood, age 6 to 12 years: At this age children begin to understand the concepts of irreversibility, universality, nonfunctionality, and causation. They may, however, believe that death is caused mostly by external forces such as accidents, although they are beginning to understand that it also occurs via internal (illness) causes.

  
  
• •
  

  Adolescence, older than 12 years: Adolescents may have a disconnect between their factual understanding of the core concepts of death and simultaneous magical thinking that it still cannot happen to them (e.g., risk taking is common at this stage).

Many of the children who require palliative care are either preverbal because of their young age or nonverbal because of their underlying illness. Although communication does occur through nonverbal means, there are many instances in which a lack of cognitive ability limits the child’s ability to comprehend and be an active partner in decisions about treatment. In adult palliative care, even if an adult becomes nonverbal, there is still a previous lived experience of the person to help guide decision making. In contrast, young children with little lived experience depend on proxy decision makers. It is often the parents, as proxy decision makers, who make major decisions for and about their children. One challenge in pediatric palliative care is to help parents make decisions that respect what is in the best interests of their child versus those decisions that may help to alleviate their own legitimate suffering. When it is difficult to separate the nonverbal child’s suffering from that of the parents, it can be helpful to reframe the situation by asking parents to try to interpret what their child may be “telling” them in actions as opposed to in words. For example, parents may interpret repeated life-threatening episodes as meaning that their child is telling them that he or she is “ready to go” and may find some solace in knowing that they have found a way to “listen” to the nonverbal messages their child is sending.

Communication with verbal children includes, but is not limited to, involving children in their own treatment decisions, listening for their preferences about the way they wish to live, and offering them the opportunity to create lasting memories for the bereaved (e.g., memory books, pictures, bequeathing cherished toys, making personalized music recordings, and paintings). The opportunity to express themselves creatively and nonverbally need not be limited to specialists in expressive therapies; simply offering crayons and paper with the instruction to “make any picture you like” and then asking the child to explain what he or she drew can be very helpful in better understanding what the child is thinking.

It is an axiom in pediatric palliative care that the unit of care is both the child and family, with the definition of family meant to include any loved one who is significant to that child. Communicating with parents, siblings, and extended family can be a challenge and will depend on the particulars of each family. Encouraging parents to include siblings in appropriate discussions and involving siblings in care may serve to improve the long-term function of the bereaved sibling.

Offering ideas about how to share meaning even in uncertain times can be helpful. For example, when a child is near the end of life, families may feel stressed by trying to time important celebrations in this unpredictable situation. It can be suggested that other families in similar situations have chosen to have the event (e.g., a birthday or holiday celebration) as soon as possible and then to repeat it on the actual calendar date if the child continues to do well.