CHAPTER 4 Palliative Care and End-of-Life Issues

The death of a child is one of life’s most difficult experiences. The palliative care approach to a child’s medical care should be instituted when medical diagnosis, intervention, and treatment cannot reasonably be expected to affect the imminence of death. In these circumstances, the goals of care focus on improving the quality of life, maintaining dignity, and ameliorating the suffering of the seriously ill child. Central to this approach is the willingness of clinicians to look beyond the traditional medical goals of curing disease and preserving life. They need to look toward enhancing the lives of the child and working with family members and close friends when the child’s needs are no longer met by curative goals. High-quality palliative care is an expected standard at the end of life.

Palliative care in pediatrics is not simply end-of-life care. There are conditions where death is not predictably imminent, and a child’s needs are best met by the palliative care approach. Children needing palliative care have been described as falling into four basic groups (Table 4-1). These conditions present different timelines and different models of medical intervention. Yet they all share the need to attend to concrete elements, which affect the quality of a child’s death, mediated by medical, psychosocial, cultural, and spiritual concerns.

TABLE 4-1 Conditions Appropriate for Pediatric Palliative Care*

CONDITIONS FOR WHICH CURATIVE TREATMENT IS POSSIBLE BUT MAY FAIL

Advanced or progressive cancer or cancer with a poor prognosis

Complex and severe congenital or acquired heart disease

CONDITIONS REQUIRING INTENSIVE LONG-TERM TREATMENT AIMED AT MAINTAINING THE QUALITY OF LIFE

Human immunodeficiency virus infection

Cystic fibrosis

Severe gastrointestinal disorders or malformations, such as gastroschisis

Severe epidermolysis bullosa

Severe immunodeficiencies

Renal failure in cases in which dialysis, transplantation, or both are not available or indicated

Chronic or severe respiratory failure

Muscular dystrophy

PROGRESSIVE CONDITIONS IN WHICH TREATMENT IS EXCLUSIVELY PALLIATIVE AFTER DIAGNOSIS

Progressive metabolic disorders

Certain chromosomal abnormalities, such as trisomy 13 or trisomy 18

Severe forms of osteogenesis imperfecta

CONDITIONS INVOLVING SEVERE, NONPROGRESSIVE DISABILITY, CAUSING EXTREME VULNERABILITY TO HEALTH COMPLICATIONS

Severe cerebral palsy with recurrent infection or difficult-to-control symptoms

Extreme prematurity

Severe neurologic sequelae of infectious disease

Hypoxic or anoxic brain injury

Holoprosencephaly or other severe brain malformations

* Premature death is likely or expected with many of these conditions.

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The sudden death of a child also requires elements of the palliative care approach, although conditions do not allow for the full spectrum of involvement. Many of these deaths involve emergency medicine caregivers and first responders in the field, and they may involve dramatic situations where no relationship may exist between caregivers and the bereaved family. Families who have not had time to prepare for the tragedy of an unexpected death require considerable support. Palliative care can make important contributions to the end-of-life and bereavement issues families face in these circumstances. This may become complicated in circumstances where the cause of the death must be fully explored. The need to investigate the possibility of child abuse or neglect subjects the family to intense scrutiny and may create guilt and anger directed at the medical team.

PALLATIVE AND END-OF-LIFE CARE

Palliative treatment is directed toward the relief of symptoms as well as assistance with anticipated adaptations that may cause distress and diminish the quality of life of the dying child. Elements of palliative care include pain management; expertise with feeding and nutritional issues at the end of life; and minimizing nausea and vomiting, bowel obstruction, air hunger, and fatigue. Psychological elements of palliative care have a profound importance and include sensitivity to bereavement, a developmental perspective of a child’s understanding of death, helping clarify the goals of care, and ethical issues. Curative care and palliative care can coexist; aggressive pain medication may be provided while curative treatment is continued in the hopes of a remission or improved health status. Palliative care is delivered with a multidisciplinary approach, giving a broad range of expertise to patients and families as well as providing a supportive network for the caregivers. Caregivers involved may be pediatricians, nurses, mental health professionals, social workers and pastoral care.

A model of integrated palliative care rests on the following principles:

• Respect for the dignity of patients and families. The clinician should respect and listen to patient and family goals, preferences, and choices. School-age children can articulate preferences about how they wish to be treated. Adolescents, by the age of 14, can engage in decision making (see Section 12). The pediatrician should assist the patient and the family in understanding the diagnosis, treatment options, and prognosis; help clarify the goals of care; promote informed choices; allow for the free flow of information; and listen to and discuss the social-emotional concerns. Advanced care (advance directives) should be instituted with the child and parents, allowing discussions about what they would like as treatment options as the end of life nears. Differences of opinion between the family and the pediatrician should be addressed by identifying the multiple perspectives, reflecting on possible conflicts, and altruistically coming to agreements that validate the patient and family perspectives, yet reflect sound practice. Hospital ethics committees and consultation services are important resources for the pediatrician and family members.

• Access to comprehensive and compassionate palliative care. The clinician should address the physical symptoms, comfort, and functional capacity, with special attention to pain and other symptoms associated with the dying process, and respond empathically to the psychological distress and human suffering, providing treatment options. Respite care should be available at any time during the illness to allow the family caretakers to rest and renew.

• Use of interdisciplinary resources. Because of the complexity of care, no one clinician can provide all the needed services. The team members may include primary and subspecialty physicians, nurses in the hospital/facility or for home visits, the pain management team, psychologists, social workers, pastoral care, schoolteachers, friends of the family, and peers of the child. The child and family should be in a position to decide who should know what during all phases of the illness process.

• Acknowledging and providing support for caregivers. The death of a child is difficult to accept and understand. The primary caregivers of the child, family, and friends need opportunities to address their own emotional concerns. Siblings of the child who is dying react emotionally and cognitively based on their developmental level. Opportunities to have team meetings to address thoughts and feelings of team members are crucial. Soon after the death of the child, the care team should review the experience with the parents and family and share their reactions and feelings. Institutional support may include time to attend funerals, counseling for the staff, opportunities for families to return to the hospital, and scheduled ceremonies to commemorate the death of the child.

• Commitment to quality improvement of palliative care through research and education. Hospitals should develop support systems and staff to monitor the quality of care continually, assess the need for appropriate resources, and evaluate the responses of the patient and family members to the treatment program. Issues often arise over less than completely successful attempts to control the dying child’s symptoms or differences between physicians and family members in the timing of the realization that death is imminent. Consensus results in better palliative care from the medical and psychosocial perspective.

Hospice care is a treatment program for the end of life, providing the range of palliative care services by an interdisciplinary team, including specialists in the bereavement and end-of-life process. Many insurance companies do not provide coverage for these services; children younger than 17 years of age make up only 0.4% of hospice admissions. Typically, hospice uses the adult Medicare model, requiring a prognosis of death within 6 months and the cessation of curative efforts for children to receive hospice services. Recently, some states have developed alternative pediatric models where curative efforts may continue while the higher level of coordinated end-of-life services may be applied.

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BEREAVEMENT

Bereavement refers to the process of psychological and spiritual accommodation to death on the part of the child and the child’s family. Grief has been defined as the emotional response caused by a loss, including pain, distress, and physical and emotional suffering. It is a normal adaptive human response to death. Palliative care attends to the grief reaction. Assessing the coping resources and vulnerabilities of the affected family before death takes place is central to the palliative care approach.

Parental grief is recognized as being more intense and sustained than other types of grief. Most parents work through their grief. Complicated grief, a pathologic manifestation of continued and disabling grief, is rare. Parents who share their problems with others during the child’s illness, who have had access to psychological support during the last month of their child’s life, and who have had closure sessions with the attending staff, are more likely to resolve their grief.

A particularly difficult issue for parents is whether to talk with their child about the child’s imminent death. Although evidence suggests that sharing accurate and truthful information with a dying child is beneficial, each individual case presents its own complexities based on the child’s age, cognitive development, disease, timeline of disease, and parental psychological state. Parents are more likely to regret not talking with their child about death than having done so. Among those who did not talk with their child about death, parents who sensed their child was aware of imminent death, parents of older children, and mothers more than fathers were more likely to feel regretful.

COGNITIVE ISSUES IN CHILDREN AND ADOLESCENTS: UNDERSTANDING DEATH AND DYING

The pediatrician should communicate with children about what is happening to them, while respecting the cultural and personal preferences of the family. A developmental understanding of children’s concepts of health and illness helps frame the discussion with children and can help parents understand how their child is grappling with the situation. Piaget’s theories of cognitive development, which help illustrate children’s concepts of death and disease, are categorized as sensorimotor, preoperational, concrete operations, and formal operations.

For very young children, up to 2 years of age (sensorimotor), death is seen as separation, and there is probably no concept of death. The associated behaviors in grieving children of this age usually include protesting and difficulty of attachment to other adults. The degree of difficulty depends on the availability of other nurturing people with whom the child has had a good previous attachment.

Children from 3 to 5 years of age (preoperational) (sometimes called the magic years) have trouble grasping the meaning of the illness and the permanence of the death. Their language skills at this age make understanding their moods and behavior difficult. Because of a developing sense of guilt, death may be viewed as punishment. If the child’s younger sibling suddenly died and the child previously wished the infant to have died, the reality of a sibling’s death may be seen psychologically as being caused by the child’s wishful thinking. They can feel overwhelmed when confronted with the strong emotional reactions of their parents.

In children age 6 to 11 years of age (late preoperational to concrete operational), the finality of death gradually comes to be understood. Magical thinking gives way to a need for detailed information to gain a sense of control. Older children in this range have a strong need to control their emotions by compartmentalizing and intellectualizing.

In adolescents (>12 years of age) (formal operations), death is a reality and is seen as universal and irreversible. Adolescents handle death issues at the abstract or philosophical level and can be realistic. They may also avoid emotional expression and information, instead relying on anger or disdain. Adolescents can discuss withholding treatments. Their wishes, hopes, and fears should be attended to and respected.

CULTURAL, RELIGIOUS, AND SPIRITUAL CONCERNS ABOUT PALLIATIVE CARE AND END-OF-LIFE DECISIONS

Understanding the family’s religious/spiritual or cultural beliefs and values about death and dying can help the pediatrician work with the family to integrate these beliefs, values, and practices into the palliative care plan. Cultures vary regarding the roles family members have, the site of treatment for dying people, and the preparation of the body. Some ethnic groups expect the clinical team to speak with the oldest family member or only to the head of the family outside the patient’s presence. Some families involve the entire extended family in decision making. For some families, dying at home can bring the family bad luck, whereas others believe that the patient’s spirit will become lost if the death occurs in the hospital. In some traditions, the health care team cleans and prepares the body, whereas in others, family members prefer to complete this ritual. Religious/spiritual or cultural practices may include prayer, anointing, laying on of the hands, an exorcism ceremony to undo a curse, amulets, and other religious objects placed on the child or at the bedside. Families differ in the idea of organ donation and the acceptance of autopsy. Decisions, rituals, and withholding of palliative or lifesaving procedures that could harm the child or are not in the best interests of the child should be addressed. Quality palliative care attends to this complexity and helps parents and families through the death of a child while honoring the familial, cultural, and spiritual values.

ETHICAL ISSUES IN END-OF-LIFE DECISION MAKING

Before speaking with a child about death, the caregiver should assess the child’s age, experience, and level of development; the child’s understanding and involvement in end-of-life decision making; the parents’ emotional acceptance of death; their coping strategies; and their philosophical, spiritual, and cultural views of death. These may change over time, and the use of open-ended questions to repeatedly assess these areas contributes to the end-of-life process. The care of a dying child can create ethical dilemmas involving autonomy, beneficence (doing good), nonmaleficence (not initiating and eliminating or mitigating harm, pain, and suffering), truth telling, confidentiality, or the physician’s duty. It is extremely difficult for parents to know when the burdens of continued medical care are no longer appropriate for their child. Pediatricians, patients, and family members may differ on when this time has come, depending on their beliefs and values of what constitutes quality of life, when life ceases to be worth living, and their religious/spiritual, cultural, and philosophical beliefs. The most important ethical principle is what is in the best interest of the child as determined through the process of shared decision making, informed permission/consent from the parents, and assent from the child. Sensitive and meaningful communication with the family, in their own terms, is essential. The physician, patient, and family must negotiate the goals of continued medical treatment while recognizing the burdens and benefits of the medical intervention plan. There is no ethical or legal difference between withholding treatment and withdrawing treatment, although many parents and physicians see the latter as more challenging. Family members and the patient should agree about what are appropriate do not resuscitate (DNR) orders. Forgoing some measures does not preclude other measures being implemented, based on the needs and wishes of the patient and family. When there are serious differences among parents, children, and physicians on these matters, the physician may consult with the hospital ethics committee or, as a last resort, turn to the legal system by filing a report about potential abuse or neglect.

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