Pediatric Palliative Care

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Chapter 40 Pediatric Palliative Care

Christina Ullrich, Janet Duncan, Marsha Joselow, Joanne Wolfe

According to the World Health Organization, “Palliative care for children is the active total care of the child’s body, mind and spirit, and also involves giving support to the family…Optimally, this care begins when a life-threatening illness or condition is diagnosed and continues regardless of whether or not a child receives treatment directed at the underlying illness.” Provision of palliative care applies not only to children with cancer or cystic fibrosis but also those with diagnoses such as complex or severe cardiac disease, neurodegenerative diseases, or trauma with life-threatening sequelae (Table 40-1). While palliative care is often mistakenly understood as equivalent to end-of-life care, its scope and potential benefit extend before and well after end-of-life care and is applicable throughout the illness trajectory. Palliative care emphasizes optimization of quality of life, communication, and symptom control, aims that may be congruent with maximal treatment aimed at sustaining life.

Table 40-1 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
High-risk solid organ transplant candidates and/or recipients such as lung or multivisceral
Chronic or severe respiratory failure
Muscular dystrophy

PROGRESSIVE CONDITIONS IN WHICH TREATMENT IS ALMOST 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

Adapted from Himelstein BP, Hilden JM, Boldt AM, et al: Pediatric palliative care, N Engl J Med 350:1752–1762, 2004.

Such comprehensive physical, psychologic, social, and spiritual care requires an interdisciplinary approach. Worldwide this is possible with creative use of professional and community providers. Organizations such as the Children’s International Project on Palliative/Hospice Services share existing clinical and scientific knowledge in an attempt to establish international standards for palliative care. In the USA, the American Academy of Pediatrics has delineated essential elements of pediatric palliative care. The National Consensus Project released its second edition of Clinical Practice Guidelines for Quality Palliative Care, endorsed by 39 medical, nursing, and social work organizations as well as the International Association for Hospice and Palliative Care.

Approximately 54,000 children (birth to 19 yr) died in 2005 in the USA. This number has remained relatively unchanged over the past several years, and half of the deaths occur in acute-care hospitals. Among children who die from cancer, about 50% die in the hospital and 50% at home. Approximately 65% of childhood AIDS deaths occur in the hospital. In many developing countries, the majority of pediatric deaths occur at home, with or without palliative care.

Pediatric palliative care should be provided across settings, including the hospital, outpatient settings, the home, and sometimes in hospice programs. In the USA, the insurance structure and frequent use of medical technology (e.g., home ventilatory support) precludes formal enrollment of a child on the hospice benefit. A growing number of home care agencies offer palliative care programs that serve as a bridge to hospice services. Some freestanding hospice houses will accept children, although many families and children prefer to be at home, if at all possible, through the end of life. Despite establishment of such programs, provision of palliative care for children is often limited by the availability of clinicians who have training or experience in caring for seriously ill children.

The mandate of the pediatrician and other health care providers to oversee children’s physical, mental, and emotional health and development includes the practice of palliative care for those children who live with a significant possibility of death before adulthood (Fig. 40-1). Many pediatric subspecialists care for children with life-threatening illnesses.

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Figure 40-1 Typical illness trajectories for children with life-threatening illness.

(From Field M, Behrman R, editors: When children die: improving palliative and end-of-life care for children and their families, Washington, DC, 2003, National Academies Press, p 74.)

Compared with adult palliative care, pediatric palliative care has:

• Smaller numbers of children who die. Professionals specializing in the care of children may only rarely encounter the death of a child.
• A broad spectrum of illnesses, including many rare diseases. In pediatrics, the wide range of often poorly understood disorders limits generalization from one disease-specific research study to another.
• Unpredictable illness trajectories with significant prognostic uncertainty. It is difficult to predict accurately the progression of illness for many life-threatening illnesses in pediatrics. Such uncertainty may compound child and family distress. Unpredictable trajectories may require palliative care services for months or years, which may not be easily sustained by some programs.
• More confusion about the integration of palliative care with life-prolonging care. Parents and professionals who care for children often consider interventions that are accepted as life-extending, but may also be palliative, such as noninvasive respiratory ventilation, blood transfusions, or parenteral nutrition.
• Parents are most often the decision-makers, but involving the child/adolescent in a manner consistent with the family communication style is also important. This triad (parent–child–interdisciplinary providers) of decision-makers also complicates the process of assessing symptoms, choosing therapies, and evaluating outcomes.
• The added emotional burden of child death. For a parent, this is unnatural and out of order and considered to be among life’s most significant tragedies. The cumulative emotional impact on pediatric clinicians is also substantial.

Medical and technological advances have resulted in an increase in the number of children who live longer, often with significant dependence on new and expensive technologies. These children have complex chronic conditions across the spectrum of congenital and acquired life-threatening disorders (Chapter 39). Children with complex chronic conditions may benefit from simultaneous palliative and curative therapies. These children, who often survive near-death crises followed by the renewed need for rehabilitative and life-prolonging treatments, are best served by a system that is flexible and responsive to changing needs.

Care Settings

Home care for the child with a life-threatening illness requires 24 hr per day accessibility to experts in pediatric palliative care, a team approach, and an identified coordinator who serves as a link between hospitals, the community, and specialists and who may assist in preventing and/or arranging for hospital admissions, respite care, and increased home care support as needed. Adequate home care support and respite care, though sorely needed, is often not readily available. Furthermore, families may feel using respite care is a personal failure, or they may worry that others cannot adequately care for their child’s special needs.

At the end of life, children and families may need intensive support. This may be provided in the home, hospital, or hospice house. Families need to feel safe and well cared for and given permission, if possible, to choose location of care. In tertiary care hospitals, most children die in the neonatal and pediatric intensive care units (ICUs). The philosophy of palliative care can be successfully integrated into a hospital setting, including the ICU, when the focus of care also includes the prevention or amelioration of suffering and improving comfort and quality of life. All interventions that affect the child and family need to be assessed in relationship to these goals. This proactive approach asks the question “What can we offer that will improve the quality of this child’s life?” instead of “What therapies are we no longer going to offer this patient?” Staff need education, support, and guidance since pediatric palliative care, like other types of intensive care, is an area of specialty. Comprehensive palliative care also requires an interdisciplinary approach that may include nurses, physicians, psychologists, psychiatrists, social workers, religious counselors, child-life specialists, and trained volunteers.

Communication, Advance Care Planning, and Anticipatory Guidance

Although accurate prognostication is a particular challenge in pediatrics, the medical team often recognizes a terminal prognosis before the prognosis is understood by parents. This time delay may impede informed decision-making about how the child lives at the end of life. Given the inherent prognostic uncertainty of a life-limiting diagnosis, discussions concerning resuscitation, symptom control, and end-of-life care planning should be initiated when the physician recognizes that a significant possibility of patient mortality exists. These conversations should not happen during a crisis, but should occur well in advance of the crisis or when the patient has recovered from a crisis, but is at high risk for others. Patients and families are most comfortable being cared for by physicians and other care providers with whom they have an established relationship; these individuals may not have had the benefit of palliative care training. The services of a palliative care team or hospice program can be consultative to the primary or subspecialty care team and/or the patient and family, and help alleviate physician discomfort in discussions regarding advanced care planning. A consultative palliative care team provides the family with an opportunity to engage in sensitive conversations that they may feel less comfortable having with their primary team, at least initially. Perhaps resulting from long-standing and highly connected relationships, primary providers and families can be mutually protective of one another, and may not engage in conversations that are perceived as promoting hopelessness. The palliative care team can help to begin conversations about difficult and emotional topics in a manner that concurrently promotes hopefulness.

The population of children who die before reaching adulthood includes a disproportionate number of nonverbal and preverbal children who are developmentally unable to make autonomous care decisions. Although parents are legally the primary decision-maker in most situations in the USA, children should be as fully involved in discussions and decisions about their care as appropriate for their developmental status. Utilizing communication experts, child life therapists, chaplains, social workers, psychologists, or psychiatrist to allow children to express themselves through art, play, music, talk, and writing will enhance the provider’s knowledge of the child’s understanding and hopes. Tools such as “Five Wishes” and “My Wishes” have proven to be useful in helping to gently introduce advance care planning to children, adolescents, and their families (www.agingwithdignity.org/index.php).

The Parents

For parents, compassionate communication with medical providers who understand their child’s illness, treatment options, and family preferences and goals is the cornerstone of caring for children with life threatening illness. During this period of time, one of the most significant relationships is that with the child’s pediatrician, who often has an enduring relationship with the child and family, including healthy siblings. Parents need to know that their child’s pediatrician will not abandon them as the goals of care shift to include palliative care. A family’s goals may shift and change with the child’s evolving clinical condition and other variable factors. A flexible approach rooted in ongoing communication and guidance that incorporates understanding of the family’s values, goals, and religious, cultural, spiritual, and personal beliefs is of paramount importance.

Pediatricians should recognize the important role they have in continuing to care for the child and family as the primary goal of treatment simultaneously may be prolongation of life and comfort, relief of suffering and promoting quality of life. Regular meetings between caregivers and the family are essential in order to reassess and manage symptoms, explore the impact of illness on immediate family members, and provide anticipatory guidance. At these meetings, important issues with lifelong implications for parents and their child may be discussed. Such discussions should be planned with care, ensuring that adequate time for in-depth conversation is allotted; a private, physical setting is arranged; and that both parents and/or others who might be identified by the family as primary supports are present. These meetings should first and foremost elicit what parents are thinking or worried about. Included is a review of what was previously discussed, listening to other concerns and issues as they are revealed, having parents repeat back what was said to ensure clarity, and responding with honest, factual answers in areas of uncertainty. By offering medical recommendations based on family goals and the clinical reality, the team can decrease the burden of responsibility for decision-making that parents carry.

Families may look to their pediatrician for assurance that all treatment options have been explored. Assisting a patient’s family to arrange a second opinion may be helpful. Listening to families and children speak about the future even in the face of poor prognosis may help keep the focus on living even while the child may be dying. Hoping for a miracle can coexist for parents even as they are facing and accepting the more likely reality of death.

Parents also need to know about the availability of home care, respite services, web-based support and information, educational books and videotapes, and support groups. Responding to parent requests or need for counseling referrals for themselves, other children, or family is essential.

While broaching the topic may seem daunting, exploration of how parents envision their child’s death, addressing their previous loss experiences (most often with death of an adult relative) and any misconceptions they may have, is often a great relief to parents. Learning about cultural, spiritual, and family values regarding pain management, suffering, and the preferred place of end-of-life care is essential before death. Even raising the thoughts about funeral arrangements, the possibility of autopsy, and tissue donation can be helpful to give parents choices and know that these things can be discussed without fear. A major worry of many parents is in how to involve and communicate with siblings as well as the child about the fact that most likely death is going to occur.

Ratings of high satisfaction with physician care have been directly correlated with receiving clear communication around end-of-life issues, delivered with sensitivity and caring; such communication included speaking directly to the child when appropriate. Communication is complicated by an assumed need for mutual protection in which the child wants to protect his or her parents and likewise the parents want to protect their child from painful information or sadness. Honoring the family’s communication style, values, spirituality, and culture, which may be impacted by the child’s personality style, is critical in these highly sensitive conversations. Evidence shows that parents who have open conversations with their child about death and dying do not regret having done so.

In communications with the child and family, the physician should avoid giving estimates of survival length, even when the child or family explicitly asks for them. These predictions are invariably inaccurate because population-based statistics do not predict the course for individual patients. A more honest approach may be to explore ranges of time in general terms (“weeks to months,” “months to years”). The physician can also ask parents what they might do differently if they knew how long their child would live and then assist them in thinking through the options relating to their specific concerns (suggest celebrating upcoming holidays/important events earlier in order to take advantage of times when the child may be feeling better). It is generally wise to suggest that relatives who wish to visit might do so earlier rather than later, given the unpredictability of the time course of many illnesses.

For the child and family, the integration of bad news is a process, not an event, and when done sensitively does not take away hope or alter the relationship between the family and physician. The physician should expect that some issues previously discussed may not be fully resolved for the child and parents (do-not-resuscitate [DNR] orders, artificial nutrition or hydration) and may need to be revisited over time. Parents of a child with chronic illness may reject the reality of an impending death because past predictions may not have been accurate. Whether they are parents of a child with a chronic illness or of a child whose death is the result of accident or sudden catastrophic illness, they may experience great anxiety, guilt, or despair.

The Child

Truthful communication that takes into account the child’s developmental stage and unique lived experience can help to address the fear and anxiety commonly experienced among children with life-threatening illness. Responding in a developmentally appropriate fashion (Table 40-2

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