CHAPTER 22

Pediatric Palliative Care: Principles and Practice

Jori Bogetz, MD, FAAP, and Richard Goldstein, MD, FAAP

The Scope and Practice of Pediatric Palliative Care

Pediatricians develop deep, meaningful relationships with patients and their families. Mostly, patient care involves the typical ups and downs of childhood with acute pediatric illnesses. But some patients are challenged by a degree of illness that requires high levels of continuous medical management, care coordination, and advocacy. These children and their families live with significant uncertainty about the future and a “roller coaster” of health experiences that may even involve the child’s death. Along with these difficulties, many pediatricians describe caring for children and families with serious illness as among the most rewarding aspects of their careers.

Palliative care is a framework that prioritizes the well-being of patients and families experiencing serious illness. This is accomplished through careful symptom management; attention to the medical, psychosocial, and spiritual needs of patients through multidisciplinary care; and establishing goals of care based on family-centered priorities. The American Academy of Pediatrics states that pediatric palliative care should be integrated starting at diagnosis and extending into hospice care and bereavement for any child with a serious illness. This care should be provided along with that provided by primary care pediatricians and in conjunction with community-based teams. These standards are illustrated in the concurrent model of palliative care, which is care that incorporates palliative care with disease-directed treatment throughout a child’s illness and extends into bereavement (Figure 22.1). Despite its growing acceptance, palliative care is often incorrectly equated with end-of-life and hospice care. Hospice is a program of coordinated services offering comfort-centered care at the end of life in the home or community setting. Although hospice and palliative care have a shared philosophy and an overlap of many of the priorities of care, palliative care should not be considered only at the end of life, nor is it exclusive of curative or disease-directed care.

Palliative care is often thought of as a specialized area of practice. Subspecialty palliative care (ie, secondary palliative care) is practiced by health professionals with additional training who often provide education and advocacy in the field of palliative medicine and provide direct patient care in quite complex clinical situations. Important elements of palliative care can be practiced by other health professionals with trusted longitudinal relationships and tremendous insights into family dynamics and priorities, however. Primary palliative care is the term used to describe the palliative care provided by nonsubspecialty health professionals. It is the most fundamental form of palliative care that patients and their families receive. The skills of primary palliative care include the abilities to hold basic discussions about prognosis and goals of care, manage basic pain and symptoms, and address family and sibling issues that arise related to the illness course. Another important part of primary palliative care is the relationship that remains between the clinician and family after a child dies. Both primary and secondary palliative care are necessary in helping children and their families live as well as possible with serious illness.

Figure 22.1. Integration of palliative care along with disease-directed treatments and continuing into bereavement.

Adapted with permission from Ferris FD, Balfour HM, Bowen K, et al. A model to guide patient and family care: based on nationally accepted principles and norms of practice. J Pain Symptom Manage. 2002 Aug;24(2):106-23.

Palliative care for children may be considered in the context of 4 major categories of serious illness: conditions for which curative treatment is possible but may fail (eg, cancer with a poor prognosis, complex congenital heart disease); conditions requiring intensive long-term treatment aimed at maintaining quality of life (eg, cystic fibrosis, muscular dystrophy); progressive conditions in which treatment is exclusively palliative after diagnosis (eg, Tay-Sachs disease, leukodystrophy); and conditions involving severe, nonprogressive disability, causing extreme vulnerability to health complications (eg, holoprosencephaly, extreme preterm birth with severe comorbidities). Many of these conditions are affected by medical advances and evolving clinical practices. The use of bone marrow transplant, gene therapy, and immunotherapy, as well as the increasing application of known therapies such as noninvasive ventilation, have important implications when counseling patients and families. These evolving norms provide opportunities for health professionals to consider categories of illness while simultaneously embracing uncertainty about outcomes for their patients in using a palliative care framework.

To implement appropriate palliative care, families often need an array of different providers, including doctors, nurses, social workers, psychosocial health professionals, developmental specialists, and spiritual supports. They often rely on a hospice or community-based palliative care program, a home nursing agency, or their primary care physician to help coordinate care. The care of children with serious illness may additionally involve representatives from school, camp, child life services, massage, hippotherapy, developmental therapists (eg, art, occupational, speech, physical), and others. Strong communication and reliable continuity within the team are essential. Ultimately, the goal of primary and secondary palliative care, as well as hospice care, is to promote the sense

that a child’s life was lived with identity and value, in contrast to a life determined by the course of illness alone.

Communicating Prognosis, Disclosure, and Decision Making

The care of children with serious illness can be marked by periods of intense uncertainty and fearful realities. Helping families determine the goals of care, appropriate treatment choices, and all aspects of planning for the child’s life is facilitated by thoughtful communication. Research has found, however, that many parents feel they receive confusing, inadequate, or uncaring communication related to prognosis and treatment. They report often feeling left to reach decisions with an understanding of medical details that is different from that of their child’s health professionals. Such decisions are especially difficult in a setting of misunderstanding, disagreement, or, worse, lack of trust. When feeling misunderstood or judged, parents may feel conflicted about stating their true perspective.

To this end, parents benefit from honest, clear communication about their child’s illness. This is a difficult task, not only because imparting bad news is an uncomfortable and complex task but also because providing an exact prognosis is fraught with challenges. The most recent systematic reviews on prognosis prediction have found that physicians are accurate only approximately 25% of the time and tend to be overly optimistic. Although more experienced health professionals tend to err less, the longer the length of a relationship with a patient, the greater the likelihood that the prognosis they share will be incorrect. Parents nonetheless seek a clear disclosure that allows them to have a sense of the future. They interpret hidden or minimal information as evidence that the health professional is withholding frightening information. In fact, parents who receive more elements of prognostic disclosure are more likely to report communication-related hope, even when the likelihood of cure for their child is low. Often the specific details about prognosis are not necessary and instead, generalities about the expected timeline (eg, hours to days, days to weeks, weeks to months, months to years) provide sufficient information for the family’s planning, coordination, and memory making. Research has also shown that earlier recognition of a poor prognosis predicts an earlier do-not-resuscitate order, decreased use of disease-directed therapies in the last months of life, and an increased likelihood of incorporating the child’s comfort as a goal. It allows for some sense of control and expression of values in overwhelming circumstances.

These difficult conversations can be facilitated through a shared understanding about how a child’s health and function have changed over time. When given time to reflect, families can often recognize these changes. Providing anticipatory care about feeding, breathing, or mobility changes related to the progression of illness can provide a more concrete, tangible basis for action in families related to their child’s increased fragility. Uncomfortable as it is, anticipating death and addressing it clearly and frankly allows some children and families to make choices about how to spend the time remaining.

Recent research shows that health professionals can help parents feel like “good parents” to their seriously ill children by letting families know that all that can be done for their child is being done and not giving families the sense that health professionals are “quitting”; respecting parental decisions; providing comfort to the child and family; demonstrating knowledge of the particular needs of the individual child/family and that the child is uniquely special; coordinating care and providing honest, factual information; inquiring about spiritual needs; and telling parents that they are seen as acting as good parents to their child. Table 22.1 outlines a stepwise approach to communicating with children and families after prognostic information has been shared to introduce palliative concepts and better understand how to support an individual child and family in these difficult circumstances. Health professionals who support families in these ways have profound effects on meaning making and bereavement after a child’s death. Even when health professionals are unsure what to say, acknowledging parents as “good parents” can show humility, build trusting relationships, and provide meaning during an incredibly difficult time.

Suffering and the Power of Hope

Initially, the topics of suffering and hope can seem to be untechnical and unprofessional to the practice of medicine. However, it is hard to imagine concepts more central to the art of healing and medicine’s true purpose. Serious illness and its treatment can cause physical distress, and research has shown that dying children have many physical symptoms, such as pain, fatigue, and dyspnea (see Chapter 14). The effects of a serious illness are much larger and more complex than a physical sensation, however. Suffering was best described by Eric J. Cassel, MD, as “the state of severe distress associated with events that threaten the intactness of a person.” This distress comes from a threat to any of the multiple aspects of personhood—”the lived past, the family’s lived past, culture and society, roles, the instrumental dimension, associations and relationships, the body, the unconscious mind, the political being, the secret life, the perceived future, and the transcendent dimension.” Some authorities recommend using terms other than “suffering” to avoid describing persons as victims or with other emotional terms that are suggestive of helplessness. Figure 22.2 shows a sample of the myriad concerning symptoms that can be interrelated for a child with serious illness, all of which must be considered when thinking about distress in seriously ill children. In caring for patients with serious illness, understanding all aspects of suffering is critical to effective care, and listening and reflection are prerequisites to the necessary healing presence required to care for dying children and their families. Pain and symptom management are foundational, but the relief of suffering requires helping a child and family struggle with issues of meaning and transcendence.

Adapted from Goldstein RD. Eliciting and communicating goals of care for seriously ill patients. EQIPP Course under development, American Academy of Pediatrics, 2018.

Hope evolves from expectations for or belief in a worthy future. It carries a sense of trust and resilience. Serious illness can make it difficult to know what to hope for, trust in, or rely on. Research increasingly shows, however, that aspects of hope and resiliency are sustained throughout the disappointments and upheaval associated with serious illness and even death. This process of evolving goals of care or changing hopes (eg, from hope for cure to hope for time with family or a trip to the beach) occurs naturally, and families often need clear communication about what to expect to engage in this process of “regoaling.” In this way, hope can be sustained when a meaningful way forward is elucidated even in the face of a dismal diagnosis or the loss of a child.

Figure 22.2. Interrelated symptoms of distress in children with serious illness.

Barriers to Palliative Care

Parenting a child with serious illness can be frightening, confusing, frustrating, and exhausting. A decision to involve palliative care professionals is in no sense a decision by parents or other health professionals to stop working tirelessly for the child. It is crucial to introduce palliative care with sensitivity and thoughtfulness. Often palliative care is inaccurately viewed as an act of abandonment or “giving up.” Research shows that often health professionals are reluctant to engage palliative supports for families because of perceptions of the family “not being ready” to acknowledge or accept a child’s poor prognosis. This is counter to the growing body of research demonstrating that parents generally accept palliative care and believe that it is offered late. This delayed involvement of palliative care specialists results in not only worse symptom management and quality of life for ill patients, but may also result in shorter survival. Additionally, children who receive palliative care supports take fewer medications, undergo fewer invasive interventions, and are less likely to die in an intensive care unit compared with those who do not receive such care.

One of the greatest obstacles to palliative care and hospice for children has historically been lack of trained specialists. This is becoming less of a barrier as more hospitals and communities have access to pediatric palliative care specialists and with broader education, advocacy, and knowledge about the field. Workforce shortages are also eased by training in primary palliative care skills for primary care pediatricians. Despite concerns about the time needed to address a family’s palliative care concerns, these practices can help pediatricians feel more useful and create deeply meaningful experiences with their seriously ill patients and families.

Another barrier has resulted from the payment model for pediatric palliative care, which historically has been based on Medicare hospice benefits that require patients to have a prognosis of 6 months or less and to forego curative care. Understandably, parents find it quite hard to give up curative efforts for their children. Although some states offer palliative care programs to remedy this, the Patient Protection and Affordable Care Act of 2010 removed the prohibition against curative treatment as a hospice benefit by or on behalf of a Medicaid or Children’s Health Insurance Program eligible child. Despite these changes, eligibility still requires a physician to certify the child as likely to die in the next 6 months. It is not uncommon for children to live beyond their expected prognosis, and in these instances children can be re-enrolled to continue receiving hospice benefits. States are still not required to provide pediatric specific hospice services, however, although doing so is increasingly the norm. Advocacy for increased access to palliative and hospice services for all children with serious illness is crucial to expanding expert palliative supports for these children and their families.

A Child’s Understanding of Death

Although death itself is an unknown experience, parents want to know what their children can understand during the dying process. Caregivers, too, wonder what a child knows as they try to anticipate needs or reckon with enigmatic statements from them. Most would agree that a child’s distress resulting from avoidance of discussions of their fears about their condition and death is especially tragic and counterproductive.

Health professionals should, when possible, communicate with children about what is happening to their bodies, while respecting the cultural and personal preferences of each family. A developmental understanding of children’s concepts of illness and death can help frame these discussions and serve to advise parents in their own conversations with their children. The understanding of a child’s concept of death can be extrapolated from Piaget’s work in cognitive development and is based on 4 stages: sensorimotor, preoperational, concrete operations, and formal operations. These developmental stages of understanding of death along with their typical chronologic ages, along with sample language to use when talking to children about illness and death, are shown in Table 22.2. Having limited cognitive abilities will affect a child’s understanding of these issues. In contrast, illness profoundly changes development, and it is not uncommon for seriously ill children to possess a precocious and advanced understanding of certain things, such as the details of their illness or the way to address their symptoms, while simultaneously being less mature in other developmental areas.

Parents can struggle with whether they should talk with their child about the child’s imminent death. Although research supports a bias toward speaking frankly to dying children, each individual situation should be considered unique and based on the child’s age, cognitive development, disease, timeline of disease, parental psychological state, and family culture. In studies of parental disclosure to children with impending cancer death, parents who talked with their child about their death had no regrets, but among those who did not speak frankly about imminent death, more than 25% regretted not having done so. During these difficult times, primary care pediatricians can provide essential contributions to the care of seriously ill children and their families by serving as a resource for these conversations and providing guidance to families. Collaboration with pediatric palliative care specialists can support pediatricians’ involvement through education and an added layer of support.

Adapted with permission from Himelstein BP, Hilden JM, Boldt AM, Weissman D. Pediatric palliative care. N Engl J Med. 2004;350(17):1752–1762.

Pediatric Primary Care at the End of Life: Normal and Extraordinary

Primary care pediatricians can play an important role in the care of children with serious illness. The presence of a constant, continuous physician who understands the child, siblings, and family holistically in the community can contribute profoundly to care as part of the medical home. Because caring for these children is a rare experience in general practice, the complexities of care can be daunting. Primary care pediatricians can benefit from creating a network with others involved in the care of the child (eg, home nurses, social workers, teachers, pediatric subspecialists, hospice) to support their direct care of seriously ill children and families throughout illness and end of life.

For seriously ill children and their families, general pediatric care from their primary care pediatrician also can be an important affirmation of the normal and can contribute to quality of life. For the child, routine health care visits, vaccinations, and developmental assessments are important, because the rationale for this care is not their disease but the positive and ordinary characteristics of childhood. The focus on development, education and learning, social engagement, play, and involvement with family and community affirms the whole child and the importance of the child’s life. The primary care pediatrician is often seen as a trusted advisor who understands the family and child in this normal context and thus “knows” them best. The pediatrician is the child’s doctor without qualification.

A trusted primary care pediatrician also has a role in complex disease management. The primary care pediatrician is in the best position to assess the family’s level of understanding and address any gaps in that understanding. Importantly, the pediatrician can help families understand complex medical information, terminology (ie, medical jargon), and other specific medical details. They can also help the medical team better understand the patient and family perspective. The primary care pediatrician can ease the adjustment between home and hospital and help ensure more seamless transitions in either direction. Relevant involvement of the pediatrician in crucial developments requires timely sharing of information among all members of the team, the generalist, and the specialist. Ideally, the primary care pediatrician should participate in hospital-based family and provider team meetings. This is especially important during meetings in which goals of care and medical decision making are the focus. Fostering collaboration between the primary care pediatrician and the palliative care specialist is essential. Currently, this can more readily be accomplished through advances in technology, such as telehealth and videoconferencing.

When a child is facing the end of life at home, the primary care pediatrician has additional responsibilities to facilitate care for the child, siblings, and family. The primary care pediatrician can work with the hospice team to continuously fine-tune approaches to pain and symptoms. Primary care pediatricians can also play a central role in delineating resuscitation orders. Research indicates that parents consider end-of-life decisions to be the most difficult treatment decisions they face. Thoughtful conversations with a trusted primary care pediatrician who has a broad perspective on the medical details as well as the family identity and priorities is invaluable. A powerful resource to guide these discussions is the document My Wishes, which offers open-ended prompts with spaces to draw pictures for young school-age children to better understand their worries and hopes. Voicing My Choices is another excellent resource for adolescents and young adults; this resource captures not only who the patient is as a person but also the patient’s end-of-life wishes. Five Wishes can be used when working with adults and is a legally recognized advance care planning tool when completed correctly. Another important form to consider completing with families is the Physician Orders for Life-Sustaining Treatment (POLST) form. The POLST form differs slightly by US state and serves as a home-based do-not-resuscitate order. For instance, emergency medical technicians in the field must resuscitate a child found in cardiac or respiratory failure unless orders exist to refrain. After a family has come to the difficult decision to limit interventions, it is important to advocate for these wishes in all settings and to complete documents to communicate these decisions. Asking parents to sign these forms can be heartbreaking, but framing their completion as a part of documenting a conversation can be helpful. Reaching out to local palliative care specialists to talk through the documents and communication strategies beforehand is equally beneficial.

Loss and After

The loss of a child or sibling changes life forever. It begins a process of bereavement, the psychological and spiritual accommodation to death on the part of the child’s family, and grief, the emotional response caused by the loss. Grief can cause distress and physical and emotional pain, but, except in cases of prolongation, it is a normal adaptive human response, not a disease. Anticipatory grief begins with the awareness of impending loss or death in parents and children with sufficient awareness and cognitive development. Palliative care attends to the grief reaction before and after death. Assessing the coping resources and vulnerabilities of the affected family system before death occurs is central to the palliative care approach.

Generally, parental grief is more intense and sustained than other types of grief. Parents may never completely accept the loss of their child. Research suggests that parents who share their problems with others during their 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. Surviving siblings also grieve. The American Academy of Pediatrics recommends that primary care pediatricians reach out to children and families at the time of loss to evaluate their bereavement and to understand the personal meaning of their loss and their process of mourning. Those involved in the care of children and families appreciate how meaningful simple things, such as condolence letters and attendance at funerals or memorial services, are to families. Notes remembering the anniversary of the deceased child’s birthday or helping the family think about developing the child’s legacy or a remembrance also contribute to improved bereavement and meaning making after a child’s death. Helping bereaved children safely remember their deceased siblings and appreciating their successes as they integrate the loss can be a powerful part of a health care visit and the long-standing caring relationship between primary care pediatricians and families. Often primary care pediatricians can be in contact with the school and other community organizations that can support bereaved siblings. When necessary, referrals to skilled mental health professionals should be offered, including referrals to bereavement specialists.

Many resources are available to support primary care pediatricians caring for seriously ill children and their families. Helpful resources include the Palliative Care: Conversations Matter campaign (www.ninr.nih.gov/newsandinformation/conversationsmatter/about-conversations-matter) and Together for Short Lives (www.together-forshortlives.org.uk/about-us/). For families, the Courageous Parents Network (https://courageousparentsnetwork.org/) is an excellent resource that describes many aspects of caring for a seriously ill child. Most importantly, pediatric palliative care specialists locally and nationally are available to support the essential work of primary care pediatricians in their care of seriously ill patients and families. Working together, the pediatrician and palliative care specialists can address the needs of these children and their families.

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