Pediatric Palliative Care in Oncology

Abstract

Pediatric palliative care aims to alleviate suffering and improve the quality of life of children with serious disease and increase support for their parents and other family members. Integration of palliative care into the routine care of children, adolescents, and young adults with cancer has resulted in improved outcomes in patients and their families. The field of pediatric palliative oncology—encompassing primary palliative care provided by the multidisciplinary oncology team as well as subspecialty palliative care provided by the palliative care team for more complex cases—is unique from palliative care in adults given its focus on care of the child and the larger family. In this review, we focus on advancements in the specific domains within pediatric palliative oncology care including family-centered communication, assessment and management of physical symptoms and distress, psychosocial concerns, and spiritual considerations of the patient, parents, and siblings.

INTRODUCTION

Although rare compared with the adult population, cancer remains the leading cause of death by disease in children. Approximately 15,590 children were diagnosed with cancer in 2018,¹ and at any one time, tens of thousands of children and adolescents are living with advanced cancer. The field of pediatric palliative oncology (PPO) has developed to help children with cancer and their families cope with the physical, psychological, social, and spiritual burdens of cancer and treatment. This burgeoning field has been bolstered by recent publications outlining psychosocial standards, care models, and strategies for integration of palliative care into pediatric oncology and unique combined training models.^2-7 Palliative care in pediatrics encompasses the same goals and care domains as palliative care in adults but uniquely focuses on serving the needs of the child and the larger family. Children exist within the context of the family; therefore, holistic care of the pediatric oncology patient also includes care of the parents, siblings, and extended family.⁸

Children with cancer who receive formal palliative care services experience improvements in physical and psychological symptoms, increased number and earlier end-of-life (EOL) care discussions, increased time between advance directive placement and death, and increased hospice enrollment.^9-15 Palliative care has also been associated with improved quality of life (QOL) for both pediatric patients^16,17 and their parents.^11,17,18 For older adolescent and young adult (AYA) patients with cancer, palliative care involvement is associated with less intensive therapy at the EOL¹⁹ and with longer overall survival.²⁰ Finally, palliative care in pediatrics may be associated with improved psychosocial outcomes in bereaved parents as a result of improvement in the child’s symptom control, receipt of less intensive EOL care, and improved preparation for EOL.^21-23

Despite an ever-growing body of literature supporting the integration of palliative care principles into routine oncology care, and acceptance of early palliative care involvement by patients and families,²⁴ many pediatric palliative care programs are weekday, business-hour operations, and more than one quarter have been in existence for less than 5 years.²⁵ The majority of PPO care is provided by the multidisciplinary oncology team (primary palliative care), with subspecialty palliative care (ie, encompassing advanced skills and training) used for management of refractory symptoms and complex psychosocial and communication issues (Fig 1).²⁶ Here, we focus on advancements in the specific domains of PPO care, whether primary or palliative caresubspecialty delivered, at more advanced stages of disease. Domains covered include family-centered communication, assessment and management of physical symptoms and distress, psychosocial concerns, and spiritual considerations of the patient, parents, and siblings.

FIG 1.

Diagram outlining the goals of longitudinal pediatric palliative care and scope of primary and specialty pediatric palliative oncology care.

FAMILY-CENTERED COMMUNICATION IN PEDIATRIC PALLIATIVE CARE

Family-centered communication promotes a dialogue between patients, families, and providers that aims to support “more effective, efficient, and empathic pediatric health care.”^27(e1141) High-quality communication is associated with parental peace of mind, feelings of being acknowledged and comforted, and greater trust in the provider.²⁸ Clear and compassionate communication is especially critical when discussing prognosis and at more advanced stages of cancer care. Not all families ask about their child’s prognosis directly, but most wish to receive prognostic information in as much detail as is available.^29,30 Prognostic understanding can empower families to revisit and reframe their short- and long-term priorities (eg, work, vacations) and treatment decisions and to focus on ways to maximize quality time together.³¹ Parents who receive detailed prognostic information report less decisional regret than do parents who receive less information.³² Children who participate in discussions around prognosis have reduced anxiety and feelings of isolation and increased adaptation to the illness.^33,34 Moreover, parents who involve their children in discussions about prognosis and impending death generally do not regret doing so.^35,36 Parents value assistance from the interdisciplinary palliative care team to understand their child’s questions and provide answers appropriate to their developmental stage and disease understanding.

AYA patients with cancer often have an advanced understanding of their own illness and preferences for care.³⁷ The amount of information requested by AYAs and the level of involvement in decision making may change over time, so patients should be asked about their desired level of involvement at each conversation.^37,38 Allowing AYAs to feel a sense of control in how information is provided to them may better prepare them to participate in future conversations.³⁹ Engagement of adolescents and family members in conversations around goals of care⁴⁰ and advance care planning (ACP) has been associated with fewer physical symptoms and decreased suffering,⁴¹ improved congruence of stated goals of EOL care, greater understanding of EOL wishes, reduced anxiety,⁴² and greater likelihood to receive early palliative care.⁴³ Preliminary results in a study assessing the effects of AYAs using the ACP guide Voicing My CHOiCES indicate that adolescents talk more to family and friends about their preferences for care than to their providers.⁴² In addition, AYAs prefer that providers raise these issues with them, rather than the other way around.⁴² Methods and example phrases for engaging AYA patients in conversations around diagnosis, prognosis, and ACP are listed in Table 1, and resources and tools to assist communication about ACP are listed in Table 2.

TABLE 1.

Methods and Example Phrases for Engaging AYA Patients in Conversations Around Prognosis and Advance Care Planning and Decision Making in Advanced Cancer

TABLE 2.

Advance Care Planning and Communication Tools for Children, Adolescents, and Young Adults

For parents of children who die of cancer, doctor-patient communication is the principal determinant of high-quality care at the EOL.⁴⁸ In fact, higher parental ratings on communication are associated with lower levels of long-term parental grief.⁴⁹ However, in a recent survey, preliminary results showed that more than 40% of bereaved parents reported not feeling prepared for the child’s medical and/or emotional needs at the EOL (L. Wiener, personal communication, December 2018). Guidance from the palliative care team on navigating these discussions has been found to improve parent perception of care.¹³ Families also benefit from reassurance from the medical team that care will be continued in the face of progressive illness.²⁹

Struggling with the uncertainty of future events and their own fears, many parents find it difficult to discuss death and dying with their child. Bereaved parents who believed their child was afraid or anxious at the EOL were more likely to report their own distress or poor QOL years later.³⁶ Children’s books (ie, bibliotherapy) may normalize the experiences of death,^50,51 facilitate communication around EOL, and provide language for parents to explain death, taking into consideration the child’s cognitive and emotional development and level of understanding.⁵²

Evidence suggests that most AYAs prefer honest disclosure regarding prognosis and EOL issues and that such disclosure can lead to less suffering.^{30,39,46,53,54} These discussions should be viewed as opportunities to elicit and honor their priorities.⁵⁵ These conversations can have important implications for how AYAs live the remainder of their life, how and where they die, and how their families prepare for and cope after their death.⁴⁴ If ACP principles and/or guides have not yet been introduced, these can be helpful (Tables 1 and 2).

ASSESSMENT AND MANAGEMENT OF PHYSICAL SYMPTOMS, SUFFERING, AND QOL IN PPO

In the nearly two decades since the landmark study examining the symptoms and suffering of children dying from cancer,⁵⁶ there has only been modest improvement in management of treatment of symptoms and associated suffering at the EOL in this population.^9,57,58 Patient symptoms and suffering also affect other family members. Global symptom burden in children is correlated with depressive symptoms in caregivers,⁵⁹ and increased somatic distress and mood disturbance in patients were associated with poorer parental emotional functioning.⁶⁰ Unlike in adult palliative care, there are no published randomized controlled trials examining the effect of subspecialty palliative care in pediatric oncology on patient and family outcomes, although several studies are currently underway.

Historically, symptom assessment and psychosocial outcomes have been measured by proxy report for pediatric patients, given concern about children’s ability to reliably understand and complete assessments as well as the lack of validated tools in this age range. However, there is poor concordance between both clinician proxy and parent proxy and patient self-report in both symptom and QOL assessments. Compared with child self-report, clinicians under-report both prevalence and severity of symptoms in patients.^60-62 Moreover, parent reports overestimate both physical and emotional symptoms in children receiving treatment.^60,63

There is increasing literature supporting the use of patient-reported outcomes (PROs) in pediatric oncology patients to better understand, assess, and treat symptoms throughout the disease course and examine the impact of care on function and QOL.^64,65 Pediatric measures from the Patient-Reported Outcomes Measurement Information System demonstrate good feasibility and acceptability among children and AYAs undergoing cancer-directed treatment and in survivorship.^66,67 PROs have recently been used to assess QOL in children with neurofibromatosis-related neurofibromas,⁶⁸ to measure symptom burden and function in children with brain tumors,⁶⁹ and to screen for caregiver distress in parents of children in the first year after cancer diagnosis.⁷⁰ PROs have been used to document changes in QOL, mood, and symptoms of anxiety during and after cognitive-behavioral psychosocial treatments in an AYA patient with cancer⁷¹ and to solicit QOL outcomes for pediatric patients enrolled in phase I and II clinical trials.⁷²

Notably, pediatric oncology providers value the use of PROs but perceive organizational barriers to routine clinical use.⁷³ In a large multisite study of Pediatric Quality of Life and Evaluation of Symptoms Technology (PediQUEST), a computer-based data collection system, 99% of patients older than 15 years of age and 96% of children 7 to 12 years of age provided self-report on symptoms and QOL.⁵⁷ These patients with advanced cancer provided self-report of symptoms and QOL over time, even during the last 12 weeks of life.^57,74 In the follow-up interventional study, reports summarizing PROs provided to the oncologist improved the PedsQL4.0⁷⁵ emotional scores and a de novo overall sickness score and were valued by clinicians, parents, and children.⁵⁸

PediQUEST Response, an intervention trial in which child and parent PROs of symptoms, distress, and QOL are provided to the primary oncology team and subspecialty pediatric palliative care team, is currently enrolling patients in a multisite randomized controlled trial. The combination of subspecialty palliative care consultation and child and parent PROs is hypothesized to lead to increased health-related QOL compared with controls. Work is underway to develop and validate a pediatric version of the National Cancer Institute–created PROs version of the Common Terminology Criteria for Adverse Events.⁷⁶ Recommendations for why, how, and where to best use PROs in pediatric oncology have recently been published.⁷⁷ PPO providers should encourage the use of, and incorporate the findings from, PROs in the care of pediatric patients with a goal to reduce symptoms and suffering and improve function and QOL.

PSYCHOSOCIAL COMPONENTS AND CONSIDERATIONS IN PPO

Psychosocial care of patients, families, and communities is a key element of palliative care in pediatric oncology patients. Recent clinical guidelines⁷⁸ and suggestions for standards of care for psychosocial palliative care⁷⁹ have emphasized the importance of routine psychosocial assessments, early introduction of primary psychosocial clinicians who can provide treatment of patients and their family members, and an interdisciplinary palliative care team.³ Delayed introduction of psychosocial services has been linked to increased suffering of patients and subsequently of the parents,³ and bereaved parents have recognized the importance of earlier introduction of psychosocial care for the patient and family.⁸⁰

The components of a comprehensive psychosocial assessment are listed in Table 3. This assessment should examine prior and current family structure and functioning, family dysfunction, and presence of other psychosocial stressors such as financial hardship.^81,82 The initial assessment should attempt to understand the broader systems (eg, schools, communities, and cultures) in which the child and family exist.⁸² Continued connection with friends and the local community is essential to child, sibling, and parent well-being,⁸⁰ and perceived social support from the family and community can be a protective factor against negative psychosocial outcomes for patients and their families.^83,84

TABLE 3.

Domains of a Comprehensive Psychosocial Evaluation and Assessment of Pediatric Oncology Patients and Their Families

On the basis of the initial assessment of psychosocial functioning, an individualized psychosocial care plan can be developed and modified as needed. For ongoing social support, cancer support programs such as hospital or online support groups and camps for children with cancer can be useful to decrease the social isolation often felt by pediatric oncology patients.⁸⁴ More targeted programs, such as Promoting Resilience in Stress Management, show promise in reducing psychological distress in high-risk populations, specifically AYAs.⁸⁵ For patients with pre-existing or current emotional and/or behavioral symptoms, the team may additionally recommend a referral to pediatric psychology for further assessment and evidenced-based interventions such as cognitive behavioral therapy, behavior therapy, or relaxation training.⁸⁶

Given the strong link between parent and child functioning, psychosocial services for parents and siblings are also an integral aspect of family-based care. Parents of pediatric patients with cancer are at higher risk for the development of post-traumatic stress symptoms than patients themselves.⁸⁷ Specific interventions targeting the reduction of distress in parents of pediatric oncology patients are currently emerging and represent an important area of research.⁸⁸

Siblings of pediatric oncology patients are at risk for psychosocial difficulty, and sibling-specific programs such as camps and support groups may be helpful in facilitating adjustment.⁸⁹ In addition, access to specialized psychosocial providers, such as pediatric psychologists in the hospital or in the community, remains an important resource. Although the overarching concepts of care delivery (eg, assessment, intervention) apply across palliative care, there are special considerations for care that will vary depending on the child’s age and developmental level.⁹⁰

Just as pediatric palliative care clinicians assess and manage physical symptoms over the course of treatment, the patient’s and family’s emotional functioning should also continue to be assessed longitudinally. Systematically capturing patients’ lived experiences demonstrates a respect for their capacities and communicates the important role patients can play in their own care.⁹¹ It also increases engagement and decreases anxiety.⁹²

During active treatment, patients with advanced cancer and their families have access to psychosocial supports, through dedicated social workers, pediatric psychologists, or other clinicians and support staff.⁹³ However, when children do not survive their disease, the abrupt end of the relationship with the care team can seem like an additional loss for the family.^23,94 Bereavement care is an evidence-based psychosocial standard of care in pediatric oncology.⁹⁵ Although data support that the loss of a child is associated with heightened risk for discord in the parental marital relationship, physical and mental health problems, and parental mortality,^96,97 routine assessment of needs of bereaved families does not systematically occur.⁹⁴ Moreover, there is often inconsistency across centers in the amount and type of bereavement support available.⁹⁴ Parents of children who died of cancer benefit from supportive contact with hospital staff during EOL and after death,⁹⁸ as well as access to both hospital-based and community-based support services.⁹⁸ Parents identify contact with other bereaved parents as being helpful⁹⁸ and indicate a need for formal grief support for surviving children.⁹⁸ Parents who receive bereavement services describe feeling cared for and supported by staff, a reduction in sense of isolation, improved coping, and personal growth; provision of services seems to be most effective for parents experiencing more complex mourning.⁹⁹ Interdisciplinary palliative care providers should collaborate with psychosocial providers to conduct routine and comprehensive assessment of the needs of all bereaved family members, including siblings and grandparents, and identify and connect families with hospital-based and community resources and support.

SPIRITUAL CONSIDERATIONS IN PPO

Spirituality in children is defined as the ability to derive personal value and transcend beyond the self through relationships with others, often parents or other family members.¹⁰⁰ Unlike many adults who may identify spirituality and religion as overlapping concepts, younger children may experience spirituality without the associated spiritual beliefs, rituals, or practices. In qualitative interviews with children with cancer about spirituality, several themes emerged, including: prayer as healing, ability to express joy and gratitute, and help in overcoming difficulties.¹⁰¹ Spirituality in children with cancer was positively associated with emotional, school, and physical functioning domains of the Pediatric Quality of Life tool.¹⁰¹ Spirituality may be especially important to AYA patients as they deal with physical and psychological adjustment to their illness, especially in the context of ongoing development and advanced illness.¹⁰²

The spiritual needs of children and AYAs should be assessed on an ongoing basis, particularly given expected changes in beliefs and outlooks based on developmental trajectory and physical and psychological changes as a result of treatment and disease.¹⁰³ In one study, parents who identified themselves as religious were more likely to talk about death with their child with advanced cancer when compared with parents who were not religious.³⁶ A study of caregivers, approximately a third of whom were parents of children who had died of cancer, suggests that religious belief may be protective in the grief experience.¹⁰⁴ Finally, bereaved siblings who used spirituality and religious beliefs and activities to cope were more likely to have worked through their grief when compared with siblings who had not used these coping mechanisms.¹⁰⁵

Despite literature that supports spirituality and religiosity as important to patients and parents and that they desire integration of these aspects into their care, few pediatricians routinely address these topics.¹⁰⁶ Physicians who identified as Christian, those who did not expect negative reactions to inquiring, and those who were knowledgeable regarding chaplains were more likely to address spirituality and/or religion with their patients and families.¹⁰⁶ Spiritual assessments and care plans that incorporate chaplains should be routinely instituted by PPO providers as a part of a larger psychosocial assessment and plan and should be reassessed at regular intervals over the treatment course. Table 1 provides example language on how to address spirituality with AYA patients.

FUTURE DIRECTIONS

Over the past two decades, mounting evidence has clearly demonstrated the need for greater attention to the physical, psychosocial, and spiritual needs of children with advanced cancer and their families. High-quality communication may facilitate greater focus on these domains and lead to improved outcomes. PROs should be strongly considered in the routine assessment of patient and family symptoms, distress, QOL, and associated functioning, and the expertise of the palliative care interdisciplinary team should be used for complex or refractory distress. To ensure the best possible outcomes in the face of pediatric advanced cancer, the next phase of PPO research needs to focus on identification of best practices and metrics for high-quality care throughout the pediatric oncology disease trajectory, including at the EOL. Furthermore, PPO providers and researchers should focus efforts on determining greater efficiency of interdisciplinary care interventions and evaluate cost effectiveness. Importantly, given the low numbers of subspecialty trained palliative care providers, which is likely to persist long term, primary PPO providers hold principal responsibility to ensure the well-being of children with advanced cancer and their families through expert clinical care, training, and innovation.

AUTHOR CONTRIBUTIONS

Conception and design: All authors

Data analysis and interpretation: All authors

Manuscript writing: All authors

Final approval of manuscript: All authors

Accountable for all aspects of the work: All authors

AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST

Pediatric Palliative Care in Oncology

The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated. Relationships are self-held unless noted. I = Immediate Family Member, Inst = My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO's conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/journal/jco/site/ifc.

No potential conflicts of interest were reported.