What Are Special Considerations for Pediatric Palliative Care?

Epidemiology of Child Mortality and Trajectories of Serious Illness

In the United States, there are approximately 43,000 pediatric deaths per year. Over 50% of these deaths occur in newborns and infants born with congenital/chromosomal conditions or among premature infants with low birthweight. In children aged 1 to 19 years of age, the leading cause of death is unintentional injuries. Palliative care teams often support these patient populations through perinatal ( Chapter 58 ) and bereavement programs.

A much larger component of the work of pediatric palliative care teams lies in caring for children living with life-limiting serious illness and their families. Although these children are at risk for significant morbidity and mortality, many live with ongoing complex chronic conditions. Complex chronic conditions are defined as “medical conditions that can be expected to last more than 12 months and involve several different organ systems or one organ system severely enough to require subspecialty pediatric care and hospitalization at a tertiary care center.” Children with complex chronic conditions constitute more than 22% of all pediatric deaths. Overall, the main categories of complex chronic conditions leading to death in children are cardiovascular (22%), malignancy (21%), congenital or genetic (19%), neuromuscular (18%), and respiratory (9%). And yet, the majority of these children have prolonged survivorship, with more than two-thirds of the patients enrolled in pediatric palliative care programs surviving more than 1 year from the time of initial consult.

Given that seriously ill children often survive for an extended period of time, it is helpful to consider their expected illness trajectory when determining what palliative care services and supports to provide. These trajectories include (1) conditions for which curative treatment is possible and may fail (e.g., cancer with a poor prognosis); (2) conditions requiring intensive long-term treatment aimed at maintaining the quality of life (e.g., cystic fibrosis, advanced muscular dystrophy); (3) progressive conditions in which treatment is exclusively directed at alleviating suffering after diagnosis (e.g., progressive metabolic disorders); and (4) conditions involving severe, nonprogressive disability causing vulnerability to health complications (e.g., holoprosencephaly or other severe brain malformations, Table 55.1 ).

In contrast to adult palliative care programs, children with cancer only make up about 20% of pediatric palliative care patients. Instead, the majority of children receiving palliative care services are those with genetic/congenital conditions (41%) and neuromuscular conditions (40%). These children are medically complex, with the majority having 5 or more daily medications and 80% needing technology supports such as gastrostomy feeding tubes, tracheostomies, and chronic mechanical ventilation to sustain their basic care needs. These children often receive care at tertiary pediatric medical centers with involvement from multiple subspecialists. Many of these children receive home nursing through home health agencies and developmental therapies through school-based programs. Understandably, the majority of these children have extraordinary care coordination needs, and a growing number of pediatric centers have complex care programs to support these children and parents through greater continuity, centralized access to care, and connection to local supports in the community.

Having a child with a complex chronic condition also strains family resources. Over 50% of families of children with complex chronic conditions report financial hardships related to their child’s illness and care needs. For many families, one parent leaves the workforce to care for their child with serious illness. These challenges and their effects on health outcomes are further impacted by race and socioeconomic status, and more work is needed on how to better support families within their communities and at end of life with these circumstances.

Hospital-, Hospice-, and Community-Based Palliative Care Programs and Location of Death

Many children with serious illness receive additional support at home and in their communities through home-based palliative care programs and concurrent care hospice. The integration of hospice and palliative care for children has been endorsed by the Institute of Medicine and the American Academy of Pediatrics. Despite this, availability of services often depends on whether there is a local palliative care or hospice provider who serves children. When home-based palliative care programs are available, they can have substantial impacts on improving care and lowering costs. These teams often include a visiting nurse, social worker, and development therapist who visit regularly to provide services around symptom management, quality of life, and care coordination. Often, these programs are a part of hospice programs with crossover of staff, which provides an opportunity to develop relationships with hospice team members ahead of the child’s hospice eligibility or end of life. Concurrent care hospice for children under 18 years of age is a provision of the Affordable Care Act. It enables children to receive life-prolonging therapies (such as chemotherapy, total parenteral nutrition, transfusions) at the same time as hospice care. Typically, stipulations are made for services outside of hospices’ provision of care. Otherwise, eligibility for hospice is similar to adults in that children need to have an expected prognosis of 6 months or less. With greater application of medical technology, it is not uncommon for children to live past these timelines and their expected prognoses. In these situations, children need to be recertified for hospice care, which often means that they need to be showing some signs of declining health. Arranging for hospice for children often involves direct conversations between the hospice team and referring teams about how services will be covered, and which teams will follow the patient for which issues.

Availability and access to hospice is a major determinant of the location of death among children. Parents also vary in their preferences regarding the location of their child’s death, as many families want to continue life-prolonging therapies for their child that are only available in the hospital. Overall, over 40% of children in the United States die in the hospital setting. Among children with complex chronic conditions, more than 80% die in a hospital and half die in the intensive care unit (ICU). Despite these data, studies of bereaved parents have found that, in retrospect, the majority would have chosen home as the most appropriate location of death for their child. Trends indicate that children with complex chronic conditions are increasingly dying at home, perhaps because of factors such as longer survival, the migration of advanced medical technology into the home, and shifting attitudes, with a greater focus on quality of life among children with life-threatening conditions. However, this trend toward home as a setting of death is not seen in Black or Hispanic children, and more work is needed to understand how to better support families with various racial and ethnic backgrounds during their child’s end of life, regardless of the care setting.

Developmental Considerations for Pain and Symptom Management