Physical Address

304 North Cardinal St.
Dorchester Center, MA 02124

Pediatric Palliative Care


According to the World Health Organization (WHO), “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 to children with a wide variety of diagnoses, including cancer, cystic fibrosis, complex or severe cardiac disease, neurodegenerative disorders, severe malformations, and trauma with life-threatening sequelae ( Table 7.1 ). Medical and technologic advances have resulted in children living longer, often with significant dependence on expensive technologies. These children have complex chronic conditions across the spectrum of congenital and acquired life-threatening disorders. Children with complex chronic conditions benefit from integration of palliative care strategies. 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 and blended goals of care.

Table 7.1
Adapted from The Together for Short Lives [formerly the Association for Children's Palliative Care (ACT)] Life-limiting/Life-threatening Condition Categories. http://www.togetherforshortlives.org.uk/professionals/childrens_palliative_care_essentials/approach .
Conditions Appropriate for Pediatric Palliative Care

Conditions for Which Curative Treatment Is Possible but May Not Succeed

  • Advanced or progressive cancer or cancer with a poor prognosis

  • Complex and severe congenital or acquired heart disease

Conditions for Which There Is Intensive Long-Term Treatment Aimed at Prolonging Life and Maintaining Quality of Life, but Premature Death Is Still Possible

  • Cystic fibrosis

  • Severe immunodeficiency

  • High-risk solid-organ transplant candidates and/or recipients (e.g., lung, multivisceral)

  • Chronic or severe respiratory failure

  • Muscular dystrophy

  • Complex multiple congenital malformation syndromes

  • Primary pulmonary hypertension

  • Severe chromosomal disorders (aneuploidy, deletions, duplications)

Progressive Conditions for Which There Is No Curative Option and in Which Treatment Is Almost Exclusively Palliative After Diagnosis

  • Progressive metabolic disorders (Tay-Sachs disease)

  • Batten disease

  • 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

  • Severe neurologic sequelae of infectious disease

  • Hypoxic or anoxic brain injury

  • Brain malformations (e.g., holoprosencephaly, lissencephaly)

Although often mistakenly understood as equivalent to end-of-life care , the scope and potential benefits of palliative care are applicable throughout the illness trajectory . Palliative care emphasizes optimization of quality of life, communication, and symptom control, goals that may be congruent with maximal treatment aimed at sustaining or prolonging life.

The mandate of the pediatrician and other pediatric clinicians to attend to children's physical, mental, and emotional health and development includes the provision of palliative care for those who live with a significant possibility of death before adulthood ( Fig. 7.1 ). Such comprehensive physical, psychological, social, and spiritual care requires an interdisciplinary approach.

Fig. 7.1
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.)

In the United States the healthcare and reimbursement structure, combined with frequent use of medical technology (e.g., home ventilatory support) or continuous home nursing, historically precluded formal enrollment of children on the hospice benefit when they were otherwise eligible (i.e., had estimated prognosis of ≤6 mo). Section 2302 of the Patient Protection and Affordable Care Act (ACA), the Concurrent Care for Children Requirement (CCCR) , eliminated the requirement that Medicaid patients <21 yr old forgo curative or life-prolonging therapies to be eligible for hospice. Although Medicaid programs in every state are now required to provide concurrent curative/life-prolonging treatment and hospice services for hospice-eligible children, development of systems to make such concurrent care a reality has been slow. A limitation of the CCCR is that it does not expand access to hospice for children with life-threatening illness who do not meet hospice eligibility criteria (i.e., have a prognosis that cannot be estimated to be <6 mo) or those not receiving Medicaid.

A number of state-based pediatric palliative care coalitions have formed in recent years to improve access to home-based pediatric hospice/palliative care services, using strategies such as Medicaid waivers or state plan amendments to increase coverage for hospice services. A growing number of home care agencies have also developed palliative care programs that serve as a bridge to hospice services for children not yet meeting hospice eligibility criteria. Some hospices have adopted an open hospice model with more flexible eligibility criteria. However, provision of hospice or palliative care for children is often also limited by the availability of clinicians who have training or experience in caring for seriously ill children.

Care Settings

Pediatric palliative care should be provided across settings, including hospital, outpatient, and home, as well as pediatric nursing facilities and sometimes inpatient hospice houses. Home care for the child with a life-threatening illness requires 24 hr/day access to experts in pediatric palliative care, a team approach, and an identified coordinator who serves as a link among hospitals, the community, and specialists and who may assist in preventing or arranging for hospital admissions, respite care, and increased home care support as needed. Adequate home care support and respite care, although sorely needed, is often not readily available because staffing or the high-tech skill required to care for these children is lacking. Furthermore, families may view using respite care as a personal failure, or they may worry that others cannot adequately care for their child's special needs or potential rapid escalation of symptoms.

At the end of life, children and families may need intensive support. About half of pediatric deaths occur in acute care hospitals, and end-of-life care may thus be provided in the home, hospital, pediatric nursing facility, 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). In some instances, when death at home for a child in the ICU is preferred, transport and even extubation at home may be possible, if clinical and logistical circumstances permit it.

The philosophy of palliative care can be successfully integrated into any 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 and provide the most meaning and sense of control and choice for their family?” instead of, “What therapies are we no longer going to offer this patient?” Staff may benefit from education, support, and guidance because pediatric palliative care, as with other types of intensive care, is an area of specialty. Regardless of the care setting, comprehensive palliative care requires an interdisciplinary approach that may include nurses, physicians, psychologists, psychiatrists, social workers, chaplains/clergy, 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 or the child. This delay may impede informed decision-making about how the child lives at the end of life. Given the inherent prognostic uncertainty of a life-threatening 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. Having these conversations in the midst of a crisis is not ideal. Whenever possible, they 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. Even in the face of long-standing and highly connected relationships, clinicians often hold assumptions about parent prognostic awareness, as well as parent readiness and willingness to have such discussions . In an attempt to protect families, clinicians may avoid conversations that they perceive as promoting distress or hopelessness. However, parents greatly value honesty, and in fact such conversations can promote parent hopefulness, as well as trust and connection with the care team. At times, therefore, a consultative palliative care team provides the family with an opportunity to engage in sensitive conversations that do not as readily occur with the primary team, at least initially.

The population of individuals who die before reaching adulthood includes a disproportionate number of nonverbal and preverbal children and adolescents who are developmentally unable to make autonomous care decisions. Although parents are usually the primary decision-makers, these youth should be as fully involved in discussions and decisions about their care as appropriate for their developmental status. Using communication experts, child life therapists, chaplains, social workers, psychologists, or psychiatrists 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 (for adults), Voicing My Choices (for adolescents), and My Wishes (for school-age children), have in practice been useful in helping to introduce advance care planning to children, adolescents, and their families ( www.agingwithdignity.org/index.php ).

You're Reading a Preview

Become a Clinical Tree membership for Full access and enjoy Unlimited articles

Become membership

If you are a member. Log in here

Related Posts