Patterns of Care at the End of Life for Children and Young Adults with Life-Threatening Complex Chronic Conditions.

OBJECTIVE: To characterize patterns of care at the end of life for children and young adults with life-threatening complex chronic conditions (LT-CCCs) and to compare them by LT-CCC type. STUDY DESIGN: Cross-sectional survey of bereaved parents (n = 114; response rate of 54%) of children with noncancer, noncardiac LT-CCCs who received care at a quaternary care children's hospital and medical record abstraction. RESULTS: The majority of children with LT-CCCs died in the hospital (62.7%) with more than one-half (53.3%) dying in the intensive care unit. Those with static encephalopathy (AOR, 0.19; 95% CI, 0.04-0.98), congenital and chromosomal disorders (AOR, 0.28; 95% CI, 0.09-0.91), and pulmonary disorders (AOR, 0.08; 95% CI, 0.01-0.77) were significantly less likely to die at home compared with those with progressive central nervous system (CNS) disorders. Almost 50% of patients died after withdrawal or withholding of life-sustaining therapies, 17.5% died during active resuscitation, and 36% died while receiving comfort care only. The mode of death varied widely across LT-CCCs, with no patients with pulmonary disorders dying receiving comfort care only compared with 66.7% of those with CNS progressive disorders. A majority of patients had palliative care involvement (79.3%); however, in multivariable analyses, there was distinct variation in receipt of palliative care across LT-CCCs, with patients having CNS static encephalopathy (AOR, 0.07; 95% CI, 0.01-0.68) and pulmonary disorders (AOR, 0.07; 95% CI, 0.01-.09) significantly less likely to have palliative care involvement than those with CNS progressive disorders. CONCLUSIONS: Significant differences in patterns of care at the end of life exist depending on LT-CCC type. Attention to these patterns is important to ensure equal access to palliative care and targeted improvements in end-of-life care for these populations.

Physician Perspectives on Palliative Care for Children With Neuroblastoma: An International Context.

BACKGROUND: Studies have shown that children with cancer globally lack access to palliative care. Little is known regarding physicians' perceptions of palliative care, treatment access, and self-reported competence in providing palliative care. PROCEDURE: Members of the Global Neuroblastoma Network (online tumor board) were surveyed. Eighty-three respondents met inclusion criteria; 53 (64%) completed the survey. RESULTS: Most respondents trained in high-income countries (HIC) but practice in low- and middle-income countries (LMIC), and care for more than five patients with neuroblastoma annually. WHO Essential Medicines in palliative care varied in availability, with incomplete access across LMIC centers. Nonpharmacologic therapies were inconsistently available. Contrary to international definitions, 17% of respondents inappropriately considered palliative care as that initiated only after curative therapy is stopped. Mean physician competence composite score (Likert scale 1-5, 5 = very competent) in providing symptomatic relief and palliative care across phases of care was 2.93 (95% CI 2.71-3.22). Physicians reported significantly greater competence in symptom management during cure-directed therapy than during end-of-life (P = 0.02) or when patients are actively dying (P = 0.007). Practicing in HIC, prior palliative care training, having access to radiotherapy, and not having to turn patients away due to bed shortages were significantly predictive of perceived competence in providing palliative care at end of life. CONCLUSIONS: An international sample identified gaps in treatment and palliative care service availability, in understanding the definition of palliative care, and in self-reported competence in providing palliative care. Increased perceived competence was associated with training, which supports the need for increased palliative care education and advocacy, especially in LMIC.

Patterns and Outcomes of Care in Children With Advanced Heart Disease Receiving Palliative Care Consultation.

CONTEXT: Although access to subspecialty pediatric palliative care (PPC) is increasing, little is known about the role of PPC for children with advanced heart disease (AHD). OBJECTIVES: The objective of this study was to examine features of subspecialty PPC involvement for children with AHD. METHODS: This is a retrospective single-institution medical record review of patients with a primary diagnosis of AHD for whom the PPC team was initially consulted between 2011 and 2016. RESULTS: Among 201 patients, 87% had congenital/structural heart disease, the remainder having acquired/nonstructural heart disease. Median age at initial PPC consultation was 7.7 months (range 1 day-28.8 years). Of the 92 patients who were alive at data collection, 73% had received initial consultation over one year before. Most common indications for consultation were goals of care (80%) and psychosocial support (54%). At initial consultation, most families (67%) expressed that their primary goal was for their child to live as long and as comfortably as possible. Among deceased patients (n = 109), median time from initial consultation to death was 33 days (range 1 day-3.6 years), and children whose families expressed that their primary goal was for their child to live as comfortably as possible were less likely to die in the intensive care unit (P = 0.03) and more likely to die in the setting of comfort care or withdrawal of life-sustaining interventions (P = 0.008). CONCLUSION: PPC involvement for children with AHD focuses on goals of care and psychosocial support. Findings suggest that PPC involvement at end of life supports goal-concordant care. Further research is needed to clarify the impact of PPC on patient outcomes.

Pocket Reference Card Improves Pediatric Resident Comfort in Caring for Children at End of Life.

BACKGROUND: Studies have shown that pediatricians in all stages of training are uncomfortable managing patients at end of life. Our goal was to create and test a portable reference card to improve pediatric resident education in comprehensive care for children nearing end of life. METHODS: We evaluated the impact of the Pediatric End-of-Life Care Management Reference Card on residents' perceived comfort and knowledge through pre- and post-intervention surveys. The preintervention questionnaires and pocket cards were distributed to all first- and second-year residents, and then a follow-up survey was provided six months later. Based on Likert scales, questions focused on self-reported understanding of palliative care principles and knowledge regarding and comfort in performing end-of-life symptom management. RESULTS: Twenty-six pediatric residents completed pre- and post-intervention surveys. Following receipt of the reference card, no significant changes were noted consistently across all groups of residents. The majority of improvements were noted when comparing second to third year residents, including knowledge and comfort related to pain management, comfort in managing secretions and nausea, and documentation following death. The first to second year residents demonstrated improvement in knowing what language to use to tell a family that their child has died. CONCLUSION: This study demonstrates that a portable reference card may be a convenient, simple, and useful component of education for pediatric residents in end-of-life care management. This reference card is a foundation from which to develop a standardized educational tool. Additional research is required to assess the impact of this type of intervention in pediatric palliative care education.

Physician and parent perceptions of prognosis and end-of-life experience in children with advanced heart disease.

BACKGROUND: Little is known about how physician and parent perspectives compare regarding the prognosis and end-of-life (EOL) experience of children with advanced heart disease (AHD). OBJECTIVE: The study's objective was to describe and compare parent and physician perceptions regarding prognosis and EOL experience in children with AHD. METHODS: This was a cross-sectional survey study of cardiologists and bereaved parents. Study subjects were parents and cardiologists of children with primary cardiac diagnoses who died in a tertiary care pediatric hospital between January 2007 and December 2009. Inclusion required both physician and parent to have completed surveys respective to the same patient. A total of 31 parent/physician pairs formed the analytic sample. Perceptions were measured of cardiologists and bereaved parents regarding the EOL experience of children with AHD. RESULTS: Nearly half of parents and physicians felt that patients suffered 'a great deal,' 'a lot,' or 'somewhat' at EOL, but there was no agreement between them. At diagnosis, parents more often expected complete repair and normal lifespan while the majority of physicians expected shortened lifespan without normal quality of life. Parents who expected complete repair with normal life were more likely to report 'a lot' of suffering at EOL (p=0.002). In 43% of cases, physicians reported that the parents were prepared for the way in which their child died, while the parents reported feeling unprepared. CONCLUSION: Both parents and physicians perceive suffering at EOL in patients who die of AHD. Moreover, parent expectations at diagnosis may influence perceptions of suffering at EOL. Physicians overestimate the degree of parent preparedness for their child's death.