Charting the Territory: End-of-Life Trajectories for Children With Complex Neurological, Metabolic, and Chromosomal Conditions

Abstract

ContextFor parents, family, or clinicians of children with rare life-threatening conditions, there is little information regarding likely symptoms, illness trajectory, and end-of-life care. ObjectivesThis descriptive analysis of a bereaved cohort recruited in the charting the territory study describes patient characteristics, symptoms, use of medications, discussion of resuscitation orders, and care provided preceding and during the end of life. MethodsOf the 275 children enrolled in the Charting the Territory study, 54 died between 2009 and 2014. Baseline demographic information, symptoms, interventions, and medical information were collected via chart review, interviews, and surveys. ResultsFifty-one of the 54 children had complete medical records. Of the seven symptoms evaluated, children were found to have an increase in median symptoms from baseline (n = 2) to time of death (n = 3). Opioids were used in the last 48 hours of life in 29 (56.9%) children, whereas only eight (15.7%) were receiving opioids at baseline. Do Not Attempt Resuscitation orders were in place at baseline in 17 (33.3%) children, increasing to 33 (64.7%) at time of death. Death occurred in a hospice setting in 16 (31.4%) children. ConclusionAlthough much emphasis on pediatric palliative care has been on supportive treatment and symptom management, when faced with a lack of sound understanding of a rare illness, the mode of care can often be reactive and based on critical needs. By developing greater knowledge of symptoms and illness trajectory, both management and care can be more responsive and anticipatory, thereby helping ease illness burden and suffering.