OBJECTIVES: For 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 . This descriptive analysis of a bereaved cohort recruited in the Charting the Territory (CTT) study describes patient characteristics, symptoms, use of medications, discussion of resuscitation orders and care provided preceding and during the end-of-life .
METHODS: Of the 275 children enrolled in the CTT study , 54 died between 2009 and 2014 . Baseline demographic information, symptoms, interventions and medical information were collected via chart review, interviews and surveys .
RESULTS : 51 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 .
CONCLUSIONS: While 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.