The end-of-life experience of patients with cystic fibrosis and the role of pediatric palliative care consultation

Abstract

Objectives * Describe patterns of medical care provided to patients with cystic fibrosis (CF) around the end of life. * Explore the roles a pediatric palliative care consultation service serves for patients with CF who ultimately die from their disease. Original Research Background. While major advances have been made in the care of patients with cystic fibrosis (CF), it remains a life-limiting disease associated with significant morbidity. Little is known about the patterns of end-of-life (EOL) care for this population and the role pediatric palliative care consultation can serve. Research Objectives. To characterize EOL care for patients with CF who received pediatric palliative care consultation since the inception of the Pediatric Advanced Care Team (PACT) at Boston Children’s Hospital (BCH). Methods. We identified patients, age >5 years, who died of CF-related disease between 2009 and 2014. Patient, clinical, and PACT consultation characteristics were abstracted from the medical record. Results. Fourteen patients with CF who died were identified, 57% female; mean age at death was 20.6 years (range 14.8 to 42.4 years). Most (86%) received a PACT consult. Median time between PACT consult and death was 152 days (range 34 days to 7.4 years). Content of PACT consultations most commonly included symptom management, goals of care, advance care planning, and psychosocial support. Among all who died, (93%) died in the ICU; 86% had a tracheostomy or were intubated, and 100% received non-invasive or mechanical ventilation leading up to death. Seven patients who died in the hospital (54%) had resuscitation status orders documented a median of 9.1 days prior to death (range 0.4 to 39.1 days). Conclusion. The majority of patients with CF who died received formal pediatric palliative care consultation, often well before death. Most patients also received intensive life-sustaining interventions immediately prior to death. Implications for Research, Policy or Practice. There appears to be a role for palliative care consultation to help foster communication at the EOL for patients with CF. Further exploring the role and timing of palliative care consultation for patients with CF may help healthcare providers identify areas of improvement in the assessment and implementation of their patients’ goals of care.