End-of-life practice patterns at U.S. adult cystic fibrosis care centers: A national retrospective chart review

Abstract

Background: There are many challenges to providing end-of-life care (EOLC) to people with cystic fibrosis (CF).

Methods: Chart abstraction was used to examine EOLC in adults with CF who died between 2011 and 2013.

Results: We reviewed 248 deaths from 71 CF care centers. Median age at death was 29years (range 18-73). While median FEV1 was in the severe lung disease category (FEV1<40%), 38% had mild or moderate lung disease in the year preceding death. The most common location of death was the intensive care unit (ICU, 39%), and 12% of decedents were listed for lung transplant. Fewer of those dying in the ICU personally participated in advance care planning or utilized hospice or Palliative Care Services (p<0.05).

Conclusions: Adults dying with CF in the United States most commonly die in an ICU, with limited and variable use of hospice and Palliative Care Services. Palliative care and advance care planning are recommended as a routine part of CF care.

Keywords: Advance care planning; Cystic fibrosis; End-of-life care; Hospice; Lung transplant; Palliative care.