Advance care planning in cystic fibrosis: Current practices, challenges, and opportunities

J Cyst Fibros. 2016 Jan;15(1):96-101. doi: 10.1016/j.jcf.2015.08.004. Epub 2015 Sep 8.


Background: Studies in cystic fibrosis (CF) report late attention to advance care planning (ACP). The purpose of this study was to examine ACP with patients receiving care at US adult CF care programs.

Methods: Chart abstraction was used to examine ACP with adults with CF dying from respiratory failure between 2011 and 2013.

Results: We reviewed 210 deaths among 67 CF care programs. Median age at death was 29 years (range 18-73). Median FEV1 in the year preceding death was 33% predicted (range 13-100%); 68% had severe lung disease with FEV1<40% predicted. ACP was documented for 129 (61%), often during hospitalization (61%). Those with ACP had earlier documentation of treatment preferences, before the last month of life (73% v. 35%; p=<0.01). Advance directives were completed by 93% of those with ACP versus 75% without (p<0.01); DNR orders and health care proxy designation occurred more often for those with ACP. Patients awaiting lung transplant had similar rates of ACP as those who were not (67% v. 61%; p=0.55). The frequency of ACP varied significantly among the 29 programs contributing data from four or more deaths.

Conclusions: ACP in CF often occurs late in the disease course. Important decisions default to surrogates when opportunities for ACP are missed. Provision of ACP varies significantly among adult CF care programs. Careful evaluation of opportunities to enhance ACP and implementation of recommended approaches may lead to better practices in this important aspect of CF care.

Keywords: Advance care planning; Decision making; End-of-life care; Lung transplant.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Advance Care Planning* / organization & administration
  • Advance Care Planning* / statistics & numerical data
  • Attitude of Health Personnel
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / psychology
  • Cystic Fibrosis* / therapy
  • Female
  • Health Knowledge, Attitudes, Practice
  • Health Personnel* / education
  • Health Personnel* / psychology
  • Humans
  • Male
  • Needs Assessment
  • Professional-Patient Relations
  • Surveys and Questionnaires
  • Terminal Care* / methods
  • Terminal Care* / psychology
  • United States