Cystic Fibrosis: More Access to Advanced Care Planning, Palliative Care Needed

Doctor comforting patient
Doctor comforting patient
Palliative care access may be limited for patients with cystic fibrosis.

Adults with cystic fibrosis (CF) may have limited access to end-of-life resources such as palliative care, according to a study published in the Journal of Cystic Fibrosis.

Researchers identified 248 adults with cystic fibrosis who had died in 71 CF care centers, at an average age of 29 years. Data were collected retrospectively to assess end-of-life care and practices for adult patients with CF.

Study results showed the most common location at time of death was the intensive care unit (ICU; 39%), with the common causes of death being cardiac or respiratory failure (n=210; 85%). Fewer patients who died in the ICU participated in advanced care planning compared with patients who died in other locations (P =.006), and fewer patients in the ICU used hospice or palliative care services (P <.001).

In addition, fewer patients listed for lung transplant received hospice or palliative care services compared with patients who were not listed for lung transplant (20% vs 55%; P <.001).

The investigators concluded that given these findings, clinicians should be encouraged to address advanced care planning and palliative care as a routine part of follow-up care for patients with CF. Significant milestones in life such as transitioning from pediatric to adult care, graduation, marriage, and the birth of a child are identified as good opportunities for these assessments.

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Chen E, Homa K, Goggin J, et al. End-of-life practice patterns at U.S. adult cystic fibrosis care centers: a national retrospective chart review [published online August 14, 2017]. J Cyst Fibros. doi:10.1016/j.jcf.2017.08.010