Advance Care Planning Experiences and Preferences among People with Cystic Fibrosis

Abstract

Background: Advance care planning (ACP) is recommended for people with cystic fibrosis (CF), yet guidance for optimal implementation is lacking.

Objective: To assess ACP-related thoughts, comfort level, and preferences among people with CF to guide evidence-based routine implementation of ACP in the CF clinic.

Design: A cross-sectional survey assessed ACP-related experiences and preferences.

Subjects: Thirty-eight adolescents and adults with CF from an urban CF center.

Results: Few subjects reported talking to their CF team about ACP care preferences (5%) or completing advance directives detailing desired medical treatments (11%). However, most participants worried about living with advanced disease (84%) and felt comfortable discussing ACP preferences with CF providers (92%). Subjects largely preferred that ACP conversations occur when they are generally healthy, in the outpatient setting, and with any familiar CF team member. Disease severity was not associated with frequency of worry about living with advanced disease, comfort level with ACP discussions, or ACP setting preferences.

Conclusions: People with CF worry about advanced disease and feel comfortable discussing ACP, but need more guidance to understand and document ACP choices. CF patient experiences and preferences support implementation of an early, active approach to ACP for people with CF.

Keywords: advance care planning; advance directive; cystic fibrosis; end-of-life care; palliative care.