The psychological burden of cystic fibrosis

Curr Opin Pulm Med. 2016 Mar;22(2):187-91. doi: 10.1097/MCP.0000000000000244.


Purpose of review: Cystic fibrosis (CF) is the most common genetic, life-shortening illness among white populations. Management of the disease requires a complex, time-consuming treatment regimen. The purpose of this review is to highlight current research examining the psychological burden of CF, including psychological distress, social challenges, treatment burden, and adherence to daily treatments.

Recent findings: Individuals with CF and their parent caregivers report elevated symptoms of depression and anxiety. Recent international guidelines (Cystic Fibrosis Foundation and European Cystic Fibrosis Society) recommend annual screening of these symptoms using the Patient Health Questionnaire-9 (PHQ-9) and Generalized Anxiety Disorder-7 (GAD-7) scales. Symptoms of depression have been associated with decreased adherence, lower quality of life, and higher healthcare costs. Adherence to pulmonary medications has been found to be 50% or less and decreases with age. Poor adherence has been associated with higher healthcare costs, more frequent hospitalizations, and worse quality of life.

Summary: Individuals with CF face unique challenges that can lead to psychological burden. Screening for these symptoms and developing effective interventions to improve adherence are the key targets for the next 5 years of research.

Publication types

  • Review

MeSH terms

  • Anxiety
  • Caregivers
  • Cystic Fibrosis / psychology*
  • Cystic Fibrosis / therapy
  • Depression
  • Humans
  • Parents
  • Quality of Life / psychology