Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease

J Cyst Fibros. 2020 May;19(3):344-354. doi: 10.1016/j.jcf.2020.02.015. Epub 2020 Feb 27.


Background: Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD.

Methods: The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate.

Results: The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues.

Conclusions: These recommendations are intended to be paired with previously published management guidelines for the overall CF population, with the objective of reducing practice variability and improving overall care, quality of life, and survival in those with ACFLD.

Keywords: Advance care planning; Advanced lung disease; Cystic fibrosis; Lung transplantation; Palliative care; Respiratory failure.

Publication types

  • Practice Guideline

MeSH terms

  • Advance Care Planning
  • Airway Management / methods*
  • Comorbidity
  • Critical Care / methods*
  • Cystic Fibrosis* / physiopathology
  • Cystic Fibrosis* / psychology
  • Cystic Fibrosis* / therapy
  • Disease Progression
  • Humans
  • Lung Transplantation / methods*
  • Palliative Care / methods
  • Patient Acuity
  • Patient Care Management / methods*
  • Psychosocial Intervention / methods*
  • Quality of Life*
  • Severity of Illness Index