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

Siddhartha G. Kapnadak, Emily Dimango, Denis Hadjiliadis, Sarah E. Hempstead, Erin Tallarico, Joseph M. Pilewski, Albert Faro, James Albright, Christian Benden, Shaina Blair, Elisabeth P. Dellon, Daniel Gochenour, Peter Michelson, Baharak Moshiree, Isabel Neuringer, Carl Riedy, Teresa Schindler, Lianne G. Singer, Dave Young, Lauren VignolaJoan Zukosky, Richard H. Simon

Research output: Contribution to journalArticlepeer-review

42 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)344-354
Number of pages11
JournalJournal of Cystic Fibrosis
Volume19
Issue number3
DOIs
StatePublished - May 2020

Keywords

  • Advance care planning
  • Advanced lung disease
  • Cystic fibrosis
  • Lung transplantation
  • Palliative care
  • Respiratory failure

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