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 Vignola; Joan Zukosky; Richard H Simon

doi:10.1016/j.jcf.2020.02.015

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.

Authors

Siddhartha G Kapnadak¹, Emily Dimango², Denis Hadjiliadis³, Sarah E Hempstead⁴, Erin Tallarico⁴, Joseph M Pilewski⁵, Albert Faro⁴, James Albright⁶, Christian Benden⁷, Shaina Blair⁸, Elisabeth P Dellon⁹, Daniel Gochenour¹⁰, Peter Michelson¹¹, Baharak Moshiree¹², Isabel Neuringer¹³, Carl Riedy⁶, Teresa Schindler¹⁴, Lianne G Singer¹⁵, Dave Young¹⁶, Lauren Vignola⁶, Joan Zukosky¹⁷, Richard H Simon¹⁸

Affiliations

¹ Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle, WA, USA. Electronic address: skap@uw.edu.
² Division of Pulmonary, Allergy and Critical Care, Columbia University Medical Center, New York, NY, USA.
³ Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁴ Cystic Fibrosis Foundation, Bethesda MD, USA.
⁵ Pulmonary, Allergy, and Critical Care Medicine Division, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA.
⁶ Community Advisor to the Cystic Fibrosis Foundation, Bethesda, MD, USA.
⁷ Medical Faculty, University of Zurich, Zurich, Switzerland.
⁸ Emory University/Children's Healthcare of Atlanta, Atlanta, GA, USA.
⁹ Department of Pediatrics, Division of Pulmonology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
¹⁰ Pulmonary Diagnostics & Respiratory Therapy Services, University of Virginia Medical Center, Charlottesville, VA, USA.
¹¹ Division of Allergy, Immunology and Pulmonary Medicine Department of Pediatrics Washington University School of Medicine St Louis, St Louis, MO, USA.
¹² Division of Gastroenterology, Hepatology and Nutrition, Department of Medicine, University of North Carolina, Charlotte, NC, USA.
¹³ Division of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.
¹⁴ Rainbow Babies and Children's Hospital University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
¹⁵ Division of Respirology, Department of Medicine, University Health Network, University of Toronto, Toronto, ON, Canada.
¹⁶ University of Utah Adult Cystic Fibrosis Center, Salt Lake City, Utah, University of Utah College of Pharmacy, Salt Lake City, UT, USA.
¹⁷ Washington University School of Medicine Adult Cystic Fibrosis Program, St Louis, MO, USA.
¹⁸ Division of Pulmonary & Critical Care Medicine, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.

PMID: 32115388
DOI: 10.1016/j.jcf.2020.02.015

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.

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