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. 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.
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.
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.
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