Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

Kathleen J Ramos; Patrick J Smith; Edward F McKone; Joseph M Pilewski; Amy Lucy; Sarah E Hempstead; Erin Tallarico; Albert Faro; Daniel B Rosenbluth; Alice L Gray; Jordan M Dunitz; CF Lung Transplant Referral Guidelines Committee

doi:10.1016/j.jcf.2019.03.002

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

J Cyst Fibros. 2019 May;18(3):321-333. doi: 10.1016/j.jcf.2019.03.002. Epub 2019 Mar 27.

Affiliations

¹ Division of Pulmonary, Critical Care, and Sleep Medicine, Dept of Medicine, University of Washington, Seattle, WA, USA. Electronic address: [email protected].
² Department of Psychiatry and Behavioral Sciences, Behavioral Medicine Division, Department of Medicine, Pulmonary Division, Duke University Medical Center, Durham, NC, USA. Electronic address: [email protected].
³ National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Dublin, Ireland. Electronic address: [email protected].
⁴ Division of Pulmonary, Allergy & Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA. Electronic address: [email protected].
⁵ Cystic Fibrosis Foundation, Bethesda, MD, USA.
⁶ Cystic Fibrosis Foundation, Bethesda, MD, USA. Electronic address: [email protected].
⁷ Cystic Fibrosis Foundation, Bethesda, MD, USA. Electronic address: [email protected].
⁸ Cystic Fibrosis Foundation, Bethesda, MD, USA. Electronic address: [email protected].
⁹ Division of Pulmonary and Critical Care Medicine, Dept of Medicine, Washington University School of Medicine, St. Louis, MO, USA. Electronic address: [email protected].
¹⁰ Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado - Anschutz Medical Campus, Aurora, CO, USA. Electronic address: [email protected].
¹¹ Division of Pulmonary, Allergy, Critical Care Medicine and Sleep, Dept of Medicine, University of Minnesota, Minneapolis, MN, USA. Electronic address: [email protected].

Abstract

Objective: Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF.

Methods: The CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care. A focus group of lung transplant recipients with CF and spouses of CF recipients informed guideline development.

Results: The committee formulated 21 recommendation statements based on literature review, committee member practices, focus group insights, and in response to public comment. Critical approaches to optimizing access to lung transplant include early discussion of this treatment option, assessment for modifiable barriers to transplant, and open communication between the CF and lung transplant centers.

Conclusions: These guidelines will help CF providers counsel their patients and may reduce the number of individuals with CF who die without consideration for lung transplant.

Keywords: Advanced lung disease; Communication; Lung transplant; Modifiable barriers; Referral; Transplant.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

MeSH terms

Cystic Fibrosis* / diagnosis
Cystic Fibrosis* / surgery
Decision Making, Shared*
Humans
Lung Transplantation* / ethics
Lung Transplantation* / methods
Lung Transplantation* / psychology
Patient Selection*
Practice Guidelines as Topic
Referral and Consultation / organization & administration*

Grants and funding

K23 HL138154/HL/NHLBI NIH HHS/United States