Lung Transplant Referral for Individuals with Cystic Fibrosis
Publication Date: March 27, 2019
Last Updated: March 14, 2022
Recommendation Statements
The CF Foundation recommends routine clinician-led efforts to discuss disease trajectory and treatment options, including a discussion of lung transplantation. (, , )
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The CF Foundation recommends CF care team initiated discussion regarding lung transplantation with all individuals with CF and an FEV1 <50% predicted. (, , )
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The CF Foundation recommends the use of up-to-date CF-specific transplant resources to promote understanding of the transplant journey and to minimize misconceptions regarding outcomes. (, , )
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The CF Foundation recommends that CF clinicians develop relationships with peers at partnering transplant centers to:
• optimize the transition to transplant, starting with referral
• understand transplant center-specific practices, including navigating complex socioeconomic barriers to transplant
• maintain ongoing communication about clinical status of individuals listed or approaching transplant listing (, , )
• optimize the transition to transplant, starting with referral
• understand transplant center-specific practices, including navigating complex socioeconomic barriers to transplant
• maintain ongoing communication about clinical status of individuals listed or approaching transplant listing (, , )
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The CF Foundation recommends that the individual's CF care team elicit and address CF-specific psychosocial and physical concerns about lung transplantation to facilitate transition to transplant. (, , )
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The CF Foundation recommends that modifiable barriers to lung transplantation be addressed preemptively to optimize transplant candidacy; however, unresolved barriers should not preclude referral. Potentially modifiable barriers may include but are not limited to: nutritional status, diabetes management, physical inactivity or deconditioning, adherence behaviors, mental health issues, substance use, and psychosocial factors. (, , )
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The CF Foundation recommends the CF and lung transplant care teams acknowledge and provide support for mental health concerns regarding the referral and evaluation process for transplant that are unique to individuals with CF. (, , )
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For individuals with CF who are 18 years of age and older, the CF Foundation recommends lung transplant referral no later than when:
• FEV1 is <50% predicted and rapidly declining (>20% relative decline in FEV1 within 12 months) OR
• FEV1 is <40% predicted with markers of shortened survival OR
• FEV1 is <30% predicted. (, , )
• FEV1 is <50% predicted and rapidly declining (>20% relative decline in FEV1 within 12 months) OR
• FEV1 is <40% predicted with markers of shortened survival OR
• FEV1 is <30% predicted. (, , )
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For individuals with CF who are under the age of 18 years, the CF Foundation recommends lung transplant referral no later than when:
• FEV1 is <50% predicted and rapidly declining (>20% relative decline in FEV1 within 12 months) OR
• FEV1 is <50% predicted with markers of shortened survival
• FEV1 is <40% predicted. (, , )
• FEV1 is <50% predicted and rapidly declining (>20% relative decline in FEV1 within 12 months) OR
• FEV1 is <50% predicted with markers of shortened survival
• FEV1 is <40% predicted.
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For individuals with CF and an FEV1 <40% predicted, the CF Foundation recommends an annual 6-min walk test (6MWT), assessment of need for supplemental oxygen, and venous blood gas to screen for markers of severe disease that may warrant transplant referral. (, , )
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For individuals with CF who are 18 years of age and older with FEV1 <40% predicted, the CF Foundation recommends a baseline echocardiogram to screen for pulmonary hypertension. (, , )
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The CF Foundation recommends lung transplant referral, regardless of FEV1, when there are markers of shortened survival, including:
• 6MWT distance <400 m OR
• hypoxemia (at rest or with exertion) OR
• hypercarbia (PaCO2 >50 mmHg, confirmed on arterial blood gas) OR
• pulmonary hypertension (PA systolic pressure >50 mmHg on echocardiogram or evidence of right ventricular dysfunction in the absence of a tricuspid regurgitant jet). (, , )
• 6MWT distance <400 m OR
• hypoxemia (at rest or with exertion) OR
• hypercarbia (PaCO2 >50 mmHg, confirmed on arterial blood gas) OR
• pulmonary hypertension (PA systolic pressure >50 mmHg on echocardiogram or evidence of right ventricular dysfunction in the absence of a tricuspid regurgitant jet). (, , )
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The CF Foundation recommends lung transplant referral for adults with CF with a BMI <18 and FEV1 <40% predicted while concurrently working to improve nutritional status. (, , )
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The CF Foundation recommends lung transplant referral for individuals with FEV1 <40% predicted and >2 exacerbations per year requiring IV antibiotics or 1 exacerbation requiring positive pressure ventilation regardless of FEV1. (, , )
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The CF Foundation recommends lung transplant referral for individuals with FEV1 <40% predicted and massive hemoptysis (>240 mL) requiring ICU admission or bronchial artery embolization. (, , )
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The CF Foundation recommends lung transplant referral for individuals with FEV1 <40% predicted and pneumothorax. (, , )
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For females with CF, especially those who are younger, the CF Foundation recommends special consideration for lung transplant referral even when other thresholds have not been met. (, , )
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For individuals with short stature (height <162 cm), the CF Foundation recommends special consideration for lung transplant referral even when other thresholds have not been met. (, , )
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The CF Foundation recommends CF clinician consultation with local and geographically distant lung transplant centers for individuals with microorganisms that may pose a risk for lung transplantation (e.g. Burkholderia cepacia complex, nontuberculous mycobacterium, certain molds such as scedosporium). (, , )
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Before determining that an individual is not a transplant candidate the CF Foundation recommends consultation with at least two transplant centers, one of which should have experience addressing the individual's potential contraindications to transplantation. (, , )
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For transplant candidates, the CF Foundation recommends communication between the CF and lung transplant care teams at least every 6 months and with major clinical changes. (, , )
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Recommendation Grading
Overview
Title
Lung Transplant Referral for Individuals with Cystic Fibrosis
Authoring Organization
Cystic Fibrosis Foundation
Publication Month/Year
March 27, 2019
Last Updated Month/Year
January 29, 2024
Supplemental Implementation Tools
Document Type
Consensus
External Publication Status
Published
Country of Publication
US
Document Objectives
Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF.
Inclusion Criteria
Female, Male, Adolescent, Adult, Child
Health Care Settings
Hospital, Operating and recovery room, Outpatient
Intended Users
Social worker, respiratory therapist, psychologist, nurse, nurse practitioner, physician, physician assistant
Scope
Counseling, Management, Treatment
Diseases/Conditions (MeSH)
D003550 - Cystic Fibrosis
Keywords
cystic fibrosis, lung transplant, Advanced lung disease