Lung transplant referral considerations for individuals with cystic fibrosis

The cystic fibrosis (CF) Foundation issued guidelines to promote timely lung transplant referral for people with cystic fibrosis (pwCF) in 2019. Since then more has been published to help refine this complex decision. The goal of this review is to summarize the recent literature informing disease severity in CF, barriers to referral for pwCF and guide timely and appropriate lung transplant referrals.

Existing guidelines utilize the degree of airflow limitation as the primary criteria to refer for lung transplant, yet this variable has some prognostic uncertainty. Novel prognostic tools may provide more reliable metrics for predicting who with CF is at greatest risk of dying from their lung disease and could be used as an indicator for when to refer. In addition, updated analyses of national registry data have highlighted the significance of hemoptysis, low body mass index, and extra-pulmonary organ failure, as important prognostic markers. PwCF with these complications have historically been under-referred for lung transplant despite data suggesting lung transplant can be safe for some in these populations. Early referral should be considered in the presence of these complications.

This review builds on existing guidelines by incorporating novel data to better determine when lung transplant referral is most appropriate. Improved prognostic tools are still needed to decrease the chances of pwCF dying without consideration of lung transplant. It is still unclear how novel therapies for CF may change the need and timing for lung transplant referral.