Survival in individuals with cystic fibrosis (CF) has improved substantially, even before treating the underlying genetic defect encoding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein.

CF lung disease causes most of the morbidity and mortality, with lung transplantation considered the ultimate treatment option for individuals with progressive advanced CF lung disease (ACFLD).

Since 2012, newer drugs called CFTR modulators have become available, revolutionizing CF care by targeting the underlying defect in CF that causes decreased CFTR protein synthesis, function, or stability.

Even though randomized controlled trial data for CFTR modulators in individuals with ACFLD are lacking, clinical usage of these drugs in selected individuals shows encouraging results, leading to removal of patients from transplant waiting lists and fewer lung transplants for performed for CF.

New consensus guidelines for the selection of CF candidates for lung transplantation should take the new therapies into consideration regarding timing of referral and listing for transplant.