Background: Cystic fibrosis (CF) is characterized by progressive lung disease that can lead to respiratory failure requiring lung transplant (LT). CFTR modulators (CFTRm) improve and/or preserve lung function and may reduce the need for LT.

Aim: This retrospective, observational study assessed associations between CFTRm approval and LT trends in the US.

Methods: Using data from the Scientific Registry of Transplant Recipients, rates of LT, pre-LT mortality, and removal from waitlist for improving condition in people with vs without CF were compared 1) prior to any CFTRm approval (1/1/09-1/30/12); 2) after ivacaftor (IVA), lumacaftor (LUM)/IVA, or tezacaftor (TEZ)/IVA but before elexacaftor (ELX)/TEZ/IVA approval (1/31/12-10/20/19); and 3) after ELX/TEZ/IVA approval (10/21/19-12/31/21).

Results: After ELX/TEZ/IVA approval, LT and pre-LT mortality rates substantially decreased in the CF group, while the LT rate increased and pre-LT mortality rate was stable in the non-CF group (Table). During this time, rate of removal from waitlist for improving condition substantially increased in CF but remained stable in the non-CF group.

Conclusions: In the US, LT and pre-LT mortality rates were reduced in people with CF after ELX/TEZ/IVA approval. Fewer CF-related LTs may have increased the proportion allocated to those without CF.