WS15.01Post-lung transplant survival for people with cystic fibrosis before and during the elexacaftor/tezacaftor/ivacaftor era

Abstract

Objective

Evaluate survival outcomes after lung-transplant (LTx) for people with cystic fibrosis (CF) in the United States (US) before and after the introduction of elexacaftor/tezacaftor/ivacaftor (ETI). Published US CF Foundation Patient Registry (CFFPR) data showed that almost half of LTx recipients were prescribed ETI prior to LTx in 2020-2022. At the 2024 North American CF Conference, the Toronto Program presented reduced survival for LTx recipients with CF in the ETI era.

Methods

LTx recipients with CF between Oct 1, 2016 and Dec 31, 2022 were identified using the Scientific Registry of Transplant Recipients dataset (the US national transplant registry), with follow-up through Jan 2024. Prior to the European CF Society annual scientific meeting, data will be linked with the CFFPR to allow for determination of ETI status for recipients at the time of LTx. Pre-ETI era was defined as Oct 1, 2016 to Sept 30, 2019 (ETI FDA-approved in the US in Oct 2019). We summarized demographics in both eras using descriptive statistics and calculated Kaplan-Meier estimates of one-year survival after LTx by transplant year, starting in Jan 2017.

Results

There were 803 LTx recipients with CF, 637 in the pre-ETI era and 166 in the ETI era. Recipients were 47% male, 95% White, 4% Black, and 7% Hispanic compared to 54%, 92%, 7%, and 14% in the pre-ETI vs ETI era, respectively. The proportion with Medicaid insurance increased from 20% to 27% between eras. The median (IQR) age was 31 years (25-38) pre-ETI and 31 years (25-40) in the ETI era. Qualitative assessment of 1-year survival estimates showed a decline in survival in the ETI era, which was most pronounced in 2021 (Table).

Conclusions

Post-transplant survival for people with CF may have worsened in the ETI era. There are shifts in some patient characteristics but not significantly older age. Understanding whether survival estimates differ for people on ETI vs. not prescribed ETI at the time of LTx is a critical next step.