*Purpose: Little data is published describing the safety or efficacy of elexacaftor/ivacaftor/tezacaftor in lung transplant recipients. Even though it is not expected to offer pulmonary benefits in lung transplant recipients with cystic fibrosis (CF), it may improve extrapulmonary manifestations.

*Methods: This retrospective case series summarizes the clinical experience of 7 lung transplant recipients on elexacaftor/ivacaftor/tezacaftor therapy.

*Results: Patients were 39 +/- 11 years old, 71% male, and 7.6 +/- 7 years post transplant when starting elexacaftor/ivacaftor/tezacaftor. After 211 +/- 89 days on therapy, 4 (57%) note improvement in at least one domain: 4 (47%) report improved GI symptoms, 2 (29%) report improvement in sinus symptoms, 2 (29%) had pancreatic enzyme dose reductions. Of the 5 patients with CF-related diabetes, 3 (40%) report improvement in diabetes control. Most patients required a tacrolimus dose reduction after starting elexacaftor/ivacaftor/tezacaftor. In order to maintain the same tacrolimus trough goal, doses were adjusted from 9.3 +/- 5.9 mg/day at baseline to 7.4 +/- 5.3 mg/day after dose titration was complete; this was an average 20% dose reduction. There were no cases of allograft rejection. With regard to drug-related side effects severe enough to warrant intervention, 2 (29%) experienced leukopenia and 1 (14%) experienced transaminitis. Of the two with leukopenia, one improved with dose adjustment to other medications whereas one required elexacaftor/ivacaftor/tezacaftor discontinuation. The discontinuation rate for any cause was 1 patient (14%).

*Conclusions: In sum, elexacaftor/ivacaftor/tezacaftor may offer a benefit for management of the extrapulomonary manifestations of cystic fibrosis after lung transplantation. Enhanced monitoring for tacrolimus dose adjustments and drug-related side effects is warranted.

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