The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic
fibrosis post-liver transplant: a case series
Abstract
Introduction Cystic fibrosis (CF) related liver disease (CFLD) manifests
as a wide spectrum of hepatobiliary disease and can progress to need
liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is
a cystic fibrosis transmembrane conductance regulator (CFTR) modulator
which has superior efficacy compared to previously approved modulators.
Use of elx/tez/iva, should be approached with caution in individuals
with CFLD or following liver transplantation due to possible increases
in LFTs and drug-drug interactions with several immunosuppressant
medications. Objective The purpose of this case series was to explore if
the use of elx/tez/iva is safe and tolerable in patients with CF
post-liver transplantation. Methods A retrospective case series
including patients prescribed elx/tez/iva following liver
transplantation and an immunosuppressive regimen consisting of drug
therapy metabolized by P-glycoprotein was completed. Results Ten
patients at six CF centers with a median age of 22.1 years (range
14-43.4 years) and median time from transplant of 6.9 years (range
0.6-22 years) were included. Most patients (8, 80%) received a reduced
or full dose of elx/tez/iva for a mean duration of 10.4 months (range
7-12 months). Fluctuations in LFTs occurred in all patients (10, 100%)
and led to therapy discontinuation in two patients (20%). Elx/tez/iva
initiation resulted in elevations in tacrolimus trough concentration in
7 patients (70%). Most patients who tolerated elx/tez/iva had
symptomatic and quality of life improvement, increased body-mass-index,
and maintained or improved lung function. Conclusion Initiation of
elx/tez/iva in patients with CF who received a liver transplantation may
be safe with clinical benefits.