P301 Step-wise introduction of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and liver cirrhosis Child-Pugh A or B using clinical- and therapeutic drug monitoring: a case series

Abstract

Serum liver test abnormalities are described as a common adverse effect of elexacaftor-tezacaftor-ivacator (ETI) in patients with Cystic Fibrosis (pwCF). In the phase I registration studies the PK of ETI have been compared between non-CF people with hepatic impairment and healthy individuals. In the former group exposure of ETI was increased and therefore a reduced dose in pwCF and cirrhosis Child-Pugh B is recommended. To our knowledge, there are no data on the exposure of ETI in pwCF and cirrhosis Child-Pugh A or B. In this case series we describe seven pwCF and cirrhosis Child-Pugh A or B where ETI was gradually introduced using clinical and therapeutic drug monitoring (TDM).