WS19.03 Triple therapy for Cystic Fibrosis (elexacaftor/tezacaftor/ivacaftor): desensitization after an adverse reaction with severe skin rash

Abstract

Background: Cystic fibrosis (CF) is an autosomal recessive disorder of the CF transmembrane conductance regulator (CFTR) gene. CFTR modulators are novel approved therapies and triple therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is the current gold-standard for patients with at least one F508del mutation. CFTR modulators are usually well tolerated but some adverse effects may occur, including skin rash, that usually does not require drug discontinuation. Rash is more common in women, particularly those on hormonal contraception.