Cystic Fibrosis Transmembrane Conductance Regulator Modulators During Pregnancy: A Case Series

Cystic fibrosis (CF) is the most common genetic disease in the United States (US) and, with the development of newer therapeutics, there is increased fertility among women with CF. We present a series of pregnant patients taking novel CF transmembrane conductance regulator (CFTR) modulators and summarize pertinent clinical considerations. All women conceived within four months after starting elexacaftor-ivacaftor-tezacaftor. Pulmonary function was stable before and during pregnancy. One patient developed transaminitis necessitating discontinuation of the medication mid-trimester. All patients delivered healthy neonates between 36-38 weeks of gestation with uncomplicated postpartum courses. No birth defects were encountered. Given that newly introduced CFTR modulators may increase fertility among CF patients, contraception counseling, pulmonary function monitoring, liver function monitoring, and multi-disciplinary care are important pillars of management.