Rebecca Scott, Amy Downes, L. Weitnauer, Rebecca Dobra, Natasha Singh, Rachel Robinson, Emily Diable, S. Collins, Elaine Bowman, Thomas Tobin, Andrew Jones, Nicholas J. Simmonds, I. Felton
OBJECTIVE: A comprehensive review of maternal, obstetric and neonatal outcomes in pregnancies in females with cystic fibrosis (fwCF) following the introduction of Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy in a novel, dedicated CF-Maternal Health service. DESIGN: Retrospective data review from a CF-Maternal Health service between September 2020 and February 2025. SETTING: A large adult-CF service in London, UK. POPULATION OR SAMPLE: Pregnant fwCF attending the Royal Brompton Hospital CF Service. METHODS: Review of CF-Maternal Health service data. MAIN OUTCOME MEASURES: Maternal, obstetric and neonatal outcomes. RESULTS: Fifty-three fwCF completed 67 pregnancies, with 69 infants born. There were no stillbirths, neonatal or maternal deaths. ETI-therapy was reported in 81% of pregnancies. In fwCF without CF-Diabetes, 57% developed gestational diabetes. Hospital admission to treat an infective pulmonary exacerbation was required in 31% of pregnancies. Forty-five percent of pregnancies delivered vaginally; 78% of babies were born at term. A major congenital abnormality was diagnosed in 4% of infants. Baseline lung-function correlated positively with birth-weight and gestation at birth. CONCLUSIONS: FwCF have improved maternal, obstetric and neonatal outcomes since the introduction of ETI-therapy, within a dedicated CF-Maternal Health service.