Reproductive Considerations in Females With Cystic Fibrosis

To synthesize current evidence on fertility, pregnancy outcomes, and contraceptive safety in females with cystic fibrosis (CF) in order to support patient-centered family planning decisions for CF care teams.

Narrative review of the literature addressing female reproductive health in CF, encompassing anatomical, hormonal, and functional contributors to fertility; assisted reproductive technology (ART) outcomes; the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy; pregnancy risks and monitoring; and contraceptive considerations.

Infertility has historically affected approximately 37% of females with CF (FwCF) who attempt conception-more than twice the general population rate-driven by mechanisms including altered cervical mucus, ovulatory dysfunction, and impaired uterine pH. Despite this, FwCF who pursue ART achieve clinical pregnancy and live birth rates comparable to age-matched controls. Since FDA approval of elexacaftor/tezacaftor/ivacaftor in 2019, pregnancy rates among FwCF have more than doubled. Pregnancy carries elevated risks of preterm delivery, low birth weight, and gestational diabetes, particularly among those with prepregnancy FEV1 <50%. Approximately 50% of pregnancies in FwCF are unintended, underscoring the need for tailored contraceptive counseling that accounts for drug interactions with CF medications and thromboembolism risk in those with implanted ports.

Female reproductive health in CF is shaped by disease-specific mechanisms that historically reduced fertility, though ART outcomes are comparable to the general population and CFTR modulator therapy has substantially improved pregnancy rates. Pregnancy in FwCF requires careful risk stratification and monitoring. Given the high rate of unintended pregnancy, proactive and individualized contraceptive counseling is an essential component of comprehensive CF care.