Pregnancy in Patients With Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive, monogenic disorder that affects individuals of all races and ethnicities. It was historically viewed as a pediatric disease, but with comprehensive care and therapeutic advances, adults with CF now outnumber children living with CF in the United States, and the median predicted life expectancy is approximately 61 years. Since 2010, a new drug class called CF transmembrane conductance regulator (CFTR) modulators has emerged and led to improved health outcomes for most people with CF in the United States. These new medications have likely contributed to a doubling of pregnancies among women with CF in recent years. In the setting of increased fertility, people with CF should consider contraception when pregnancy is not desired. For people with CF who are seeking pregnancy, preconception consultation with expertise in maternal-fetal medicine and genetics and a dedicated CF team is important because of the increased morbidity among pregnant people with CF. Breastfeeding is possible and encouraged when taking CFTR modulators, although the pediatrician should monitor the infant for possible side effects given established transfer through breast milk. Single-gene noninvasive prenatal testing is emerging and offers a new strategy to screen for fetal CF, which is increasingly important with emerging evidence that transplacental transfer of CFTR modulators may delay, prevent, or reverse multiple CF manifestations for the fetus with CF. Research is ongoing to study pregnancy and parenthood in the modern era in people with CF.