Professor University of Florida College of Medicine Gainesville, Florida, United States
Objective: Cystic fibrosis (CF) is a genetic disease that had previously made pregnancy near impossible. With advancements in therapy, pregnancies in women with CF are increasing. We aim to describe the maternal and fetal outcomes in a large cohort of pregnancies with CF in the U.S.
Study Design: We utilized the National Inpatient Sample and extracted cases of CF-associated pregnancies and deliveries. We described the characteristics and perinatal outcomes of pregnant women with CF and compared them to general non-CF pregnancies in the U.S. from 1998-2021. Trends were analyzed with Poisson regression and rates were analyzed with chi-squared test.
Results: There was a total of 11,496 pregnancies with CF. From 1998 to 2021, hospitalization rates of CF pregnancies and deliveries have increased by 5-fold and 7-fold, respectively (Figure 1). Median age was 26 years in the CF pregnant cohort compared to 28 years in the non-CF cohort (p< 0.0001). Median length of hospital stay was longer in the CF cohort (3 days vs 2 days; p< 0.0001). Maternal deaths occurred at significantly higher rates in the CF cohort (0.496% vs 0.013%; p< 0.0001). Rates of diabetes (6.79% vs 1.11%; p< 0.0001), obesity (4.59% vs 3.81%; p=0.049), hepatitis C infection (0.51% vs 0.28%; p=0.033), cirrhosis (0.435% vs 0.006%; p< 0.0001), intravenous drug use (1.16% vs 0.61%; p< 0.001), and coagulopathy (1.24% vs 0.32%; p< 0.0001) were significantly higher in the CF pregnancy group compared to the non-CF pregnancy group. Poor fetal growth was more common in the CF cohort (3.05% vs 2.28%; p=0.013). Rates of pre-eclampsia, eclampsia, and intrauterine fetal demise were similar between the two groups (p >0.05). Among the 11,496 CF pregnancies, 6,786 (59.0%) had record of delivery. Rates of stillbirths (1.31% vs 0.76%; p=0.020) and preterm births (17.30% vs 7.83%; p< 0.0001) were higher in the CF cohort. Maternal death during delivery was also higher in the CF cohort (0.147% vs 0.006%; p< 0.0001).
Conclusion: CF pregnancies and deliveries are increasing over time, but morbidity and mortality in this group is higher than in the general pregnant population.
