Medical and Obstetric Complications Among Pregnant Women with Cystic Fibrosis

Objective: The objective of this study was to estimate the nationwide prevalence of cystic fibrosis (CF) in pregnancy and determine what medical complications exist at delivery among pregnant women with CF.

Study Design: The Nationwide Inpatient Sample (NIS) was queried for all delivery-related discharges. Women with CF were identified by International Classification of Diseases, 9th revision, Clinical Modifications codes and compared with women without CF. The prevalence of selected severe medical complications was compared between the 2 groups (NIS years 2008-2010) using multivariable logistic regression and the linear change in prevalence of CF at delivery determined (NIS years 2000-2010).

Results: From 2000 to 2010, there was a significant linear increase in the prevalence of CF at delivery from 3.0 to 9.8 per 100,000 deliveries, in 2000 and 2010, respectively (R(2) = 0.92, P < .0001). From 2008-2010, there were 1119 deliveries to women with CF and 12,627,627 to women without CF. Women with CF were more likely to be white (P < .0001) and have diabetes (odds ratio [OR], 14.0; 95% confidence interval [CI], 11.8-16.7) or asthma (OR, 5.1; 95% CI, 4.3-6.1). Multivariable logistic regression demonstrated that women with CF were more likely to die (adjusted OR [aOR], 76.0; 95% CI, 31.6-183), require mechanical ventilation (aOR, 18.3; 95% CI, 10.8-31.2), or have pneumonia (aOR, 56.5; 95% CI, 43.2-74.1), acute renal failure (aOR, 17.3; 95% CI, 9.1-32.6), preterm labor (aOR, 2.2; 95% CI, 1.9-2.6), or an adverse composite CF outcome (aOR, 28.1; 95% CI, 21.8-36.3).

Conclusion: Pregnant women with CF are more likely to die, require mechanical ventilation, and have infectious complications compared with women without CF, although the absolute risks are low and these events are relatively rare.