Both survival and health outcomes have improved for individuals with cystic fibrosis (CF) over the past 2 decades. In 2002, the median expected survival age for CF patients in the United States was 31.6 years; women had a lower median survival age of 29.0 years. Because of these improvements, clinicians are now required to address fertility, family planning, and pregnancy issues with their patients, Although early case reports and surveys of pregnant women documented poorer health outcomes during and following pregnancy, investigators have subsequently reported more favorable outcomes. In these reports of matched cohorts, no difference in lung function decline was noted between pregnant and nonpregnant patients. In the largest of these analyses, pregnancy was not an independent predictor of survival; however, women with lower lung function, diabetes, or poorer nutritional status in both the pregnant and nonpregnant groups had decreased survival. comments
In a more recent analysis of the Cystic Fibrosis Foundation patient registry data (1987 to 1997), women who became pregnant did not demonstrate a significantly shortened survival time, even in the subgroup of patients with an FEV1 of < 40% predicted. These findings are supported by reports from Canada, the University of Minnesota, and Scandinavia. The Scandinavian report also suggested that CF-related therapies to treat pulmonary and nutritional needs are used more intensively during pregnancy.
Taken together, these reports suggest that, at least for women who have mild CF lung disease, pregnancy may present minimal additional risk. However, in counseling patients and their partners, it is incumbent on clinicians to present a balanced picture, a view not only of the risks of pregnancy and the impact of childbearing on the health of the mother with CF, but also of the additional care that might be anticipated during and following the pregnancy. We used data from the Epidemiologic Study of Cystic Fibrosis (ESCF) longitudinal encounter-based data set to identify changes in care that occurred during and after pregnancy as well as nutritional and pulmonary outcomes in pregnant women with CF.