Current pregnancy outcomes in women with cystic fibrosis

Abstract

Women with cystic fibrosis (CF) now achieve a greater life expectancy and therefore have greater expectations from life. Literature reporting pregnancy outcomes in CF is still sparse. There remains a legacy of advising women with significant disease to avoid pregnancy. We aimed to assess current maternal and fetal outcomes in women with CF with varied pre-pregnancy lung function. Retrospective case note review of data from 15 pregnancies in 12 women with CF receiving care at a specialist centre between 2003 and 2011. Descriptive statistics were used for the quantitative data. The forced expiratory volume (FEV₁) and forced vital capacity (FVC) were calculated and shown as the percentage of their predicted values for BMI, height and age. Changes in lung function pre, 6, and 24 months post delivery were calculated with the paired t-test. Mean maternal age was 28.9 (range 21-36, CI 26.8-31). Maternal FEV₁ at booking ranged from 27 to 80% predicted (mean=63.6%, CI 54.62-71.38%). Cystic fibrosis-related diabetes (CFRD) was present in 8 of 14 (live birth) pregnancies. Average gestation at delivery was 38 weeks. There was a 100% vaginal delivery rate (11 spontaneous vertex, 2 ventouse, 1 forceps). Average fetal birth weight was 2.97 kg (range 2.2-3.83 kg, CI 2.72-3.23). The differences between the maternal pre- and 6 months post-pregnancy mean FEV₁ (p=0.136) and FVC (p=0.225) were not statistically significant. With careful multidisciplinary antenatal and intrapartum management, successful outcomes have been obtained in this group of women with CF.