Pregnancy outcomes in the current era of cystic fibrosis care: A 15-year experience

Background: With improvement in clinical care and longer survival of patients with cystic fibrosis (CF), pregnancy has become commonplace. However, the impact of pregnancy on maternal health and fetal outcomes requires ongoing review. Methods: A retrospective study of 20 pregnancies from 18 women with CF during the period 1995-2009 was performed. Changes in lung function, body mass index (BMI) and development of gestational diabetes were recorded. Fetal outcomes and maternal survival were examined, and the influence of pre-pregnancy parameters on outcomes was evaluated. Results: Mean maternal age at pregnancy was 29 +/- 5 years with a mean pre-pregnancy forced expiratory volume in 1 s (FEV1) of 65.6 +/- 20.8% predicted. Eleven of 20 pregnancies had a pre-pregnancy FEV1 < 60% predicted. During pregnancy, FEV1% predicted fell by 4.8% (CI 1.6-7.9), but recovered to baseline within 6 months post-partum. Mothers gained a mean weight of 7.6 +/- 3.2 kg, and gestational diabetes developed in 43% of women. All women delivered live births apart from one therapeutic abortion. Five infants were preterm, and two mature infants had low birth weight. Three mothers either died or required lung transplantation after pregnancy (range 2.5-8.0 years). FEV1 < 60% predicted and BMI < 20 kg/m2 were significant predictors of fetal complications. Conclusion: Most women tolerated pregnancy well without major complications despite many having at least moderate lung function impairment. Pre-pregnancy FEV1 and BMI were important predictors of outcomes.