Pregnancy and cystic fibrosis - A case-controlled study

Study objectives: Changes in cardiorespiratory and pulmonary function that occur with normal pregnancy along with increased maternal and fetal demands related to cystic fibrosis (CF) may augment morbidity for the woman with CF. Status prior to pregnancy is implicated in pregnancy outcome and maternal life expectancy postpartum. The purpose of this study was to investigate the effect of pregnancy on these patients' course during pregnancy and document prepregnancy status and 2-year postpregnancy survival. Design: Patients with documented pregnancies were matched to nonpregnant CF patients of similar age (+/- 2 years), severity of airflow obstruction (percent predicted forced expiratory volume in 1 min [+/- 15%]), weight (+/- 10 kg), height (+/- 5 cm), and pancreatic sufficiency status at 1 year preconception. Patients: Using their 1-year preconception data, seven women with CF and with documented pregnancies were matched to nonpregnant control subjects. All patients were pancreatic insufficient. Interventions: Weight, forced expiratory volume in 1 min (% FEV1), FVC, Schwachman-Kulczycki (S-K) and Brasfield scores, sputum cultures, pregnancy outcome, and pulmonary exacerbations were followed from 1 year preconception, during pregnancy, and 2 years postpregnancy. Measurements and results: Mean weight gain during pregnancy was 5.2 kg. There were no differences between the groups in the rate of decline for pulmonary function or S-K scores over time. Greater rate of decline was noted in the pregnancy group, however, for body weight and Brasfield scores in the postpartum interval. One patient in the pregnancy group died 6 months postpartum. Conclusions: Pregnancy has little adverse effect on patients with stable CF, but poor outcomes can occur in individuals with more advanced disease.