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 fet al demands related to cystic fibrosis (CF) may augment morbidity for t he 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' co urse during pregnancy and document prepregnancy status and 2-year post pregnancy survival. Design: Patients with documented pregnancies were matched to nonpregnant CF patients of similar age (+/- 2 years), sever ity of airflow obstruction (percent predicted forced expiratory volume in 1 min [+/- 15%]), weight (+/- 10 kg), height (+/- 5 cm), and pancr eatic sufficiency status at 1 year preconception. Patients: Using thei r 1-year preconception data, seven women with CF and with documented p regnancies 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 Brasfie ld scores, sputum cultures, pregnancy outcome, and pulmonary exacerbat ions 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. G reater rate of decline was noted in the pregnancy group, however, for body weight and Brasfield scores in the postpartum interval. One patie nt in the pregnancy group died 6 months postpartum. Conclusions: Pregn ancy has little adverse effect on patients with stable CF, but poor ou tcomes can occur in individuals with more advanced disease.