Cystic fibrosis (CF) is a common inherited autosomal recessive disorder, caused by a gene defect on chromosome 7. Viscous mucus in the lungs results in chronic colonization with various organisms that cause recurrent respiratory infections. The chronic inflammation leads to progressive bronchiectasis, a decline in respiratory function, and eventually respiratory failure causing death. Bowel, pancreatic, and hepatobiliary organs are also affected, with additional complications of diabetes mellitus and biliary cirrhosis. Survival with CF has dramatically improved over the last decade because of improvements in care of the respiratory system and nutrition. About 50% of women with CF can now expect to live to over 38 years of age. With increased life expectancy, more women with CF wish to conceive, but the published data on pregnancy outcome in patients with CF are sparse. This retrospective review was undertaken to assess pregnancy outcomes over the last 8 years in women with CF and a wide variation of prepregnancy lung function.
A multidisciplinary team that included several CF specialists and an obstetric anesthetist provided patient care with pulmonary function monitoring and aggressive treatment of infective exacerbations of lung disease. Growth scans were obtained every 2 to 4 weeks from 26 weeks’ gestation. Spontaneous labor and vaginal delivery were advised if the patient had no obstetric contraindications. Oxygen was administered if the patient became breathless or oxygen saturation was less than 95%. Outcome measures included maternal age, body mass index before and after pregnancy, forced expiratory volume at 1 second (FEV1), forced vital capacity (FVC), need for intravenous (IV) antibiotics, incidence of maternal diabetes, mode of delivery, gestational age at delivery, and fetal Apgar scores and birth weight, and maternal and fetal complications and survival.
From 2003 to 2011, 12 women (average age, 28.9 years) had 15 pregnancies; 5 patients had prepregnancy counseling, and outcomes of all 15 pregnancies were known. Fourteen (93.3%) resulted in live births, and 12 (86%) were at term (>37 weeks). No stillbirths, neonatal deaths, or early maternal deaths occurred. One fetus had acrania noted at 14 weeks’ gestation, and the pregnancy was surgically terminated; she had a normal delivery and live birth in the subsequent pregnancy. One child had CF. No patients with CF had preexisting pulmonary hypertension or cor pulmonale. One mother died 12 months after delivery because of complications of CF (FEV1 27% before delivery). The mean prepregnancy body mass index, FEV1, and FVC were 21.4 kg/m2, 63.6%, and 83.2%, respectively. Diabetes was present in 8 women (57%), preexisting CF-related diabetes in 4 women (28.5%), and diabetes diagnosed during pregnancy in 4. Seven patients needed at least 1 course of IV antibiotics in pregnancy; 3 patients (21%) had chronic infection with Pseudomonas aeruginosa. All women had a vaginal delivery (spontaneous vertex in 12, ventouse [vacuum] delivery and forceps delivery in 1 each). Epidural analgesia was used in 11 patients (78.6%). No postpartum hemorrhages or third-degree perineal tears occurred. Average fetal birth weight was 2.97 kg. With data from 11 pregnancies, the mean FEV1 fell by 6.4% and FVC by 9.7% during pregnancy. The difference in the FEV1 before and 6 months after pregnancy was 1.9%, but that for FVC was 12.6%; these differences were not statistically significant. The differences between the mean FEV1 and FVC before and 24 months after pregnancy were 5.2% and 7.7%, and neither was statistically significant.
Pregnancy in women with CF is increasingly common, and this incidence will rise as the health of women with CF continues to improve. Lung function may not be the only important predictive factor in pregnancy outcome, and the stability of a woman’s lung function when she becomes pregnant may be equally important. The psychological aspects must be included in any counseling offered to women with CF. Managing parenthood with a chronic life-limiting disease must be thoroughly discussed. Research with larger numbers of women may offer current accurate prepregnancy counseling for women with CF.