The Outcome of 72 Pregnancies in 55 Women With Cystic Fibrosis in the United Kingdom 1977???1996

Abstract

Objective To identify pregnancies in women with cystic fibrosis and describe obstetric, infant and maternal medical outcomes in relation to the severity of maternal disease. Design Retrospective study, based on casenotes. Setting Eleven cystic fibrosis centres in the United Kingdom. Population Pregnant women with cystic fibrosis. Methods Single observer medical and obstetric casenote review categorising maternal cystic fibrosis (e.g. genotype, pancreatic, hepatic and diabetic status) and pre‐pregnant severity (e.g. weight and lung function) and noting fetal outcome and maternal morbidity. Main outcome measures Completed pregnancies and pregnancy losses, fetal outcome and complications, maternal morbidity, such as changes in weight, lung function, pulmonary infections during and after pregnancy. Relation of outcomes to severity of maternal cystic fibrosis. Results From 72 pregnancies identified, the outcomes were known for 69; there were 48 live births (70%) of which 22 were premature (46%); 14 therapeutic abortions (20%); and 7 miscarriages (10%). There were no stillbirths, neonatal or early maternal deaths. Three major fetal anomalies were seen, but no infant had cystic fibrosis. At the conclusion of our study three pregnancies were still continuing. Prematurity with increased fetal complications and maternal morbidity with infection, declining lung function and poor weight gain were associated with poor pre‐partum lung function. Conclusion Pregnancy occurs in women with cystic fibrosis of all degrees of severity. Outcomes for the infant are generally good but are variable for the mother. Predicting outcome on the basis of maternal severity is difficult but lung function appears to be the most significant determining factor. Pregnancy may be normal in women with normal lung function (forced expiratory volume > 80%). However, it may adversely affect mild and moderate lung disease due to cystic fibrosis and should be avoided in pulmonary hypertension, cor pulmonale and when forced expiratory volume < 50% predicted. Ideally, all pregnancies should be planned with prior counselling and monitored by dedicated cystic fibrosis teams, including obstetricians who are experienced in managing high risk pregnancies.

Changes in pulmonary function during pregnancy: a longitudinal cohort study

“…13 Studies have reported a direct relationship between maternal FEV1 during pregnancy and infant birthweight, 14,15 and an inverse relationship with intrauterine growth retardation, 14 gestational hypertension, and preterm birth in asthmatic women. 15 In pregnant women with cystic fibrosis, low FEV1 is associated with preterm delivery, [16][17][18] and with a greater loss of lung function during pregnancy. 17,18 As a consequence, pregnant women with pulmonary disease need regular monitoring of symptoms and measures of lung function by spirometry in order to optimise their lung function throughout pregnancy.…”

Management of a parturient with respiratory failure secondary to cystic fibrosis

“…The respiratory parameter most frequently described as being predictive of outcome is the forced expiratory volume in 1 s (FEV 1 ). Thresholds vary -different studies have linked poorer outcome with figures of less than 50% [4], 60% [5] or 70% [6] of predicted. Gilljam et al [4] also noted that infection with Burkholderia cepacia was associated with a poorer outcome.…”

“…Gilljam et al [4] also noted that infection with Burkholderia cepacia was associated with a poorer outcome. Others have advised against pregnancy in a context of pulmonary hypertension or cor pulmonale [6].…”

Contraception and pregnancy in cystic fibrosis

“…23 In a study of 26 term and 22 preterm live deliveries in women with CF the mean maternal weight gain of those pregnancies reaching term was 8.9 kg and only 2.6 kg in those delivering prematurely. 24 Careful consideration should be given to appropriate vitamin supplementation. Folic acid should ideally be used for 3 months preconceptually and for the first trimester of pregnancy to reduce the risk of fetal neural tube defects.…”

“…24 Fourteen (20%) pregnancies were terminated. The data showed that the prepregnancy %FEV 1 and %FVC predicted were significantly associated with gestational age, maternal weight gain and infant birthweight.…”