Pregnancy in Patients with Cystic Fibrosis

Abstract

As more patients with cystic fibrosis reach adulthood, questions arise about the potential hazards of pregnancy. We reviewed the medical records of eight women with cystic fibrosis who had a total of 11 completed pregnancies and had been evaluated within 1 year before conception. In five women (Group 1), the overall maternal condition was little affected by the pregnancy, and in three women (Group 2), the mother's condition deteriorated during and after pregnancy and did not return to the pregravid state. With regard to pregravid status, significant differences between patients in Group 1 and Group 2 were found in Shwachman-Kulczycki clinical scores, weight for height values, Brasfield chest radiograph scores, and pulmonary function. A quantitative assessment of pregravid nutritional and pulmonary status is useful in counseling women with cystic fibrosis about the risk of pregnancy.

Respiratory diseases in pregnancy bullet 4: Women with cystic fibrosis and their potential for reproduction

“…Occasional case reports39-45 continued to appear until the first major review in 1980 of 129 pregnancies from the National CF Registry in the USA46 closely followed by a detailed study in Pennsylvania, the first to consider specifically the effect of lung function,47 and a third from Canada in 1991 48. These three have been the foundation on which commonly accepted practice has been based and led to a series of articles reviewing management49-51 and case reports detailing special management issues— notably, anaesthetic techniques in labour33 52 to minimise maternal respiratory embarrassment, intravenous hyperalimentation to maintain adequate weight gain,10 32-34 and breast feeding 53-57.…”

“…49-51 The most comprehensive to date, which reviews 15 studies,4 33 34 36-40 42 44 46 47 67-69 is by Kent and Farquharson 70. They looked in particular for pregnancies completed beyond 20 weeks, the incidence of spontaneous abortion, the proportion of preterm births, the perinatal mortality rate, and the maternal death rate at 6 and 24 months.…”

“…More recently the number and proportion of successful mothers with pancreatic insufficiency is increasing47 48 64 66 71and, as nutritional care has improved, pancreatic status has become a less significant variable,62 64 although pancreatic sufficiency is still associated with better overall outcome 66…”

Pregnancy and the lungs

“…Whereas men with the disorder are usually infertile, women can conceive. Patients with good antenatal pulmonary function and nutritional status have a high likelihood of a favourable maternal and fetal outcome 27. The best predictor of bad maternal outcome is considered to be a forced expiratory volume in one second <60% and termination of pregnancy should be recommended to those with poor functional status.…”

Contraception and pregnancy in cystic fibrosis

“…32 Other small case series have also reported an increased incidence of preterm delivery and maternal death in women with worse pre{existing lung function. 33,34 In view of the potentially devastating consequences of an unwanted pregnancy for those with severe CF some authors have advocated termination of pregnancy in women with poor lung function (FVC less than 50% predicted value) or progressive pulmonary deterioration. 35 However successful pregnancies have been reported even in those with poor lung function.…”