Abstract

PIP: As more women with cystic fibrosis (CF) live to childbearing age, more become pregnant and deliver healthy infants. A 1980 review shows 129 pregnancies in 100 CF women. 80% were full term. The perinatal mortality rate was 8.5% (almost all deaths were premature infants). 18% of the mothers dies within 2 years of delivery, but none died during pregnancy. This mortality rate matched the expected rate for nonpregnant CF women at the same age. If CF women are in sound health and want to have children, physicians should encourage them to do so. Despite rumors to the contrary and theoretical problems with dehydrated cervical mucus, women with mild CF have little difficulty conceiving. Overall contraception issues are the same for both CF women and non-CF women. A few differences do exist, however. CF women should prevent unwanted pregnancy because an abortion poses special risks for them and the child adds more demands on a woman who often needs hospital care. Unpredictable absorption in the intestines makes oral contraceptives unreliable for CF women. A CF woman must consider timing, family support, and genetics of the father when planning a pregnancy. Pregnancy may not affect lung function greatly because lung volume in CF women depends on the condition of the airways rather than the size of the thoracic cage. In pregnant women with severe CF, minute ventilation cannot rise enough so hypercapnia occurs, and blood volume and cardiac output may increase 50% in the 3rd trimester. All these changes could trigger cor pulmonale in these women. Pregnancy is contraindicated for CF women with raised PaCO2, and SaO2 of 90%, and cor pulmonale. Physicians should manage pregnant CF women the same as they would other women, but increase emphasis on controlling pulmonary infection and adequate nutrition. They should also avoid teratogenic drugs and drugs with no proven record.