Outcome of pregnancy in sickle cell anemia and sickle cell-hemoglobin C disease. An analysis of 181 pregnancies in 98 patients, and a review of the literature.

The outcome of pregnancy has been analyzed in 72 women with sickle cell anemia (SS) and 26 women with sickle cell-hemoglobin C disease (SC), part of an unselected population of 148 women over 18 years of age with these hemoglobinopathies, who have been followed at a sickle cell disease clinic for 8 years. In SS women, 22% of first pregnancies were aborted spontaneously, and the overall early fetal loss was 19.2%. A similar figure was calculated from the literature since 1956. In SC women, 12% of first pregnancies were lost, but the overall early fetal loss was only 8.9%. The perinatal mortality, under quite variable conditions of prenatal care and delivery, was 10.2% in SS women and 2% in SC women. There were no stillbirths or midterm deaths in utero among SC women, but these accounted for most of the perinatal mortality in SS women, particularly in first and second pregnancies. There was one neonatal death in each group, but eight third pregnancies in SS women were completed without perinatal mortality. A remarkable finding among the SC women was the number of successful pregnancies, ten in one woman, and many of the pregnancies were completed without the supervision of a physician. Our findings were compared with those in the literature, and the conclusion that we drew is that termination of pregnancy and sterilization of young women are not generally indicated solely on the basis of these hemoglobinopathies.