Sickle cell anemia and pregnancy

Abstract

The present study is a review of eleven cases of sickle cell anemia associated with pregnancy observed on the Obstetrical Service of the Johns Hopkins Hospital over a 20-year period (1927–1947). No deaths occurred in these eleven mothers during the period of the pregnancy or puerperium. Three of thirteen viable infants died, giving a fetal mortality of 23.1 per cent. These figures justify a more optimistic outlook than hitherto noted in sickle cell anemia associated with pregnancy, since the previous literature reports an average maternal mortality rate of 21 per cent in thirty-three cases. Fifty-nine viable infants were born of these thirty-three mothers with nine deaths, giving a fetal mortality rate of 15.2 per cent.Sickle cell anemia occurred in one in 1,296 pregnant Negro women. Repeated hospitalization during the prenatal period was necessary. The average number of transfusions per patient during the pregnancy and puerperium was 5.2. Twelve reactions occurred in fifty-two transfusions given these patients (23.0 per cent). Only single instances of thrombosis and abdominal crises were observed during the pregnancies. Three cases were complicated by toxemias of pregnancy. Analysis of eleven cases did not support the idea that pregnancy exerted an untoward effect on the disease of sickle cell anemia.X-ray pelvimetry was studied in four cases, of which three had convergent side walls with heavy, blunted ischial spines. The pelves revealed a tendency toward funneling and anthropoid architecture with straight sacra and narrow subpubic arches. Further x-ray studies should be made.The treatment of sickle cell anemia associated with pregnancy demands constant medical observation and frequent hospitalization for investigation and treatment of concurrent toxemias and febrile states. Liberal use of penicillin, sulfonamides, and blood transfusions is needed to reduce both the maternal and fetal mortality rates. With the addition of the present study to the thirty-three previously reported cases in the literature, a total of forty-four cases have been observed up to the present time. Seven maternal deaths (or 15.9 per cent) have occurred with a fetal mortality of 16.6 per cent and a total fetal loss of 33 per cent. Therapeutic abortion and sterilization are seldom indicated in patients with sickle cell anemia associated with pregnancy.