OUTCOME OF PREGNANCIES COMPLICATED BY SICKLE-CELL AND SICKLE-C HEMOGLOBINOPATHIES

Retrospective analysis was made of office and hospital records of pati ents with sickle cell hemoglobinopathies. Blood products were transfus ed only when indicated for symptomatic anemia, severe anemia with a he matocrit less than 18%, sickle crisis, cardiovascular instability, and preoperatively. The Fisher exact test and the Student t test were use d for statistical analysis; P <0.05 was considered significant. All me an values are reported +/- 1 standard deviation. From 1981 to 1991, 40 patients with sickle cell hemoglobinopathies had a total of 61 single ton pregnancies: 36 were complicated by SS disease (SSD), 22 by sickle cell disease (SCD), two by sickle-thalassemia, and one had CC disease (CCD). Only patients with SSD and SCD are reported here. The mean mat ernal age was 24.3 +/- 5.3 and 19.5 +/- 0.6 years in patients with SSD and SCD, respectively. There was a high occurrence of preterm labor ( 45% and 20%), preeclampsia (20% and 8.7%), pain crisis (50% and 34.2%) , pulmonary complications (25% and 16.7%), and cesarean sections (52.6 % and 37.1%) in SSD and SCD, respectively. An average of two units of blood was required by 43.1% of the patients. Two patients with SSD had unpreventable deaths. The mean gestational age at delivery was 35.5 /- 4.3 and 37.0 +/- 3.7 weeks (P <0.05), and the mean birthweight was 2443 +/- 926 and 2997 +/- 807 g (P <0.05), respectively. There were tw o intrauterine fetal deaths and one neonatal death in the SSD group an d one neonatal death in the SCD group. The perinatal mortality was 10. 5% and 2.9%, respectively. Despite advances in perinatal medicine and hematology, conservative management of sickle cell disease in pregnanc y is still associated with significant maternal and perinatal morbidit y and mortality.