Outcome of Pregnancies Complicated by Sickle Cell and Sickle-C Hemoglobinopathies

ABSTRACT

Retrospective analysis was made of office and hospital records of patients with sickle cell hemoglobinopathies. Blood products were transfused only when indicated for symptomatic anemia, severe anemia with a hematocrit less than 18%, sickle crisis, cardiovascular instability, and preoperatively. The Fisher exact test and the Student t test were used for statistical analysis: P<0.05 was considered significant. All mean values are reported ± 1 standard deviation. From 1981 to 1991, 40 patients with sickle cell hemoglobinopathies had a total of 61 singleton 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 maternal 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 P<0.05), and the mean birthweight was 2443 ± 926 and 2997 ± 807 g (P P<0.05), respectively. There were two intrauterine fetal deaths and one neonatal death in the SSD group and 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 pregnancy is still associated with significant maternal and perinatal morbidity and mortality.