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.
