Maternal and Infant Outcomes in a Subset of Patients with Sickle Cell Disease in South Carolina

Sickle cell disease (SCD), which disproportionately affects minorities, increases complications during pregnancy. Severe maternal mortality is increased in women with SCD, including morbidity related to the disease and other nondisease-related complications. It also can have devastating complications for fetuses, with increases in premature birth and low birth weight. This study aimed to describe the characteristics of women with SCD in South Carolina, with a specific focus on fetal and maternal outcomes and complications. The secondary aim of this study was to identify the effect of maternal characteristics on birth outcomes, including social determinants of health.

A secondary analysis of women from a single institution, the Medical University of South Carolina, which was part of the registry from the multi-institutional Sickle Cell Disease Implementation Consortium, was conducted. Patient demographics, self-reported pregnancy history, hydroxyurea use, and maternal and fetal outcomes were collected from patient-reported survey data. In addition, the number of vaso-occlusive episodes surrounding their pregnancies was collected for analysis.

Fifty-nine percent (116/195) of the female participants reported ever being pregnant. Seventy-two percent had live births, 15.8% had miscarriages, 1.8% had stillbirths, and 6.1% had an abortion. The mean age was 22.3 ± 4 years, with no difference in markers of severity between the groups. Most women were HbSS genotype with high rates of pain in the last year. No difference was found in age, education, employment, or income between these groups of women. Women in the nonlive birth cohort had higher body weights (78.7 versus 72.1 kg, P = 0.045). The Distressed Community Index was used as a marker for social determinants of health and was similar between the two groups, with a majority of both cohorts (61.7% and 52%) living in "at risk" and "distressed" communities. Complications related to SCD were high, including 43% of women experiencing pain during pregnancy, 5.2% developing acute chest syndrome, and 22.4% requiring transfusion. An additional 11% experienced preeclampsia. Unfavorable infant outcomes included 49% of the infants being premature and 40% having babies weighing less than 5.5 lb at birth.

High rates of complications to both mother and infant were found in the women with SCD. Although few statistically significant predictors were found, by identifying and addressing specific needs of pregnant women with SCD, we can work toward reducing fetal and maternal mortality in an already vulnerable population.