Background:

Despite significant advancements in medical care, maternal and perinatal mortality rates remain alarmingly high among pregnant individuals with sickle cell disease (SCD). Preliminary evidence indicates that prophylactic transfusions could potentially enhance maternal and perinatal outcomes. However, the limited number of studies and their methodological limitations underscore the need for further research. Therefore, a retrospective analysis was performed to evaluate the incidence of maternal and perinatal complications among transfused pregnant patients with different sickle cell genotypes.

Methods:

A retrospective analysis was performed of medical records from New York City Health and Hospitals/Kings County, an urban hospital serving an underserved population. The study included pregnant patients diagnosed with sickle cell disease between January 1, 2015, and January 1, 2024. Patient data were obtained using the hospital's EPIC and Quadramed software systems. Statistical analysis was conducted to evaluate maternal sickle cell complications, obstetric complications, and perinatal outcomes, stratifying the data by genotype, mean hemoglobin level, race, and age.

Results:

This study included a predominance of patients with homozygous sickle cell disease (HbSS), constituting 69% of the study population. Most of these patients (66%) were over 35 years old. The HbSS group had lower mean baseline hemoglobin levels at approximately 7.0 g/dl, which remained stable throughout their pregnancies. In contrast, patients with heterozygous sickle genotypes (HbSC and HbS Beta+ thalassemia) had baseline hemoglobin levels, averaging around 9.5 g/dl and 7.0 g/dl, respectively, which stabilized at about 8.0 g/dl during pregnancy. The requirement for blood transfusions was highest among HbSS patients, with 69% receiving transfusions, compared to 44% of HbSC patients and 25% of HbS Beta+ thalassemia patients, highlighting differing medical needs.

Obstetric complications were more prevalent in HbSS patients compared to other genotypes. Pre-eclampsia was observed exclusively in the HbSS group (21%), with no cases in the HbSC and HbS Beta+ thalassemia groups. Postpartum hemorrhage was observed in 17% of the HbSS patients, 44% of the HbSC patients, and 0% of the HbS Beta+ thalassemia group. Cesarean sections were common across all groups, with rates of 55% in HbSS, 56% in HbSC, and 50% in HbS Beta+ thalassemia patients. Miscarriages occurred in 10% of both HbSS and HbSC patients, with none in the HbS Beta+ thalassemia group.

Fetal complications varied among genotypes. Intrauterine growth retardation (IUGR) was predominantly noted in HbSS patients (7%). Fetal distress was more prevalent in HbSC patients (22%) compared to 3% in HbSS patients, with none in HbS Beta+ thalassemia patients. Intrauterine fetal death was a concern in 3% of HbSS and 22% of HbSC patients, but none in the HbS Beta+ thalassemia group.

Vaso-occlusive episodes during pregnancy were reported in 29% of all patients, with 34% in the HbSS group, 11% in the HbSC group, and 25% in the HbS Beta+ thalassemia group. Comparative analysis between transfused and non-transfused HbSS patients showed that the transfused group had lower hemoglobin levels and a higher incidence of vaso-occlusive episodes. Obstetric and fetal complications were comparable between the two groups, indicating a complex association between transfusion therapy and adverse outcomes.

Conclusion:

This retrospective analysis highlights the intricate association between pregnancy-related complications in sickle cell disease and transfusion therapy. The data suggest a correlation between lower mean hemoglobin levels and adverse outcomes, indicating the need for further investigation to determine the best management strategies for pregnant individuals with SCD. Prospective studies that incorporate comprehensive clinical and laboratory assessments are essential to better understand the role of prophylactic transfusions in mitigating maternal, fetal, and SCD-related complications. These efforts are critical to advancing evidence-based practices and improving outcomes for this vulnerable patient population.

Disclosures

No relevant conflicts of interest to declare.

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2024
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