Description of criteria for near miss in high-complexity obstetric population with sickle cell anemia: an observational study.

Introduction

Sickle cell disease (SCD) is one of the most common monogenic diseases worldwide, affecting approximately 30 million people across the globe. It is one of the most common hemoglobinopathies affecting pregnancy, particularly in the black population. Pregnant women with SCD, in conjunction with the physiological changes of pregnancy, have an increased risk of developing severe maternal and fetal complications.Materials and methods: A descriptive, observational study was conducted retrospectively to determine the maternal and neonatal disease burden of pregnant women with SCD between 2011 and 2016. The study included pregnant patients with SCD admitted to the hospital, with confirmed diagnosis through hemoglobin electrophoresis. Patients having no confirmatory testing of the disease were excluded.

Results

In all, 54% (34 patients) had no previous diagnosis of sickle cell anemia or presence of sickle cell trait, and so an in-hospital diagnosis was carried out through hemoglobin gel electrophoresis, whereas the remaining 46% (28 patients) had already a peripheral diagnosis. In total, the following profiles were obtained: Hb AS 62.9% (39 patients), Hb SC 17.7% (11 patients), Hb SS 16.1% (10 patients), Hb AC 3.2% (2 patients), and Hb CC 0% (0 patients). Vaso-occlusive pain crises constituted the most common cause of hospital admission antepartum (54.8% of the general population), occurring in 100% of the patients with Hb SS and Hb AC, in 45% of the Hb SC group and in 43% of those with the Hb AS trait. The second cause of hospital admission encompassed infectious processes, which affected 45% of the general population, 30% of the Hb AS group, 60% of the Hb SS group, 77% of the Hb SC group, and 100% of the Hb AC group. The method of termination of pregnancy of 44% (27 patients) was C-section. The most common perinatal complication was preterm birth, occurring in 26% (16 patients), with the highest incidence in the Hb AC group. The second most common complication was the intrauterine growth restriction, reported in 6% (4 patients) of all patients, being most frequently in the Hb SS group.

Conclusion

Pregnant women having SCD should be assessed and managed by a multidisciplinary team composed of hematology, perinatology, and intensive care. Management should be conducted preferably in high-quality centers experienced in treating this entity, thereby decreasing the great impact of morbidity and mortality associated with SCD in the pregnant population.