Patient Characteristics
Data were collected on 781 pediatric patients with SCD age 2 to 19 years, 462 (58.7%) with genotype HbSS/HbSβ° and 319 (41.3%) with genotype HbSC/ HbSβ+. Mean age was 10.8 years, 99% African American, and 82% had public insurance. There was no difference in the male to female ratio and no difference in age among the groups. Table 1 presents participant characteristics.
Patients with HbSS+/HbSβ° had more acute chest syndrome, stroke, avascular necrosis, and more of these patients were on treatment with hydroxyurea or on chronic transfusion therapy. Patients with SCD HbSC/HbSβ+ had a higher median BMI percentile (71%) verses patients with SCD HbSS/HbSβ°, who had a BMI percentile of 48.5%. There was a statistically significant differences in the hemoglobin levels and absolute reticulocyte count among the genotype groups. Patients with SCD HbSC/ HbSβ+ had a median hemoglobin level of 10.9 g/dL and absolute reticulocyte count of 146, versus patients with SCD HbSS/HbSβ° who had a median hemoglobin level of 8.5 g/dL and absolute reticulocyte count of 306.