Abstract
Sickle cell disease (SCD) is a genetic disorder resulting from the presence of mutated hemoglobin S (HbS). Homozygous carriers will present with early manifestations of painful vaso-occlusive crises. SCD patients have been reported to be severely deficient in vitamin D (<20 ng/mL). Four years (2010–2014) of individual de-identified Sickle Cell Clinic of Southern Louisiana (SCCSL-SCD) patient records were analyzed for vitamin D status and the level of crisis-related ER/hospital utilization. To determine the dietary, and behavioral mediators of SCD-crisis in our study population, a cohort of 102 SCCSL-SCD patients were administered a survey that evaluated sun exposure, dietary behaviors, and pain frequency and severity. Patients with circulating levels of 25(OH)D3 less than 14.1 ng/mL reported having more crisis-related hospital visits per year (10) than patients with 25(OH)D3 serum levels >34 ng/mL. The result of the dietary survey detailed a relationship between patients who reported to have “Almost Never” consumed fish or milk in their diets and more frequent hospital stays and ER visits than those who reported consuming these products on a daily basis. Those who consumed these foods in their diet several times a month also had fewer ER visits when compared to the “Almost Never” category.
Highlights
Sickle cell disease (SCD) is a genetic disorder that is caused by the improper insertion of the amino acid valine in place of glutamate at position 6 of the hemoglobin chain [1,2,3]
Hospitalized responses were positively associated with serum vitamin D levels (Table 1) (r = 0.224, p = 0.01)
There was no significant association between the self-reported prescription of vitamin D supplements and having serum vitamin D levels >20 ng/mL (22% sufficient vs. 13% sufficient, p = 0.334) (Table 2)
Summary
Sickle cell disease (SCD) is a genetic disorder that is caused by the improper insertion of the amino acid valine in place of glutamate at position 6 of the hemoglobin chain [1,2,3]. This seemingly small error results in significant hemoglobin instability, solubility changes, and morphological changes in the form of misshapen red blood cells that are incapable of normal oxygen exchange. HbS gene is present among approximately 8% of African-Americans; 1 out of 500 births will have homozygous HbSS/SCD. The heterozygous HbS carrier is asymptomatic and benefits from resistance to the parasite that causes malaria, the much rarer
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