Sickle Cell Anemia Its Epidemiology, Pathophysiology, Nutraceuticals Role: A Review

Abstract

The individual with the "SS" genotype possesses a deviant beta globin gene, resulting in the manifestation of sickle cell anemia, an inherited pathological condition. The severe symptoms of sickle cell disease are a result of a specific genetic mutation in the gene responsible for encoding the human β-globin subunit. This mutation leads to the substitution of valine for β 6 glutamic acid. The replacement of sickle cell hemoglobin (HbS) causes a significant decrease in its solubility when it is deoxygenated. The advancements in targeted molecular treatments have been driven by the significant advancements in our understanding of the biology of sickle cell disease (SCD) and its various repercussions since its discovery in 1910. Sickle cell disease (SCD) is a condition where the flow and lifespan of red blood cells are impacted by a mutated form of hemoglobin called hemoglobin S. This mutation occurs when a single amino acid in the β-globin chain is replaced, causing the hemoglobin to form polymers. During the early phases of treating sickle cell anemia, patients are commonly prescribed hydroxyurea, folic acid, amino acid supplements, penicillin prophylaxis, antimalarial prophylaxis, and blood transfusions to stabilize their hemoglobin level. They face significant expenses and hazards. However, there is a positive development: the investigation of medicinal plants for their ability to prevent sickling has yielded significant financial rewards. Laboratory experiments have demonstrated that this alternative therapy involving nutraceuticals can effectively reverse the process of sickling and also decrease the occurrence of crises.