Sickle cell anemia

An approach through nutritional support

Abstract

Sickle cell anemia (SCD) is the most frequent form of structural hemoglobinopathy, of an autosomal recessive nature, caused by a mutation in the Hemoglobin Beta Subunit (HBB) gene encoding for the beta globin chain (?-globin), as a product of a substitution in the chain of a glutamic acid by valine in the sixth position, called hemoglobin-S (HbS). It tends to occur more frequently among people whose lineage can be linked to Sub-Saharan Africa, the Middle East, the Mediterranean, India, the Caribbean, Central and South America. As part of its pathophysiology, erythrocytes are seen in the shape of a sickle, generating a polymerization of hemoglobin molecules, leading to stifness and instability of erythrocytes, which produces acute and chronic multisystemic manifestations; consequently, the susceptibility to infections, appearance of chronic anemia, hemolysis, episodes of vascular occlusion and painful crises are increased. Currently, there is no cure available for the pathology. However, it has been shown that adequate multidisciplinary management can contribute to improve the symptoms, reducing the exacerbation of the disease and the quality of life of these patients. This review aims to emphasize the importance of nutritional support in the treatment of sickle cell anemia.  In addition to standard treatment, patients can receive better healthcare, decrease the exacerbation of the disease, and enjoy a better quality of life by food and dietary supplementation.