Pathophysiology and physical activity in patients with sickle cell anemia

Sickle cell anemia (SCA) is an inherited blood disorder caused by a single mutation on the β-globin gene leading to the synthesis of an abnormal hemoglobin S (HbS). Under deoxygenation, HbS may polymerize leading to red blood cell (RBC) sickling, hemolysis, vaso-occlusion and ultimately chronic organ damage. The metabolic changes occurring during a physical exercise may initiate sickling and painful vaso-occlusion. Although, few studies suggested that exercise could have beneficial effects in SCA patients, actually there is no accurate recommendation for physicians to prescribe an exercise program in this population. SCA patients are usually encouraged to exercise on a symptom-limited basis. This review discusses the basic theoretical principles that could be used for exercise training in patients with SCA