How is sickle cell anemia treated

How is sickle cell anemia treated?

• Goal hemoglobin for patients with sickle cell anemia and the correction rate of the anemia are recommended to be no more than 10 to 10.5 g/dL and less than 1% to 2% per week, respectively. Higher hemoglobin levels and more rapid correction of anemia may precipitate a vasoocclusive crisis.

• Anemia correction may be achieved with blood transfusion or the use of erythropoietin stimulating agents (ESA). Blood transfusions provide a higher proportion of HbA compared with stimulating endogenous red blood cells with an ESA. Additionally, the use of ESA may be associated with increased vasoocclusive risk. ESA dosing may need to be higher in individuals receiving hydroxyurea due to the inherent bone marrow suppressive effect of the latter. However, it has also been suggested that the addition of ESA may allow administration of higher doses of hydroxyurea and improved fetal hemoglobin levels. Hydroxyurea is used in the treatment of SCD to increase the synthesis of fetal hemoglobin, which, unlike HbS, does not sickle.

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