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The therapeutic management of children with SCA focuses on preventing vaso-occlusive episodes and infection as well as other complications. Functional asplenia (decrease in the ability of the spleen to function appropriately) places the child at significant risk for serious infection with Streptococcus pneumoniae or other encapsulated organisms. Prophylactic antibiotics in the young child and appropriate immunization in all children with SCA can reduce the risk of serious infection (Field & Vichinsky, 2020). Treatment of vaso-occlusive episodes focuses on pain control. Oxygen administration is necessary during episodes of crisis to prevent additional cell sickling. Adequate hydration with intravenous fluids is critical. Close monitoring of Hgb, Hct, and reticulocytes determines the point at which transfusion of PRBCs becomes necessary. Electrolyte analysis is also necessary to ensure that appropriate amounts of electrolytes are present in the serum. When RBCs are administered, there is the potential for hemolysis of the cells, thus increasing the potassium level in the serum. Antibiotic therapy is necessary when infection is present. Box 46.3 describes additional medical treatments that are needed in some children.
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