Treatment of the Chronically Transfused Patient (4)

Hypersplenism/Splenectomy
Meticulous patient records including units and cc's of blood transfusions, and hemoglobin values are maintained to allow for calculation of blood requirements every six months. Splenectomy is recommended in most cases when red cell requirements exceed 200 cc/Kg/year if the child is older than five years. If the child is under five years the risk of infection is balanced against the degree of hypersplenism. Certain patient's families' beliefs find removal of organs unacceptable. The relentless forcing of splenectomy may lead to families removing patients from comprehensive care. Therefore, splenectomy must be approached with extreme cultural sensitivity. In severe hypersplenism in the young child, partial splenectomies have been performed to preserve splenic function while decreasing hypersplenism. We recently reported our successful experience with this in selected patients. Following splenectomy, a detailed plan for the management of fever is implemented, including plans for transportation to the hospital once fever is noted.

Infection
In addition to the infections associated with hepatitis described below, some patient populations can be at particular risk for tuberculosis and HIV infection, depending on living situations and socioeconomic level. Often urban immigrant thalassemia patients and their families have an increased risk of communicable diseases such as tuberculosis. These patients require extensive family education, screening and treatment. <next>

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