Therapeutic management of a 6-year-old child with hereditary spherocytosis (HS) should include which therapeutic intervention?
a. Perform a splenectomy.
b. Supplement the diet with calcium.
c. Institute a maintenance transfusion program.
d. Increase intake of iron-rich foods such as meat.
ANS: A
Splenectomy corrects the hemolysis that occurs in HS. The splenectomy is generally reserved for children older than age 5 years with symptomatic anemia. Supplementation with calcium does not affect the HS. Additional folic acid can prevent deficiency caused by the rapid cell turnover. A maintenance transfusion program suppresses red blood cell formation. At this time, the risks of transfusion are greater than those of a splenectomy. Iron supplementation does not influence the course of HS.
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