Hereditary spherocytosis secondary prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2] Associate Editor(s)-in-Chief:

Overview

The administration of vaccines including pneumococcal, hemophilus influenzae, meningococcal and influenza should be given two to three weeks before splenectomy. Folic acid supplementation as well as oral penicillin is also suggested for postsplenectomy patients untill reaching adulthood.

The administration of vaccines (pneumococcal, haemophilus influenzae type b, meningococcal group C and influenza) is indicated 2 to 3 weeks before splenectomy.^[1]
Postsplenectomy patients should also be given 1mg of folic acid daily for preventing secondary folic acid deficiency and oral penicillin (penicillin V) for preventing secondary infection until reaching adulthood.
In general, once the diagnosis and baseline severity of hereditary spherocytosis in a child are established, it is not necessary to perform repeated blood tests unless there is an additional clinical indication (such as intercurrent infection and pallor, or an increase in jaundice).^[2]
Avoidance of precipitants of hemolysis (such as sulfa drugs or quinines) and certain foods (such as fava beans) may help prevent hemolysis.