This is the only specific treatment available for hyperhemolytic episodes where only the liver is involved. Other support measures, including fluids, electrolytes, vitamin K, and antibiotics, should be provided based on observed abnormalities. Many advocate splenectomy in children over five with one episode of sequestration because of a very high recurrence rate. Others will delay splenectomy until the second episode. Pneumococcal vaccine and meningococcal vaccine are indicated before surgery.
Prophylactic penicillin is indicated after surgery.
Individuals that do not have their spleens removed should be maintained on chronic transfusions for a presently unknown period of time. These are continued until the child is two. They are then stopped and splenectomy performed for recurrences after acute management. Recurrences on transfusion or alloimmunization should prompt splenectomy in most cases. This area has been controversial because of the desire to maximize immune function and avoid surgery without exposing the child to undue risk of death from recurrence and complications of splenic sequestration.