The objective of supportive therapy is to maintain sufficient hemoglobin levels to prevent bone marrow expansion and bony deformities and to provide sufficient RBCs to support growth and normal physical activity. Transfusions are the foundation of medical management, with a goal of maintaining the hemoglobin level above 9.5 g/dl, an aim that may require transfusions as often as every 3 weeks.
The advantages of this therapy include (1) improved physical and psychologic wellbeing because of the ability to participate in normal activities, (2) decreased cardiomegaly and hepatosplenomegaly, (3) fewer bone changes, (4) normal or near-normal growth and development until puberty, and (5) fewer infections.