What is the optimum hemoglobin level?
The Hb level is kept between 9-10 g/dL with transfusions given at intervals of 2-4 weeks. Lower hemoglobin levels may impair proper growth. Higher hemoglobin levels (>14 g/dL) may carry the risk of thrombotic complications and need more aggressive chelation therapy. In presence of Hb <5 g/dL or in presence of congestive heart failure, packed red blood cells (PRBC) amount is given in aliquots, 5 mL/kg at one sitting with diuretic use.
