Quick Reference: Sickle Cell Anemia
Sickle cell hemoglobinopathy is a single-gene recessive disorder that involves an abnormality of the hemoglobin (Hgb) molecule. This disorder causes the red cell to sickle under certain circumstances. A “sickled” cell cannot undergo normal respiratory functions and they interfere with normal blood circulation.
- Both the pheresis and the partial exchange transfusion are done in the operating room pre-op with the cell saver.
- Blood is withdrawn initially at a rate of 100 ml/min directly into the cell saver bowl. The blood must be anticoagulated as it is being withdrawn from the internal jugular vein. The plasma can be returned to the patient.
- Volume expanders (Albumin or Voluven) can be administered to replace blood volume removed. Packed RBCs can be added to the circuit to maintain hematocrit.
- Before bypass, Y the venous line so the patient’s blood (aprox. 2 liters) can be withdrawn and processed (PPP and PRP).
- Intra-operative cell saver is not advised in patients with SS unless the Hb-S is in a very low percentage.
- Maintain PO2 >300mmHg, SvO2 > 75% and SaO2 >97%.
- Prevent acidosis and keep blood flow rates high and keep pH >= 7.40.
- Normothermia is encouraged, but if hypothermia is needed, the temperature should not go below 30 degrees C.
- Cardioplegia should be given normothermic or tepid.
- Maintain arterial pressure >65mmHg.
