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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.
  1. Both the pheresis and the partial exchange transfusion are done in the operating room pre-op with the cell saver.
  2. Blood is withdrawn initially at a rate of 100 ml/min directly into the cell saver bowl. The blood must be anticoagulated as it is being withdrawn from the internal jugular vein.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.
  3. Volume expanders (Albumin or Voluven) can be administered to replace blood volume removed.  Packed RBCs can be added to the circuit to maintain hematocrit.  
  4. Before bypass, Y the venous line so the patient’s blood (aprox. 2 liters) can be withdrawn and processed (PPP and PRP).
  5. Intra-operative cell saver is not advised in patients with SS unless the Hb-S is in a very low percentage.
  6. Maintain PO2 >300mmHg, SvO2 > 75% and SaO2 >97%.
  7. Prevent acidosis and keep blood flow rates high and keep pH >= 7.40.
  8. Normothermia is encouraged, but if hypothermia is needed, the temperature should not go below 30 degrees C.
  9. Cardioplegia should be given normothermic or tepid.
  10. Maintain arterial pressure >65mmHg.

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