Surgery in a patient with sickle cell disease may precipitate a serious complication. Careful perioperative management can minimize the risk to the patient and speed post-operative recovery,


Patients at risk:

(1) sickle cell anemia (hemoglobin SS)

(2) sickle cell-hemoglobin C (hemoglobin SC)

(3) sickle cell-beta thalassemia (hemoglobin S-beta thal)


Perioperative management is most important for:

(1) Surgery under general anesthesia, especially emergency surgery

(2) Eye surgery.



(1) The patient should be admitted 12-24 hours prior to surgery for hydration with intravenous fluids.

(2) Hydration should be maintained during and following surgery.


Blood Transfusion:

(1) For surgery other than minor procedures done under local anesthesia: The patient should be transfused to increase the hematocrit to 30% prior to surgery.

(2) Replace blood loss during and following surgery.


Pulmonary Function:

(1) Perform pulmonary function testing prior to surgery. Administer a bronchodilator if there is evidence of bronchospasm.

(2) Perform incentive spirometry postoperatively.



(1) Monitor oxygen therapy during the procedure with a pulse oximeter. Be careful to prevent intraoperative oxygen desaturation to minimize the risk of postoperative acute chest syndrome.

(2) Postoperatively monitor pulse oximetry until the patient is awake.

(3) Postoperative observation may need to be overnight or longer, depending on the extent of surgery. The postoperative acute chest syndrome has a peak occurrence 48 hours following surgery. Patients with inadequate preoperative preparation or emergency surgery may benefit most from increasing the postoperative observation period.



(1) Prevent hypothermia during and following surgery.

(2) Monitor for postoperative fever and treat probable cause aggressively.


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