Sickle Cell Anemia (Disease and Trait)

Anesthesia Implications

Anesthesia Implications

Avoid triggers – factors that would cause a sickle cell crisis: dehydration, hypoxemia, hypothermia, hyperthermia, or acidosis.

CBC and Type/Cross – Preoperatively monitor the CBC and obtain type and cross. Monitor the hemodynamic state carefully and provide adequate hydration. Give transfusion of cross-matched red blood cells to replace the surgical losses. Hemoglobin should be no less than 10-11 g/dL. Preoperative transfusions are debatable as the infusion may increase blood viscosity and thereby lead to end-organ damage.

Keep SaO2 high – Sickling of hemoglobin cells happens in sickle cell trait and sickle cell disease when PO2 levels reach 20-30 or 30-40 mmHg respectively.

Pain control – Pain related to the condition may be present. This typically manifests in the back, chest, or joints and can range from mild to severe. This is also the primary reason these patients present to the emergency room. This is due to sickled cells obstructing of one or more vessels. Keep pain under tight control – and avoid any of the sickle-cell triggers: dehydration, hypoxemia, hypothermia, hyperthermia, or acidosis

Sickledex – If family history is suggestive of sickle cell anemia that has not yet been diagnosed, a test called the Sickledex may be obtained. The patient must be greater than 6 months of age to take this test.

Pathophysiology

Autosomal recessive genetic disease of the beta-globin gene. Sickle cell disease differs from sickle cell trait.

Sickle cell trait is a heterozygous disorder which is far more common than sickle cell disease (10% in African Americans versus 1% of African Americans respectively). With sickle cell trait, only 30-50% of the hemoglobin exhibits type S hemoglobin whereas the majority of the hemoglobin molecules are hemoglobin type S with sickle cell disease.

Essentially, with sickle cell disease, sickling happens more readily and widespread than it will with sickle cell trait.

Sickle cell crisis is characterized by hemolytic anemia, vasoocclusion, severe pain, and organ damage.

African-American patients presenting for a cholecystectomy may have undiagnosed sickle cell disease as cholelithiasis is a common complication arising from chronic hemolysis.

According to Nagelhout, patients presenting to ambulatory surgical centers should meet the following criteria:
There should be no major organ damage as a result of sickle cell anemia
There should not have been a sickle cell crisis for at least a year
The patient should be compliant with the prescribed medical regimen
When the patient is discharged, they should reside no further than 15 minutes travel to a medical facility that can treat the patient
The patient should have close follow-up care after the surgery

References

Nagelhout. Nurse anesthesia. 5th edition. 2014.