Key concepts

0 Sickle cell disease (SCD) is an inherited disorder caused by a defect in the gene for hemoglobin. Patients may have one defective gene (sickle cell trait [SCT]) or two defective genes (SCD).

01 Although most often seen in persons of African ancestry, other ethnic groups can be affected.

SCD involves multiple organ systems.

Prophylaxis against pneumococcal infection reduces death during childhood.

Hydroxyurea has been shown to decrease the incidence of painful crises. However, the patient population that receives hydroxyurea should be carefully monitored.

® Chronic transfusion therapy programs have been shown to be beneficial in decreasing the occurrence of stroke in children with SCD.

Patients with fever greater than 38.5°C (101.3°F) should be evaluated and appropriate antibiotics should include coverage for encapsulated organisms, especially pneumococcal.

® Pain episodes can usually be managed at home. Hospitalized patients usually require parenteral analgesics. Analgesic options include opioids, nonsteroidal anti-inflammatory agents, and acetaminophen. The patient characteristics and the severity of the crisis should determine the choice of agent and regimen.

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