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  • Sickle cell disease (SCD) causes a chronic hemolytic anemia associated with acute and chronic vaso-occlusion.

  • Baseline hemodynamic and laboratory values in patients with SCD can be confused with sepsis.

  • Serum creatinine levels of 1 to 1.5 mg/dL often indicate significant renal dysfunction.

  • The most common intensive care management problems in patients with SCD include the acute chest syndrome (ACS), very severe anemia, sepsis, stroke, priapism, splenic sequestration, or right heart failure associated with ACS and/or acute severe hemolytic anemia.

  • The ACS is a form of acute lung injury that occurs in 10% to 20% of patients hospitalized in vaso-occlusive pain crisis, often caused by fat embolization syndrome or pneumonia.

  • Secondary pulmonary hypertension, defined by right heart catheterization and often unrecognized, occurs in 10% of adult patients with SCD and worsens during sepsis, vaso-occlusion, and other medical stress.

  • Red cell transfusion is an important treatment for most patients with SCD requiring intensive care management.

  • Rapid exchange transfusion is indicated for central nervous system events, serious respiratory disease, or multiorgan failure.

  • Transfusion management in patients with SCD requires investigation of alloimmunization history.

  • Preoperative red cell transfusion and detailed supportive care are advisable for significant surgery in patients with SCD.


Sickle cell disease (SCD) is highly prevalent in the United States, affecting 1 in 500 African American infants. It is common in individuals of African, Caribbean, Mediterranean, Middle Eastern, and Asian Indian descent. It is a genetic disorder with an autosomal recessive inheritance pattern. SCD is often called “the first molecular disease” because the biochemical alteration in sickle hemoglobin described by Linus Pauling in 1948 was one of the first lesions identified at the molecular level for a human disease. Sickle hemoglobin forms rod-like polymers in deoxygenated red cells in areas of the circulation with low oxygen tension, acidosis, or hyperosmolarity. Sickle hemoglobin polymerization causes a host of secondary molecular and cellular changes, many of which impair blood flow and contribute to tissue damage. The microcirculation can be acutely or chronically impaired in virtually any organ in the body, resulting in the characteristic crisis pattern of intermittent pain and acute organ injury superimposed on the gradual development of chronic organ failure.

Despite the early progress in a molecular understanding of SCD, its treatment remained largely palliative for many decades. In recent years, the longevity of patients with SCD has been prolonged by the institution of prophylactic penicillin treatment and immunization to decrease mortality rate from pneumococcal sepsis. Chronic transfusion therapy for selected patients has improved outcome, and acute transfusion therapy is the central intervention for most complications requiring admission to the intensive care unit (ICU). Hydroxyurea, the first prophylactic treatment approved by the Food and Drug Administration for SCD, decreases disease severity and mortality rate.1,2 Three new agents have been approved in recent years, each providing prophylactic benefit through antioxidant, antiadhesive, and antipolymerization mechanisms. It is now well ...

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