RT Book, Section A1 Kato, Gregory J. A1 Gladwin, Mark T. A2 Schmidt, Gregory A. A2 Kress, John P. A2 Douglas, Ivor S. SR Print(0) ID 1201808646 T1 Sickle Cell Disease T2 Hall, Schmidt and Wood’s Principles of Critical Care, 5th Edition YR 2023 FD 2023 PB McGraw Hill PP New York, NY SN 9781264264353 LK accesssurgery.mhmedical.com/content.aspx?aid=1201808646 RD 2024/10/09 AB KEY POINTSSickle 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.