Critical Care Management of Adult Patients With Sickle Cell Disease: A Contemporary Review

Abstract Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting approximately 100,000 individuals in the United States and 300,000 newborns globally each year. Despite improvements in preventive care and increased life expectancy, individuals with SCD continue to experience a lifespan approximately 20 years shorter than the general population, with a substantial proportion of deaths occurring in the intensive care unit (ICU). The leading indications for ICU admission are acute chest syndrome (ACS) and severe vaso-occlusive crisis (VOC), followed by sepsis, stroke, fat embolism syndrome, multisystem organ failure (MSOF), renal failure, and hepatobiliary emergencies. Reported ICU mortality rates range from 3% to 13%, with adverse outcomes observed in up to 20% of admissions. Independent predictors of complicated ICU courses include acute kidney injury, tachypnea, low mean arterial pressure, and deviation of hemoglobin from baseline. Red blood cell transfusion, either simple or exchange, remains central to ICU management, although it carries risks such as alloimmunization and delayed hemolytic transfusion reactions. This review synthesizes ICU-specific epidemiologic data, the 2025 French critical care guidelines, and emerging evidence on fluid resuscitation, transfusion strategies, pulmonary hypertension, venous thromboembolism, advanced pain management, and perioperative optimization to provide a practical framework for intensivists managing critically ill adults with SCD.