Care of the Critically Ill Pediatric Sickle Cell Patient

Sickle cell disease is the most common inherited disease in the United States. Through the effects of hemolysis and vaso-occlusion, it has the potential to incite critical illness involving multiple organ systems. Children with sickle cell disease are at risk of multiple types of shock resulting in a need for ICU care. Our youngest patients with sickle cell disease are at highest risk of infection due to lack of splenic function, and this can present with septic shock. Hypovolemic shock can occur secondary to severe acute anemia as seen with splenic sequestration or a delayed transfusion reaction. As one ages, the risk of cardiac dysfunction – diastolic and systolic dysfunction as well as pulmonary hypertension – can result in cardiogenic shock. In addition to shock, patients with sickle cell disease are at risk for respiratory failure from acute chest syndrome as well as acute neurologic deterioration from stroke. For these reasons, critical care physicians must be familiar with the unique management of sickle cell complications in order to provide the best possible care for this vulnerable population.