Sickle Cell Disease in the Emergency Department: Complications and Management

Abstract

Sickle cell disease is the most common blood disorder in the United States, affecting 100 000 people. A genetic mutation creates hemoglobin S. In the deoxygenated state, hemoglobin S polymerizes, creating sickled hemoglobin. Sickled hemoglobin causes a cascade of complex pathophysiologic events that lead to hemolysis, chronic anemia and endothelial damage. This results in clinical complications, end organ dysfunction and a shortened life expectancy. The acute nature of many sickle cell complications makes the emergency department a common setting where sickle cell patients present. Common complications (vaso-occlusive episode, fever, acute chest syndrome, stroke) and less common complications (splenic sequestration, priapism, aplastic crisis, ocular emergencies) will be discussed. Public health implications will be discussed briefly.