Sickle Cell Disease

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that can cause complications throughout the lifespan, most notably pain crises, cerebrovascular accidents, and pulmonary complications such as acute chest syndrome. SCD treatment has long been characterized by chronic transfusions and hydroxyurea, but there are several new emerging therapies that seek to prevent such complications. In this chapter, we describe pathophysiology, manifestations, and diagnosis of SCD as well as management of the disease and common complications.