The Acute Chest Syndrome of Sickle Cell Disease

It has become a cliché to say that sickle cell anemia is the first “molecular disease.”1 The substitution of valine for glycine in β-globin has been known as the cause of sickle cell anemia since 1957.2 That this specific knowledge has not resulted in a rationally designed, specific cure remains a disappointment. What is not disappointing is that the study of sickle hemoglobin and sickle red cells has provided important information about the pathophysiology of the disease, and this information forms the basis for rationally designed clinical care.The polymerization of sickle hemoglobin is central to the disorder (Figure 1). . . .