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Sickle Cell Disease And Other Hemoglobinopathies: Approaches To Emergency Diagnosis And Treatment

December 2001

Abstract

Sickle cell anemia (SCA) refers to a family of genetic disorders that results in the production of hemoglobin S. Thalassemia is a mutation that impairs the synthesis of hemoglobin but not its structure. Variants of both diseases exist, depending on the genotype. The most common hemoglobinopathies, in descending order of frequency, are sickle cell disease (SCD), hemoglobin S/C disease, sickle-beta+thalassemia and sicklebeta0thalassemia.1

While there are over 350 types of abnormal hemoglobins,2 the most clinically important of these disorders is SCD. In the United States, one of every 600 people of African descent has SCD.3

The costs of caring for patients with SCD are significant. There were 75,000 hospitalizations per year between 1989 and 1993 for patients with SCD, representing an annual cost of $470 million.4 Patients with SCD are frequent users of the ED, as well. In one study of patients who came to the ED five or more times in a 12-month period, those with SCD had the highest likelihood of return visits of any group with chronic conditions—even more than those with renal failure or COPD.5

As a result of improved treatment, most patients with SCD survive long into adulthood, but they still encounter a lifetime of complications, including chronic hemolytic anemia, recurring bouts of pain, and other sequelae of vascular occlusion.1 Children still die from the complications of SCD—mostly from infection, complications of acute splenic sequestration, or acute chest syndrome (ACS). Patients who present to the ED with SCD offer several challenges to the treating physician. First and foremost is offering sufficient treatment for the patient's pain. Equally important, though, is determining the cause of the patient's pain. A diagnosis of SCD does not rule out other conditions; moreover, SCD predisposes its victims to certain complications that demand prompt and effective treatment. Finally, patient disposition must be handled carefully on a case-by-case basis. Despite the many challenges inherent in treating SCD patients, one thing is certain: As ED crowding increases, emergency physicians will play an ever-expanding role in the care of SCD patients.

This issue of Emergency Medicine Practice explains the basic terminology, pathophysiology, natural progression, and expected complications of SCA. Appropriately, the article emphasizes the ED evaluation and management of SCD.
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