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Understanding Sickle Cell Disease
Gaye Riddick-Burden, CRNP, BC, is a nurse practitioner at Thomas Jefferson University Hospital in Philadelphia, and is responsible for the day-to-day operations of the Sickle Cell Day Unit. Samir K. Ballas, MD, is professor of medicine and pediatrics at Thomas Jefferson University, director of the Sickle Cell Center at Jefferson and director of the Sickle Cell Program of the Commonwealth of Pennsylvania for the Philadelphia region. Here, they discuss the pathophysiology of sickle cell disease, its diagnosis, management, complications, therapies, and prognosis. This lecture and the post-test is worth 1.5 credit hours. The authors note that sickle cell anemia is one of the most common inherited blood disorders in the US, affecting one in every 600 live births in the African American community. They define sickle cell disease (SC) as one of a group of autosomal recessive disorders that affect the hemoglobin and red blood cell structure and function. These autosomal recessive disorders include sickle cell anemia, hemoglobin SC disease, and sickle beta-thalassemia. The lecture continues with a definition of hemoglobin, its various types – A, S, and F – and of the process of vaso-occlusion in which sickle cells become lodged in the micro- and macrovascular system. Vaso-occlusion causes the majority of clinical manifestations of sickle cell disease. An early manifestation is dactylitis, or hand-foot syndrome. Objective signs which affect some 50% of patients, include fever, leukocytosis, joint effusion, tenderness, hypertension, tachypnea, tachycardia, nausea, and vomiting in sickle cell disease. A further clinical manifestation is pain, the management of which, say the authors, is often inadequate due to health care providers’ unwillingness to provide enough opioid. The most common nonopioids in sickle cell disease are acetaminophen and nonsteroidal anti-inflammatory (NSAIDS) drugs. Manifestations of an acute chest syndrome, the second commonest cause for hospitalization after acute episodes of pain, are managed with antibiotics. The authors discuss life-threatening complications including splenic sequestration, an enlargement of the spleen due to red blood cells becoming entrapped there, as well as stroke and cerebral hemorrhage … the latter being the subject of the Stroke Prevention Trial in Sickle Cell Anemia (STOP). The lecture concludes with a discussion of transfusion therapy in sickle cell disease and its indications and complications, and notes that bone marrow transplant in sickle cell disease is the only curative treatment. |
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