Providers Weigh In: Exchange Transfusion Therapy is a Key Intervention in Managing Sickle Cell Disease
Thursday, September 08, 2016
Sally Campbell-Lee, MD
Sickle Cell Disease (SCD) is an inherited lifelong blood disorder that affects the hemoglobin found in red blood cells. When a person inherits one sickle cell gene and one normal gene, they are said to have the sickle cell trait.
"One in 12 African Americans is a carrier of the sickle cell trait. The sickle cell trait is not a disease, but having it means that they may pass it on to his or her children," says Dr. Sally Campbell-Lee, director of Transfusion Medicine at UI Health. "If both parents have the sickle cell trait, there is a 25% chance that their baby can be born with sickle cell disease." The red blood cells of individuals with sickle cell disease contain hemoglobin S, an abnormal type of hemoglobin. These cells become sickle-shaped and can block blood flow to tissues, causing severe pain, damage to the body's organs, and even death. Sickle cell pain crises can happen frequently or infrequently, from several times a year to every few years. They also can develop into more severe complications, such as acute chest syndrome. Exchange transfusion can be life-saving in these situations. During exchange transfusion, blood is drawn from the individual with SCD through a venous access device and placed into a machine that separates the blood's components. A calculated amount of red blood cells is removed from the patient and replaced with normal donor red blood cells. This exchange reduces the amount of sickle red cells and provides added oxygen-carrying capacity to blood. It may be done in an emergency for patients having complications, such as a stroke or acute chest syndrome. Exchange transfusions also are done on a monthly basis in an outpatient setting to help maintain a safe level of abnormal (sickled) cells and an adequate number of normal red cells to prevent anemia. "Used correctly, exchange transfusion can prevent strokes and certain complications of sickle cell disease. However, it remains an underutilized therapy due to the need for specialized equipment and trained nursing staff," says Campbell-Lee. "Our special exchange transfusion program at UI Health has reduced complications and improved the quality of life of many sickle cell disease patients by reducing frequent hospitalizations." The Sickle Cell Disease Management program at UI Health is led by Drs. Sally Campbell-Lee and Victor Gordeuk.