Published: February 18, 2021 | Updated: April 5, 2021
Each year, about 1,000 babies are born in the U.S. with sickle cell disease (SCD), one of the most common genetic disorders. Curative options for SCD include bone marrow transplantation and the emerging gene therapies that are available currently mostly in research settings. Red cell exchange is a specialized form of sickle cell treatment that accomplishes removal of the patient’s sickled red cells with normal cells without significantly raising the total hemoglobin. This minimizes iron overload, which would be the case with simple transfusions where healthy red cells are simply transfused to the patient. Patients can get significant iron overload in a year’s time on simple transfusion and can require chelation therapy to get rid of the excess iron, which can burdensome to the patients and families.
Healthy red blood cells are round, and look somewhat like doughnuts in shape. SCD causes red blood cells to be shaped like a sickle, which looks like a crescent-shaped moon. The sickle-shaped red blood cells are hard and sticky, and can deprive the body’s tissues of oxygen or prevent oxygen from reaching tissues. This lack of oxygen can cause damage to cells and the body’s organs and cause symptoms such as pain, anemia, stroke or kidney failure.
Red blood cell exchange helps people with SCD by removing the sickled red blood cells and replacing them with healthy red blood cells from donor blood. Red blood cell exchange is typically done on a monthly basis to help prevent complications of SCD. The treatment also can be used when someone experiences a sickle cell crisis. A sickle cell crisis happens when the sickle cells clump together and block blood vessels, causing severe pain. The pain commonly happens in the spine, arms, legs, chest or abdomen.
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The treatment occurs by a patient’s blood being removed through a needle or catheter. The blood is then cycled through a machine which separates the blood by cell type: red cells, white cells, platelets and plasma. The red cells are sifted, and healthy red blood cells, taken from donor blood, are circulated into the patient’s other blood products (white cells, platelets, plasma).
The blood cell separator removes less than 8 ounces of blood at any one time. Patients may feel a little dizzy or lightheaded during the process, but patients are monitored throughout the exchange. The therapy can last from two to three hours, but can be shorter or longer depending on a patient’s unique situation.
Norton Children’s Cancer Institute, affiliated with the UofL School of Medicine, is the largest and only pediatric health care system in Kentucky offering red blood cell exchange for sickle cell treatment. We offer a lifespan approach to sickle cell care, helping children with sickle cell disease transition to adult care by offering education and an individualized care plan that helps young adults feel confident to own their health care.