Sickle cell disease, also referred to as sickle cell anemia, is a genetic disorder of the hemoglobin protein in blood. It gets its name from the shape red cells take on when they are in a low-oxygen state, which resembles that of a sickle. Sickle cell anemia affects roughly 70,000 people in the United States alone. However, the disease primarily affects African Americans, with one in every 500 being afflicted. It is important to do our part to help those suffering, with one of the few proven treatments: blood transfusion.
According to Dr. William Crews, medical director of laboratory services at Carter BloodCare, sickle cell anemia causes hemoglobin to malfunction in low-oxygen states, such as when blood goes through capillaries. When this happens, the cells are prone to clumping together, making it harder for them to pass through and bring oxygen to the body. Hemoglobin S, the type of hemoglobin found in people with sickle cell anemia, also shortens the lives of red blood cells, which is why most people with the disease are also anemic. For comparison, normal red blood cells last for approximately 120 days, while those affected with sickle cell anemia live for only 30 to 60 days.
The most common symptom of sickle cell disease is pain, primarily in the joints. When the pain occurs, it is known as a sickle cell crisis and requires the person suffering to receive IV fluids and pain medication. Another common symptom is what is known as acute chest syndrome, which happens when the pain is localized in the chest, making it difficult for people to breathe. Splenic infarct, a condition that causes tissue death in the spleen due to a shortage of oxygen, is another prevalent side effect of sickle cell anemia. The most serious complication of the disease is a stroke, which commonly occurs during childhood.
When serious complications occur, the best way to treat people with sickle cell anemia is what is called an exchange transfusion. Exchange transfusions are performed by connecting the patient to an apheresis machine that removes a patient’s sickle-cell blood and replaces it with donated, healthy blood. The apheresis process essentially “cleans” out the sickle-cell blood; however, there is still a gene present that creates the sickle cells. When an exchange transfusion is fulfilled, the goal is that less than 30 percent of the blood is irregular.
It is only through the blood given by volunteer donors that those with the disease are able to live a normal life. Traditionally, an exchange transfusion requires a minimum of four units of blood, making it that much more important for blood to be available. Although there are small pockets of people in the Mediterranean who suffer from sickle cell anemia, the vast majority of those affected are in the African American community.
“It is important for us to get African-American donors to help those with sickle cell anemia,” Dr. Crews says. “At Carter BloodCare, we try to go out and educate potential donors. We schedule meetings with church groups, community service groups, and other things like that. We want to spread awareness to others.” In addition, Carter BloodCare does their best to match-up blood antigens from donors to see if they are able to be matched with sickle-cell patients in the community. “We do this with donors who identify as African American on their donor record. If they are a match, we let them know. We actually send them a letter to let them know they could be a huge help.”
Sickle cell anemia is a debilitating condition that those afflicted cannot control. To those with it, blood transfusions can make the difference between living in constant pain and being able to live a normal life. “If you aren’t a regular donor, consider taking the time to give blood,” says Dr. Crews. “It is a constant struggle for us to provide these units for hospitals and the patients who need it. It is such a big help to the community, especially to those living with sickle cell disease, when African-American donors give regularly.”
Whether it is for patients living with sickle cell disease or you’re looking to do something helpful for your community, it is always the right time to give life.