Kristen Burns, social worker for UVA’s Comprehensive Pediatric Sickle Cell Program, contributed this post.
June 19 is World Sickle Cell Day. The National Institutes of Health reports that approximately 80,000 Americans have this disease, and one in 500 African-Americans are born with it.
Every baby in Virginia is tested for sickle cell anemia. It’s important to test early for sickle cell disease in order to initiate treatment that may prevent significant complications of this disease.
What Is Sickle Cell Anemia?
Sickle cell anemia is a genetic disorder that affects red blood cells in the body. It’s caused by defective hemoglobin, which creates a change in the shape of red blood cells.
Healthy red blood cells are round and disc shaped. This shape allows them to move easily and carry oxygen to different parts of the body. People with sickle cell anemia have red blood cells that become sickled, or shaped like a crescent moon.
The red blood cells also become sticky. These changes cause the red blood cells to get stuck together and block blood from flowing properly throughout some parts of the body.
These blockages cause what is known as a pain crisis, which is extremely painful. Pain crises are the most common complication of sickle cell anemia. There are some things that can help a person with sickle cell avoid a pain crisis:
- Drinking water
- Staying warm
- Resting when tired
Pain crises are treated with aggressive oral, and occasionally intravenous, pain medications.
Where Did Sickle Cell Anemia Come From?
There is a connection between sickle cell anemia and malaria, which is a serious and sometimes deadly disease that is most common in tropical and sub-tropical regions, including parts of Africa, South and Central America. People who carry a sickle cell gene are more likely to survive an acute malaria infection. If two people have a sickle cell gene, they have a 25 percent chance of producing a child with sickle cell anemia.
You can determine your “sickle status” by having your doctor perform a simple blood test called a hemoglobin electrophoresis.
Is It Curable?
Currently, the only cure for sickle cell anemia is bone marrow transplantation. This procedure is only available to those patients whose disease is very difficult to manage and who have a sibling with matched bone marrow.
However, children with sickle cell anemia receive comprehensive health care to help prevent potential complications of this disease. Comprehensive sickle cell centers are the foundation to promote living fully with sickle cell disease by providing management of complications, health maintenance, education and social support.
Comprehensive Sickle Cell Care for Kids in Charlottesville
At UVA, pediatric patients can meet monthly with a team that includes:
- A doctor
- A nurse practitioner
- A social worker
- An education consultant
Our blood disorders team also sees patients at any time for management of expected complications of sickle cell anemia. We serve patients in Charlottesville and surrounding areas, such as Harrisonburg and Lynchburg.
Support for Sickle Cell Patients
Every year, we offer a chance for our patients and their friends and families to come together with health care providers to learn more about sickle cell anemia and support each other. On June 11, we’ll hold our ninth annual Sickle Cell Family Education Day at Triple C Camp.
- A guest speaker who will provide education about the process of transitioning to an adult sickle cell program and the importance of comprehensive adult sickle cell care
- Activities to promote team building, personal confidence and achievement and self esteem building.
Learn more about this disease from the Sickle Cell Information Center.
Has sickle cell anemia touched your life? Leave a comment below to share your story.