Reduce Your Risk > Non-Controllable Risk Factors > Sickle Cell Disesase
Sickle cell disease (SCD) is the most common inherited disease among African Americans. In the United States, sickle cell anemia affects about 72,000 people. Most people affected are African Americans. The disease occurs in about:
* One in every 600 African-American births
* One in every 1,000–1,400 Hispanic-American births.
As the parent of a child with SCD, you may know that SCD can lead to pain, anemia, serious infections and damage to major organs. But you may not know that nearly 11 percent of children born with the most severe form of sickle cell disease will have a stroke by age 20.1
How does sickle cell disease relate to stroke?
What tests can show if my child is at risk for stroke?
What treatments prevent recurring strokes?
How can I help reduce my child’s risk for stroke?
Where can I get more information?
How does sickle cell disease relate to stroke?
Sickle cell disease is a red blood cell disorder. Hemoglobin is the protein in red blood cells that carries oxygen. It underlies the problem in sickle cell disease.
Normal blood cells are round like doughnuts and flexible. They squeeze through small blood vessels to deliver oxygen to the body’s tissues. The sickled hemoglobin sticks together to form long rods in the red cell when it gives away oxygen. These rigid rods can change the red cell from round and flexible to a shape like a sickle (farm tool used to cut wheat). The sickled cells tend to stick to and damage the inner wall of blood vessels. They also clog the flow of the blood. In the brain, this can cause a stroke.
Children with SCD are 200 to 400 times more likely to suffer a stroke, compared to children without SCD. The Cooperative Study of Sickle Cell Disease showed that stroke is a constant threat after age 2, but the incidence is highest in the middle of a child’s first 10 years of life.
What tests can show if my child is at risk for stroke?
Children over age 2 who have sickle cell anemia should be screened for stroke using an ultrasound test called Transcranial Doppler. TCD is a device that uses painless and harmless ultrasound (sound waves) to find areas of abnormal blood flow in the brain’s blood vessels. When sickle cell damage narrows the blood vessels, the blood flows faster through the narrowed area and makes a louder noise. This means that a stroke is more likely.
A large national study called the Stroke Prevention Trial in Sickle Cell Anemia (STOP) performed in the 1990s, showed that children with abnormal TCD who receive regular (about monthly) blood transfusions have a 90 percent lower risk of stroke. So TCD is an important test. Combined with blood transfusion, it can prevent disabling stroke. TCD is recommended yearly during early childhood. But it may not be needed as often, depending on your child’s results. Your child’s provider also may order other tests, such as magnetic resonance or MR of the brain to better understand the risk of stroke or other problems.
If your child has sickle cell disease, ask your child’s doctor about using TCD and MR screening to test the brain for stroke. TCD is available at most major health centers.
What treatments prevent recurring strokes?
Up to two-thirds of children with SCD and stroke will have more strokes unless they’re treated.
Transfusions can prevent a first stroke when the TCD is abnormal, and they can generally prevent second and third strokes in patients with SCD who have already had a stroke.2 A transfusion will help replace the sickled red blood cells in your child’s body with normal donor cells.
When the first stroke occurs, most children get a transfusion to reduce the blocked blood flow caused by the sickle cells. After that, doctors suggest monthly transfusions due to the high risk of another stroke. Transfusions should continue for at least three to five years.3
After about two years of monthly blood transfusions, people will have too much iron in their bodies. Iron chelation (removal) therapy with deferoxamine (Desferal) helps keep the body healthy by allowing it to pass excess iron out through the urine.
Transfusions help prevent stroke and reduce other complications of SCD. But there are drawbacks. Talk to your doctor about the risks and benefits for your child. It’s best to talk to healthcare professionals experienced in transfusing people with sickle cell disease and managing iron overload.
How can I help reduce my child’s risk for stroke?
The best defense against a first or subsequent stroke in children who have SCD is to effectively manage their disease. Even if you can’t prevent the first stroke, you can help reduce your child’s risk of permanent damage from another stroke. Work with your doctor to develop a treatment plan that’s right for your child.
Where can I get more information?
Talk to your doctor. Learn all you can about managing your child’s SCD and find out your child’s risk for stroke. Share this information with everyone in your child’s network to get help quickly in an emergency.
For more information on SCD, contact the Sickle Cell Disease Association of America at 1-800-421-8423.
You can find information on the STOP study, use of TCD and sites trained to perform TCD for children with sickle cell disease at the Medical College of Georgia Web site.
The links on this page are provided for convenience only, and are not an endorsement or assurance of the entities or any products or services.
[1] Cooperative Study of Sickle Cell Disease.
[2] STOP Study. (NEJM 1998 reference and Medical College of Georgia Web site.)
[3] NIH Publication No. 95-2117, Revised December 1995 (3rd Edition) National Institutes of Health, National Heart, Lung, and Blood Institute