Sickle Cell and Stroke in African-American Children
- Created on October 31st, 2008
- By Lisa Ramirez-Johnson
Like many six-year-olds, Ashton Taylor loved watching the Disney Channel, enjoyed drawing and showed a passion for ballet and tap dance. Her energetic disposition; however, masked a medical menace: the blood disorder sickle cell disease.
Ashton was diagnosed with the disorder at birth. Her young life was consumed with medical appointments, hospitalizations and daily medication regimens. Ashton had just completed kindergarten and was scheduled to perform in a dance recital the week she suffered a stroke.
On June 1, 2006, she complained of stomach pain. On June 2, she was admitted to the hospital. On June 6, she was transferred to another facility and given blood transfusions. The following day, Ashton appeared to be improving and even had enough energy to draw a green flower with a yellow stem. On June 8, she complained of pain and fever throughout the day. That evening she suffered a stroke, and on June 14, she died of complications from it.
“I didn’t know what was happening or why,” said her mother Stephanie Taylor of Walterboro, South Carolina. “I did not foresee a stroke happening to my daughter.”
The Taylor’s youngest daughter Peyton, 4, also has sickle cell disease. Her parents are concerned she may also have a stroke, and they are proactive advocates for her medical care.
The most common underlying risk factors for childhood stroke are sickle cell disease and congenital or acquired heart disease, according to a new American Heart Association/American Stroke statement — “Management of Stroke in Children” — published recently in Stroke: Journal of the American Heart Association.
Children with sickle cell disease are 200 to 400 times more likely to suffer a stroke, compared to children without the disease. In the United States, sickle cell disease is most commonly found in African Americans and occurs in about one in every 600 African-American births. The Cooperative Study of Sickle Cell Disease found that stroke is a constant threat after the age of 2, but the incidence is highest in the middle of a child’s first 10 years of life.
“We have known for a long time that children with sickle cell disease are at high risk of stroke,” said Robert J. Adams, M.S., M.D., co-author of the American Heart Association/American Stroke Association statement and Director of the Medical University of South Carolina Stroke Center. “What we did not know was how to predict who was going to have a stroke.”
Children over age 2 who have sickle cell disease should be screened for stroke using an ultrasound test called Transcranial Doppler, Adams said. 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.
“The TCD is a strong predictor of which children are at highest risk. This means we have a better idea of who will have stroke, and we can target them for prevention more effectively,” Adams said. TCD is available at most major health centers.
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 blood transfusions have a 90 percent lower risk of stroke. Combined with blood transfusion, it can prevent disabling stroke. Physicians also may order other tests, such as magnetic resonance, or MR, of the brain to better understand the risk of stroke or other problems.
Joy McKee, of Canal Winchester, Ohio, was told that her daughter, Mayah, had sickle cell disease shortly after the birth. On May 6, 2005, her five-year-old suffered a stroke. Mayah has made gains in recovery, does well in school and enjoys swimming, but has difficulty pushing the pedals on her bike and maintaining balance while riding.
McKee advises parents of children with sickle cell to be aware of their risk of stroke, follow the recommended treatment, and inquire about new treatments and trials.
“Parents need to educate themselves and know exactly what to do if a stroke happens,” McKee said. “The sooner they react, the sooner the child can get treatment to minimize the risk of permanent damage.”
More information about sickle cell disease, TCD, and transfusions are available through a downloadable document of the Association’s Power to End Stroke campaign, or by visiting strokeassociation.org/power.