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Monthly Blood Transfusions Can Prevent “Silent” Strokes In Children With Sickle Cell Anemia

In children with sickle cell anemia, some red blood cells are malformed, shaped like a crescent instead of a disc. This makes them more likely to clump together, causing organ damage, strokes, and pain.
National Institutes of Health
In children with sickle cell anemia, some red blood cells are malformed, shaped like a crescent instead of a disc. This makes them more likely to clump together, causing organ damage, strokes, and pain.

An international study initiated by Washington University has found that giving monthly blood transfusions to children with sickle cell anemia can significantly reduce their risk of what are known as “silent” strokes.

In children with sickle cell anemia, some red blood cells are malformed, shaped like a crescent instead of a disc. This makes them more likely to clump together, causing organ damage, strokes, and pain.
Credit National Institutes of Health
In children with sickle cell anemia, some red blood cells are malformed, shaped like a crescent instead of a disc. This makes them more likely to clump together, causing organ damage, strokes, and pain.

Unlike regular strokes, which have sudden, overt symptoms like difficulty speaking or numbness in an arm or leg, silent strokes can only be detected with an MRI scan, so they generally go unnoticed by parents and physicians.

But according to study co-author Michael Noetzel, a pediatric neurologist at Washington University, silent strokes can have serious consequences for a child’s health and development.

Not only do they make children more susceptible to full-blown strokes, they damage the parts of the brain involved in learning, making it harder for children to keep up with their peers in school.

“Our recommendation is that every child with sickle cell disease ― by at least school age ― have an MRI scan, to look for what is now a treatable condition,” Noetzel said.

Almost 1 in 3 children with sickle cell anemia experience silent strokes.

They can also suffer from infections, delayed growth and episodes of severe pain that can last for weeks.

Noetzel said the children in the study who got monthly blood transfusions not only had fewer strokes, they also had fewer “pain crises” requiring hospitalization.

“So there was also a secondary but very important impact on their overall level of disability, the way sickle cell affects not only the brain but the entire body,” Noetzel said.

Monthly transfusions are already the standard treatment for children with sickle cell anemia who experience overt strokes, also called infarcts.

“Our study was the first and the only one that’s demonstrated that transfusions are also beneficial if you only have a silent infarct,” Noetzel said.

Frequent blood transfusions also carry some risks, including infections, allergic reactions and the buildup of too much iron in the bloodstream, which can damage the heart and liver.

They can also be burdensome, requiring caregivers to miss work and sometimes travel long distances to take children to a hospital or clinic.

Noetzel said in the case of this research, private insurance, or more often, Medicaid, covered the cost of the brain scans and transfusions.

Sickle cell anemia is an inherited condition that in the U.S. mostly affects African Americans. There is no cure.

The current study is published in The New England Journal of Medicine.

Follow Véronique LaCapra on Twitter: @KWMUScience

Copyright 2021 St. Louis Public Radio. To see more, visit St. Louis Public Radio.

Véronique LaCapra first caught the radio bug while writing commentaries for NPR affiliate WAMU in Washington, D.C. After producing her first audio pieces at the Duke Center for Documentary Studies in N.C., she was hooked! She has done ecological research in the Brazilian Pantanal; regulated pesticides for the Environmental Protection Agency in Arlington, Va.; been a freelance writer and volunteer in South Africa; and contributed radio features to the Voice of America in Washington, D.C. She earned a Ph.D. in ecosystem ecology from the University of California in Santa Barbara, and a B.A. in environmental policy and biology from Cornell. LaCapra grew up in Cambridge, Mass., and in her mother’s home town of Auxerre, France.
Véronique LaCapra
Science reporter Véronique LaCapra first caught the radio bug writing commentaries for NPR affiliate WAMU in Washington, D.C. After producing her first audio documentaries at the Duke Center for Documentary Studies in N.C., she was hooked! She has done ecological research in the Brazilian Pantanal; regulated pesticides for the Environmental Protection Agency in Arlington, Va.; been a freelance writer and volunteer in South Africa; and contributed radio features to the Voice of America in Washington, D.C. She earned a Ph.D. in ecosystem ecology from the University of California in Santa Barbara, and a B.A. in environmental policy and biology from Cornell. LaCapra grew up in Cambridge, Mass., and in her mother’s home town of Auxerre, France. LeCapra reported for St. Louis Public Radio from 2010 to 2016.