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The two strokes occurred shortly after a single abnormal transcranial Doppler study, which uses ultrasound to measure blood flow through the brain, and before confirmatory tests could be performed.

Drs. Adams and Virgil C. McKie, Professor Emeritus of Pediatrics, identified the painless, relatively inexpensive transcranial Doppler as a way to identify children at risk in a New England Journal of Medicine article in 1992. Their work to identify and help at-risk children began after Dr. McKie came to Dr. Adams with concerns that some of his young patients with sickle cell disease were experiencing strokes.

End points for the STOP II study included a reversion to an abnormal transcranial Doppler or a stroke and almost half the children in the halted transfusion arm experienced an end point within 10 months, some in as few as two months, Dr. Adams says. He noted data on nine patients in the halted transfusion arm were censored because they either resumed transfusion or began taking Hydroxyurea, a drug to treat the pain crises that are a hallmark of sickle cell disease.

One child who had a stroke had his first abnormal Doppler about eight months after halting transfusions and a stroke 14 days later. The second stroke occurred in a child eight days after his first abnormal study.

"I think it's clear that some people are going to look at these findings and suggest if we do Doppler exams often enough, we can get children off transfusions," says Dr. Adams. "But children in STOP II had frequent Doppler exams, much more frequently than likely would be used in usual clinical practice, and there were still problems." Participants had Doppler exams at least every 12 weeks and more frequently when anything abnormal was found.

Of the 209 children enrolled in the two STOP studies, 20 had strokes and all those children had abnormalities on their most recent exam. This
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Contact: Toni Baker
tbaker@mcg.edu
706-721-4421
Medical College of Georgia
29-Dec-2005