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Stroke risk returns when children with sickle cell disease stop transfusions

confirms the technique's efficacy as a stroke indicator before transfusions are started and after they are stopped, according to the study.

"These results suggest that if stroke is to be prevented after transfusion is stopped, transcranial Doppler examinations must be performed at frequent intervals and transfusions resumed expeditiously," Dr. Adams writes.

Two children in the original STOP had strokes despite being on transfusion, probably because their disease was so advanced when treatment began, Dr. Adams says. STOP, which followed 130 at-risk children, also was halted early because of the dramatic findings of a 90 percent reduction in stroke risk. A physician advisory issued by the NHLBI in September 1997 recommended regular transcranial Doppler studies in children age 2-16 with sickle cell disease and that transfusion be considered for those at risk.

That's what Dr. McKie, who now works part time in the MCG Pediatric Sickle Cell Clinic, recommends today.

"I'm not surprised at how effective transfusion is," Dr. Adams says, noting that most children tended to do well on transfusion, with higher hemoglobin levels, fewer pain crises and cases of acute chest syndrome, better liver function and just generally growing and feeling better.

However, transfusions are not without problems. No transfusion-related infections were reported but there were nine transfusion reactions reported in STOP II, including one that required hospitalization.

Also, iron overload, which damages vital organs, necessitated chelation therapy five nights a week for most transfusion recipients to remove excess iron. Patients and families don't like the time-consuming and expensive therapy, Dr. McKie says, noting an oral therapy approved this year by the Food and Drug Administration may make chelation more palatable.

Many fundamental questions remain, including why these 10 percent of children have problems with stroke and how transfusion
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Contact: Toni Baker
tbaker@mcg.edu
706-721-4421
Medical College of Georgia
29-Dec-2005


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