STOP I demonstrated transfusions reduced stroke risk by 92 % in high risk children and TCD is used to select patients for transfusion. STOP II showed rising TCD velocities, especially above 200 cm/s cutoff, led to more strokes upon transfusion discontinuation.
Eleven percent stroke by age 20.
25 percent by age 45
Recommend at least annual TCD between ages 2-16
mortality with hemorrhage is 24-65 percent
Associations with hemorrhage: Hypertension, recent transfusion last 14 days, steroids, possibly NSIAA
nonatherosclerotic
point mutation valine for glu in Beta polypeptide chain
younger patients tend to get ischemic strokes, older patients hemorrhagic strokes, two thirds recur
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