Despite the fact that the problem of stroke in sickle cell disease (SCD) patients have been recognized for more than 75 years, viable prevention and treatment options are just now being investigated. Nearly 4000 persons with SCD were followed in 23 US clinical sites over a 10-year period for the Cooperative Study of Sickle Cell Disease, which yielded recent data on prevalence and incidence.
The total age-specific incidence of the first stroke in SCD (homozygous sickle cell anemia) is minimal (0.13 percent) at ages less than 24 months, climbs to slightly more than 1% at ages 2 to 5 years, and then reduces to 0.79 percent at ages 6 to 9. The risk of a stroke decreases until a second peak arises. A person's risk of a brain infarction diminishes over time until it hits a new peak at the age of 50 when it jumps to around 1.3 percent.
Although cerebral hemorrhage can occur in young children with SCD, it is uncommon in older children and adults. According to the Cooperative Study of Sickle Cell Disease, previous transient ischemic events, low steady-state hemoglobin values, rate and recency of episodes of acute chest syndrome, and high systolic blood pressure are all risk factors for infarction.
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