Stroke recurrence in children with sickle cell disease treated with hydroxyurea following first clinical stroke

Susanna Bortolusso Ali1, Michelle Moosang, Lesley King

  • 1Sickle Cell Unit, TMRI, University of the West Indies, Jamaica. susanna.ali@uwimona.edu.jm

Insights

Hydroxyurea (HU) is an effective alternative to chronic transfusions for preventing stroke recurrence in children with sickle cell disease (SCD). This intervention significantly reduces stroke risk and associated complications when blood transfusions are not feasible.

Area of Science:

  • Pediatric Hematology
  • Neurology
  • Public Health

Background:

  • Stroke is a major complication of sickle cell disease (SCD) in children.
  • Chronic transfusion therapy is the standard for preventing stroke recurrence.
  • Access to transfusions is limited in many developing regions.

Purpose of the Study:

  • To evaluate hydroxyurea (HU) as an alternative to transfusion for preventing stroke recurrence in children with SCD.
  • To assess the impact of HU on stroke recurrence, mortality, and disability in this population.

Main Methods:

  • Retrospective cohort study of 43 children with a first clinical stroke between 2000 and 2009.
  • Comparison of outcomes between children who received HU and those who did not.
  • Analysis of stroke recurrence, mortality, disability, and educational needs.

Main Results:

  • Only 1 of 10 children on HU experienced stroke recurrence (2/100 person-years) versus 20 of 33 not on HU (29/100 person-years).
  • HU use was associated with a significantly lower risk of stroke recurrence (HR 9.4, P=0.03).
  • The non-HU group had higher rates of mortality, moderate-severe physical disability, and special educational needs.

Conclusions:

  • Hydroxyurea is a viable and effective intervention for preventing stroke recurrence in children with SCD.
  • HU offers a crucial alternative when chronic transfusion programs are impractical or unavailable.
  • This approach can mitigate severe neurological deficits and improve long-term outcomes in pediatric SCD patients.

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