Stroke Recurrence in Nigerian Children With Sickle Cell Disease: Evidence for a Secondary Stroke Prevention Trial

Abstract

Background: To improve the quality of care for children with sickle cell anemia in Kano, Nigeria, we initiated a standard care protocol in 2014 to manage children with strokes at Aminu Kano Teaching Hospital.

Methods: The standard care protocol requires that children with acute strokes be treated with hydroxyurea at a fixed dose of 20 mg/kg/day within two months of the stroke.

Results: Twenty-nine children with sickle cell anemia and initial stroke were identified based on clinical World Health Organization criteria from 2014 to 2017. Follow-up was a median of 1.04 years (interquartile range 0.43 to 1.83 years) to either July 2017 or a second stroke, corresponding to an initial stroke incidence rate of 0.88 per 100 patient-years. Eight children had a recurrent stroke, six of whom were prescribed hydroxyurea 20 mg/kg/day by two months after initial stroke. Two children died. Six of the recurrent strokes occurred within six months of the initial stroke, two before hydroxyurea prescription. The stroke recurrence rate was 17.4 events per 100 patient-years. Adherence was approximately 60%, partly because families had to pay for hydroxyurea. Stroke incidence is probably underestimated because despite formal training for stroke detection during the quality improvement period, no participant had assessment using a standardized pediatric stroke scale and neuroimaging was not available.

Conclusions: In children with sickle cell anemia, a high rate of initial and recurrent strokes exists in a low-resource setting. Ongoing needs include training to detect strokes with an objective stroke assessment and government-supported free access to hydroxyurea for stroke prevention.

Trial registration: ClinicalTrials.gov NCT02560935 NCT02675790.

Keywords: Hydroxyurea; Mortality; Sickle cell disease; Stroke; Stroke recurrence.

Copyright © 2019 Elsevier Inc. All rights reserved.

Figures

Figure 1:. Kaplan-Meier plot of time to stroke recurrence after first stroke (n=29).

Figure 2-

Time to second stroke showing 75% of children having stroke recurrence within 6 months of the first stroke.

Figure 3:. Modified Rankin Scale for Children with Stroke.

The bar graph shows the frequency of modified Rankin scale scores for n=29 Nigerian children with sickle cell disease.