1. Ages 3 to 9 y (inclusive) at enrollment |
2. Confirmed laboratory diagnosis of HbSS or HbS-B0 thalassemia (available within 1 wk of enrollment) |
3. Hb ≥ 6.0 g/dL |
4. No history of HU use for >6 mo |
5. Not acutely ill at enrollment: fever, respiratory infection, sickle crisis within the previous 2 wk, or blood transfusion within the past 90 d (temporary exclusion criteria) |
6. Had attended the Mulago Hospital SCA clinic ≥2 times in the previous 4 y, or ≥1 time in the previous 2 y if age ≥2 y (to help ensure attendance of study visits) |
7. Parent and child are able to speak and understand English or Luganda (a predominant language in Kampala) |
8. No history of neurological abnormality known before the age of 4 mo (to avoid early non-SCA brain injury) |
9. Child was not currently enrolled in a clinical intervention trial |
10. Child and parent/legal guardian were willing to participate (if child was ≥8 y of age for assenting) |
11. Absence of previous stroke by standardized stroke-focused PedNIHSS examination by a study physician∗ |
1. Ages 3 to 9 y (inclusive) at enrollment |
2. Confirmed laboratory diagnosis of HbSS or HbS-B0 thalassemia (available within 1 wk of enrollment) |
3. Hb ≥ 6.0 g/dL |
4. No history of HU use for >6 mo |
5. Not acutely ill at enrollment: fever, respiratory infection, sickle crisis within the previous 2 wk, or blood transfusion within the past 90 d (temporary exclusion criteria) |
6. Had attended the Mulago Hospital SCA clinic ≥2 times in the previous 4 y, or ≥1 time in the previous 2 y if age ≥2 y (to help ensure attendance of study visits) |
7. Parent and child are able to speak and understand English or Luganda (a predominant language in Kampala) |
8. No history of neurological abnormality known before the age of 4 mo (to avoid early non-SCA brain injury) |
9. Child was not currently enrolled in a clinical intervention trial |
10. Child and parent/legal guardian were willing to participate (if child was ≥8 y of age for assenting) |
11. Absence of previous stroke by standardized stroke-focused PedNIHSS examination by a study physician∗ |
HbS-B0, compound heterozygote HbS and Hb Beta0 thalassemia; HbSS, homozygous HbS.
Pediatric National Institutes of Health Stroke Scale.13