Background Chronic blood transfusion is the standard treatment for secondary stroke prevention in sickle cell anaemia (SCA) but this treatment option poses major challenges in resource-poor countries of the world, especially malaria-endemic ones.

Objective To compare the outcomes after a first clinical stroke, with and without treatment with hydroxyurea (HU).

Methods Over a 6-year period, Nigerian children with SCA who had suffered a first stroke was studied. Outcomes in those who received HU (25mg/kg/day) were compared with those whose parents declined both HU and chronic transfusion.

Results Thirty-two children, all with haemoglobin SS phenotype (SCA) presented with stroke and one died of haemorrhagic stroke at presentation. Age at first clinical stroke (Mean +/- SD) was 7.58 (+/-2.33) years. Thirteen children received HU while 18 declined HU therapy. The secondary stroke incidence of 7/100 person years in the HU group was significantly lower than the 28/100 person years in the non-HU group (P = 0.001, OR 3.808, 95% CI 1.556, 9.317). Children who did not receive HU were more likely to drop out of school and to have moderate-severe motor disabilities requiring caregiver assistance for activities of daily living.

Conclusion In settings where facilities for chronic blood transfusion are not accessible or feasible, HU therapy should be considered for secondary stroke prevention in children with SCA. Since, as far as we are aware, this is the first use of HU in a malaria-endemic setting, the possible impact of HU on drug-resistance in malaria needs to be carefully studied.

Reference

1. Lagunju I, Sodeinde O, Telfer P. Prevalence of transcranial Doppler abnormalities in Nigerian children with sickle cell disease. Am J Hematol. 2012 May; 87(5):544–7. doi: 10.1002/ajh.23152. Epub 2012 Mar 28. PubMed PMID: 22460323.