Hydroxyurea lowers transcranial Doppler flow velocities in children with sickle cell anaemia in a Nigerian cohort

Abstract

HU appears to significantly reduce TCD velocities in Nigerian children with SCA and elevated velocities ≥170 cm/sec with beneficial effect on the haematological profile. HU may provide an effective approach to primary stroke prevention, particularly in Africa.

Sickle cell anemia in sub-Saharan Africa: advancing the clinical paradigm through partnerships and research

“…One important question is whether fixed low-dose therapy (10-20 mg/kg per day) is more practical than escalation to maximum tolerated dose (20-30 mg/kg per day), because medication optimization requires frequent monitoring, dose adjustments, and clinical expertise. 72,73 Small retrospective studies suggest hydroxyurea will have beneficial effects on both primary and secondary stroke prevention in Africa, 74,75 similar to earlier findings from Jamaica. 76 Prospective research regarding hydroxyurea therapy is now underway in Africa, and Table 3 highlights currently registered clinical trials.…”

Coagulation abnormalities of sickle cell disease: Relationship with clinical outcomes and the effect of disease modifying therapies

“…It has been shown to reduce the frequency of acute painful episodes, dactylitis, acute chest syndrome, hospitalizations, and the need for blood transfusions in children and adults with sickle cell anemia (161,162). Observational studies have reported a reduction of TCD velocity (163–165), rate of first stroke (166) and the rate of stroke recurrence (167–170) in SCD patients treated with hydroxyurea. The Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial, was a randomized, non-inferiority trial comparing transfusions and iron chelation to hydroxyurea and therapeutic phlebotomy for children with sickle cell anemia, stroke, and iron overload, with a composite primary endpoint allowing an increased stroke risk but requiring superiority for removing iron (171).…”

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

“…Recognizing this significant barrier, several investigators evaluated hydroxyurea therapy as an alternative to regular transfusions for stroke prevention. Based on several studies that consistently demonstrated a decrease in TCD measurements with hydroxyurea therapy [66][67][68][69][70][71], the NHLBI sponsored the Transcranial Doppler With Transfusions Switching to Hydroxyurea (TWiTCH) trial. For primary stroke prevention, the TWiTCH trial randomly allotted children with hemoglobin SS and hemoglobin S/beta-0-thalassemia with elevated TCD velocities without strokes and were on a regimen of regular transfusions to receive either regular blood transfusion therapy or hydroxyurea therapy escalated to maximum tolerated dose.…”