Building capacity to reduce stroke in children with sickle cell anemia in the Dominican Republic: the SACRED trial

Abstract

Background c The prevalence of sickle cell anemia (SCA) in the Dominican Republic is approximately 4 times that in the United States. c In the pediatric sickle cell clinic at Hospital Infantil Dr. Robert Reid Cabral (HIRRC) located in the capital city of Santo Domingo, Dominican Republic, 5% to 10% of children with HbSS (homozygous for hemoglobin S) develop primary stroke, with substantial morbidity and mortality. c Transcranial Doppler (TCD) ultrasonography has been shown to effectively identify the risk for primary stroke in the United States but is not routinely accessible or available in the Dominican Republic. c Hydroxyurea is a potent disease-modifying therapy for SCA that can reduce TCD velocities and prevent conversion of conditional to abnormal velocities. Hydroxyurea is available in the Dominican Republic, but health care providers do not have experience with dosing, and most families cannot afford the cost.

Stroke Prevention with Hydroxyurea Enabled through Research and Education: A Phase 2 Primary Stroke Prevention Trial in Sub-Saharan Africa

“…In addition, we found lower mean hemoglobin concentration, oxygen saturation, serum creatinine, and mean corpuscular volume in children with elevated TCD velocities, confirming that previously identified markers of disease severity correlate with elevated TCD velocities in the sub-Saharan African context. This higher overall prevalence of elevated TCD velocities in our Tanzanian cohort is similar to the prevalence of elevated TCD velocities documented among children with SCA in East Africa [24], West Africa [31], and the Caribbean [32, 33]. Whether this higher prevalence reflects biological, genetic, or environmental differences is unclear, but highlights the need for reliable TCD screening to prevent pediatric stroke.…”