source: Blood Advances

year: 2018

authors: Luisanna M. Sánchez, Rosa M. Nieves, Teresa Latham, Susan Stuber, Judy R. Luden, Gabriela S. Urcuyo, Melissa E. Berges, Carla Florencio, Carla Gonzalez, Paola Del Villar, Adam Lane, William Schultz, Neelum Jeste, Rafael Mena, Russell E. Ware

The prevalence of sickle cell anemia (SCA) in the Dominican Republic is approximately 4 times that in the United States.

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.

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.

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.

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