Typically, monthly blood transfusion therapy is an option for children with sickle cell anemia (SCA) followed by hydroxyurea therapy. As blood transfusion therapy is not feasible in Africa, different strategies were investigated for stroke prevention in SCA.
Stroke risk in children with sickle cell anemia (SCA) is 100 times times that for children without SCA, according to the American Stroke Association1. Most strokes in children with SCA are ischemic and are largely asymptomatic. If SCA stroke is not treated, 80% of children who have it will have another stroke within three years2.
In high-income countries, children with SCA and abnormal transcranial Doppler velocities get blood transfusions once a month for at least a year. This lowers their risk of having a stroke by 90%. About half of the 300,000 SCA children born each year live in Nigeria. Unfortunately, most Nigerian children do not have access to monthly blood transfusion therapy.
To address this significant disparity, a team led by Dr. Michael R. Debaun, a professor at Vanderbilt University, conducted the Stroke Prevention in Nigeria (SPIN) trial, single-arm feasibility trial using moderate-dose hydroxyurea therapy for children with abnormal transcranial Doppler measurements. Following that, a phase III, double-blind, parallel-group, multi-center randomized controlled stroke prevention trial, called the Sickle Cell Disease in Nigeria (SPRING)trial was conducted.
The trial findings demonstrated that moderate-dose hydroxyurea (20 mg/kg/day) therapy for secondary stroke prevention results in a 66% relative risk reduction. Based on these trials, researchers established a state-supported stroke screening service and treatment for over 20,000 children in Kano, Nigeria. Over the last ten years, they have also built a group of academics who are working to improve the lives of children and adults with SCA in Nigeria.
References
American Stroke Association. (Sep 21, 2021). Sickle Cell Disease and Pediatric Stroke Risk.
https://www.stroke.org/en/about-stroke/stroke-in-children/sickle-cell-disease
Debaun, M. R. (2022). PRIMARY STROKE PREVENTION IN CHILDREN WITH SICKLE CELL ANEMIA LIVING IN AFRICA: THE FALSE CHOICE BETWEEN PATIENT-ORIENTED RESEARCH AND HUMANITARIAN SERVICE-PART II. Transactions of the American Clinical and Climatological Association, 132, 236-248. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9480538/
