Stroke Prevention in Young Adults With Sickle Cell Anemia

Study point of contact

Djamila L Ghafuri, MD, MPH
[email protected]
Michael R DeBaun, MD, MPH
(615) 936-1762
[email protected]

Locations

1 United States site

1 Nigeria site

Age

16 to 26 Years

Study type

Observational

Gender

All

Interventions

Drug

Compensation

Unknown

About the study

Sickle cell disease (SCD) is the most common genetic disease, affecting about 25 million
people worldwide. Approximately 150,000 Nigerian children are born each year with sickle cell
disease (SCD), making it the country with the largest burden of SCD in the world. Recent
advancements in care for children with SCA have translated into improved survival of children
in both high and low-resource settings. However, more complications of SCD are seen in those
who survive to adulthood. Silent cerebral infarcts (SCI) and strokes are among the most
devastating complications of SCD, affecting 40% and 10% of children, respectively.

The overall goal of this study is to extend the Investigator’s successful capacity-building
effort in the assessment of neurological morbidity in children with SCD living in northern
Nigeria (Kano) to young adults with SCD living in the same region. About 50% of all adults
with SCD live in Nigeria. Despite the high prevalence of SCD in Africa, the neurological
morbidity is not well characterized, limiting opportunities for primary and secondary stroke
prevention strategies. At least 50% of young adults with sickle cell anemia (SCA), the most
severe form of the disease, will have SCIs and an estimated 10% will have strokes, based on
studies in high-resource settings. In high-resource settings, screening for abnormal
transcranial Doppler (TCD) velocities in children with SCA, coupled with regular blood
transfusion has resulted in a 92% reduction of relative risk for strokes. Despite this
effective strategy, regular blood transfusion therapy does not seem sustainable in
sub-Saharan Africa due to shortages and the risk of transfusion transmissible infections.
Additionally, there is a lack of evidence-based stroke prevention strategies in young adults
with SCA, either in the high-income or in low-resource settings. Based on the foregoing, the
Investigators propose to determine the prevalence of neurological injury (overt stroke,
transient ischemic attacks, and silent cerebral infarcts) in young adults at the transition
age from 16-25 years. The Investigators will also, for the first time, assess conventional
risk factors of stroke in the general population to determine whether a different prevention
strategy is required to reduce the incidence of neurological injury in this high-risk
population.

participation requirements

1. Patients with hemoglobin S-S or Sβ0 thalassemia confirmed by hemoglobin
electrophoresis or High-Performance Liquid Chromatography (HPLC);

2. Participant is 16 through 25 years of age;

3. Informed consent from participants above 18 years, and informed consent from a parent
or legal guardian and assent of participants aged < 18 years (assessment can take place up until the 26th birthday); 4. Participant resides within an hour driving distance from the medical center to facilitate weekly phone calls between the scheduled monthly clinic visits; 5. Participant is willing to be enrolled and followed for the duration of the study.

participation restrictions

1. Young adults with co-morbidities that may have an impact on neurological status, such
as epilepsy;

2. Young adults enrolled in clinical trials upon entry;

3. Participants with an implanted defibrillator or certain other implanted electronic or
metallic devices contraindicated for MRI;

4. Young adults with known HIV diagnosis;

5. Any other condition or chronic illness, which in the opinion of the site’s Principal
Investigator (PI) makes participation ill-advised or unsafe.

Locations

  • Nashville, Tennessee, United States, Vanderbilt University Medical Center, 37232-9000
  • Kano, Nigeria, Aminu Kano Teaching Hospital