The Epidemiology of Silent and Overt Strokes in Adults With Sickle Cell Disease: a Prospective Cohort Study

Study point of contact

Djamila L Ghafuri, MD, MPH
[email protected]
Leshana Saint-Jean, PhD
[email protected]


4 United States sites


> 18 Years

Study type






About the study

Sickle Cell Disease (SCD) is a rare disease occurring in an estimated 100,000 individuals,
often poor and underserved, in the US. Silent and overt strokes contribute significantly to
morbidity in adults with SCD, resulting in functional impairment, challenges with school and
job performance, and premature death. Five NIH-funded randomized controlled trials have
identified therapies to prevent silent and overt strokes in children with SCD, including
monthly blood transfusion therapy (for preventing initial and recurrent strokes) and
hydroxyurea (for preventing initial strokes). Despite the observation that at least 99% of
children with SCD in high-income countries reach adulthood, and approximately 60% of adults
will experience one or more strokes (~50% with silent strokes and ~10% with overt strokes),
no stroke trials have established therapeutic approaches for adults with SCD. For adults with
SCD, inadequate evidence-based guidelines exist for secondary stroke prevention strategies.
Applying stroke prevention strategies in children may not be effective for stroke prevention
in adults with SCD, particularly given the high rate of co-morbidities. Identifying subgroups
of adults with SCD and higher incidence coupled with the contribution of established stroke
risk factors in the general population (smoking, diabetes, obesity, renal disease) will
provide the requisite data required for the first-ever phase III clinical trials focused on
secondary stroke prevention in adults.

participation requirements

1. Participants with sickle cell disease on hemoglobin analysis and/or other confirmatory
documentation of phenotype

2. Patients ≥ 18 years of age

3. Patients followed regularly (at least two visits per year) in the hematology clinics

4. Patients who have demonstrated adherence with follow-up visits for ≥ 3 years

5. Patients willing to be followed prospectively for a minimum of 3.5 years and agree to
a standard care exit MRI/MRA of the brain, as well as MRI/MRA every 12 to 18 months or
participation in VUMC AHA trial with Dr. Jordan as PI. These are adults with SCA aged
18-40 years at study entry, enrolled with any infarct status (none, SCI or overt
stroke) and followed prospectively.

6. Willingness to comply with study protocol, routine clinic visits

participation restrictions

1. Participants judged to be non-compliant by the hematologist based on previous
experience in terms of clinic appointments and following advice

2. Participants with contraindications to MRI, including individuals with
MRI-incompatible foreign metal objects


  • Birmingham, Alabama, United States, University of Alabama at Birmingham, 35233 [Recruiting]
  • Saint Louis, Missouri, United States, Washington University School of Medicine, 63110 [Recruiting]
  • Nashville, Tennessee, United States, Vanderbilt University Medical Center, 37232-9000 [Recruiting]
  • Nashville, Tennessee, United States, Vanderbilt University Medical Center, 37232 [Recruiting]