Establishment of Functional MRI Imaging Parameters for Use in the Evaluation of Sickle Cell Disease

Study Overview
=================
Brief Summary
-----------------
Patients with sickle cell anemia (SCA) are at an increased risk for damage to brain tissue due to their disease. The investigators are interested in how blood flow and cerebral inflammation are different in SCA patients and how that affects brain tissue- the investigators will use a relatively new set of dynamic MRI techniques to evaluate these parameters. The investigators will image participants with both SCA and matched controls with non-invasive MRI.

Detailed Description
-----------------
Patients with sickle cell anemia (SCA) are at increased risk for episodes of stroke, both overt clinically evident and subclinical lesions only seen on imaging, which have associated morbidity and mortality. In addition, SCA patients demonstrate relatively poorer cognitive performance compared to their peers without SCA that is believed to be related to the episodes of stroke, but may be present even in their absence. This study is designed to explore potential risk factors in patients with SCA that will identify predictors of cerebral damage that may also be modifiable. Elevated blood flow in cerebral arteries and increased inflammation are believed to be related to both ischemic lesions and cognitive findings but have not yet been clearly proven. We aim to use new MRI techniques which target cerebral blood flow and inflammation to identify differences in SCA patients and peers and follow this with an initial exploration of the association between these pathologic findings and cognitive deficits. The investigator does not assign specific interventions to the subjects of the study.

Official Title
-----------------
Establishment of Functional MRI Imaging Parameters for Use in the Evaluation of Sickle Cell Disease

Conditions
-----------------
Sickle Cell Anemia

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Patient with HbSS/HbSB0, age between 18 and 55 years or age/gender/race/education matched peer Exclusion Criteria: Previous history of a stroke/transient ischemic attack, neurosurgery, head trauma, seizures, pulmonary embolism, deep-vein thrombosis, bleeding/clotting disorders, current or previous use of anticoagulation medications

Ages Eligible for Study
-----------------
Minimum Age: 18 Years
Maximum Age: 55 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
Accepts Healthy Volunteers

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| SCA Patients<br>Asymptomatic sickle cell anemia patients will undergo imaging/neurocognitive testing and be compared to controls | |
| Control Patients<br>Asymptomatic sickle cell anemia patients will undergo imaging/neurocognitive testing and be compared to controls | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Cerebral blood flow (CBF) measured by continuous arterial spin labeling (CASL) MRI | CBF will be measured by continuous arterial spin labeling (CASL) MRI | Day 1 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Cogstate Testing | Exploration of the association between CBF values and cognitive performance deficits | Day 1 | 
| Mean diffusivity/Fractional anisotropy (MD/FA) evaluated by diffusion tensor imaging (DTI) MRI measurements | MD/FA will be evaluated by diffusion tensor imaging (DTI) MRI measurements | Day 1 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Inflammation, Blood Flow, Cognition