Evolution of cerebral hemodynamics and metabolism across the early lifespan in patients with sickle cell disease

Sickle cell disease (SCD) is an inherited hemolytic anemia with altered hemodynamics and increased stroke risk. However, lifetime trends in SCD cerebral hemodynamics and metabolism are poorly understood. We used non-invasive functional measures of cerebral blood flow (CBF) from arterial spin labeling, and oxygen extraction fraction (OEF) from T2-Relaxation-Under-Spin-Tagging MRI, to quantify hemo-metabolic patterns in controls (n=64) and SCD patients (n=125) across the early lifespan (6-40 years). CBF decreases with age in healthy controls (-3.2 mL/100g/min per decade) but increases in patients (5.2 mL/100g/min per decade). OEF was elevated in SCD, showing a similar slope with age as controls.