Seminars

Sickle cell disease (SCD) is a debilitating genetic disorder affecting 70,000 Americans and millions globally that induces chronic inflammation and vascular dysfunction and causes multiple organ damage as a result. The pathophysiology of SCD is quite complex and involves altered interactions between blood cells and endothelial cells lining the vessel walls, altered mechanical properties of blood, blood cells and blood vessels, and altered tissue properties in affected organs. Although the molecular defect associated with aberrant sickle hemoglobin is well understood and the polymerization of sickle hemoglobin and sickling of red blood cells has been extensively studied, effective treatment remains elusive. We apply innovative engineering approaches and technologies to better understand conditions that contribute to vaso-occlusion, a hallmark of the disease, and the relationship between inflammation, vascular remodeling, vascular biomechanical abnormalities and bone tissue abnormalities. Results from these studies will enable the development of new therapies and provide clinicians with therapeutic opportunities for improved management of individuals with SCD.