Hemoglobin and Haptogoblin in Sickle Cell Disease

Project Description Narrative:

This project will examine the impact of intravascular hemolysis on cardiovascular dysfunction in sickle cell disease (SCD). The major symptomatic complications of SCD are related to the cardiovascular system, with vaso-occlusive crisis and pulmonary hypertension amongst them. This project will study the mechanism of cardiovascular complications that occur as a result of increases in plasma hemoglobin content. Sickle cell disease is a debilitating, life-shortening disease that is prevalent amongst the African American (1 in every 500 children) and Hispanic American (1 in every 1,500 children) populations. The results from this proposal will add to the understanding of the importance of haptoglobin/hemoglobin interaction in normal cardiovascular health and emphasize the deleterious complications of enhanced hemolysis.