Sickle Cell Disease and Cardiovascular Risk- Red Cell Exchange Trials (SCD CARRE)

Project Rationale

Sickle cell disease (SCD) is the most common inherited blood disorder in the U.S. It mainly affects African Americans (1 in 500) and Hispanic Americans (1 in 36,000). People with SCD are born with a defect in hemoglobin, which is a protein in red blood cells that carries oxygen throughout the body. In SCD, the red blood cells become rigid and sticky and look like a C-shaped farming tool called a sickle. Sickle cells can get stuck in small blood vessels and block the flow of blood and oxygen to organs within the body. When the blood vessels are blocked, patients experience extreme pain. In addition, adults with SCD have a high risk of organ damage and early death.

The purpose of this study is to carefully evaluate the effects of giving long-term red blood cell exchange transfusion to a group of patients with SCD with certain risk factors for severe organ damage or early death. We want to know if regular exchange blood transfusion will minimize complications in patients with SCD or reduce the chances of new serious problems occurring in patients with sickle cell disease.

Project Goals

• To test the therapeutic efficacy of automated RBC exchange transfusion of ABO Rh Kell matched blood and standard of care compared with standard of care, in patients at high mortality risk defined by high TRV, the combination of moderately high TRV and high plasma NT-proBNP, or the presence of chronic kidney disease, on the number of episodes of clinical worsening of SCD requiring an acute health care encounter (non-elective infusion center/ER/hospital visits) or resulting in death over 12 months.

• To evaluate if the addition of automated RBC exchange transfusion of ABO Rh Kell matched blood to standard of care reduces end-organ heart, lung, and kidney injury, improves exercise capacity (measured by six-minute walk distance and home accelerometry), and improves cardiovascular function, health related quality of life, and pain compared with standard of care. 

• To assess the effects of the intervention on TRV, NT-proBNP and measures of diastolic left hear.

For more information about SCD CARRE, please click here.