Iron Mediated Vascular Disease in Sickle Cell Anemia Patients

Patients with sickle cell anemia often require blood transfusion as part of the treatment for
their disease. Each teaspoon of transfused blood contains about 5 mg of iron and the levels
of iron in sickle cell patients increase rapidly with each transfusion. While iron is
necessary for many bodily functions, too much iron damages blood vessels, liver, hormone
producing glands (pancreas, pituitary and thyroid) and the heart. It is important to know how
iron damages blood vessels because most of the problems experienced by sickle cell anemia
patients (stroke, kidney failure, pulmonary hypertension, heart disease) result from blood
vessel damage. In this trial, iron in the liver, pancreas, and kidney will be measured
noninvasively by MRI while vascular function will be measured by ultrasound and tissue
Doppler. Patients will be recruited primarily from the greater Los Angeles area, although
patients from greater distances will be allowed to participate.

Inclusion Criteria:

- Age > 13 years

- Documented diagnosis of sickle cell anemia (SS, SC, Sß0, Sß+)

- Transfused no more than 8 times in a year.

Exclusion Criteria:

- Cardiac pacemaker, implantable neurostimulator or other MRI incompatible device.

- History of extreme claustrophobia in MRI machine or other reason for inability to do
MRI without sedation.

- Inability to be positioned on the MRI table for sufficient time to complete the MRI
exams.

- Any medical or psychological condition that, in the opinion of the local investigator,
would make it unsafe or ill-advised for the subject to participate.

- Currently not receiving chronic transfusion therapy, defined as greater than 8
transfusions per year.