Antioxidant Therapy to Reduce Inflammation in Sickle Cell Disease

People with sickle cell disease have more inflammation (a response of body tissues to injury
or irritation) than people without sickle cell disease. This inflammation can be measured
in the blood by checking the level of a protein called CRP as well as other changes we see
in blood due to inflammation (such as changes in platelets and other cells). There is even
more inflammation during sickle-related complications (like pain or acute chest syndrome).
We want to test if inflammation in people with sickle cell disease can be reduced by the use
of antioxidant compounds.

Antioxidants are nutrients (certain vitamins, minerals and enzymes) that can counteract the
effects of oxidative stress arising from free radicals in our cells. The formation of free
radicals is a normal cell process, but uncontrolled oxidative stress can cause problems for
us. One such harmful problem is inflammation.

We know from other research studies that antioxidants help with some conditions related to
inflammation. In this study the antioxidant being tested is a combination of alpha-lipoic
acid and acetyl-L-carnitine, both of which are natural parts of many of the foods we eat and
are needed by our cells to make energy from food.

Inclusion Criteria:

- Proven diagnosis of sickle cell disease, either Hb SS or Hb S Beta zero thalassemia
genotype

- Age at entry at least 14 years. Younger children will not be included since the
combination alpha-lipoic acid and acetyl-L-carnitine tablets are not available in a
smaller dose at this time.

Exclusion Criteria:

- More than 3 packed red blood transfusions in the past 12 months

- Coexisting illness that could contribute to inflammation. These include chronic
hepatitis, lupus, arthritis, inflammatory bowel disease, chronic osteomyelitis, and
other similar conditions.

- Acute sickle cell disease related symptoms requiring a hospital visit in the past 4
weeks

- Women who are pregnant, attempting to get pregnant, or breast feeding

- Active participation in other investigational drug or device studies

- Participants who start hydroxyurea or regular transfusion therapy during the course
of the study on the recommendation of their primary hematologist will be ineligible
for further participation.