Antioxidant Therapy to Reduce Inflammation in Sickle Cell Disease

The purpose of this study is to determine whether alpha-lipoic acid and acetyl-L-carnitine will lower systemic inflammation in patients with Sickle Cell Disease by reducing oxidative stress, which will result in a decrease in the frequency of vaso-occlusive pain episodes and improve their quality of life.

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 C reactive protein 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.

Antioxidant, Inflammation, Sickle Cell Disease, Oxidative Stress, Cytokines, anti-inflammatory, Acetylcarnitine

C-Reactive Protein (CRP) was measured by a clinical laboratory. Measuring the quantity of serum CRP is helpful in recognizing inflammatory states.

Inclusion Criteria: Proven diagnosis of sickle cell disease, either homozygous sickle disease 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.