Mito Cocktail

“Mito Cocktail” is the name given to a variety of vitamins and supplements which are commonly used by adults and children who have been diagnosed with mitochondrial disease. While there is no cure for mitochondrial disease, many types of the disease including mitochondrial myopathy, mitochondrial cytopathy, MELAS, PDCD, or Complex I, II, III, and IV are responsive to specific vitamin and supplement therapies.

Consult with your physician before beginning or altering any medication, vitamin or supplement regimen.

The supplements and vitamins used by Mito patients are often high doses and could require a patient to take up to 50 different pills per day. A compounding pharmacist (find one in your area) through the International Academy of Compounding Pharmacists (IACP), can create a “cocktail” in a liquid, capsule or other form that combines the pure powdered form of the prescribed supplements and vitamins. The final medication is usually a much smaller amount than if otherwise taken, and can even be flavored to improve palatability. By avoiding fillers common in over the counter tablets, an individual’s allergy or dietary restrictions can be accommodated. The exact compound, including dosage and ingredients, is determined by the patient’s physician and differs depending on an individual Mito patient’s diagnosis, clinical symptoms, weight.

Most Common Ingredients of the Mito Cocktail

CoenzymeA relatively small organic (i.e., carbon-containing) molecule whose presence is required in order for the biological activity of an enzyme to occur. Thus, coenzymes are a particular kind of cofactor. Q-10
Coenzyme Q-10 (Coenzyme Q10A relatively small fat-soluble organic (i.e., carbon-containing) molecule, found in membranes throughout the in the body. It readily transports both electrons and protons. Its many essential functions, CoQ10, CoQ-10, CoQ, ubiquinone, Q-Gel®), is a fat-soluble vitamin-like substance present in every cell of the body and serves as a coenzyme for several of the key enzymatic steps in the production of energy within the cell. It also functions as an antioxidant protecting against accumulation of harmful free radicalsUnstable, highly-reactive atoms or molecules, generated in living organisms by metabolic reactions, mainly in the mitochodrion. These can damage DNA, proteins and other molecules, in the mitochondria, which is important in its clinical effects. Many patients report increased energy while using Coenzyme Q-10, and thus it is a common “front-line” approach to supporting children and adults with mitochondrial disease. Frequently reported side effects include stomach upset and sleep disturbance, so pharmacists typically recommend taking Co Q-10 doses earlier in the day, with food. Therapeutic levels may need time to be established, so patients may not see an immediate beneficial effect. In addition, the excess of the substance that is not used is stored in the fat cells, so proper dosing is important.

Complex Vitamins

Some B-vitamins are cofactors which participate in important mitochondrial reactions. Most of the B-vitamins have a bitter taste and more palatable if flavored. B-vitamins are water soluble; that is, they are excreted if not used, and the benefit from taking these vitamins should be felt immediately.

• Vitamin B1 (Thiamine)A critical B vitamin that supports energy metabolism and nerve function.
  This is a water soluble vitamin which helps break down carbohydrates so the body can better use them, helps with growth and maintenance of muscle tone, and aids memory. The only possible side effect sometimes noted is drowsiness.
• Vitamin B2 (RiboflavinIt is a type of vitamin B. It helps in red blood cell production and aids in the release of energy from proteins.)
  Also a water soluble vitamin, B2 is necessary for energy production in the mitochondria and increases muscle performance as well as helping maintain healthy mucous membranes, skin, hair and nails. The only side effect noted is the tendency to turn urine an orange color. Given in the form of Riboflavin Biphosphate can improve the taste of this vitamin.
• Vitamin B3 (NiacinSupplement that aids in its deficiency that leads to slow metabolism and an intolerance to the cold. It is made and used by your body to turn food into energy.) Occasionally used, B3 can often cause flushing of the face so it is generally given separately first to see if any side effects will occur before it is added to the cocktail.
• Vitamin B6 (Pyridoxine)A B vitamin that assists in amino acid and fatty acid metabolism and red blood cell production. It is essential for normal brain development and keeping the nervous and immune system healthy. and Vitamin B12 (Cobalamine) These B vitamins are frequently part of the compounded mix which Mito patients may use.

Other Antioxidants
Antioxidants decrease free radical accumulation in the cells and therefore are used for Mito patients as well. Alpha Lipoic AcidA small, vitamin-like organic molecule,made in the mitochondria, and found in every cell. It plays multiple essential roles. It is a necessary co-factor for the activity of five different enzyme compl is probably the most commonly prescribed anti-oxidant used in the Mito cocktail.

• Vitamin C
  This is used for its help in the healing process and to ward off infections but can cause some stomach upset and occasionally headaches when the dose is increased.
• Vitamin E
  This protects cell membranes and improves neurological function. Usually the dose is no higher than 400 – 600 mg per day for an adult. This can interfere with coumadin/warfarin medications, so be aware of this.
• Vitamin K1
  This vitamin must be prescribed by a physician and used with caution, as there is a very small safe range for the dosage of this vitamin.
• L-Carnitine
  Also prescribed by a physician, L-Carnitine helps transport fatty acids and improve the strength and tone of muscles. Side effects may include diarrhea, and a fishy odor which may be excreted via the sweat glands. Some patients report decreased fatigueThe overall feeling of tiredness or lack of energy. It is not the same as simply feeling drowsy or sleepy. Being fatigued means having no motivation or energy. and energy improvements by taking L-Carnitine. It is taken in either  tablet or liquid form and is usually taken separate from the compounded cocktail.
• Creatine
  Creatine helps maintain muscle mass and increases energy for cells. Its side effects include diarrhea and drowsiness. Dosages range from 5 grams/day for children to 10 grams/day for adults and is generally compounded into liquid or capsule form.

All of the vitamins and supplements noted above are added or not added to a cocktail as specified by a patient’s need. Each cocktail is patient specific. Please work with your doctor to determine the exact ingredients to be used in the compound that will be most beneficial for you.