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MitoAction

MitoAction

Support, Education, Outreach and Advocacy for Children and Adults Living with Mitochondrial Disease

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Mito Cocktail

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A Mito Cocktail (also known as a MitochondrialRelated to the mitochondria. Cocktail or Mitochondrial Disease Cocktail) is 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. To find a doctor who specializes in the treatment of mitochondrial diseases, click here.

A Mito Cocktail is Often Compounded

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 can create a “cocktail” in a liquid, capsule or other form that combines the pure powdered form of the prescribed supplements and vitamins. This allows the final medication to be taken in much smaller amounts than if taken individually, 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, and other factors.

A Mito Cocktail (also known as a Mitochondrial Cocktail or Mitochondrial Disease Cocktail) can help mito patients control many symptoms of their disease.

Most Common Ingredients of the Mito Cocktail

A Mitochondrial Disease Cocktail is customized for an individual patient, based on diagnosis, symptoms and more. The following ingredients are commonly found in mito cocktails:

Coenzyme Q-10

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 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) 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.

B-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.
  • 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. 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 (PyridoxineA supplement helps with neuropathy and is a component of neurotransmitter synthesis.) and Vitamin B12 (Cobalamine) These B-vitamins are frequently part of the compounded mix which Mito patients may use.

Other Antioxidants

Antioxidants are molecules that protect the body from damage caused by free radicals, unstable molecules that can lead to inflammation and disease.

  • 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
    Alpha-lipoic acid is a universal antioxidant. In addition, it can support the regeneration of other antioxidants, like glutathione, Vitamin C and Vitamin E.
  • Vitamin C
    Vitamin C 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
    Vitamin E protects cell membranes and improves neurological function. Usually the dose is no higher than 400-600 mg per day for an adult.
  • Vitamin K1
    Vitamin K1 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. Some patients report decreased fatigue 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.

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.

Find a Mito Doctor

To make it easier for mito patients to find a doctor who specializes in Mito, MitoAction maintain a list of experts who treat and research mitochondrial diseases. Click here to find a Doctor near you or one who sees patients nationwide via telehealth.

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