| | | Mitochondrial Myopathies... What Are They?
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 Mitochondrial myopathies are a group of neuromuscular diseases caused by damage to the mitochondria - small, energy-producing structures found in almost all cells in the body that serve as the cells' "power plants." Nerve cells in the brain, muscle cells and heart cells require a great deal of energy, and thus appear to be particularly damaged when mitochondrial dysfunction occurs. Some of the more common mitochondrial myopathies include Kearns-Sayre syndrome, myoclonus epilepsy with ragged-red fibers (MERRF), and mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes (MELAS) and Friedreich's Ataxia. The symptoms of mitochondrial myopathies include muscle weakness or exercise intolerance, heart failure or rhythm disturbances, dementia, movement disorders, stroke-like episodes, deafness, blindness, droopy eyelids, reduced ability of the eyes to move, vomiting, and seizures. The disorders range in severity from progressive weakness to death. The age of onset ranges from birth to adulthood, with the majority of cases occurring before the age of 20. Exercise intolerance or muscle weakness usually develops by the age of 20. During physical activity, muscles may become easily fatigued or weak. Muscle cramping may rarely occur. Nausea, headache, and breathlessness are also sometimes associated with mitochondrial myopathies.
What is the prognosis?
The prognosis for patients with mitochondrial myopathies varies greatly from patient to patient because disease progression depends largely on the type of disease and the degree of involvement of various organs.
Are there any treatments?
Although there is no specific treatment for any of the mitochondrial myopathies, physical therapy may extend the range of movement of muscles and improve dexterity. Vitamin therapies such as riboflavin, coenzyme Q10, vitamins C and K, Thiamine, Alpha Lipoic Acid and carnitine (a specialized amino acid) may improve function in some patients. If you are considering vitamin therapies discuss the issue first with your local health care professional. If you get the go-ahead, we suggest starting with Q-Gel CoQ10.
Why Q-Gel CoQ10?
Because, Q-Gel CoQ10 is hydrosoluble and it gets into your system very quickly. Before Q-Gel was available, it was taking Dr’s doing clinical trials on CoQ10 one to two years to get therapeutic blood levels of CoQ10. With Q-Gel, they are achieving therapeutic blood serum levels of CoQ10 in one month or less. Note that we also have Q-Gel available in liquid in a bottle for those patients who cannot swallow a soft gel.
Children’s Q-Gel CoQ10 Dosages:
Normally healthy children should NOT need to supplement their diet with CoQ10. Dosages for children with mitochondrial diseases and other conditions that could be helped with Q-Gel CoQ10 should be discussed with your child’s Doctor. Per our senior scientist, a starting point you may want to discuss would be 3 mg. per pound of the child’s body weight. For example, if the child weighs 20 pounds, start with 20 X 3 mg = 60 mg per day in divided doses, i.e. 20 mg 3 times daily.
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