One of the first supplements that patients with mitochondrial disorders are prescribed is CoQ10, also known as Coenzyme Q10 or ubiquinone. The main reason that CoQ10 is so highly relied upon with mitochondrial dysfunction is that it is such a major player in cell function and the production of ATP – the actual “energy” that the mitochondria make on which our cells run.
As you know, there is no cure or specific treatment for mitochondrial dysfunction, but many clinicians believe that the use of high-dose CoQ10 with their patients may, at the least, slow the degeneration of the mitochondria and stabilize bodily functions in those disorders. While the research to support this hypothesis is not overwhelming, there are more and more research papers which tend to support the use of CoQ10 in mitochondrial disorders.
One such paper states:
. . . that oral administration of coenzyme Q10 increases both brain and brain mitochondrial concentrations. They provide further evidence that coenzyme Q10 can exert neuroprotective effects that might be useful in the treatment of neurodegenerative diseases.
Coenzyme Q10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects. Proc. Natl. Acad. Sci. USA, Vol. 95, pp. 8892–8897, July 1998.
On a more personal level, my daughter has been taking CoQ10 for almost two years now – ever since she was diagnosed with Complex I OXPHOS mitochondrial disorder. While I have not seen major improvements in her condition, I also have seen that, at least in her case, her cognitive and developmental abilities have continued to improve as she has gotten older – even when faced with severe regressions in the face of both illness and progression of her disease. I don’t want to roll the dice to find out if it makes a difference by stopping CoQ10 supplementation.
There is a clinical trial currently underway, however, that will hopefully shed even more light on this subject and is specific to mitochondrial dysfunction. The study was started in 2007 and has an anticipated completion date of May, 2009. There are three main objectives of the multicenter, prospective, randomized, double-blind, placebo controlled crossover trial of oral CoQ10 in children with biochemically proven deficiencies of complex I, III or IV:
1) Proving that supplementation of CoQ10 is safe and more effective in improving outcomes than a placebo.
2) Determining the effectiveness of CoQ10 in improving motor function and quality of life for the population.
3) Determining the safety of CoQ10 supplementation on the target population.
This clinical study is also supported in part by the National Institutes of Health.
My hope for this study? That CoQ10 will FINALLY be accepted as a medically significant treatment for children with mitochondrial disease and its cost will be covered by insurance companies, most of whom currently view CoQ10 as a “supplement” and “experimental” and not supported by peer reviewed literature and research.
So, to answer my question… we will be continuing CoQ10 . . .