Mitochondria are very small, rod-like bodies that live in the cytoplasm of most cells and play a large role in energy production. The cytoplasm is the large “living space” surrounding the nucleus of the cell. It’s where most of the major players of cell function reside, especially DNA. There are thousands of mitochondria in the body and they are located in virtually every cell except red blood cells. The mitochondria are the “power house” of the cell in which they take in nutrients and break them down, which helps create energy for the cell. This creation of energy is known as mitochondrial respiration. The number of mitochondria in each cell depends upon what the cell needs to do. For example, a nerve cell requires less energy than, say, a muscle cell which needs a massive amount of energy for growth and function.
Mitochondrial Diseases
When the mitochondria within a cell malfunction, it can be detrimental to the body and possibly fatal if symptoms aren’t treated adequately. The mitochondria are vitally important for the energy that is needed for the body to function properly and sustain life. If mitochondria fail, cellular death or injury follow and major organ systems begin to shut down. These diseases most often occur in the heart, liver, brain, muscles, kidneys, and respiratory systems, but can happen anywhere in the body. They are more common in children, but adult onset has become more common in recent decades.
Symptoms of mitochondrial disease include, but are not limited to, loss of motor function, muscle weakness and pain, swallowing difficulties and other GI problems, liver disease, cardiac disease, diabetes, seizures, vision and hearing problems, failure to thrive or grow, susceptibility to infection, and developmental delays. It is important to keep in mind that these symptoms could stand alone or be part of a syndrome, but if a reason for the symptoms is unknown, workup for mitochondrial disease may be helpful in determining cause and developing a treatment plan.
The cause of mitochondrial disease is not entirely clear, and studies have shown that it develops because of mutations in the mitochondrial DNA, both inherited and spontaneous.
While mitochondrial specific diseases are less common the number of children diagnosed each years is rising. In addition, many diseases not directly defined as mitochondrial disorders are being linked to dysfunction within the mitochondria. Alzheimer’s, Parkninson’s, ALS, obesity, diabetes and cardiovascular diseases are all being investigated for their links to mitochondrial dysfunction. Autoimmune disorders have also been implicated in mitochondrial dysfuntion including Sjogrens syndrome, MS, lupus and rheumatoid arthritis.
Treatment for Mitochondrial Diseases – A Nutritional Approach
Unfortunately, there is no cure for mitochondrial disease; however, there are some treatments that can help alleviate symptoms and slow down progression of the disease by re-energizing the cells, so to speak. Most of the symptoms can be treated individually with medications and other conservative treatments; however, dietary therapy has shown to be greatly beneficial for patients with mitochondrial disorders.
As above, these diseases usually occur in children, so patients with this diagnosis will need a pediatrician specialized in genetics and metabolic diseases. Adults with the disorder can seek out metabolic specialists as well. While there is no cure for the disorders, a proper diet can go a long way to alleviating symptoms and helping cellular function and growth. However, it is important to note here that it is imperative to speak with both a dietitian and the treating geneticist or physician to develop an appropriate diet plan because there could be underlying conditions that would be exacerbated by this dietary therapy. A dietitian who specializes in metabolic disorders would be most beneficial in these cases.
One thing to be sure to avoid is fasting (prolonged periods without food). For patients with mitochondrial disorders, lack of nutrition can actually increase symptoms of cellular injury or death and cause more problems, so fasting should never be an option for these patients. Therapists recommend several small meals throughout the day rather than the typical 3-meal-a-day approach. Eating before bedtime is even considered okay. Patients with severe mitochondrial disease may need to be hospitalized if they become ill and cannot take in or hold down food (such as with the flu or other GI condition).
A high-fat diet is recommended for patients with mitochondrial disorders. While there is some debate regarding its efficacy, many patients and studies have shown positive results. Most patients with mitochondrial disorders are underweight, so gaining weight would be a good thing. Medium-chain triglyceride oil fats are recommended, such as butter and coconut oil. Since there is conflicting evidence depending on patient metabolism and underlying conditions, this may also go the other way in which certain types of fats need to be reduced. This is why a dietitian who specializes in these disorders will be most helpful. The patient will need blood work and labs to determine what kind of fats their body will and will not need to help treat the disorder.
Another important nutrient to pay attention to is iron. Theoretically, iron creates free radicals which can be extremely harmful in patients with mitochondrial disorders. The free radicals could “poke holes” in the mitochondria, making the disorder and symptoms much worse and more difficult to treat. For this reason, excess iron should be avoided.
Vitamin C intake should be monitored closely because it enhances iron absorption from the GI system. Vitamin C is a good antioxidant and can be helpful in patients with mitochondrial disorders, but this needs to be monitored closely if the patient takes in iron-rich foods.
Conclusion
Mitochondrial disease is complicated and can be debilitating if not treated appropriately. Mitochondria are being implicated in many diseases that are affecting the majority of Americans today. These small organelles within the body’s cells are vital to proper functioning, so if the disease is diagnosed or dysfunction is suspected, talk with a metabolic specialist immediately about developing a proper dietary therapy plan.
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