Mitochondria, often referred to as the “powerhouse of the cell”, are structures located inside most human cells that produce 90% of our total energy needs. Through a process called oxidative phosphorylation, mitochondria combine the oxygen we breath with the macronutrients we eat to create cellular energy, called ATP. Mitochondrial disorders arise when insufficient amounts of ATP are created, damaging cells and compromising tissue and organs function.
Mitochondrial Disorders are the result of genetic mutations, which can be either inherited or caused by environmental aggressors. Inherited mutations are generally more widespread, and impact multiple organ systems. Symptoms can include muscle weakness, diabetes, hearing and vision loss, kidney failure, heart disorder, and dementia. Mutations caused by the environment are generally confined to specific organ regions and buildup over time, leading to certain forms of cancer and an increased risk of age-related disorders, such as heart disorder, Alzheimer disorder, and Parkinson disorder.
Mitochondrial disorder is difficult to diagnose as it manifests differently depending on the individual. If three or more organ systems are involved, mitochondrial disorder should be suspected. Although mitochondrial disorder primarily affects children, adult onset is becoming more common. Evaluation of a child thought to have a mitochondrial disorder includes a thorough medical history, physical and biochemical testing, MRI, and muscle biopsy. Once a diagnosis is made, the treatment plan can be determined. There is no known cure for mitochondrial disorder, though recent studies testing the Ketogenic Diet and its effect on mitochondrial disorder show promising results.
A Case for Keto
Nutrition has been the primary therapy for mitochondrial disorders along with supplements and medications. Unfortunately, as with all rare disorders, there are few studies to examine which therapies are best.
While the mechanism of action is still unknown, the Ketogenic Diet shows promise for fighting the effects of Mitochondrial disorder by increasing the rate of mitochondrial biogenesis, thereby reducing the energy deficiency that is the hallmark of the disorder. In addition to this increase in total mitochondria, the diet may also increase the energetic output of individual mitochondria, further reducing the energy deficiency. Several case reports have been published recently showing benefit of Ketogenic Diet therapy for mitochondrial disorders including Complex I, II, III and IV.
Think the Ketogenic Diet is right for you? Talk to your doctor before adopting a Ketogenic Diet, or connect with one of our qualified diet professionals to determine a course of action that is right for you.
What are the benefits of Ketosis?
Achieving a state of ketosis can have many benefits from treating chronic illnesses to optimizing performance. While the benefits are well documented, the underlying mechanism of action is not entirely known. The diet seems to enhance the ability of mitochondria, the power plants of our cells, to deliver our bodies’ energy needs in a manner that reduces inflammation and oxidative stress. Through optimizing the way our body uses energy, we fortify our bodies’ ability to take on the ever-growing stressors of our modern way of living.
Can a high fat diet be healthy?
According to David Diamond PhD, “Saturated fat does not clog the arteries: Coronary heart disease is a chronic condition, the risk of which can be effectively reduced from healthy lifestyle interventions. We have known for 150 years that a high carbohydrate diet contributes to fat in the blood and contributes to obesity.” He goes on to say, “the poor science that was used to support the idea that increased fat intake increases cholesterol and damages arteries was authored by Ancel Keys who had no background in nutrition, but did have a BA in Economics and a PhD in Fish Physiology. Keys’ theory that fat in diet causes increased risk for high cholesterol and heart attack was based on extremely flawed science and “became dogma without ever being rigorously assessed.” “A generation of citizens has grown up since the Diet/Heart Hypothesis (of Ancel Keys) was launched as official dogma. They have been led by the greatest scientific deception of our times: the notion that consumption of animal (saturated) fat causes heart disease.” George Mann MD, Nutrition Today, 1985. “The belief that atherosclerosis is due to high cholesterol has been perpetuated by powerful forces using tactics to preserve the profits and reputations of those who promote them.” Paul Rosch M.D, Scandinavian Cardiovascular Journal, 2008. “The diet heart hypothesis is sustained by social, political and financial institutions which have little to do with science and established success in public health.” Uffe Ravnskov MD PhD, 2008.
Ready to get started? Learn the basics…
The Ketogenic Diet, also referred to as the ketosis diet, or Keto for short, is a way of eating that mimics the effects of fasting. By consuming a diet rich in quality fats, adequate in protein, and low in net carbohydrates (total carbs minus fiber), the body’s metabolism begins to utilize fat as its main source of fuel, rather than carbs. This shift has profound effects on metabolism for both the sick and healthy alike. The diet shows promise for improving or reversing many neurological conditions and metabolic disorders. For the healthy, the diet represents a tool for preventing chronic disease, as well as optimizing cognition and body composition (i.e. fat loss).
What is ketosis?
The term ketosis refers to a byproduct of the breakdown of fat into useable energy, called ketone bodies, or ketones for short. This fat can be derived directly from the food we eat, or adipose tissue stored throughout your body (otherwise known as body fat). Ketones are used directly by the body to power itself. This breakdown of fat into useful energy is similar to the process that dietary carbohydrates undergo in producing glucose to fuel the body. In other words, ketones are to fat what glucose is to carbohydrates. Ketosis is defined as having blood ketone levels > .5 millimolar/L.
How do I get into ketosis?
There are two methods to make the metabolic shift from using glucose to ketones as your main source of energy.
Fasting – the method of complete cessation of caloric intake for a prolonged period of time has been used to treat disease as far back as 400 B.C. when Hippocrates, the Father of Modern Medicine, employed the method for a myriad of ailments. Though this should be done under medical supervision, fasting is a safe, effective (and, some would say, the easiest) way to get into ketosis, quickly. For the average adult, a 48-hour fast will generally result in ketosis. After this fast, adopting a Ketogenic Diet will allow you to stay in ketosis. We recommend starting the fast at least 3 hours before bedtime on the first day, and eating at the same time 2 days later. While fasting means many things to many people, we define it here as the total restriction of macronutrients. We recommend boosting water consumption in order to avoid dehydration, and many find black coffee or plain tea to help maintain focus and performance during the fast. Children go into ketosis much faster and therefore can be started on the diet without fasting.
Diet – adopting a high fat, moderate protein, and low net-carb diet, will result in ketosis, and will take 2-3 weeks to achieve this state, as defined above. The diet is most basically explained by the ratio of macronutrients (fat, protein and net-carbs) in your diet, as it relates to fat. A classic Ketogenic Diet has a ratio of 4 parts fat, to 1 part protein + carbs (referred to as a 4:1 ratio). This 4:1 ratio is the high end of the spectrum as it relates to fat intake, though modifications to the diet can see this ratio go as low as 2:1. The ratio you adopt depends on the therapeutic benefit you are trying to achieve as well as the diet that is achievable for your lifestyle. We will go into diet options below, but you can also link to them here.
How long should I be on the diet?
We at the Charlie Foundation believe that a 3-month commitment to the diet is a minimum commitment to allow your body to fully acclimate to the new fat based fuel source. Since most people following a western diet are not proficient at metabolizing fat optimally, this period allows the body time to become “fat-adapted”, utilizing dietary fat efficiently and effectively. There are a variety of nutritional plans that will enable a ketogenic lifestyle, and flexibility is one of the hallmarks of the diet that make it easy to adopt as a life-long tool to enhance your health. Our nutritionists can help figure out both the short and long-term options best suited for you and your lifestyle.
Am I a candidate for the Ketogenic Diet?
While the short answer is yes for the majority of people consuming a western diet, we urge you to consult your general practitioner prior to making the switch to Keto. The Charlie Foundation will provide you with the information and tools necessary to adopt the diet, and partnering with your doctor during this process will ensure the most therapeutic outcome. We also suggest that you connect with a diet professional who can help you form a plan in collaboration with your doctor, who may be less familiar with the diet.
Types of Ketogenic Diets
There are a variety of diets that will allow you to get into ketosis. The major differentiating factor between them all is the amount of calories that come from protein, carbs and fat, which are what we call “macronutrients”, or nutrients in our food that have a caloric value. The three macronutrients differ in many ways, namely, their caloric values, as well as how the body uses them. Fat is the most calorically dense macronutrient, having 9 calories per gram, compared to 4 calories per gram for both carbs and protein. In a homeostatic state, the body utilizes fat and carbs for energy production, while it uses protein to rebuild the cells of our body. While this is generally the case, an overconsumption of protein can lead the body to break down the excess protein into glucose (which is what carbs break down into)
- Classic Ketogenic Diet
- Modified Ketogenic Diet
- MCT Oil Diet
- Modified Atkins
- Low Glycemic Index Diet (LGIT)
- Intermittent Fasting
If you think you can benefit from adopting a Ketogenic Diet, we encourage you to consult one of our dietitians, visit a participating hospital, or contact us so that we may guide you towards the most fruitful option given your specific needs.