March 8, 2018

Treating Metabolic Disorders Using the Keto Diet

Treating Metabolic Disorders Using the Keto Diet

Ketogenic diets bring about a metabolic condition that is similar to a fasted state while still allowing for adequate nutrition. When completed correctly, they can do this without entering a catabolic state, which is a condition where the body begins to break down muscle tissue for fuel. For individuals with various inherited metabolic disorders, the keto diet can serve as a relief from many of the symptoms of these disorders.

Understanding the Metabolism

The metabolism refers to a collection of chemical processes that take place in the body to handle energy needs. Your metabolism dictates how well the body can break down carbohydrates, proteins and fats into smaller compounds to create fuel for the body. It also works to transform extra nitrogen into waste products that the body excretes. Metabolic functions also break down of convert chemicals to provide the right substance to help support cells.

Essentially, your metabolism is responsible for how effectively your body works to break down food into components that the body can then use to fuel, break down and build the structures in the body. Enzymes and proteins play an important role in the functioning of the metabolism. Components that the body can't use, it gets rid of. If the body can't use all of the nutrients from a meal, it can store many of the compounds for use at a later time.

People who believe they have a slow metabolism are typically simply referring to the fact that they continue to gain weight despite their diet and exercise efforts. A slow metabolism is very rare since the metabolism is simply a collection of processes that result in the conversion of food to energy. While it's possible to have a slow metabolism, it's a very rare condition.

Factors that are more likely to cause weight gain include your sex, age, body size and composition. Muscle mass plays a large role in how many calories you'll burn while at rest. Even people with more fat on their body will often burn more calories at rest. Additionally, eating too many calories, an inactive lifestyle, genetics and medications may also play a role. It's also important to get enough sleep to ensure the body is able to repair itself appropriately.

Common Metabolic Disorders

With a basic understanding of how the metabolism in the body works, it's easier to understand how the metabolism can support your weight loss goals. Many metabolic disorders result in a lack of enzymes to process certain foods. This often manifests in the form of a food intolerance rather than a true food allergy. It's usually a defective gene that causes the metabolic disorder.

With hundreds of possible metabolic disorders, your doctor must work with you on an action plan to help treat your disorder. It's important to seek treatment since a missing enzyme may result in a buildup of toxins or some substance that the body needs will not be produced. In most cases, if you have a true metabolic disorder, it means both of your parents were also carriers of the dysfunctional gene that is responsible for the disorder.

It's estimated that metabolic disorders only affect less than one-tenth of a percent of the population. The most common kinds of metabolic disorders include lysosomal storage disorders where waste products aren't properly broken down inside the cells. Galactosemia occurs when there is an inability of the body to break down sugar galactose. Maple syrup urine disease, which causes a buildup of amino acids. A tell-tale sign of this condition is that the urine smells like syrup. Phenylketonuria (PKU) where a lack of the PAH enzyme leaves high levels of phenylalanine in the blood. Glycogen storage diseases that lead to low blood sugar levels, muscle pain and weakness.

While all of these diseases must be treated by a doctor, glycogen storage diseases stand ready to gain the most benefit from a ketogenic diet. Research indicates that by reducing the load of simple sugars on the body, it's possible to achieve a reduction in symptoms. High-protein, high-fat diets with sufficient levels of MCTs produce ketones that are therapeutic for individuals who suffer from these conditions. These conditions are most often caused by a low activity of the phosphorylase enzyme.

Historical Treatment Attempts

High-protein diets have been mentioned as a treatment option for glycogen storage disorders since 1945. In an early case, doctors were able to increase nitrogen turnover by providing beef and bread late in the evening. This improved the overall energy levels of the patients. For a long time, this was the predominant method of care for sufferers.

In the 1980s, this premise was taken one step further. Protein levels were increased from about 13 percent of total energy up to around 20 to 25 percent of total energy. This necessitated a reduction in carbohydrates as well. Other approaches indicated a diet that uses 37 percent protein, 61 percent carbohydrates and 2 percent fat. All of these approaches showed benefits for the patients.

In a case of a brother and sister who both had a potentially fatal glycogen storage disease, the sister died at 11 months of age. In an effort to save the brother, a treatment utilizing D, L-3-hydroxybutyrate and a high-fat ketogenic diet were administered. Before the diet, the brother was moving towards the same fate as his sister. The diet was able to rescue the brother and he recovered.

The Problem of Carbohydrates

The issue for people who suffer from glycogen storage issues is that carbohydrates seem to overload the system. The body ends up experiencing a high-glycemic load, and it is unable to recover from this condition. Replacing the carbohydrates with either protein or fat helps to resolve the condition. This process effectively dilutes the carbohydrates in the system and the patient experiences an improvement.

Doctors in the 1960s did not understand the long-term health effects of a diet that was high in simple sugars. Enhancing the state of ketogenesis and gluconeogenesis results in a better overall level of health by reducing the glycemic load of a meal. Simply adding protein or fat to a meal will lower the glycemic index. As long as enough carbohydrates are included to eliminate the risk of hypoglycemia, the body is able to maintain a healthier state.

When the body switches away from depending on carbohydrates to supply glucose to the body for energy, it can process the small amounts of carbohydrates more effectively. Since a glycogen storage issue results in too much blood glucose, it's necessary to reduce carbohydrates. On a keto diet, the majority of the fuel will be provided through ketones and a few carbohydrates will keep glucose levels stable.

While reducing carbohydrate intake is important, it's crucial to avoid going the route that too many dieters take. They feel that is a simple reduction in carbohydrates makes me feel better, that a complete elimination of carbohydrates will provide the answer to all of their problems. However, the proper balance of carbohydrates, fat and protein must be maintained. Going too low will result in low glucose levels in the blood, which can create its own set of problems.

Treating Metabolic Disorders Using the Keto Diet

Getting the Right Carbohydrates

In addition to reducing the overall level of carbohydrates in the body, it's also important to get the right kind of carbohydrates. For the best results, aim to consume foods that have a low glycemic index to get the best results. Oatmeal, pasta, sweet potato, corn, yam, legumes, lentils and stone-ground whole wheat are foods which a low glycemic index. All non-starchy vegetables, dried beans and cereals tend to have low glycemic indexes.

For the best results, it's crucial to concentrate on the quality of all the foods in your diet. Simple reducing your carbohydrate intake to about 50 grams will result in a state that should help improve glycogen storage problems. This is part of the reason that the keto diet is so effective at treating these metabolic disorders. The higher level of protein and fat keeps blood sugar steady.

Glycemic control should be an aim of all individuals. It can help patients who have hepatitis with cirrhosis and individuals who have diabetes. While a shift to a ketogenic diet should help alleviate many symptoms, it's also important to realize that certain foods may keep you below the 50-gram carbohydrate recommendation while still spiking your blood sugar.

Stick to a keto diet and avoid foods with refined sugars for the best results. White bread, corn flakes, bran flakes and pretzels are all foods to avoid. While rolled or steel-cut oatmeal is acceptable, you should avoid instant oatmeal. It's also a good idea to avoid pineapple, melon, rice cakes, popcorn, saltine crackers, pumpkin and Russet potatoes.

Combining Foods

It's important to note that fat and fiber can lower the glycemic index of a food. In most cases, the more cooked and processed a food is, the higher its glycemic index will end up being. When you combine a high-glycemic food with a lot of fat or fiber, then it can reduce the overall impact of a high-glycemic index diet.

If you're going to count nothing else in your diet, you need to count the number of carbohydrates. This will help you improve your overall carbohydrate levels and ensure you are getting a better overall proportion of macros. The best diet for someone who is suffering from a metabolic disorder seems to be a high fat, medium protein and low carbohydrate diet.

For the typical person on a keto diet who doesn't have a metabolic disorder, the manner in which carbohydrates are consumed won't have a major impact on their overall weight loss and the effectiveness of the diet. When you're trying to manage symptoms, the best way to do this is by scattering your carbohydrates out throughout the day. Overloading on a single high-carbohydrate meal while still maintaining only 50 grams of carbohydrates in the day won't help you manage your condition.


For someone who is suffering from a metabolic condition, the diet won't solve every issue. If you're missing a crucial enzyme, your doctor may be able to help you restore your system with a carefully prescribed probiotic. While a ketogenic diet should be discussed with your doctor, it's important to remember that there is plenty of evidence to suggest the diet does help relieve symptoms.

Ketogenic diets are often the diet of choice for people who are suffering from specific kinds of metabolic conditions. When it comes to glycogen storage diseases and mitochondrial energy supply disorders, the ketogenic diet can provide an incredible benefit for people who are suffering. As a final word of caution, careful monitoring is important since catabolism can create a dangerous situation for someone who has a metabolic disorder.  GFXXMCMD151997672