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Imagine running along and all of a sudden feeling a sharp, tight sensation in your calf or quad muscle. A painful cramp like this can stop you dead in your tracks. This super intense pain and hard contraction can turn your muscle into a rock. There are many causes of cramps but generally, most are able to be tracked through something missing in your energy system to fuel your muscles correctly.

Most people don’t realize that we have 3 different energy-generating systems in our bodies. I am fortunate to have a degree in sports science, which has helped me understand and experiment with training to shift my body’s energy-burning system. Understanding how to regulate my body’s energy flow has helped me manage my Pompe disease.

Pompe disease is a rare, inherited, and often fatal disorder that disables the heart and skeletal muscles. It is caused by mutations in a gene that makes an enzyme called acid alpha-glucosidase (GAA). Normally, the body uses GAA to break down glycogen, a stored form of sugar used for energy. In Pompe, mutations in the GAA gene reduce or completely eliminate this essential enzyme. Excessive amounts of lysosomal glycogen accumulate everywhere in the body, but the cells of the heart and skeletal muscles are the most seriously affected.

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In very simple terms, glycogen is the stored energy in your body that fuels it. What happens when you have Pompe disease is that the body cannot break down this energy to use as fuel to keep it going, so it starts to breakdown muscles in the body to keep it going, which ends up causing huge amounts of pain, muscle trauma and weakness due to losing muscle mass so rapidly. 

When I was diagnosed in 2015, there was very little information given to me by my doctors as they also knew so little about this condition. My first step was what everyone I believe does, go onto Google and research more about what this condition is and how it affects the human body as well as the prognosis for the future. I was terrified at first, as all the information online speaks about the struggle for survival once diagnosed with this disease, but in searching medical journals and speaking with many other Pompe disease patients, I soon learned that most of the literature is related to the infantile onset of Pompe disease. 

Everything I read pointed to the accumulation of glycogen being the causal factor of issues in the body. I know that glycogen is made up of carbohydrates and sugars, so immediately I thought let me try and cut out these as much as possible in my diet. This made a substantial impact; gradually I started to feel my body function a bit better and some relief in pain.

The next thought process I went through was knowing that glycogen is essential in fueling my body and wondering where was my body now going to obtain energy to keep it moving without causing too many complications. Having knowledge of the 3 different energy systems in our body, I immediately knew that I had to cut down on all high-intensity exercise and movements, which cause the body to use our anaerobic glycolytic system.

I aimed to try and train my body to make use of only its aerobic energy system, which requires a combination of glycogen, fat reserves, and sometimes a small portion of protein. The main focus was to channel my body into making use of fat as a fuel over using glycogen as much. It was a difficult, long process of training my body to become fat adapted but like anything in training, patience and consistency are everything.

I set out to perform longer cycle and swim training in a fast state. This fast state is when you do not eat for fuel before or during training and only consume water while training. I used to drink a strong black coffee before training, which would help to stimulate fat oxidation and maximal aerobic adaptation. I was training my body to mainly make use of its intramuscular triglycerides over glycogen.

I have seen huge improvements with this type of training as it has helped my body function more effectively on fat and not rely on glycogen as much, which has most certainly stopped my body from breaking down muscle as quickly. The most important aspect to remember when adopting this training is you have to be very controlled and disciplined.