We all know it is healthy for us as humans to perform some form of physical activity for a minimum of 30 minutes every day. It does not matter what you do when you do it, or how you do it. It just matters that you do it and you do it every single day. It is this simple approach that could save your life.
Doing what I love, some form of physical exercise, may sound simple to many and if I loved doing it then why was it so hard. When I say I love physical exercise, I mean almost every bit of adventure out there. I participated in cycling, open water and pool swimming, surfing, paddling, rock climbing, trail running, and hiking.
Pompe disease is a rare inherited disease that inhibits your body to generate a protein that breaks down a complex sugar, called glycogen, for energy. Too much sugar builds up and damages your muscles and organs. Pompe disease causes muscle weakness and it mostly affects the liver, heart, and muscles.
Even before I was diagnosed in 2015, I was still trying to participate in as many activities as I could. Some days, however, it was hard to get out of bed. I would have gone cycling or surfing the day before and the next day I was in so much pain that I could not function normally and get along with my day as usual. I would spend a few days resting and not doing any activities until the pain subsided a bit to allow me to exercise again.
I would exercise gradually and slowly start building myself back up again after each bad ‘episode’ of extreme pain. I noticed that when I had this extreme pain that I would lose a lot of weight quite drastically. I first thought it was because I was not eating that much when I was not able to exercise. Then as I slowly began to build my body back up my weight would return to normal.
Further, I realized that when I was experiencing these episodes I was losing muscle mass in a matter of days. Then it would take many weeks and sometimes months to build back up to my normal weight. So, it would take almost 6-8 times longer to generate new muscle than my other friends or athletes around me.
Upon being diagnosed with having Pompe, I only then learned that all the exercise I was still trying to maintain was actually helping my body stay relatively strong. The exercise actually helped in keeping my glycogen levels lower, at least at a level where I could function fairly normally.
Pompe disease causes an excess accumulation of glycogen in the body. We are able to store glycogen in our bodies as it is the fuel that keeps our whole body going. It feeds all our muscles including our heart and lung muscles. However, in the case of having Pompe, my body does not stop storing the glycogen. It is this overflow effect that causes immense damage to my body’s cells and leads to muscle breakdown and weakness, which results in extreme pain in the body due to muscle myopathy.
Some days the exercise I did was walking in a pool, other days I was able to surf, and then I found cycling. I soon learned after trial and error, that cycling has a very low impact on my body and as long as I did not cycle with very high intensity, I was able to exercise for longer than any other exercise I was trying to do.
I have learned through everyday real experiences how my bike has taken me through places I would never have dreamed of, whether it be an emotional state or a baron, vivid landscape that I am riding through.
I started cycling more and more. Through cycling I gained the confidence to start doing smaller events. I did these events with the aim of raising awareness about the many rare diseases and trying to find others who need help in finding a diagnosis much as I did.
This goal is all I think about now and I started looking at all sorts of events to promote awareness. I started with longer single-day cycling events and then challenged myself to a 2-day mountain biking event, then 3-day events, and even gained the confidence to bravely take on an extreme event I once dreamed about doing, The Absa Cape Epic. It is known as the world’s toughest 8-day mountain bike stage race, the Tour de France of mountain biking.
My doctor told me it would not be possible to build up my body enough to do such an event. I had to train consistently for 10 months to build myself up for this test on my body. I realized that cycling causes far less stress on the body and my body could mainly rely on fat as a fuel source and that would result in better energy production so that my body would not break down when trying to make use of glycogen.
I managed to finish the Cape Epic and that has been the biggest challenge and achievement in my life. I went there to complete the event when many people said I couldn’t. And I reached a huge amount of people in the process, spreading awareness for rare diseases.
At the end of the Epic, I was awarded a prize called the ‘Conquer As One’ award. This award went to someone who completed the Epic and made an impression and a difference in other people’s lives. This award was chosen out of 1400 people and it got me great coverage for further awareness creation.
Every stage I finished in this race I learned something new about my body, about the disease, and that gave me strength knowing that I was doing it for a worthy purpose.
I finally found something positive that I could do with having this disease. Whether it be to inspire others to embrace and appreciate an active lifestyle, or help give people with a rare condition a little bit of hope and perhaps a path to test out in achieving a higher quality of life.