Pursuing my calling

Ever since I can remember I have been of the mindset that I have been given Mucopolysaccharidosis (MPS) II Hunter Syndrome for a higher purpose. A gift in many ways. I know it sounds strange to call a genetic progressive (and extremely rare) disease a gift. But that is how I honestly feel. The life I have been given – the experiences, opportunities, people I have met, and friendships I have made (and continue to each and every day) is amazing. I know it’s the person that ultimately creates the life they want to live, but MPS II Hunter Syndrome is part of me. I don’t think I would have had the experiences and live this amazing life if I did not have this painful disease. Of course, MPS II Hunter Syndrome provides many struggles, but to be honest I think the good has outweighed the bad. I am not saying there haven’t been tough days – trust me I have had many and will continue to do so. For those who know me though I don’t live a life where my disease defines me, but rather I believe my disease is just part of me. I truly believe this disease is my calling to make change in the lives of those with MPS II, MPS diseases, and all rare diseases.

I often get asked “Simon, how do you have such a positive attitude?” and my response is often “Better to be positive than taking the easy way out.” We all could feel sorry for ourselves and that would be such a waste and shame. We all have potential, it’s just a matter if you choose to use it or not. Remember there is always people that are worse off than you, so you have make the best of what you have been given. As India’s first prime minister, Jawaharlal Nehru said: “Life is like a game of cards. The hand that is dealt you represents determinism; the way you play it is free will.” Yes, I was not given a great deck of cards in having MPS II Hunter Syndrome, but I work with what I have and created a wonderful life for myself. I have amazing family / friends and now a new mission to make change through my iBellieve Foundation.

Back in the spring of 2010, I decided to make the very difficult decision to leave my terrific job at Right To Play, the leading international humanitarian and development organization using the transformative power of sport and play to build essential skills in children and thereby drive social change in communities affected by war, poverty and disease. As extremely passionate as I am about Right To Play (RTP) and the incredible work that RTP Founder, President CEO Johann Koss and his team do, I knew in order to make change (and achieve my personal goals) for MPS II Hunter Syndrome and the rare disease community I needed to be 100% dedicated to my iBellieve Foundation. I have taken with me huge learnings from my time at RTP, which have already and will continue to serve me well with iBellieve. The support of my friend Johann Koss and all my friends at RTP has made the transition smoother than I could ever imagine. I am a supporter for life of Right To Play – they are changing the lives of so many children worldwide. I know personally the impact that sport has made in my own life and the work RTP does is making the world a better for all of us to live.

The idea for iBellieve has built up over the last few years with my involvement in the MPS and rare disease community, beginning with my charity Bike 4 MPS ride down Vancouver Island in summer of 2002.

In November 2003 my life would change forever when I enrolled in a Phase III clinical trial for the enzyme, iduronate-2-sulfatase (I2S), that my body is lacking and ultimately causes all MPS II Hunter Syndrome medical complications. As there was no Canadian trial site, I had to make the difficult decision to leave my home in Victoria, British Columbia in order to move to Toronto and commute once a week to Chapel Hill, North Carolina for the next two years. At the University of North Carolina Medical Center, under the tutelage of MPS specialist Dr. Joseph Muenzer, I would receive weekly enzyme infusions. It was hard to leave my life behind in Victoria, but honestly there was no real choice as my family and I had traveled the world looking for a therapy/treatment that I was now actually going to receive. It was a very emotional and nerve wracking time, as well as exhausting, but I can say 7 years later that the clinical trial changed my health for the better. I don’t know if I would be here right now if I was not able to receive the genetically modified I2S enzyme (Elaprase) developed by Shire Pharmaceuticals.

Of course, participating on the trial had its ups and downs, with frights of initial allergic reactions that thankfully could be controlled by pre-medications. Looking back on the trial experience, it was exhausting traveling to Chapel Hill, North Carolina every week and I often wonder how we did it. But in the end it has drastically improved my health and stopped the progression of MPS II Hunter Syndrome. It does not regress anything that has already happened with the 26 years of cellular waste build up on my body; however my breathing, flexibility, mobility, energy level have all improved, and my organs have gone back to normal size. These are improvements that I never thought possible. More importantly, the younger MPS II patients, such as Trey Purcell, have benefited even more as they will not have as much cellular waste build up the younger they receive Elaprase. This treatment is our “cure” right now, but through my iBellieve Foundation it is my goal to fund research to cure MPS II Hunter Syndrome (and ultimately all MPS diseases) once and for all, so no patient (and families) have to live with the devastating effects of this painful disease any longer.

Once I returned from the weekly travel to Chapel Hill and was receiving my weekly enzyme infusions at Hospital for Sick Children in Toronto, as part of the clinical trial extension, I was told by the Canadian government that they would market Elaprase, but the Ontario government would not pay for Elaprase treatment for me and the other eligible MPS II patients in Ontario. There are less than 10 eligible MPS II patients in Ontario and 30 across Canada. As healthcare funding is a provincial mandate, each province has to handle drug funding separately. Another problem that goes hand in hand with Canada being the only developed country in the western world with no orphan drug policy. Elaprase is completely unaffordable to the average Canadian, but the federal government had no problem marketing Elaprase but the patients who need it most cannot get access to get treated. The only drug available to save my life and had been evaluated by every MPS II Hunter Syndrome specialist and proven to stop progression for this deadly disease, but now I will not be treated. After I had sacrificed so much of my life and seen the amazing benefits Elaprase has had on my health and the lives of younger MPS II patients, there was no way I was taking no for an answer. I had a plan to get Elaprase funded and hopefully it would work.

My good friend, Steve Nash, has seen me grow up with MPS II Hunter Syndrome and has witnessed the incredible difference Elaprase has had on my health. When he heard what was happening with funding difficulties, Nashy was more than happy to support and help put a Public Service Announcement (PSA) together. Once we released the PSA, major Canadian media publications, such as Globe & Mail and CTV Canada AM, were taking notice of this important health issue. This is only my story and difficulty with Elaprase funding, but unfortunately I am not alone in this struggle. No treatment for a rare disease has ever been funded in Canada without patient advocacy. There is also no funding available in the Health Canada budget for rare disease treatments or rare disease research.

When the Canada AM interview aired, supposedly the Ontario Health Minister’s phone was ringing off the hook. Finally our issue was being heard! Within ten days, I had an answer. Via snail mail, of course. My treatment was being funded by the Ontario government and Elaprase would be made available to other eligible MPS II Hunter Syndrome patients in Ontario. As a result of these advocacy efforts, MPS II Hunter Syndrome would also be the test for the new model of how drugs for orphan diseases would be evaluated in Ontario. This was definitely a victory! But we have a long way to go to create an Orphan Drug Act in Canada and give access and funding to available and new treatments for all rare diseases in Canada. Regardless of where you live in Canada you should be given access to available treatments – we should not be made prisoners in our own province. Last time I checked we are Canadians regardless the province in which we live.