Many of you will have seen my Facebook post on the new Slice TV program, The Undateables, last week.
The program title really aggravated me as it personifies everything that is wrong with our society. Sure, it may be a reality show on dating and disabilities; however, why do the program’s participants need to be described in a negative context. Have our societal morals and values sunk so low that we consider any one with a so called “disability” (or differences) to be unattractive and therefore “undateable”?
As an individual with a disability and also an advocate for persons with disabilities, I find the program name offensive. It goes against everything persons with disabilities want in life, which is to be treated equally. In a society where it is hard enough on all levels, including love & romance, the media should not be portraying disabilities in a negative light.
In today’s world I am saddened that mainstream media still allows this type of behavior to continue; however, it is really a societal issue as we are the ones that watch these programs thereby driving up ratings and media dollars to feed the machine that pumps out this garbage.
Unfortunately the media is the root of the problem as persons with disabilities continue to be portrayed in less than favourable light than their so called able bodied counterparts. Until the media and our global society’s perception of disability changes, the struggle will continue.
Perception is reality until change.
I have lived my life as viewing my differences not as deficiencies and looking at my ability not my “disability”. I wish our global society would do the same.
As Canadian hero, Terry Fox, said “I may have a disability, but it’s not a handicap.”
Thankfully I have not been bullied too much in my life, however; remembering those rare occurrences still sting. One such occurrence happened recently when I was sneered at by a group of young adults while waiting in line at McDonalds. I am not going to lie, it hurt. The majority of the stares and/or sneers are water off my back, especially if they’re young kids as they are just interested and inquisitive.
Of course, I am very aware that I look different. But don’t we all? There is no such thing as “perfect” in my vocabulary. It just does not exist and the reality is we are all different and each and every one of us has a challenge or obstacle to overcome.
It may be that I am more aware now, but I do feel our society is far less tolerable of others than it used to be. Let’s remember differences are not deficiencies. It is not your place to put someone else down. We all have an equal right to be a contributing member of society.
Next time you see someone who is “different”, you have a choice. I recommend you send a smile, not a sneer. You might actually make yourself feel good.
I believe if we are more tolerable of each other, treat everyone fairly, we’ll have a much better world in which to live.
iBellieve – do you?
I have been “juicing” for 10 years now, but unlike some sportsmen we know, whose “juice” was destructive, my “juice” is productive and life-saving. Sorry Lance, Roger, Mark…etc!
10 years ago my life changed forever and set me on a journey unlike any other. 10 years later, after many highlights and low lights, clinical trials, different infusion sites, funding challenges, I now receive my weekly infusions at home. These enzyme replacement infusions have stopped the progression of my disease and have kept me alive – they continue to save my life.
None of us can determine our longevity; MPS II (Hunter syndrome) clearly marked my short stay. At the age of 25 I knew time was not on my side: statistically, I should have been six feet under. Yep, I had beaten the odds and I was the “best case scenario” but my disease was slowly progressing and taking over every cell in every part of my body.
It has never been easy to be told repeatedly throughout my life that I will not see the next year let alone the next day. Of course, MPS II is a progressive disease, so each day, week, month, and year I get is a bonus and a blessing but I was not chalking down the days to my departure. My physical outlook was not good, but this did not change my demeanor, attitude, and personality. I was still Simon!
My family had looked high and low all over the world for many years looking for MPS II treatment and therapies that didn’t exist and my mother would say “courage does not need to roar, the lamb will rise to be the lion.” The lion proved to be the Elaprase, my treatment and with your help this lion will roar louder as we move toward the cure. In the words of Journey “Don’t Stop Believing!”
What did I have to lose to be part of the MPS II enzyme replacement clinical trial? I was grateful to be accepted into the trial, especially at 25, and to be going to see world expert Dr. Muenzer at UNC Medical Center in Chapel Hill, NC every week on the trial. I was in good hands and at the very least if this infusion trial didn’t work out I could catch some great college hoops there.
Leaving my life and hometown of beautiful Victoria, BC was one of the hardest things I had to do. Going on the trial provided nervous excitement, and a chance of a longer life but I was also leaving behind my career and friends. I wasn’t ready to move to Chapel Hill, so I decided to move to my birthplace of Toronto. Not many people leave Victoria to move to Toronto. Usually the other way around. Our family had done just that in 1990! Thankfully some family, best friend, and the Leafs were in Toronto to make the transition easier but not the weekly travel to North Carolina, which was a whole different ballgame! Have you seen ‘Up In the Air’ with George Clooney? That is indicative of the two year weekly travels with my Mom.
I am so fortunate to have such a caring and loving Mom, who would not even consider or allow me to go through this alone. She put everything in her life on hold and made the weekly treks with me. Looking back I often question how we did it, but at the time you just do it. It became routine. Like clockwork. Each week we took the Sunday afternoon flight and returned after the infusion on Monday night. We knew every Air Canada front line staff, Toronto & Raleigh – Durham airport staff, and all the passport/immigration officers. The familiarity was nice as you could build a relationship with each of these individuals. They became part of the journey and shared a small, but important, part of the experience.
Looking back on that first infusion and walking into University of North Carolina Medical Center I had a lot of nervous energy and anticipation for what was to come in the next few years. My stomach was in knots, but excited that this experimental drug had the potential to give me the chance of some additional time. I had faith in Dr. Muenzer, his team, and Transkaryotic Therapies (TKT now Shire HGT). I had sacrificed a lot to participate in this trial and knew in my heart this was the right thing to do: I may have nothing to sacrifice if I didn’t do it —- it would not be only my health I would lose but my life. I am still here to tell the tale and so glad I made the decision to be a part of it.
I could write for hours about all the different experiences, amazing moments, and incredible people I have met in the last 10 years, but instead I thought I would provide my Top Ten and Bottom Five list, in no particular order.
- All the UNC Medical Center staff – friendships for life
- Meeting other MPS II patients and families
- Spending time with my Mom
- Airline & Hotel points
- Chick –fil-A
- College Basketball – UNC and Duke
- PF Chang’s
- Carolina BBQ
- Mark Kennedy’s trip to UNC and our road trip to Charlotte to visit Whits
- Infusion reactions
- Multiple IV injections – IV team is good, but MPS II patients have bad veins
- MRI tests
- North Carolina weather – they cancels a flight for a bit of frost? LOL
- RDU Airport Terminal (pre-construction)
Now ten years later, what an amazing journey my life continues to be. Sure I may not have been dealt the best “cards”, but what’s important is how I continue to play those cards. As one of my former professors and mentors, Dr. Martin Collis, always says: “The doors to life swing on small hinges.” It is so true. We must take what life brings our way and make the best of it. We never know where one small decision may lead us.
I really feel I have turned a not so good hand into the best hand in poker – a royal flush. Thank you to all the wonderful people in my life – my family & friends – who make it possible.
I look forward to the next ten years (and more)! I plan to be thanking all of you for making the cure a possibility – goodbye MPS II – no more of this devastating disease – more life – more joy and laughter and more friends like you who help make everything so much better. Thank you and please join the iBellieve Foundation and support MPS II research. www.ibellieve.com
“Do not go where the path may lead, go instead where there is no path and leave a trail.” – Ralph Waldo Emerson
I have never liked or referenced the word “normal”. What really is normal? If you ask me it sounds plain and generic. Aren’t we all uniquely different in our physiological make up? Isn’t that what makes the world special? Sure some of us are pre-disposed to challenges, be it physical, medical, or emotional, that make our lives different from the average person and more difficult. However, I have always been of the mindset that these “challenges” make us stronger. Differences are not deficiencies!
I often, today especially, give a lot of thought to my ability to cope with my rare and life threatening disease, MPS II (or Hunter syndrome), and how I have been able to persevere all these years despite the long list of medical challenges. To be honest though, I don’t know anything different. My physical being is what I have been given and I have to use what I have to my uttermost ability.
“People are always blaming their circumstances for what they are. The people who get on in this world are they who get up and look for the circumstances they want, and, if they can’t find them, make them.”- George Bernard Shaw
My “normal” is MPS II; however, it does not define Simon Ibell. It is the packaging, but it is not the whole person. My life is not defined by my disease. I have had to live within (and surpass) the medical challenges it has presented me time and time again. This is my single biggest obstacle in life, probably bigger & tougher than most people, but that’s what it is: an obstacle to some and a stepping stone for me.
My ability to continually push the boundaries put in front of me has been my ongoing desire to push myself to my limits and achieve any and all of my dreams I have for myself.
The way my family and friends dealt with my grim MPS II diagnosis instilled in me from my infancy has allowed me to believe in myself, regardless of the continuous medical obstacles MPS II presents me. The way my parents handled this initial diagnosis paved the way for my positive attitude to conquer this devastating disease. They have always taken the road of inclusion for me since that awful day, which has been my great asset.
My Mom never took NO for an answer and always believed in me and my abilities. She has instilled in me so many great qualities, but the number one thing I always mention is inclusion. I was included in everything growing up, from school, arts, sports, and community activities, which was the starting point for building my confidence. Her belief in me not only gave me the confidence in myself, but also helped others in my life to have further confidence in me and my abilities and the understanding that his disease would not stand in my way. It’s for this reason when I look in the mirror every morning that I see Simon not MPS II. This disease is only part of me, but it is not who I am. I am bigger than the disease, which never has and never will define me.
The road has not and probably will not be an easy one. I often have my down moments and wish things were easier. But at the end of the day I always remind myself that there is a greater purpose for me having this disease. I think I have come to realize that this is to inspire others with their own personal challenges and to reach my lifelong goal to cure MPS II through the iBellieve Foundation.
I get joy, especially when I surpass my doctors’ expectations; not only medically or survival, but other expectations with regard to my accomplishments and status. Regardless of such accomplishments, I am judged because of my looks. The constant stares results in judgment, which is only remedied when I speak and hold a conversation. In essence, content and not packaging is only fair judgment.
“Perception is reality until change”
As yet, the full story is untold. To date, I have had an amazing life with wonderful experiences, friends, and family. There is only one way to live life; to be positive, to work and play within our means, physical and otherwise. As long as I can remember, I have faced adversity head on and did not let it stand in my way. I will (and have) found a way to accomplish what I want to do in this life. MPS II may be seen as my obstacle, but everyone has their own MPS II (aka. obstacle) to overcome.
I know we will find a cure for MPS II, but I hope I can also inspire our supporters and others to overcome their own “MPS II”. We all have a challenge/s. Mine happens to create havoc with my entire body, is more profound and visible but I will never allow it to devastate my mind as it has with many that suffer from this terrible disease that has robbed too many children of a chance for life.
“Do not spoil what you have by desiring what you have not; remember that what you now have was once among the things you only hoped for.” – Epicurus
Please take a moment today to recognize those that suffer (and have suffered) with one of seven MPS diseases and think about joining our fight to find a cure. www.ibellieve.com
I believe – Do You?
We can. We do. We will.
Over the course of my life I have been told over 8 times that I have just a year left to live. Today, on my 35th birthday, I celebrate my life, and each and every person in it.
Here’s a snapshot of my first 35 years, including the creation and development of the iBellieve Foundation to find a cure for MPS II (Hunter syndrome) and 50 other related lysosomal storage disorders (LSDs).
- December 6, 1977: I am born at Toronto General Hospital
At the time, I was the longest baby on record at TGH, born healthy and without any complications.
- May 1979: I am diagnosed with MPS II (Hunter syndrome)
Early on, my parents were told I would not live to see my 5th birthday, then 10th birthday… I’ve been told approximately 8 times I would not see the next year.
Despite this grim outlook, my Mom and family would not take ‘No’ for an answer and provided me with all opportunities to be included in every facet in life, including: education, arts, community, and sports.
- August 1981: My sister Olivia is born
- September 1983: I had multiple surgeries for hernias and hearing tubes
I saw many specialists during his early childhood and teen years. My family and I traveled all over the world looking for therapies and/or treatments to support my long term health, but nothing existed.
This all occurred well into 1990s.
- June 1988: I got my first set of hearing aids as a result of the progressive nature of MPS II.
- March 1990: Moving to British Columbia for a potential treatment
My family moves west to Victoria, BC to be closer to Seattle for a potential bone marrow transplant. Unfortunately, my family finds out, at the 11th hour, that a bone marrow transplant will not work for MPS II patients.
I enrolled at St. Michaels University School (SMUS), a top Canadian private school. I am the first student to attend the school with a visible disability.
- April 1990: BC Children’s Hospital Visits
I visited medical specialists at BC Children’s Hospital in Vancouver about every month. These included: cardiology, pulmonary, endocrinology, rheumatology, etc. and lasted until 1996.
- April 1992: Bullying at SMUS
I suffer a bullying attack from four students who make a grotesque caricature of me in a wheelchair going over a cliff with statement “SMUS is not for the deformed”.
Note: I have never used a wheelchair.
I work with these students to improve their self esteem. Two of these students are eventually expelled, but two become acquaintances as years pass at SMUS.
- June 1992: Awarded SMUS Student Council Cup for Attitude & Effort
- September 1992: Sports at SMUS
I start Grade 9 and have to give up participating in contact sports due to size disproportionate with his peers and the potential harm it may cause. Coaches in baseball, soccer, basketball, hockey, and rugby, were always cognizant of my medical challenges and made sure he was never hurt during play.
In Grade 9 & 10: I manage Junior Boys Basketball and rugby teams. Travels on rugby tour to UK & Ireland in Grade 10. I was also awarded the SMUS ‘Soldiering On’ Cup in Grade 10 and managed the Senior Boys Basketball and rugby teams in Grade 11 & 12.
- March 1996: Awarded 1996 Kinsmen Youth Courage Award by the Kinsmen Foundation of British Columbia. The award was presented by Betty Fox (Terry’s Mom) in Vancouver
- June 1996: Graduated from SMUS as a British Columbia Academic Honour Roll Student
I was also awarded the SMUS Headmaster Award for Courage, SMUS Golf Team Award for Sportsmanship, and numerous individual academic distinctions in Grade 12 and throughout my time at SMUS
- July 1996: Decide to stay in Victoria and attend UVIC instead of Richard Ivey School of Business at University of Western Ontario
- September 1996: Start at University of Victoria (UVIC) in Commerce program and becomes the youngest manager of University of Victoria Men’s Varsity Basketball Team in history
- March 1997: UVIC Men’s Varsity Basketball Team wins CIAU (now CIS) National Championship in Halifax
- August 1997: I travel as manager of the Canadian National Student Team competing in the Jones Cup in Taiwan
- September 1997: YTV Achievement Award for Courage
I was the runner-up for this annual award for courage.
- January 1998: Human Potential – Dr. Martin Collis
I take undergraduate elective course, Human Potential, taught by Dr. Martin Collis (Pioneer of Wellness). Dr. Collis inspires me to share my story and is the critical catalyst to the beginning of my public speaking career.
By September 1998, inspired by Dr. Collis and my love of sport, I change my degree from Commerce to Recreation & Health Education (Sport Administration) in UVIC’s Faculty of Education – School of Exercise Science, Physical, and Health Education.
- May 2002: My First MPS Conference
I attend my first MPS Conference and it was an emotional and overwhelming experience as up until that point, I had only met one person with MPS.
I also meet Dr. Joseph Muenzer (University of North Carolina Medical Center – Chapel Hill, NC). I stay in touch with Dr. Muenzer and keeps updated on his innovative work for MPS II (Hunter syndrome).
- June 2002: I graduate with Distinction from UVIC
- June – July 2002: Bike 4 MPS
Bike 4 MPS was a bike ride that took place over 10 days from Port Hardy to Victoria. Inspired by Canadian icon Terry Fox, I saw the ride as a metaphor for the daily struggle for those living with MPS.
Many of my family, friends, including Steve Nash, Simon Whitfield, and Roland Green, joined the bike ride and participated in school talks along the way. Also, major contributions from Dallas Mavericks owner, Mark Cuban, helped increase awareness and support for Bike 4 MPS.
- March 2003: Awarded ‘Spirit of Sport Story of the Year’ Award at the Canadian Sport Awards
Olympic gold medalist Beckie Scott and figure skaters Jamie Sale & David Pelletier were also nominated in same category.
- May 2003: Awarded Golden Jubilee Commemorative Medal for Her Majesty Queen Elizabeth II
- July 2003: Nominated for David Foster Vancouver Island Award of Excellence
- October 2003: Elaprase and the UNC Medical Center
I put my Master’s Degree and career on hold in Victoria and move back to Toronto in order to participate in a Phase III Clinical Trial for Idursulfase (Elaprase) to potentially improve my life living with MPS II.
I would commute once a week for the next 1.5 years+ to UNC Medical Center (Chapel Hill, North Carolina).
- December 2003: Elaprase Reaction
I suffer severe reactions to the enzyme replacement therapy. There was worry that I may be allergic to the enzyme that could save my life. Thankfully, test results show that everything was ok to continue with the trial, but that I would have to be pre-medicated prior to receiving the enzyme infusion.
- November 2004: Elaprase
I begin to see the incredible difference Elaprase is making in my own physical health, but also the younger patients participating in the trial. Elaprase does not change any of the medical complications that have already occurred, but it improves one’s quality of life and stops the progression of MPS II.
Benefits include improved breathing (pulmonary), organ size decreases (back to a “normal” size), increased flexibility, energy level, and speech enunciation, to name a few.
- July 2005: Elaprase Trial Extension
As part of the Elaprase trial extension, my weekly infusions move back to Toronto at the Adult Pediatric Unit at Sick Kids Hospital. Instead of weekly travel to Chapel Hill, North Carolina, I visit with Dr. Muenzer for regular check ups every couple months.
- October 2005: Simon works at Right To Play as Manager, Athlete Relations & Communications
I worked there until April 2010, before establishing the iBellieve Foundation.
- August 2008: Elaprase Eligibility in Ontario
Due to the lack of a Canadian Rare Disease Plan & Orphan Drug Policy, the Province of Ontario refuses to pay for Elaprase for eligible Ontario patients. My good friend, Steve Nash, and I put together a media campaign to fight for me (and other Ontario MPS II patients) to gain access to Elaprase funding. We were successful in our efforts and Elaprase was funded for all eligible Ontario patients.
As a result, MPS II became the initial model for how orphan drugs are evaluated in Ontario. At that time no rare disease patient had their treatment funded without patient advocacy. Canada is the only country in the developed world WITHOUT a Federal Healthcare policy for the rare disease community. Approximately 1 in 12 Canadians (or 2.8 million) suffer from one of 7000 different types of rare diseases.
- September 2010: I launch the iBellieve Foundation to find a cure for MPS II (Hunter syndrome) & 50 related lysosomal storage disorders (LSDs)
- February 28, 2011: iBellieve launches ‘Be Fair 2 Rare’ campaign in the House of Commons in Ottawa on International Rare Disease Day
- February 2012: iBellieve hosts its first event at Tappo restaurant in Toronto’s Distillery District
- February 2012: I am honoured as 2012 University of Victoria Distinguished Alumnus, Faculty of Education
- October 2012: I am honoured as 2012 St. Michaels University School Distinguished Alumnus
- November 2012: iBellieve hosts ‘Can You Spell MPS II?’ photo shoot at Twist Gallery in Toronto
#iBellieve there is a way! We can. We do. We will.
As for many of us across Canada and around the globe, especially in the sports world, we are still in shock with the sudden passing of our dear friend Randy Starkman. I am completely devastated and saddened by his death and can only imagine what Mary, Ella, and the entire Starkman family are experiencing right now. I know tomorrow, Randy’s funeral, will be an extremely difficult day.
Randy & I first met 5 + years ago initially through our friend, Mark Brender, when Mark & I were both working at Right To Play. Even though Randy & I first dealt with each other in a professional sense through my job at the time as Athlete Relations & Communications manager at Right To Play Canada, we became fast friends. I’ll miss our socializing and many conversations about everything from sports, music, arts, and life. As a fellow fan and supporter of Canadian amateur sport and the Olympic Games, Randy’s stories and life experiences were incredibly fascinating to hear. I was always happy to listen and be in his company for hours. He was an incredible human being and the most genuine person I have ever met.
I’ll never forget the special dinner we had at Randy, Mary, and Ella’s house a couple years ago with my Mom, and our friends Beckie (Scott) and Spencer (Wynn). Randy made homemade cannelloni and the conservation (with wine) was endless and as enjoyable as the meal. As my Mom said afterwards, “It’s a rare occasion where you can meet someone new and know you respect them immediately.”
Randy was SO dedicated to his work and Canadian amateur athletes to ensure each of them had their stories told – not just every four years when the Summer or Winter Olympic Games came around. Randy followed all these athletes, as friends, he cared deeply for each of them and was on their team. He was one of them, in the trenches each & every day. He understood them and what it took to excel in Canadian amateur sport and he made sure the rest of Canada did too. As displayed in the last couple of days, you can see how well loved and respected Randy was by all these athletes and the journalism community. He was one of kind. Randy may never have won an Olympic medal, but he was an Olympian in every sense of the word. He epitomized the Canadian Olympic values of Excellence, Fairness, Fun, Respect, Leadership, Human Development, and Peace. He owned the podium!
As Adam (van Koeverden) mentioned in an earlier tribute, Randy personified the second meaning of the word Champion – “a person who fights for or defends any person or cause.” Not only did he do this for Canadian amateur athletes, he did this for many great causes, including Right To Play, Flat Stanley, and Be Fair 2 Rare™.
Right To Play is widely supported by athletes worldwide for the incredible work they do to improve the lives of children in some of the most disadvantaged areas of the world by using the power of sport and play for development, health and peace. Randy was an avid supporter and wrote many stories to support Right To Play, including athlete visits to Right To Play programs across Africa, Asia, the Middle East, and South America. Most recently, Adam van Koeverden & Clara Hughes’ visit to Mali. Randy was integral to putting Right To Play on the map in Canada and communicating the power of sport to so many wonderful Olympians and all of Canada.
Personally, Randy was an amazing supporter of me and my current efforts to find a cure for my condition MPS II (or Hunter syndrome) as well as advocating for a Canadian Rare Disease Plan through my Be Fair 2 Rare™ campaign. He wrote many articles to support my efforts and continually encouraged me to write more and to share my story & thoughts with others. The last time we saw each other was a few weeks ago at the official launch event of my Be Fair 2 Rare™ campaign on February 29, 2012 – International Rare Disease Day (see photo below).
I have cried several times since hearing the news on Monday, including when reading the wonderful tributes. Randy was a special man and left his mark on everyone he met. I will miss him dearly and will remember him forever.
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 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.