Justin

Sometimes life can be serendipitous. Case in point - MPS VI is so rare that only 4 or 5 known cases exist in all of Canada, a country of 35 million people. This week, I received an email from Justin Van Herrewegen, pictured above with Isaac at the Tim Horton's this evening, a 29 year old MPS VI sufferer who lives only an hour away from us.

Justin was first diagnosed with MPS VI when he was two years old - a time when there was little or no research being done on rare diseases and the outlook didn't look promising for those affected with this disorder. And while Justin was told he had a "mild" form of the disease (Isaac, apparently, has an advanced form), his experiences with MPS have been anything but.

Since age two, Justin has had numerous surgeries that stem from the effects that MPS has had on his body, including double hip replacements, heart surgery, and spinal cord surgery. He has lost hearing in one ear and, most recently, has had a tracheotomy that has resulted in him losing his ability to speak.

Justin contacted us this week because he was recently turned down for funding of the life sustaining treatment (Enzyme Replacement Therapy) that he requires by the Province of Ontario, and he didn't know where to turn for help. We went through the same battle for Isaac 4 years ago, and we are happy to help Justin fight today.

The government's rationale for turning Justin down for treatment is presented in a flawed argument - an argument that should be looked at closer and reversed in order to provide Justin with the Health Care he desperately needs in order to stave off, or at least slow down, the progression of his disease. The province contends, wrongly, that they refused funding for Justin based on "the very minimal available data on the natural history of MPS VI upon which to model potential treatment benefits." In addition, Justin's very impersonal letter of rejection from the Province continues that "there is currently a lack of data to clinical benefit to slowing and/or stopping disease progression with Naglazyme treatment of MPS VI at this time."

Clearly the Government of Ontario didn't take the time to note that MPS is a rare disease and, as such, minimal available data is all that exisits now, or will ever exist in the future. Data is hard to come by, considering there are only 4 other people in the country affected by the disease. However, this hasn't stopped Ontario from funding Isaac's treatment, and it hasn't stopped the BC government from funding treatment for one of their children. In addition, it hasn't stopped the Quebec government from providing the necessary and life sustaining treatment to their two affected treatment. In fact, it hasn't stopped numerous countries around the world from providing the necessary health care to their citizens affected with this disease, regardless of how minimal the "available data" is.

The fact of the matter is plain and simple - strong evidence exists within this province (through Isaac's experiences) and throughout the world, to show that the treatment being rejected by the government DOES slow the progression of the disease in most individuals. In addition, by slowing the ravages of the disease, ERT enhances the quality of life experienced by sufferers.

Enzyme Replacement Therapy isn't a cure - it's a lifeboat - but it's a lifeboat that Justin deserves to be on, just like the rest of us that are waiting until the day we're rescued from this incredible journey. Treatment has saved Isaac's life - it's increased his quality of life, and we're forever indebted to the Ontario government for providing funding for our son. We now call on the province to do the same for Justin.

Truth be told, seeing Justin today reminded me that we are still in the war of our lives, and while we seem to be winning the battle, we still have a long way to go. And it scared me a bit too, which, I guess, is why I'm up in the middle of the night updating our blog.

We'll keep you posted on our progress. If you can, please help by reblogging and commenting on this post because, in politics, there is always strength in numbers.

Thanks again...with Love,

A.

This is my son, Gabriel.  Four years old, and full of energy, Gabriel has been Isaac’s  best friend and constant companion during the long journey our family has be on since Isaac’s diagnosis.  I wanted to write a quick post about him today because he often gets pushed aside when we are at Isaac Foundation events and, at times, it’s tough to see.

When Isaac was diagnosed, we were told that 1 in 4 of our children would suffer from the same disease.  Trouble was, we were already 7 months pregnant with Gabe when we got this news.  After two tense months of waiting and hoping, we got the news that Gabriel was healthy and didn’t suffer from MPS VI.

By nature, Gabriel has never wanted to be front and centre whenever people are around.  When the boys are giving their daily concerts in the living room, Gabe never wants to be John Mayer - he’s more then happy to be Steve Jordan (or, at times, Pino Pallidino).  But, truth be told, it’s Gabriel - not Isaac - that wants to be a Rock Star and follow in his hero’s footsteps.  Isaac, on the other hand, wants to be a DJ and we’ve spent countless afternoons listening to the boys plan their future collaborations together.

In public, Gabriel is shy and reserved, often using Isaac as his official spokesperson.  But if we’ve learned anything through the experiences that we’ve had together as a family, it’s that Gabriel takes everything in - he sees, hears, and feels exactly like the rest of us.  During our visits with John Mayer and Roy Halladay, Gabriel doesn’t feel much like speaking.  Last week, he did summon the courage to give John a big hug, which was a big step for our shy boy!  However, when we get back in the car, he can’t stop talking about how excited he was and wondering “when we’ll get to see John Mayer again!”

At Isaac’s school, where they constantly run fundraisers in support of The Isaac Foundation and our search for a cure, the kids always walk by Isaac with a high-five, a “Hi Isaac!”, or drop over for a hug.  Often times, Gabriel gets left behind and I struggle with how to handle things.  But Gabe is resilient, and it doesn’t seem to bother him.  I think this quality in Gabriel, wanting the best for his brother but not wanting anything for himself, is what endears me to him and his personality that much more. I think it’s ME that struggles most when I see him being ignored, and I think I just have to learn to adapt to it.

Though quiet, Gabriel has been the perfect friend for Isaac during some difficult times.  They spend each week at the hospital together, often snuggled up in the hospital chair as Isaac receives his enzyme infusion.  They share a bed in their “big boy room”, and one of my greatest joys is listening to them as they lay in bed and chat until they are tired enough to fall asleep. I hear the sweetest things they say to each other and, listening to them late into the evening, I just know everything will be OK in our lives because we all have each other.

Thanks again for your support,

Best,

A.

Questions

"Does this mean I'll get away from having MPS VI now?"

This is the question that Isaac posed to Ellen before dinner tonight. We had just received a call from Dr. John Hopwood, one of the leading researchers on the planet in regards to lysosomal storage disorders. Dr. Hopwood was calling to discuss some of the findings in his latest research project, a project partially funded by The Isaac Foundation, and Ellen was explaining some of the exciting news with Isaac when he asked the question.

This has been happening more and more lately, and it's been very difficult for me to try and answer. Not because I don't have an answer for him, and not because I don't have hope, but it's difficult because I know how very much Isaac is thinking about his disease, and I can see how he is struggling to understand how and why he is different then the other kids around him. He wants be the same height as his brother, and he wants to be able to climb to the top of the climbers without having to have mom or dad there to spot him. And he wants to not have to go to the hospital anymore. But mostly he just doesn't want to have MPS VI, and it breaks my heart to see him struggle.

Thankfully, Ellen is handling things well - she's a pro, really, and I envy her strength as she calmly discusses Isaac's disease with him and as she patiently shows him that MPS simply makes him that much more special. And then he'll smile and say "you're right!" and the struggle is done until next time.

I can't wait for the day when Isaac asks that same question and we can emphatically say "Yes!" What a wonderful celebration it will be.

The photo above was shot by John Mayer last week during our stop-in to say hello. Was too cute not to post, and I don't think he'll mind.

Thanks again for your support. You can't imagine how much we appreciate it.

With Love,

A.

Celebrity, Media, and The Truth

Celebrity is a curious thing. You work hard at what you do, people fall in love with you, your product, and the image you created, and you're on top of the world. Next, those same people that helped you make it big, with the help of the "media", start the process of tearing you down. Piece by piece they work their own craft, finding and distorting the small flaws in you that are (or should be) inherent in all of us, until they're satisfied that you can be judged by the public on the basis of rumour, innuendo, and lies, and not by hard work you put into your craft, the changes you effect in the world around you, or the impact you make on those less fortunate than yourself. Such is the case of John Mayer, and it's a shame that the media refuse to promote him for the kind-hearted soul that he is.

I logged online this morning and caught an article by Craig Outhier in the Phoenix New Times that calls Mayer the "douchiest douche of all time", "dismissible", and "uncharitable". Furthermore, Outhier goes on to suggest that the now infamous playboy interview that John did in February was simply a way for Mayer to further his career and spark interest in himself and pad the number of followers he has on his twitter page - a naive and foolhardy suggestion at best, and an outright lie, mischaracterization, and devious attempt to attract his own attention at worst. Regardless, Outhier's "article" in today's Times seems to accurately reflect the mission of most "media" outlets in today's age - build up, pile on, take down, and move on.

Outhier, and the other "journalists" that troll through celebrities lives and fixate themselves on the flaws of people like Mayer, force themselves to look past the good they do with their time and the impact they have on others, in order to sell print and make money. It's cowardly journalism at it's finest, and I'd like to believe that writers like Outhier didn't begin their career believing they would be doing their profession proud by publishing such biased and embellished trash and calling it news.

John Mayer is a favourite for news-outlet fodder, a favourite for biased bloggers to target and tear-down. Conveniently, however, these outlets leave out honest deeds that Mayer does whenever they write their tripe, and the decision makers that OK the publication of such drivel should be shamed because good writers don't overlook the truth before them, and good writers present the whole story.

For me, the whole story on John Mayer, his life, and his career should include the past 10 years he's spent honing his craft in the music business, crossing genre's and creating a unique style of sound that will last for generations. For me, the whole story on Mayer should include the countless children (like my son) that he has spared time for in order to make their world a better place, if even just for a moment. It should include the high-fives, the hugs, the smiles, and the words of encouragement he's given to those in need of the simple things in life. It should include all that he does for others, even if he doesn't go out of his way to broadcast it himself.

The John Mayer I know isn't a "douche". He's kind and caring, and sincere. The John Mayer I know was mortified and hurt at the interview that was published in February and would NEVER seek to further himself through other people's hurt. The John Mayer I know takes pride in helping others, and goes out of his way to make my son smile. As I type this entry, I'm listening to Isaac ask his mom why he has to have MPS VI, and it's breaking my heart. Of late, he's been struggling to understand why he drew this difficult lot in life, and I sometimes don't know what to tell him. But in the same breath, he's been able to find some small comfort in his unfair disease because of the love and care he's found in people like John Mayer. John's not "dismissible" or "uncharitable" by any standards, and the proof is in my son's smile.

If there's hope for the profession and for journalistic integrity, I challenge Outhier and those like him to put aside their bias and write a real, balanced story on Mayer. Salvage your integrity and do what you trained to do, which is write the truth, because to me, knowing what I know of John Mayer, you've done everything but.

P.S. I snapped the photo to the left this morning while Isaac was at the hospital. Instead of dragging his IV pole into the playroom, he chose to lay in his chair and listen to his hero on the iPad, a lasting glow from his time at the show last night.

P.S.S There's also a photo of John signing a guitar for our charity to auction off at our upcoming Gala, co-signed by Isaac himself.

Take Care and, as always, Thanks for your support.

A.