Run for a Cure – Training – Ontario

Today’s short and slow run took me through the streets of downtown Toronto, and past the Ontario Legislature. I used to think that real change happened in that building – then my son was diagnosed with a rare disease. Suddenly, it became evident that we have a health care system that touts itself as the best system in the world, but only if you don’t need access to expensive medicines to stay alive. It dawned on me early in this battle that real change doesn’t happen in that Legislature or by the people elected to work there – it comes from us. It comes from the people who need it most.

Had we not worked so hard to change public policy, the people in that Legislature would have sat back and watched our son die. Same holds true for the other kids diagnosed with MPS in this Province. The people in that Legislature believe that treating symptoms is better than treating the disease – because it’s cheaper to do so and helps to keep their budgets in check. But tough action, determination, and a brave fight by our kids and families has helped ensure access for many who need it. There is a new battle brewing, one that will take place in the very near future – a battle for access to treatment for our MPS VIA kids. I’ll be there to lead the charge because I believe in a health care system that is fair and equitable for everyone – even if you happen to have a rare disease, even if you need access to an expensive medicine to stay alive.

So – this warm up run is dedicated to the incredibly brave families I’ve met here in Ontario, and to the patients who are the bravest of all. I’m so lucky to know you and I’ll be running for you in Ottawa. This is for Isaac, Jasper, Lillian, Jack, Avery, Luke, Zane, Derric, Dawn, Riley, Tyler, Jordan, and Ayub!

Godspeed, Heather. You Will Be Missed!

As the Alberta Election race enters the final hours, so too does the official time in office for one of the most honourable and compassionate souls I have ever met. Once the results are announced tonight, the retirement of Heather Forsyth officially begins, bringing an end to a highly successful political career.

I met Heather a few years ago when I was struggling to find help for a little girl, Aleena Sadownyk, who needed immediate access to life-saving treatment.  Because this treatment is expensive (the fourth most expensive drug in the world), we were getting nowhere with the PC government, at that time led by Premier Alison Redford.  Desperate for help, I contacted Heather to see if there was anything she could do in her capacity as the Official Opposition Health Critic.

The moment I spoke with Heather, I knew I had connected with someone that would do everything in her power to help this little girl.  She listened to everything I had to tell her, keyed in on the most important facts of our case, and sprang to action immediately after our initial phone call was over.  I was impressed, and had Hope again for helping little Aleena.

Now, a quick aside.  Over the years, I’ve had the pleasure (sometimes) of working with many politicians throughout the country.  Oftentimes, these politicians offer their help during advocacy cases to further their own stature within political circles – helping with these advocacy cases usually translates into scoring political gain against the government of the day, and members of the Official Opposition jump at the chance to score such points.  I’m not naive in the slightest – I understand that many politicians get involved in my advocacy pushes because of the prospect to score those sought after blows against the government.  And I’ve never minded that fact – I’ll do anything to help our kids suffering from rare diseases, and if a politician wants to help out – for whatever reason – who am I to turn down the help offered, especially when we are talking life and death situations for our kids?

4d5f8030f60e11e2ad2b22000ae80c6b_7While I was impressed with the speed that Heather and her team sprang to action to save Aleena, I was more impressed that I felt she was doing it out of a love for helping people, and a passion for doing what’s right.  Moreover, I truly felt she was working as hard as she did because of the heartache and heartbreak she felt for what Aleena and her family were dealing with.  However, my close friends were skeptical – they have heard me speak often about the help I’ve received in the past and they were sure Heather took this case on for those same reasons.  They were sure she was doing this for political gain and for personal ambitions within that political world.  I vehemently disagreed – I consider myself a good judge of character, and I was sure that Heather was one of those rare people in the world of politics who were helping because they could, and because she felt that it was incumbent on her to make a difference in the life of this little girl if she were able.

And you know what?  My first impression was right.

Shortly after Aleena had her treatment approved – solely due to the hard work of Heather and her team led by Matt Solberg – Heather shared with me that she would not be seeking re-election the next time the Province when to the polls.  Win or lose, Heather didn’t have a political future she needed to worry about.  Political gain didn’t play into the situation at all, though I felt that was the case all along.Aleena is doing incredibly well today – she had her life saved by one of the most caring and compassionate people I’ve ever met – ever – in the world of politics.  When she looks back on a career filled with a long list of accomplishments – from serving in Cabinet to being the Leader of the Official Opposition – I know that Heather will be able to remember Aleena and the impact she had on her life as being one of the most satisfying and rewarding moments.

I’m sad to see Heather leave the world of politics.  She’s one of a kind – one of the few that go into the office everyday to try and make the world a better place for those around them.  She’s always been honest, truthful, kind, caring, and compassionate.  And she saved the life of a little girl.

0b6291d2079211e3943422000a9f1416_7The Alberta Legislature is better for having Heather serve amongst its storied halls, and the people of Alberta are better for having her represent them for so very long.  Personally, I’m a better person for knowing her, and I’m proud to call her Friend.

Godspeed, Heather Forsyth.  Enjoy your retirement, it’s well deserved.  Thank you for your service and your love for our kids battling rare diseases.  We will be sure to send you updates on Aleena as she makes her way through school, as she gets her first job, as she walks down the aisle on her wedding day.  Thank you for your kindness, for your love.  We will forever be indebted to you for that, and more.

Run For a Cure – For Jack Higginson

This warm-up run is dedicated to Jack Higginson, a sweet little boy battling MPS II. This run was a little longer, and a bit more difficult. But thinking of the pain and heartbreak Jack and his family suffer through day in and day out as they battle this disease gave me the motivation to keep going and finish strong.
Families dealing with the diagnosis of MPS II have the double agony of diagnosis coupled with the excruciating wait until age 6 or 7, an age where they will know whether their child is battling cognitive impairment as a result of the disease. 

MPS is a tough battle, and the strength, courage, and determination it takes to battle through a diagnosis of Hunter Syndrome is something that only the strongest people can undertake. Jack’s family have been incredible advocates and fighters for our kids – the strongest people I know. They are kind, caring, and compassionate, and will be a big reason why we find a cure for Hunter Syndrome! This one was for you, Jack. And for you lovely Higginsons! See you on race day!

To Change The World – Treatment Approved in New Brunswick!


Never 
doubt that a small group
 of thoughtful, committed citizens can change the world; indeed, it’s the only thing that ever has. – Margaret Mead

The quote above is one that we have displayed prominently on our website, and they are words we live by.  Existing in the world of rare diseases requires one to have such a mindset – to make a difference in the world of rare diseases, you have to begin with a small group of people.  Here in Canada, Isaac is one of just thirteen individuals that suffer from MPS VI.  In the broader family of MPS, we’re still talking less than 250 people battling the disease country-wide.  That’s 250 people out of 35 million (or just .0007% of our population, for our mathematician supporters!)  In essence, we’re a small group.  But we think big, and truly believe that we can change the world for our kids.

I have this quote on our website for many reasons – one of them being to remind myself that things are possible, even when we’re facing odds that most people would bet against.  Little did I know, that quote would help a government approve a treatment for a little girl who desperately needs it.  Little did I know, that quote would help save her life.

The photo above is of Kamie Babineau, a beautiful little girl from a town near Moncton New Brunswick.  Kamie’s mom, Parise, connected with me in December 2014, worried that her daughter was suffering from MPS VI – the same disease that Isaac has. The same disease that Jasper and Aleena and Violet have.  The same disease we’ve worked tirelessly to find a cure for, the one we’ve had to battle governments to provide life-saving treatment for.  Parise was obviously worried for her daughter – she sent me a photo of Kamie and one of Aleena that she found in a newspaper report and felt they shared the same features.  One glance at Kamie’s photo told me she was on the right track, but confirmatory tests needed to be done before we could begin the process of setting up treatment for her.  Confirmation of MPS VI was given at the end of January, and an application for reimbursement for treatment went into the government of New Brunswick in early March.

If you’ve been following our blog, you’ve seen how difficult it is to access treatment for MPS VI in Canada.  It’s expensive (the third most expensive drug in the world, according to this website and many others like it,) and governments are reluctant to cover the cost of the treatment for our kids.  Reluctant, even though it’s life-saving.  Reluctant, even though it gives our kids the chance at a healthy life.  Reluctant, even though it staves off the ravages of the disease, slows down or halts its progression.  Reluctant, even though the cost of not treating the disease is sometimes far more costly than treating it when you take into account the costs associated with managing the multi-systemic failures that ensue in children without access to therapy.  If you need some reminders on how difficult it is for our kids to get access to treatment in this country, have a look through this collection of news articles and stories.  To summarize – it’s difficult, and treatment is rarely approved without a fight.

DSC_0144I had been told that New Brunswick would be especially difficult to deal with.  Indeed, I had already reached out to the Province a few months earlier upon learning that a boy with MPS IVA needed access to treatment and the provincial bureaucrats wouldn’t accept the application for funding because they didn’t have a process in place to review the application.  I called and stated my concerns and frustration that an application wouldn’t even be accepted for this child, let alone reviewed and denied.  I was told to wait until the bureaurcratic process of a CDR Review was completed and that they would not consider any request for treatment until that time (that review gets completed in 2 days from now!)  So, I was prepared for a difficult start to accessing treatment for Kamie, and I wasn’t wrong when I began the process a few weeks ago.

I again called the bureaucratic branch at the Ministry of Health in New Brunswick that looks after pharmaceuticals, and talked to the same person I had talked with many months earlier.  I patiently explained the situation.  I had previously sent an email with background information (it wasn’t read until I was on the phone with them), and explained that there were already 9 other children in Canada receiving the same treatment that Kamie desperately needed.  I offered to provide all the resources they could ever need to produce a review of the application, including the International Treatment Guidelines (which assert that treatment should begin immediately after diagnosis for best outcomes and that the primary way to deal with this disease is to provide the treatment we were seeking), and the recent 10 Year Resurvey Data (which shows the incredible outcomes for patients with access to treatment over a 10 year period).  I also sent in a full folio of MPS VI and other articles showing the benefits that treatment brings to kids suffering from this disease.  I offered to connect them with the leading MPS VI experts throughout North America to help them with the data.  I expressed and provided ample evidence to show that symptoms that appear prior to treatment beginning cannot be reversed.  Many of these symptoms are devastating, and I expressed that we were racing against time to stave off such symptoms and provide Kamie with the quality of life that she deserved, not the quality of life that a disease untreated would leave her with.  At the end of our conversation, I asked how the application would be reviewed and when an expected decision would be rendered.  I was told, quite bluntly, that they had “no idea.”

This isn’t uncommon when dealing with this disease and applications for reimbursement of treatment.  When a disease affects .0007% of a population, we can’t expect governments to know everything about the disease and the process that would be used to review applications for treatment.  However, what should be expected is that these types of situations are taken seriously.  What should be expected would be for the bureaucrats to spring into action, figure out what review process was needed to deal with the situation, with an immediate and expeditious review undertaken.  This is not what happened, unfortunately.  Two weeks passed with the same update – they had no idea how they would review the file.  In fairness, they did express their hope that it would be approved, but couldn’t figure out exactly how to initiate the file set before them.

DSC_0201From here, my impatience got the better of me.  But it’s an impatience that stems from necessity – necessity to get treatment initiated immediately, the knowledge that Kamie’s disease is progressive and unrelenting, the hope that she can get the help she needs before her body is further deteriorated by MPS.  This impatience is where the story changes for the better.

I placed a call into the Minster of Health, Mr. Victor Boudreau.  I’ve been fortunate to work with may of the Ministers throughout the country, and I hoped he would take the time to get back to me, hear my concerns, and put a plan in place to look after Kamie’s application.  Mr. Boudreau had his staff look into the issue.  We connected numerous times over email, and I provided him with details about the frustratingly slow action being taken by the Ministry.  What ensued thereafter is something I’ve not seen in this country, from any official dealing with treatment for MPS VI – a determined effort to review the application for funding, and a promise for a prompt resolution/decision for the family.

We scheduled a direct meeting with Mr. Boudreau at his constituency office in Moncton.  I hopped on a plane, met with Parise and her husband, and planned out how we were going to handle the meeting.  In previous fights for treatment for other kids, we learned to be prepared for anything, and we learned to show governments that we would do anything to help save our children.  We were prepared to let Mr. Boudreau know that we weren’t going to go away, that we would do everything necessary to help save Kamie.  We were in this for the long haul because treatment for Kamie was our only Hope for her future.

At the meeting, Mr. Boudreau began by telling Parise that cabinet had approved treatment for Kamie.  Just like that, her life was changed.  Just like that, her life was saved.  In two days – two days! – the application was reviewed by Mr. Boudreau’s team, a presentation was put to the government’s cabinet, and an approval was passed.  Quick action by Mr. Boudreau changed the lives of this family forever, and I’m incredibly indebted to him for it.

How does this story relate to the quote above?  The Minister told us that he was on The Isaac Foundation website to research the disease, our organization, and read about the work we’ve been doing for kids across this country.  Displayed prominently at the top of our page is Margaret Mead’s quote, he told us.  Minister Boudreau informed us that Premier Gallant, the leader of the government in New Brunswick, uses that quote all the time around the cabinet table.  He uses it to inspire his government to do what is right for people.  He told us that he took that quote from the website when presenting Kamie’s case to the Premier and told him that his government could be the “small group of people” in this situation.  They could be the ones to change the world, even if just for one family.

In all my dealings with governments, bureaucrats, and politicians over the past decade, I have rarely been as impressed as I was with Minister Boudreau on Monday morning.  He saw a problem, saw a need for prompt action, saw the need to help a family in need, and went about doing everything he could do to make things better.  While I’m sure it’s too early for him to understand what his actions, his passion, and commitment to make a difference really means, I know from experience that he will look back on this and see this as part of the legacy he’s left behind, part of the good he was able to do while in office.  Saving someone’s life will remain with you forever and, usually, change who you are for the rest of your life.  Just ask Heather Forsyth, leader of the Wildrose party in Alberta, the person who worked tirelessly to save the life of Aleena Sadownyk.  Just ask Elizabeth Witmer, the person who did everything in her power to save my son Isaac’s life.  I hope Minister Boudreau looks back on this with pride, and I hope his family can learn just how much of a difference he made to the life of a little girl this week.

I know this entry is long, but before I sign off I want to leave you with the other quote I live my life by, one that guides me wherever I go, whenever I get the chance to meet with families battling a rare disease like we are.  It comes from my favourite book, To Kill A Mockingbird, from a character whom I wish I could be more like in this world – Atticus Finch.  In this novel, Atticus works hard to teach his children about courage.  He leads by example, and hopes his children realize that they can make a difference in this world if they truly believe in what they are doing, no matter what the consequences may be for themselves.  Atticus tells his daughter that:

“Courage is not a man with a gun in his hand. It’s knowing you’re licked before you begin but you begin anyway and you see it through no matter what. You rarely win, but sometimes you do.”

In many ways, this is exactly what we face when we’re dealing with rare diseases and treatment for our kids.  The initial diagnosis is devastating for families.  Knowing a treatment exists provides hope, so much hope during those dark days.  Families are devastated again when they find out that governments deny access to those treatments – take that hope away – solely based on financial considerations.  Expensive treatments aren’t the fault of patients and families, but they bare the consequences.  Oftentimes, we fight with grace and courage to get the access to treatment we need, against all odds.  Rarely do we win, but sometimes we do.  We did yesterday, with the help of Minister Boudreau, and I can’t thank him enough.

So – where do we go from here?  Well, there is another child suffering from MPS in his Province who needs access to treatment.  He’s the same boy I called the Province about many months ago without success.  Perhaps the application we tried to submit many months ago can now be reviewed, and hopefully Mr. Boudreau can do what he can to assist.  With help, perhaps the little boy in need can now get the help he deserves.  I know I’ll keep trying to make it happen, and I hope to be met halfway by the Government of New Brunswick.

Thanks for hanging in to the end of this entry.  It’s a long one, but one of the most important ones we’ve ever logged.

Till next time,

A.

Foundation Poster

 

 

Revisiting an Old Post – My Inspiration To Serve

Hi Everyone,

As I get set to head to Saskatchewan (still my favourite Province to visit!) to meet with the Minister of Health and other political leaders about a family requiring access to therapy, I thought I would re-post an old entry I wrote from Saskatoon, at one of the most peaceful spots I visited on my journey.

I wrote this as I was making my first decision to enter the world of politics, as I sat by the grave of the great John Dieffenbaker in Saskatoon and wrote back and forth to Stuart Mclean, a friend of mine and a great Canadian in his own right.  I posted this in May of 2009 and wrote about my passion and excitement to help shape Canada for the better, feelings I continue to hold today, though in a different light.  I take my role in the world of Rare Diseases very seriously, and will do everything I can to protect our kids who desperately need our help, who desperately need access to treatments we’ve already approved here in Canada.  I owe it to my own son to continue fighting for our kids, and I’ll do so until we find the cure we’ve been searching for all these years.

Enjoy.

May, 2009

Hey Folks,

I am currently sitting at one of my favourite places in Canada, beside the tombstone of John Diefenbaker, Canada’s 13th Prime Minister.  I first fell in love with this spot at this time last year, when I travelled to Saskatchewan on a class trip with my grade 8 class.  The week had been a long one, and a busy one, and I managed to escape here for lunch and some respite on my final day in town.

I sat here, in the very spot that I am typing from now, and wrote a journal entry to a friend of mine back in Ontario.  He has a radio show on CBC called The Vinyl Cafe.  At the time, entering public life was an idea that had been pulling at me for some time, since before my son Isaac got sick.  I think the atmosphere here inspired me and I just had to put down on paper…er…email what was going through my mind.  Here’s what I wrote…

June, 2008

Dear Stuart,

After a long and arduous week, I have finally arrived at the Diefenbaker centre at the University of Saskatchewan. I had two things that I wanted to see while I was away; the vast expanse of prairie farmland and the final resting place of the enigmatic Chief himself.

Being locked up in paperwork forced me to miss our two-day trip through the countryside and down to Moose Jaw, hence removing the chance for me to see the land that W.O Mitchell so elequently wrote about in his masterpiece, Who Has Seen The Wind. But I was determined to get here today and get a glimpse of the life that was John Diefenbaker.

As I type, I am laying in the grass beside the tombstone and final resting place of the great man himself. A gopher is watching my every move as the prairie sun beats down upon me. Diefenbaker must have selected this resting place for himself, knowing that those visiting his grave would have to look over and about the land he loved so much.

As I aspire for a life in public office, I’m inspired by the man whose grave I’m quietly visiting. I love that I am here and being reminded that he never gave up his dream of serving the people, never lost his drive to fight for what he believed in, and always remembered his roots.

This exchange has brought me a full week in Kingston, where I was able to teach our kids about how incredible John A. was for our city and country. Now I get a true glimpse at what the Chief meant to the people in the West. I see who he fought for; the disadvantaged, the minorities, the real people that have built our great nation, and I’m proud that he is recognized with this museum, and I’m proud that people still stop by here to pay him homage.

I will come back here sometime, in the near future or a long way down the road, if only to keep my inspiration for a life of service in politics at the forefront of my mind.

 

See you when I see you,

A.

Stuart emailed me back almost immediately, and included a journal entry that he had written from the grave side of Sir John A. MacDonald who was, of course, Canada’s first Prime Minister.  Here’s what HE wrote…

June, 2008

Andrew,

Thanks for the picture. I visited his house in Prince Albert a couple of months ago.  A couple of years ago I  spent a few hours one afternoon by Macdonald’s grave in Kingston, Ontario and wrote a script for the show.

to wit:

I am writing these words in a child’s scribbler sitting under a pine tree that is growing by the gravesite of Sir John A Macdonald. I have come here by train along the same railbed perhaps that he once rode, maybe even gazing at the same fields, the same farms, perhaps the very same trees, on the two hour morning run in from Toronto.

It took less than five minutes and costs less than five dollars to get here by taxi from the train station. And now sitting here with my coat spread on the grass beneath me a spider working its jerky way across my right knee I can hear the whistle of trains as they shunt through the station below me.

This grave that I have meant to visit for years is marked by a simple stone cross, no more than three feet high.  The government sign marks his grave as a Canadian Prime Minister. But the sign also says the grave sight was fixed up in 1982  — which is when the black wrought iron fence was run around it, I decide.

And the stone cross that I have come to see seems too polished to have weathered many Canadian winters. And I am thinking, as I sit here in 2002, 111 years since he died, in a world that is often too shiny for my taste, that I never got the impression he fretted over heavy weather or would be offended by the work of the wind.

I would like to tell him as I sit under this tree and feel the warmth of the June sun on my face, that I was glad to be born in this corner of the world that he coaxed into being. That we are still here and that we know ourselves to be among the lucky.

But mostly I would like to tell him that I travel by train whenever I can, and that he could still travel by train if he wanted; still all the way to the pacific, and on the way over the prairie where

I would like to tell him, if he wanted he might still, if the conductor was feeling good, ride between the cars and throw the top half of the door open so he could stick his head out and feel the wind on his face and count the ducks in the ponds as he went by.

And I would like to tell him that just last weekend I found myself walking by railroad tracks with a young man, a boy still in school who turned to me and said, “I like the smell of trains.” I would like to tell him that.

But mostly I would like to tell him that I wasn’t the only person sitting here on this June afternoon on this pleasant hillside by his grave thinking these things.

– Stuart

Our exchange made me smile and capped off one of those moments in life when things come clearly into perspective.

It felt good to be here then, and it feels good to be here once more.  I hadn’t realized that I would have the opportunity get back here so soon.  It’s another beautiful day on the Prairies, and the “Land of the Living Skies” is truly living up to its name.  To my left, I can see downtown Saskatoon on the horizon, and to my right, nothing but trees and nature.  I am sitting with two friends and we are all just taking in the serenity of this spot beside Dief’s grave.

I am touched by the amount of people that walk by and then stop for a moment of silence at the final resting place of John Diefenbaker.  I watched the first few people stop and then continue along on their walk through the campus.  But I wanted to know why they were here, and from where, so I started to engage them in conversation.  The first man that I spoke with, as I sat in the grass with my laptop, was from Toronto, simply visiting Saskatoon for a few days until he reached Regina and his business meeting.  He had a video camera and a digitial camera with him and he was filming the walk up to Dief’s grave.  He paused for a moment and took in the atmosphere, the warm Prairie sun, and the sound of the wind rushing through the trees.  He seemed almost awe-struck, and told me that he just had to get to this spot before he headed south.

The next group that I talked to was an elderly couple with their two friends.  They came up to visit from the south of the province, almost as far south as the border.  The one gentleman told me that he came here as a student many years ago, when there were only 2500 people registered at the University.  His daughter was a retired professor from Queen’s University in Kingston, Ontario, the city that I’m visiting here from with my grade 8 class.  His daughter would have taught my wife in the Faculty of Nursing, a fact that pleased both him and his wife.

Canada is a small country once you start talking to people.  Don told me that Diefenbaker once came to his father’s house for coffee, simply because his dad had invited him to.  And he told me that Dief would do that often, visit people in their homes, because he was just a regular person, just like everybody else.  As Don stated, “he had his problems too, but that’s also just like everybody else.”

I think this is why I admire Diefenbaker as much as I do.  There are those out there that would object to Dief’s name being printed on this blog site, the blog of a hopeful candidate for the Liberal Party of Canada.  But what made Dief great wasn’t the fact that he was Conservative, nor was it the fact that he was even the Prime Minister.  To me, Dief was great because he worked hard to represent the people that he cared for most; the people that needed it the most.  Dief was a politician for the people, something we don’t see enough of in today’s rough and tumble world of politics.  Dief cared about the “ordinary Canadian”, the people that had struggles but were relentless in their determination to persevere.  Dief was a community politician, and he turned his difficult upbringing into a passion and penchant for helping those who needed guidance.

History has, and always will, allow us to look at our heroes in a different light, away from the glare of public scrutiny, and the pressure to step in line with the status quo.  I hope there will be a day when people can look back at the things that Diefenbaker accomplished for our country and can appreciate that he always had the betterment of the nation as his goal.  Dief looked out for us, and provided countless citizens with a sense of hope for their future without ever asking for anything in return.  THIS should be the everlasting quality that  we seek from our politicians today, for it’s this quality that will help us to grow and evolve as a nation.

Stuart wrote of the people that were sitting at MacDonald’s grave with him and his longing for MacDonald to know that they were there for him.  I too have been touched by the people that have stopped by for a few moments with Dief, a final acknowledgement that he made a difference in our world, and a thank you for giving himself to those who needed it most.

I hope I can give back to my community someday and have the opportunity to work hard to help our country get back to community representation and a government “of the people, by the people, and for the people.”

I’m about to open up Obama’s “Audacity of Hope”, and I can’t think of a better place to read it than here by the graveside of one, John Diefenbaker.

So long from Saskatoon.

A.

10693630_328239647353454_1314403896_n

Dr. Cengiz Karsli

Hi Everyone,

There are people in this world who do extraordinary things, simply by doing what they love to do, by the way they interact with others.  People who change lives without knowing it, make the world better just by being in it.  Dr. Cengiz Karsli is one of those people.

Cengiz has been Isaac’s Anesthesiologist since the very start – since Isaac was 18 months old and about to undergo a massive spinal-cord decompression surgery.  We’ve written about him often from time to time, and many of you have asked about him (and if you should ensure you get him for your own children!)

He’s always one to respond to emails when we need him, and goes out of his way to ensure he’s available to be here for Isaac and our family for surgeries.  Ellen and I couldn’t imagine going through the stress of one of these surgeries without knowing that he is on hand to look after our son.  He’s the calming presence we need before things begin, he’s the utmost professional as the surgery gets started, and he’s one of the finest Doctors at Sick Kids in Toronto, so we’re rest assured knowing that Isaac is safe in his care.  Once all is said and done, he finds us quickly and lets us know how things went in the warm way that parents need.  We arrived at the hospital this morning at the height of our stress.  We left feeling relieved, relaxed, and thankful.  We have Dr. Cengiz Karsli to thank for that.  And we will never be able to thank him enough.

Cengiz was only supposed to be on call this morning.  But upon hearing that Isaac was going to be in surgery, he scheduled himself in for the procedure to look after him, and then he stayed on until 1 pm.  As we were heading out of the hospital tonight, Cengiz was coming back in – he’s on call for the next 12 hours as well.  I’m sure he’s tired after a long day and staring at an even longer night.  But he caught us as we were heading out the door and checked in on our boy, patiently answered all of Isaac’s questions, and bid us all a good night.

There aren’t many people like Cengiz in this world, someone who goes out of their way to make things easier for kids and families.  Many MPS specialists are moving to a system where only 1 or 2 experts are used for surgeries on our kids.  Their airways usually dictate that only the best should be involved with these procedures.  We’re lucky that we have the best, and that he’s a wonderful person at the same time.

Isaac is now resting comfortably at our hotel for the night.  We’re back tomorrow for a check in on his eye and his infusion.  I’ll keep everyone updated as I can.

Thank you to everyone who checked in on us today – we truly appreciate your support.

Best,

A.

Russian Roulette

Hi Everyone,

I’m sitting here, once again, in the OR waiting room at Sick Kids Hospital in Toronto.  I can’t recall how many times I’ve sat in this room, waiting for news on my son.  But I do remember how awful it is in here – I’ve often called this place the worst place on earth – it always feels stuffy, the atmosphere grim, the energy of each and every one of us in here drained completely.  And the stress.  The stress permeates the room from the moment you enter.  It heightens in everyone as soon as a doctor appears in the door – each of us wondering if it’s news for us, and if that news will be good.  The stress breaks once parents get word that their children are now ready to be seen, but only for a short while.

They’ve tried to change this room over the years.  It’s received a fresh coat of paint – it seems lighter in here, and there is a Christmas tree in the corner to make people feel at home and cheery.  There is a TV on the wall that updates all the patients and the stage they are at…In Holding, in the OR, or Finished.  But it’s the same place – and I’m sitting in the same seat that I always do while I’m here.  It’s not the room that makes this place feel awful, it’s the mood.  And that will never change.

We’re here together – Ellen, Gabriel, and I, and dealing with things together like we always do.  But we almost didn’t have Gabriel with us.  For some reason, we thought the stress, the long day – the unknown –  would be too much for him and we arranged for him to stay with Nanny while Ellen, Isaac, and I came on our own.  But it was also stressful to think about him being left alone and worrying about his brother, and wondering what was going on while we were away.  He woke up yesterday and told us he wanted to be there with us. He’s Isaac’s best friend – his support system, and we need to be here together.  I’m so glad he came, and watching the boys spend time together before the surgery was one of the loveliest things I’ve ever seen.

This is the place that parents of kids with MPS dread the most.  Surgeries are tough on kids, but made all the more difficult for our kids with MPS because of their comprised airway – airways that continue to deteriorate with each passing year.  Sitting here, I can’t help likening these frequent visits to Russian Roulette, a thought that’s been nagging at me for weeks now, ever since we got word that Isaac’s transplant was set for today.  It’s a grim thought – one that I don’t want to have and one that scares me.  How long until the chamber isn’t empty?  I don’t want to know.

We’re lucky, however.  More lucky than most parents in our position.  Isaac’s airway has been slowly rebuilding itself since we started him on Elmiron, the JnJ drug that we worked so hard for him to receive.  We know it’s doing wonders – his hands are straightening out, his spine is doing better, his joints are less stiff.  And his airway – it looks almost like a “normal” 10 year old airway now, something we could never have expected a few short years ago.

But that old familiar feeling of dread comes back to us all once we enter this room, once we sit and watch the door for our doctor to arrive.  I wondered aloud yesterday if this weakness of mine makes me less qualified to do the work I’m doing now on behalf of our kids throughout this country, whether this vulnerability or fear (or whatever you call it) makes me less able to support other families as they go through similar battles.  The answer came quickly, from a wonderful Mom who I’ve been lucky to get to know over the past few months.  Via text, she said:

“This makes you a great patient support person because you are honest enough to share your true emotions and know what a roller coaster ride it is. You are super human ; ) but you are human too.”

Those few sentences made me feel so much better about being afraid, and it’s comforted me as I sit and type away on our blog.  I should have told her that before making her read it here 😉  And she’s right…I am human.  And it’s OK to be afraid.

So far, it’s been an hour and a half since Isaac went back.  We have full trust in the man that’s looking after him right now – Dr. Cengiz Karsli, Isaac’s longtime Anesthesiologist (pictured below with Isaac this morning.)  We’re lucky to have him each time we go through this process, and it makes us feel a bit better knowing we’ve got the best person to care for our son.  We’re hopeful that we’ll win this round again, and the stress levels can come down a bit as we help our son heal.

I’ll update more on Isaac’s status as soon as I know more.  Thanks, as always, for your support.  It means the world to Ellen, Gabriel, Isaac, and I.

With Love,

A.

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Transplant Tomorrow

Hi Everyone,

Sorry for the delay in posting – we’ve been busy with work, family support, and working hard to access treatments for kids across Canada that need help. At the same time, we’ve been taking a few weeks to spend time together has a family and get ready for Isaac’s corneal transplant, set to take place tomorrow morning.

I wasn’t going to post anything until all was said and done. But I received a few notes over the past week from wonderful supporters asking how we are doing. They noted that we’ve been posting a lot about the work we are doing on behalf of other families and were wondering how Isaac and the rest of our family was doing. Fair enough, and I appreciated the kind notes.

To say the least, we’re nervous – afraid really – of all that comes with this procedure. It’s daunting to think about the possibilities, so we’re concentrating on the positives and the potential benefits that this will give to Isaac. Imagine – he may be able to see the stars again, something that he stopped being able to see a few years ago.  We are thrilled that his vision may be improved and we desperately hope everything will go smoothly.

I’ll do my best to update how things are going. All 4 of us are heading into Sick Kids Hospital this evening, with the transplant set for early tomorrow morning. We’ve got the best team possible looking after Isaac’s care, including his longtime Anesthesiologist Dr. Cengiz Karsli, pictured with Isaac below.

Thanks for checking in. I’ll update when I can.

A.

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On Leadership, Compassion, and Danielle Smith

Hi Everyone,

Just wanted to drop in to comment  on the recent Alberta election and the aftermath that the PC Sweep of all 4 seats has had on the political landscape there.  To say the least, I’m dumbfounded that the results of the by-election have led to questions about Wildrose leader Danielle Smith’s leadership.

To begin, for those that don’t know me, I’ve always been known as a staunch Liberal.  I’ve run as a Liberal nomination candidate, have been deeply involved in riding associations, and have even been the campaign manager for a strong candidate running for President of the Federal Liberal Party.  Most of my friends identify me alongside the Liberal Party, and whenever talk over a glass of wine or dinner turns to politics, I’m often turned to in order to offer the Liberal Perspective on things.

But to label me as a Liberal wouldn’t be entirely accurate.  I like to believe that I dedicate my life to helping protect the most vulnerable in our society – the ones that need a hand up; the people who need support so that they can reach their full potential in life.  Many parties identify with those beliefs, though some more than others. Obviously, I put my passion into practice when I decided to become a teacher, with my focus being on educating our future.  And more obviously, that focus changed, quite dramitically, when my son was diagnosed with a very rare and progressive disease.  On that fateful day in 2006, I promised my son that I would do everything in my power to protect him, to help him battle his disease.  And to help him win.  Along the way, I’ve been fortunate to play a small role in helping other children and other families fight back against this terrible disease, and along the way I’ve met some incredible people – people just as passionate about fighting for those in need as I am.  Danielle Smith is one of those people.

Under Danielle’s leadership last summer, the life of a little girl suffering from a rare disease was saved.  The government did not want to take action to provide little Aleena with the treatment she needed.  Indeed, her request for approval of her treatment was denied by Alberta Health.  Heather Forsyth, the opposition health critic for the Wildrose Party, worked tirelessly to help get that decision reversed.  And Danielle Smith was with us the entire way, supporting our efforts and ensuring time was allotted in the Legislature to hold the government to account for their inaction.  Heather and Danielle’s efforts made news from coast to coast, with National organizations covering the plight of Aleena. Because of her commitment to this family in need, Danielle helped save this young girl’s life.  And today, Aleena is thriving, growing, and living a rich, full, and happy life.

Danielle didn’t have to allocate the resources of her party or her time during Question Period to helping this one child.  But she did, because it was the difference between suffering or not for Aleena;  the difference between life or death.  Danielle ensured help was available because it was the right thing to do, and I was incredibly impressed with the way she committed herself and her party to wholehearted and unconditional support of Aleena and her family.

Since that time, I’ve had the opportunity to revisit the legislature and connect with Danielle in person.  And she’s renewed her commitment to fight for Albertans suffering from rare diseases, she’s committed to holding the government to account for their lack of action to help our kids – she’s committed to protecting the most vulnerable of Albertans – our sick children fighting a battle they need help to win.

After watching Danielle’s commitment to Aleena, I’ve watched the policies she’s put in place for her party – health care reform, ending extra school fees that have become an added burden on families, protecting taxpayers, and the list goes on and on.

So to those of you out there questioning Danielle’s leadership in the wake of the four by-election loses this past week (losses in what have always been considered SAFE PC SEATS, I may add), I ask you this – what Leadership qualities ARE you looking for if you feel Danielle isn’t the person you need?

To me, having someone at the helm of your party who has shown a strong commitment to those in need, who has demonstrated her ability to help the most vulnerable in our society, who believes that change can happen if people work together, and who continues to show a passion to protect our social safety net, would be a true blessing.  Danielle is a good person, and represents everything the Wildrose Party has come to stand for over the past few years.

General Douglas MacArthur once said, “A true leader has the confidence to stand alone, the courage to make tough decisions, and the compassion to listen to the needs of others. He does not set out to be a leader, but becomes one by the equality of his actions and the integrity of his intent.”  Centuries earlier, Napoleon said “A leader is a dealer in hope.”  From what I’ve seen and experienced over the course of the past two years, Danielle Smith embodies these definitions perfectly.

Far be it for me to tell the Wildrose what to do about their party and about their leader.  But please remember this before you pass judgement at the upcoming leadership review – Winston Churchill lost 5 elections, Lincoln lost 8.  True leaders aren’t judged by wins or losses, they are judged by quality of character, ideals, compassion, and vision.  More often than not, true leaders end up on the winning side of things eventually – sometimes patience is required, but leaders tend to win out.  And Danielle Smith is a true leader, one you should be proud and are lucky to have.

So, before you vote, take a step back and see the forest for the trees.  I bet you’ll find you are in good hands with Danielle.

Respectfully,

A “Liberal” from Ontario.

Isaac Update – Corneal Transplant

Hi Everyone,

I’ve just returned from one of the most rewarding weeks I’ve ever had in my role as Executive Director of The Isaac Foundation.  I’ll save updating on all that was the MPS and Adulthood Conference for another blog posting.  For now, I wanted to give you an update on Isaac’s health as the conference, for me, was overshadowed by the news we received yesterday (and while I was still away) that Isaac requires and will be undergoing double corneal transplants soon.

This news was shocking and upsetting to us, but not surprising.  We’ve always known that this was on the horizon – or that the possibility of this was on the horizon.  But he’s been so stable in his health during the past 6 months that it left our minds as a possibility.  But stable isn’t quite the right word – he’s improved since January, improved considerably.  I guess this is why the news we got yesterday was a tough to take.

First off – Isaac’s being incredibly brave.  Please know that.  Me?  Not so much, but I’ll stay strong because it’s what I have to do.  He expressed very eloquently that how his eyes are important to him because he need to be able to read (he’s a veracious reader and it’s unimaginable to him that that could be put in jeopardy.)  He wants the surgery to protect that hobby, which is both incredibly cute and heartbreaking.

Now – corneal transplant…what is this and why are we here now?

Kids with MPS accumulate a buildup of cellular waste in their bodies known as glycosaminoglycans, or GAGS.  Enzyme Replacement Therapy (ERT) helps to break those GAGS up and clear them out of the body.  But sometimes ERT doesn’t prevent the clouding (caused by the GAGS) that takes place in the eyes. And Isaac has always had corneal clouding.  Quite severe, in fact, but it’s been severe since he was very young.  In fact, if you were to look at Isaac’s eyes today, they don’t have much colour – they are grey due to the clouding.

Isaac’s glasses have corrected his vision over the course of the past numbers of years.  The clouding has continued to get worse – to the point where the doctors haven’t been able to see into his eyes for about 2 years.  But still – his vision has been stable.  A big change happened, however, over the past 6 months.  Vision in his left eye has deteriorated considerably – 20/30 down to 20/80, and this is quite concerning.  The result?  A decision to move on with the transplants that we thought were still a number of years off, in the very least.

Doctors are very good at doing this surgery, though I have to admit that I’m still very scared and worried.  But our MPS community is amazing, and many parents are rallying around us with love and support – something I’m truly grateful for.  And Isaac’s friend since childhood, who also suffers from MPS, has had both of her cornea transplanted.  Her family will be a great resource for us as we embark on this latest battle, and she will be able to talk to Isaac about what to expect.

And quite serendipitous – when I arrived home, there was an invitation in my email for me to tour one of the best (if not THE Best) transplant clinic for kids with MPS in the US.  I’m excited to attend and, perhaps, garner a bit of information on the process along the way.  The clinic specializes in bone-marrow transplants and stem-cell transplants, but they be a wealth of information for me for this comparatively smaller and less serious procedure.

Anyhow – I feel a tad better now that I’ve written this and the decision has been made.  It was hard to get the news while I was away.  All I wanted to do was hug my boy, struggle together with my family, and be here for each other when we needed it most.  It was a tough night last night, and a long flight home.  But as soon as I arrived at the school to pick up my boys, they both jumped into my arms and hugged me for what seemed like an eternity.  And Isaac looks good, and strong, and – as he always does – brave.  He can do this, and so can I.

Isaac shared the news with his best friend at school, Amy.  I’m so thankful that he has someone he can trust to talk to, and I’m sure it made him feel better to share the news with his friends.

I’m listening to Danny Michel as I type this blog update – poignant because he’s playing at our upcoming Gala For A Cure.  The song below is called “Just The Way I Am”, and it’s providing the perfect soundtrack for my frame of mind right now as I think about Isaac, his bravery, and the joy I know he takes in having a supportive friend to talk to when things get tough.  It’s below for you to listen to as well – it really is a beautiful (and perfect) song for this posting.

Thanks for letting me ramble on.  I’ll update with more information when I can.  Thanks for always being here for us and our kids.

With Love,

A.

Always a trooper. #Brave

Quick…well not that quick, visit to Sick Kids Hospital in Toronto today for Orthopaedic Surgeon followup. It was a 4 hour wait (really, this needs to be fixed) to see the surgeon, but he had great news!

Isaac’s kyphosis (curvature of the spine) has improved by 4%, and his slipped disc has corrected itself. While we can’t be certain, we’re convinced it’s the effect that Elmiron has had on him.

Truly grateful.