"BLACK ICE"

For most of you reading this, you'll understand what I mean when I use the phrase black ice.
Have you ever been travelling down the road, most likely in the winter time, behind the wheel of your automobile, paying close attention to the weather conditions but for the most part the air is clear, no snow flakes at all to be seen.  The road itself looks bare, no snow build up just bare pavement.  So you continue to tour along at pretty good speed cause you've got places to go and people to see.  Then something happens that makes you have to slow down in a hurry.  Could be an animal jumps in front of you, the vehicle in front makes a sudden stop or needs to turn, pedestrian or cyclist maybe.....  Whatever it is you find yourself having to take your foot off the gas and use the brake petal instead.  But it's no problem cause the road is clear remember?  Or so you thought....
As you press harder and harder on the brakes to get your vehicle to stop you discover that what you thought was bare pavement is actually coated with a very thin, unnoticeable layer of ice.  BLACK ICE as they call it.  And as you probably well know, ice under skidding tires makes you keep on sliding.  Totally uncontrollable, you might as well take your hands right off the wheel and take a prayer position because that's all that's going to help you now.
Around and around you may go, and when you finally do come to a complete stop, if you're lucky no harm has come to you, your passengers or your vehicle.  OR you might end up in a ditch or you might have an impact with whatever it was that made you have to brake in the first place.  Either way, once you have stopped completely, you can then assess what has transpired since you began to spin uncontrollably.  That's usually about the same time you get that nervous, worry feeling inside.  Quite often if feels as though someone had sucker punched you in the gut and it can be quite a while before you are able to calm down and that feeling subsides.
We had "black ice" occurrence yesterday while on our weekly trip to London.  It wasn't exactly as described above or as you might think.  We didn't have trouble with driving or with the road under us at all.  Our occurrence happened while on the road of life and a life dealing with MPS.
We have be motoring along in our journey with Jasper and MPS.  Not sure you could ever call it being in cruise control but it almost feels like it at times.  He's taken to his treatments well, never had side effects and most importantly, all of the specialists we have been seeing for check ups have been happy with his progress, or in the case of MPS, lack of progress.  Things have been stable for Jasper in all respects and when things are stable you start to just cruise along, going day to day.
Until yesterday, as I said, we were forced to take our foot off the gas and apply the brakes.  As soon as we did, we hit a large patch of "black ice."
Jasper had an appointment to have his MRI repeated since it had been 12 months since his last one.  So, up early we got, left home at 6:30am to begin our day.  Seemed like forever but by 11:15am Jasper was asleep and then Pam and I went back to the waiting room to of course, wait.
When it was finally over, we waiting for a bit more, until Jasper was alert enough to head over to our usual spot in PMDU for his infusion.
While his infusion was running we got a visit from the Neurosurgeon.  She had with her a print out of that day's scan as well as a shot from last years to compare.

BLACK ICE.

A common symptom of MPS is spinal cord compression.  Waste builds up and cause the spinal canal to tighten up and quite often end up compressing on the cord itself.  Obviously, any unnatural pressure on the spinal cord can cause any number of neurological problems and the only way to fix or prevent it is very risky and invasive decompression surgery.
In Jasper's MRI from last January there was noticeable room in his canal, as you could see room for fluid in and around the spinal cord.
This year however, there wasn't any room in there.  Things looked tightened right up.  There isn't any evidence of compression yet, and the Dr was happy with his reflexes and how easy they were to find etc.  But.....  they were a bit puzzled by the lack of room and afraid that we might be headed for some eventual compression.
We're spinning uncontrollably.  As Carrie Underwood so properly puts it in her song, we have not choice but to put our hands up and let Jesus take the wheel.  It's out of our control and entirely in his now.  We put our faith and our hope in him completely.

 So, rather than waiting another year for follow up MRI, we are going back in 6 months.  Again, hoping that things don't worsen any further.
Of course, there's always a chance that the MRI wasn't 100% accurate or that the radiologist and neurosurgeon aren't reading it correctly in regards to his condition.  It is true, they don't see MPS all that often, if ever, so they have no one else to compare it with in London.  And there's a chance that if Jasper grows, he may stretch out his spine etc it might open up a bit more on his own.  We're pressing harder and harder on the brakes to get us to stop the wild ride.
Those are the bright sides, those are the possibilities that we can hope for.  On the other hand, what we fear is that it will tighten up more and force the need for risky surgery.
Now that we're done spinning and have come to a complete stop, we now have that awful "punched in the gut" feeling.  Sick to our stomachs, can't sleep, don't want to eat etc etc.
Yes, things aren't as good as we hoped they would be, BUT we have to remember they're not as bad as they could be either, yet at least.

I realize it's been over a year since our last blog entry and most of you have probably given up on following cause there hasn't been anything to follow.  I do apologize and part of that reason is due to the fact that things have been good and we've been travelling down the hwy without worrying about bumps or black ice.

Signing off now, with hopes that none of you have to experience any kinds of deadly Black Ice out there.  Please take your time, slow down and make sure alway keep an eye out for the unexpected.
Keep in touch.
Love from the More's as always.

Change in season

It's been a very long time since I've posted.  As you know, summer was busy with everyone playing ball, now that the season has changed into fall, ball is over, and we are all now into our 'winter' activities.
I'm curling again on Thursday nights in the competitive league with the same great 3 guys as previous.
Pam is back to playing in her Bridge league every other Monday.  Daphnie is probably the busiest little lady.  Helping the little skaters on Tuesday nights, gymnastics on Wednesday, (at which she is a natural I think), Gems on Thursday evenings, and Jazz dancing on Friday evenings.
Clay has hockey Monday, Wednesday and Saturday, and Jasper started skating this year on Tuesdays. 

Thank goodness we have parents close by who are willing and able to help us shuttle kids to various activities.
As for Jasper and the Isaac Foundation, it's been a busy couple of months.  While we were camping on our holidays in August, we were kicking off "Project One Million".  www.projectonemillion.ca In an effort to raise awareness, but ultimately convince Johnson and Johnson, the makers of a specific oral drug to help us fund the clinical trial we need for our kids.
I haven't gotten an update recently from the foundation, but I hope that project is going well.
In October we once again made the trek over to Coburg ON for the 3rd annual Gala for a Cure.  Once again we were humbled to be apart of a great event.  The kids once again waited patiently through the evening until they could shake their groove on the dance floor, and they didn't disappoint. 

They boogied well past midnight until the hotel staff were closing up the ballroom on us.
Jasper met a new friend in Ron Sexsmith.

I was impressed at how personable Mr. Sexsmith is.  Not your stereotypical music celebrity.  It was great to meet him and listen to him perform.

Next came Halloween.  As you can see from the photos, we had some good costumes once again this year.  Great job to Pam for putting them all together.

This weekend brings about the second annual "More than a Spiel" curling bonspiel to help "Take out MPS".  Happy to report at this time that both draws are full.  It will be fantastic to see everyone out, and I hope everyone is able to enjoy themselves.  Unlike last year, this year's total earnings will go directly to the Isaac Foundation to help fund the research projects we have on the go right now.

Another life/child, Worth every penny

We had the opportunity to attend the National MPS conference in Alliston ON this past weekend.  We weren't going to go at first because Daphnie had her year end ball tournament on the same weekend.  When we learned that she could be done playing early on Saturday, we decide to book the hotel room and register for at least half of the conference that we could attend.
BOY are we glad we did.
We met a great family from Saskatchewan including 3 year old Violet who made the journey over to Ontario mostly for this conference and the opportunity to meet and network with some other MPS affected families, specifically the More's and the McFadyen's.
This young family just received the devestating diagnosis for their darling daughter this past February.  Since then they have been trying to sustain the funding needed to get enzyme replacement therapy for Violet in Saskatchewan.
Flashback to exactly 1 year from now.......  We were right in the middle of our quest for the very same thing here in Ontario.  We had the benefit of a precedent set by Isaac.  Violet doesn't have that 'luxury' in their province but she does have all of us on her side and in her corner 100%.

See in the photo, taken at the conference of the 3 children affected, standing together, already becoming friends.  These 3 share something in common with each other that only 6 others in all of Canada share.  Each one is very special and worth every penny.
If it comes to it again, we are prepared to launch a very public campaign to secure Violet's funding, but hopefully Saskatchewan won't be as difficult as Ontario was.
All the best as always.
The More's.

Meet Terry

Hi Everyone.  Please click the link below and read a story written about Jasper's friend Terry and the great work/effort he's put in so far toward making our life easier and better particularily while we're at the hospital, but it has an impact even when we're not.
Jasper loves Terry and I dare say the feeling is mutual.....!

http://www.lhsc.on.ca/About_Us/LHSC/Publications/Features/July13.htm

We are most grateful and very proud to express our gratitude.
We did get some 'sad' news this week while at treatment though too.  We learned that Jasper's favourite nurse (so far), Cathy is leaving the PMDU area of the hospital to accept a different position.
Cathy was one of the first nurses we had when we started, and Jasper quickly became comfortable with her, her efficiency and her methods.  And he always looks forward to visiting with Cathy.
We will miss her dearly at treatments, but of course wish her the best in her new position.  I'll try and get a photo of them both to add to the blog.
God bless all the good people at LHSC.

I Wanna Live

   

A few years ago Pam and the kids gave me John Denver's 'Essential' greatest hits double cd as a gift. I had seen a special on PBS with him and was reminded, how many of his songs I recognized and liked. But it wasn't until Jasper was first diagnosed with MPS that I realized what this song of John's really meant to me. I was driving home from work one day in the spring of 2011, not long after we learned MPS was part of our lives. With John's CD in, this song came on. It really hit me hard hearing it for the first time since learning of the diagnosis. With all the new (incomplete) information running through my heart and my mind at the time, the lyrics in this song made me cry for Jasper's sake. He begins by referring to all the hardships that kids are born and raised in places less fortunate than ours, although he never mentions illness exactly, you know it has to be included as well. But was really the chorus that made me weep for Jasper.

"I want to live I want to grow,
I want to see I want to know,
I want to share what I can give
I want to be I want to live"

All we ever ask for when children are born is that they're healthy. Doesn't matter what sex, or how big, or how much they weigh, 'as long as they're healthy.' Hearing the words in this song, and having a child with a degenerative, life threatening disease really make you realize how much you just want your children to be alive, to be happy and healthy.

Please have a listen, think of Jasper, and all the other kids affected by disease and tragic conditions as well.

Thanks.

Jasper meets a childhood icon

Hi Everyone.  Quick update today.  Jasper was fortunate enough to be at LHSC last week for the annual Teddy Bear Picnic event.  He got a cameo appearance on the London CTV news while he was stuffing his teddy bear.  He also won a bag of prizes, but the highlight of the day was getting his picture taken with Tony the Tiger.

This weekend we are golfing with the Isaac Foundation to raise more money that we've committed to 2 research grants over the next 12 months.  Stay tuned for more photos.

God bless.

Long overdue update

Hello out there.  It's been a long time since our blog had been updated so sit back, grab your beverage of choice for whatever time of day it is when you start to read this and enjoy.  Jasper has had a great few weeks of treatment.  The only issue we still normally have is putting the Emla cream on.  He doesn't like that much yet, and I'm not quite certain why.....?  but after that is on, he never flinches when they access his port, or when they take the needle out at the end either.  He's becoming a real trooper.

Hard to believe that it's almost been a year since we first learned of those horrible (when put together) 3 letters of the alphabet.  (M.P.S.)  It was last April when we first found out Jasper has some form of Muccopolysacharidosis.  Last spring/summer was crazy busy with visiting all the specialists for baseline testing.  Now, I guess we need to prepare for the yearly follow up with all of those specialists again.

Most recently, Jasper had a follow up opthamology appointment as well as a visit with the cornea surgeon.  We had seen the opthamologist in October already after only a week or two of treatment.  At that point she noted some cloudiness in Jasper's corneas, hence the suggestion to meet the surgeon, as one day it may be necessary for Jasper to have a transplant in this area.
We were surprised (as was the eye doctor herself) to hear her say that she was thinking his eyes didn't look as cloudy as they were in October!  That would be amazing to believe.  Almost too good to be true.  Naglazyme has never shown any concrete results to say that it could reverse the effect of corneal clouding, so I think that is what's keeping me from believing it's true.  I'd like to with all my heart and soul, but I believe we need to take that information with a grain of salt.  She was never able to actually measure the amount of 'cloud' back in October cause she couldn't get her gauge to work quick enough before Jasper would flinch and she'd have to start over.  Therefore, all she has to go on is her recollection and her notes that she made compared to what she saw this week.  Don't get me wrong, I still see it as a positive because at the very least, she didn't say they've gotten worse.

In addition to that benefit, we do have one measurable improvement that can be 'taken to the bank'.  Weekly at the ERT appointments, they do their best to get a urine sample from Jasper.  Beginning back in April, before treatment started.  I asked our Dr to chart the results for us and the chart revealed that since starting treatment the GAG levels in his urine have dropped by almost 70% down to just over what a normal 3 year old without MPS would measure and has stabilized there.  What this means is, while the drug isn't PERFECT at removing the GAG waste, it is helping immensly.  Almost enough to the point where there should be no more than normal waste buildup in organs, bones, corneas etc.

Aside from that, there aren't any noticable changes in J.  We had our kids ability initial visit in February as well, and they have recommended monthly physio and occupational therapy visits just to monitor, advise and help keep his joints moving well.

The HIGHLIGHT of our February by far though was the tour East on the 401.  Primary destination Campbellford ON with a stop over in Bowmanville.  Many of you know, there are 2 other cases of MPS in Ontario.  Justin is 30 years old and lives in Bowmanville with his parents.  We have been email and Facebook conversing with him over the past months, but took the opportunity to go and meet with him and his mom last weekend.  He is a fantastic individual, and I'm amazed at how well he's fought MPS VI in his 30 years.  It was very good to finally meet him in person.

After a good visit and a few photos, we loaded back up in the van and headed further East to Campbellford.  We've met Isaac and the McFadyen family a couple times already and as most of you know too, the amazing history between our families that exists beyond that.  We accomplished a lot during this overnight stay in their home, but perhaps the most important thing we did was further secure our friendship and our deep ties.

All 5 kids played exceptionally well together all weekend.  Both indoors and out.  The parents got lots of opportunity to share and swap more stories and tales and advice regarding our 2 affected boys.  Pam and I are very honoured that they have asked us to join their foundation officially as board members.  So watch out we now have even more purpose to ask you for fundraising dollars:-)
And speaking of fundraising.....It's going to be a busy summer/fall.  Stayed tuned and check back often for the next post which will contain and detail the coming events.
Take care everyone.
God bless.