"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.

Words from Grandma Kate

Grandma wanted to post something on our blog for everyone to read, so I'll add that in a minute.  First I wanted to say, I can't believe it's been more than a month since I last posted on the blog.  And here we are in the new year.  Welcome 2012.  I hope we don't have near the shocks and surprises we had in 2011.  At least not the same kind.
There's not a lot of change, and not a lot to really update.  Jasper is still taking his treatments well.  Still no negative reactions to the drug itself, thank goodness.  He still varies week to week on how well he handles process(es).  Somedays he allows the Emla cream to go on really well, and others, he has to be 'held down' while in tears when it goes on.  I wish he could understand what the cream was for, so that he'd realize the next step would be much more painful without it.  But all in all, for his age, I think he's doing amazingly well with it all.
Last week we had a different nurse, than our usual 'Cathy', and she wasn't familiar with our 'routine' so we ended up being there for an extra hour longer than usual which was frustrating, but Jasper had a rare nap while we were there too which helped pass some time for all of us.
Christmas is now past, it was a wild time at our house.  Santa was generous (I think) and the kids are having fun with their new 'toys'.
I'll leave you now with some words from Grandma Kate.  Take care everyone.  God bless!

This past Wednesday I went with Darren and Jasper to the Children's Hospital in London. It was a moving experience. Wednesday must be IV day because I think that every child there was hooked up to one.

I was very proud of both of "my" boys. Darren knows the routine down pat and was able to help get Jasper ready for the infusion. He was so calm and tender with Jasper. Jasper strutted into the hospital and knew just where he was going and what happened at each stage. He popped up on his bed at 10:45 and stayed there until his infusion was done at 5:00 p.m. The only time he put up any fuss was when the port was put in and taken out, both of which procedures are painful. The rest of the time he played games, listened to stories, napped and taught Grandma how to play Angry Birds. He's a trooper.

 I came away with an overwhelming sense of gratitude: grateful that there is an enzyme replacement therapy available for Jasper and that he can take it without any ill effects, grateful that his family lives close enough to a facility where he can get the treatment, grateful for the facility and the people there to administer the ERT, incredibly grateful that Jasper has a family willing and able to make the sacrifice to get him his treatment, grateful to communities and friends who help pay the travel expenses, and tremendously grateful to live in a province which funds a weekly treatment that costs nearly $5,000 each week.

Grandma Kate

Routine.......???

Is there ever such a thing in life, and could we now consider ourselves into one of some sort?  I'm not sure yet but it's starting to feel like it, perhaps.
Hockey practice 3 times a week, 2 of which grandma's and grandpa's have been VERY helpful in getting Clayton to and from, dance 2 times a week, again, credit grandma with getting Daphnie to and from at least one of those sessions.  This has become fairly 'routine'.
Jasper has now had 6 successful infusion treatments.  Successful in that there have been no significant drug related side effects.  While risking a 'jinx' on the success we've had so far, I have to say, I think yesterday was the best one up until now as far as Jasper's co-operation and 'willingness'.  He still put up a bit of a battle while taking his pre-meds, but as soon as he decided to take them, they were gone in no time.  No tears!  Then when it came time to access the port this time, we must have done a better job at distracting him, because he only flinched.  Again, no tears!  After that, we're back to games, books, tv etc etc to keep busy for the next 5 hours.
We also went back to the audiologist yesterday because we are getting a little stressed with hearing "what?  What?  What mommy?"  And constantly having to repeat ourselves.  The audiologist did some tests to assess his hearing ability, but right from looking into his ears, she could see some redness and noted there was definitely a buildup of fluid behind both drums.  As we suspected, she confirmed that is hearing was definitely impaired. 
I will note, that this is most likely unrelated to MPS.  Very common in kids, the fluid just doesn't drain like it should all the time.
After a few more minutes of games (tests) she determines that when bypassing the eardrums, he hears just fine.  She'll pass the info on to our Dr, and if she decides to a refer us to an ear, nose and throat Dr, she may, otherwise we'll try and let it pass on it's own, and return to audiology in a couple months time.
We have also seen the opthamalogist recently too, and she has confirmed that he definitely has some corneal clouding already.  Not severe yet, in which case they'd recommend corneal transplants.  So hopefully the ERT will help prevent any furthur clouding.
So, all in all, things have been going okay for us.  We've started to see some results from his treatments, his hair is much softer, he's lost 1" from his abdomen, and he's grown 1" since mid September.  Most importantly the GAG (waste) levels in his urine have decreased significantly.  Since we started seeing the geneticist (April) until the time he started treatments (October) his GAG levels have ranged from 22 - 64.  'Normal' range is 0.3 - 13.  After 4 weeks of treatment he measured 16.  This is very significant because there is that much less waste now being stored in organs, bones and corneas.
Think that's it for now.  Except to say again, thank you to everyone for thoughts and prayers.  We are still in awe of the love and support we've had and continue to see from people.  The fundraising dinner Pam's ball team put on was an amazing success.  This weekend the curling club in Palmerston is holding a "More Than Just a Spiel" spiel to help "Take Out MPS VI".  Both draws are full which is great, and it should be a fun night.  In a couple weeks "What's the Occasion" and "Norsco Sports" are having a sale and charity bbq where part of the proceeds are going to help Jasper and us through his treatments as well.
Cannot say thank you enough to everyone, but we'll keep trying :-)
Love from the Mores

Busy October news

October has been a pretty “crazy train” to say the least.  I apologize for not keeping the blog as current as I should be.

Beginning with the first day of October, we were fortunate enough to attend the Isaac Foundation’s Gala For a Cure.  This was truly an awesome event and we were fortunate to be accompanied by all our parents and Braden too.  It was very nice to have them go with us and support us as well as the Isaac Foundation charity.  Sitting in a ballroom filled with people who were all there for the same reason was truly inspiring.  We had a great meal, and then there were some nice words said.  Andrew honoured our family, by introducing Jasper and our family and telling everyone a little bit about our journey so far, and the struggle we had to obtain the funding for Jasper’s treatments.  He then turned the microphone over to Pam as she had written a few words as well.  Here I have included the wonderful speech she (with a little help) put together and did great job of telling:

“It was only April of this year when we heard the three letters that would forever change our world – MPS.  These three letters were something we had never heard before our son Jasper’s diagnosis. We struggled to understand their meaning and to figure out how we were going to deal with all of the information and emotions that were suddenly and quite unexpectedly thrown our way.  All of a sudden, when we looked at the face of our beautiful little boy, we no longer saw what we had seen even the day before but now we found ourselves searching his precious face, body and movements for signs and clues, analyzing his every move and word to see if we could pinpoint which type we were dealing with and how severe and quickly the disease was progressing.  We were plagued with feelings of helplessness and confusion, guilt and despair. 

It was mid July after many blood tests, urine tests, a skin biopsy, DNA mapping and many emotion-filled weeks of waiting when we were told that Jasper in fact had MPS type VI.  Our team of Doctors sent us home that afternoon with stacks of information and so many thoughts and questions swirling around in our heads it was extremely overwhelming.  When I sat down to read some of the information, I discovered at the top of one page they had handwritten a website – www.theisaacfoundation.com  I immediately pulled up the website and as they say – the rest is history!  It took me 2 or 3 attempts before I could complete an email to contact The Isaac Foundation as with each attempt came a flood of tears and emotion.  When I finally forced myself to send that first email the response I received was filled with nothing but compassion, support and encouragement.  I began asking both Andrew and Ellen a multitude of questions and their every response was brimming with wonderful information, guidance and a positive outlook.   

We immediately began the process for applying for funding to cover the Enzyme Replacement Therapy that is available for MPS type VI.  On one hand our doctors were telling us there was treatment available but on the other hand it looked like obtaining the necessary funding was going to be very difficult.  We felt sick.  We knew in our hearts we wouldn`t stop fighting until we had the answer we wanted and funding for Jasper was approved but at the same time we didn`t know where to start.  This is when we turned again to The Isaac Foundation for advice.  There are 2 statements that come to my mind whenever I think back to July and early August and our initial conversations.  The first is an email from Andrew in which he told us `rest assured you WILL get treatment paid for here in Ontario.  Isaac has set a precedent and they cannot deny a patient treatment that is already being given to another child in the province given that they suffer from the same disease`.  The second statement came from Ellen when she responded to an email with `We will do everything we can to help and trust me, my husband is a very determined man!’

It was mid August when we began to realize the true magnitude of Andrew`s determination.  Our initial application to the Ontario Ministry of Health for funding had been denied and we were nothing short of devastated.  We knew we could appeal this decision but were concerned that alone wasn`t going to be enough.  We didn`t have to express our concerns to The Isaac Foundation, as they had already anticipated our thoughts and had begun working on a press release the day before to spread the word.  You see it isn`t only determination that Andrew seems to possess but to Ellen’s email I would add the traits of dedication, knowledge and compassion as well.  The positive manner in which he kept pushing and encouraging us throughout the entire funding application process was nothing shy of amazing.  Without the Isaac Foundation we would have lost. 

One other moment along our funding application journey really sticks out in my head.  It was Wednesday evening of the week we had been denied by the government and I was chatting on the phone with Andrew and expressing some of my concerns and frustrations.  He was doing a great job of trying to keep me positive but I am not sure I was buying it at that point.  All of a sudden he said to me “hey, would you like to talk with Isaac?”  I most readily agreed and on to the phone came the happiest “Hi Pam!”  Isaac and I chatted for a couple of moments and then I asked him if he knew he had amazing parents?  He let out a bit of a sigh and said “I know, I’ve heard that before”!   I am not sure anyone realizes how much that short conversation did for me.  It picked up my spirits and gave me the encouragement and boost I truly needed at that point in time.  To hear Isaac so happy and full of life reminded me of exactly what we were fighting for. 

It is now October and when we look back on the roller coaster ride we have been on since that day in April we are amazed at how far we have come. We have had numerous ups and downs in the past few months but continue to be amazed by the support we are receiving from family, friends, co-workers, community, doctors, church and our new MPS family. As we anxiously await Jasper’s first Enzyme Replacement Therapy appointment we feel blessed for Jasper to have received the chance for treatment and what it will mean for his life. We no longer look at his every movement in search of answers and analysis but see our Jasper again. We see ways we can fundraise to find a cure, we see wonderful people that we never would have met if it wasn’t for this disease and we continually search for ways to make Jasper’s journey positive and life changing for everyone he touches.

Andrew and Ellen, you truly are amazing individuals and together with your 2 boys you make an amazing family.  As I said before, without your help we would have been lost.  Darren and I will forever be thankful for everything you have done to help Jasper and to help our family.  Jasper is scheduled to start his first treatment session next week and without you that may not have happened.  For this we are forever indebted.  Please accept our extreme thanks and the thanks of our families that are with us here this evening.  Please know that we look forward to connecting our families for as long as it takes for us to find a cure for Isaac and for Jasper and for all others affected by this disease and beyond.  We are ready and willing to step up and support the Isaac Foundation in whatever capacity we can.  We look forward to fundraising right along beside you in the search for a cure.  We are ready to hold out our hand in hopes you will reach out and take it as we walk this journey with our families.  We are so happy to be here tonight and know it is just the beginning of great things to come.  Look out Andrew some of that dedication and determination may be wearing off on us!  There is a cure out there and together we are going to find it.”

Then some of us (Clay not so much) enjoyed the sounds of Sarah Harmer playing her guitar as she sang some of her favourite hit songs.  Then it was Clay’s turn, the dance floor opened up and Clay got his groove on, and shook what his momma gave him…..just past midnight, until his parents made him go to bed.

The More kids and the McFadyen kids got along very well together and I could tell this was going to be the start of a great family bond between us. 

We began to look forward to the coming Wednesday, October 5^th.  This was to be the first day of treatment for Jasper.  That is until Tuesday morning, he began to cough.  It was with great disappointment that we had to postpone that much anticipated treatment start till the next week.

And so it arrived.  We had an appointment with the neurosurgeon on the 4^th so we went down and took advantage of the Ronald McDonald house on site at the hospital.  The neurosurgeon was happy with all that she had read about Jasper thus far, and everything she could see from him in person.  Her biggest concern was with the common spinal cord compression which often occurs in MPS patients.  She will order and MRI to be done and will likely want to repeat it every 6 months or so, to keep a close eye on it.

After a decent nights sleep in the Ronald house, we made our way across the driveway to the PMDU area of the hospital which will become our home away from home.

Jasper was very apprehensive on this first visit as you can well imagine.  Our first ‘struggle’ came when we were supposed to give him is oral pre-medication.  Tylenol and Benadryl given to help prevent or offset any minor side effects such has headache, fever, skin irritation etc, that the Naglazyme might cause.  Holding him down, and trying to keep his mouth open at the same time as he’s screaming, we force the medication in.  Not even bribing him with a popsicle helped.  By this time, the Emla cream we put on over his port to freeze the skin, had been applied more than an hour before, so it was time to access the port, or in Jasper’s words, “GET POKED”.  He did not like this part at all.  But after the initial shock, he calmed down fairly quickly.  We then began the four hour process of pumping his body with 130ml of the artificial enzyme.

On pins and needles we waited, watched, and hoped that things would go well, and he would notice little or nothing to do with this foreign matter entering his bloodstream.  Once again, our prayers were answered.  Four hours later, after a lot of games, a movie, and a brief nap, the $4775 bag of fluid was empty.  After another hour of straight saline solution, and a few more vital checks, we were cut loose.  We had to stop on the way home to get another fish of course, cause the first three were gone already. J

The coming weekend was to be another long awaited one.  One we’ve been looking forward to and working for, almost since we heard the letters MPS (together).  The Scotiabank Toronto waterfront marathon 5k event.  At this time, I want to thank you all again, who generously sponsored our team in our effort to raise funds for the MPS Society of Canada.  Together we raised over $12,000.  What an amazing feat.  I also want to thank our friends and family who took time out of their busy schedules to help with the fundraising and then joined us in Toronto either the day before, or the day of the big event.

And what an event it was.  To be one person in 6500 in the 5k event, one person among 22,000 in all three events, and one person, there raising money and walking for my own cause.  This was very humbling.  Seeing all the other people there, walking for their own reason(s), some on crutches, some using walkers or wheel chairs……  The kids in our group all did a fantastic job, there was very little complaining and lots of laughter and cheering and having fun.  Which we couldn’t have asked for anything more.  I cannot wait for next year.  I’ll be watching the Scotiabank Marathon web site for info on next years event, and waiting for the time when I can sign up.

That pretty much brings us to the second Wednesday of treatment.  This time we leave home about 7:30am to begin our day.  Things go really well at PMDU this time around.  Jasper even stepped on the scale to be weighed all by himself for the first time.  Mom and dad put the EMLA  cream on before we get into the waiting room this time, so it will be all ready to go on time.  Jasper is a big boy this week, and takes his pre-meds without a fuss, and only a little bit of crying when it’s time to get poked this time.  No crying at all when taking the band-aids off, or when taking the needle out of his port.  Mom and dad are SUPER proud of the little man.  Once again on the way home, we have to stop and pick up a new fish because yes, this one didn’t last the week either…..  good thing they’re not expensive and good thing we haven’t had time to get too attached to them either.

So, with all this going on, throw in the fact that Daphnie has started Jazz dancing on Monday evenings, and Hip-Hop dancing on Saturdays, Clay has started hockey Monday, Wednesday and Saturdays, mommy has started bridge, and daddy has started curling.  As you can see, October has been….full.  I’ll be glad when it’s over, but first…….HALLOWEEN!!!!  Pictures will follow!

Supreme Bummer

Jasper has developed a 'barky' cough last night and this morning so they have postponed his first infusion until next Wednesday now.  :-(

Port Insertion Surgery

As a father, yesterday (Sept 29/2011) will go down in history as one of THE TOUGHEST days of my life……..so far.

It was with very mixed feelings since the day the surgery was first booked, through the early part of this week when we found out surgery had been expedited till the time we were able to hold Jasper again when it was all over.
Mixed feelings because the whole while we knew that this surgery was 100% necessary to make life easier with the many infusions he’s going to have to endure now, while on the other hand………… what in the heck are we putting this poor defenceless boy through?

As you see in the photo, pre-op, he was playing the morning away with his friend Terry (Child Life) and all the toys in the pre-op waiting room.  Mom, dad and Terry are every once in a while trying to remind him about what’s about to happen using ways and words that aren’t going to upset him.

Then it’s time to go, rather than lay him in a steel caged crib to be whisked away, Jasper says he’s fine to walk in with Terry.  Mom and dad breathe a sigh of relief because we expected a fuss at that point.  I wonder…….knowing what happened to him now, if we were in that position today….would he be so cooperative as to go with Terry?  Will he ever again?
Mom or dad wasn’t allowed to go into the O.R. with him because we didn’t have time to register in the hospital’s NAP program.  Looking back, maybe it was a good thing.  Terry told us that Jasper did very well going to sleep.  He was apprehensive at first with the other strangers in the room, but they all took their time, and when he was asked, Jasper sat up on the table and then laid back, and then put the mask on without any fuss.  Hearing this makes mom and dad very proud and somewhat relieved because we could have expected a big outcry and un-cooperation.  It also made me very sad to picture that happening in my mind.  That poor dear sole, who never hurt anyone, has to go through that scary process by himself.  Both mom and dad are fighting back tears.  He’s brave and strong, why can’t we be?
It’s now just shy of 11:30am.  We go to the waiting room and watch as various surgeons come down and speak to other waiting friends and family and tell them that their loved one is ‘done’ their surgery and will soon be in recovery.  We wait a few minutes in case the anaesthesiologist has any questions, then we decide to go and grab a quick bite, since none of us had eaten anything yet.  Grabbing a drink and a muffin we go back and eat it in the waiting room because we want to be there as soon as the volunteer comes to get us to tell us Jasper is in recovery.
First, the surgeon comes by and calls us over.  “Everything went well, and it’s all done.  At first, the tube kept wanting to travel down Jasper’s arm rather than down in to the aorta, but after a few attempts, it got to where it needed to be.  We tested it and it works fine, so they shouldn’t have any trouble using it for his replacement therapy very soon.”
We feel another big sigh of relief as we sit down again and wait for them to tell us we can meet J in recovery.
A few minutes later, we get the call, a volunteer takes us over to recovery.  Jasper is lying in the crib crying and crying, not really opening his eyes, just crying inconsolably.  “owy owy owy, my tummy hurts.   Can you kiss it mommy?”  After a few short minutes of this, daddy has to sit down.  My stomach is weak at the best of times, but now, seeing and hearing these painful cries.  As if I didn’t feel guilty enough already.  I don’t think daddy can take much of this…….  They give Jasper a shot of morphine on top of the Tylenol he had going in to surgery.  That seemed to calm him down and it made him sleepy again so he had another nap.  While he was asleep, mom and dad talked to the nurses a bit, and listen as other children in the room who have had other various types of surgeries are waking up and crying in pain, and yearning for their parents to come in as well.  One little girl (Pearl) whom Jasper played with a little bit in the waiting room pre surgery, is continually crying for her mom, dad and baby sister.  We hear the nurses say to each other, “we can’t find her parents, they must have gone for a walk, or to get a coffee or something.”  My heart went out to that little girl, and I couldn’t help but think, ‘parents, if you could only hear your little girl crying out for you……’  It was tearing my heart apart, and it wasn’t even my child.
As we watched J sleep, I prayed that when he woke up, we’d get our Jasper back.  I prayed that the anesthetic would be completely worn off, that the morphine continued to help with the pain, and that he could be awake without too much discomfort.  I knew that if he woke up crying again, and still in a lot of pain that I would not be able to take that.  I recall thinking, ‘why God?  No child should EVER have to endure the pain and suffering of surgery, of any kind.  They’ve done nothing wrong, and yet, they have to suffer.’  Mommy remains strong, as daddy loses the battle against the tears.  The nurse says it’s time for him to wake up now……the moment of truth.  Does daddy have to face his little boys’ pain and anguish again?
As he wakes this time, he doesn’t frown, and doesn’t cry.  He’s thirsty.  Those familiar words, “Can I have juice?”  Daddy cries again, tears of tremendous joy and relief this time.
We stay in recovery for a another 15 mins or so, and then he’s ready to be moved to post-opp.  Daddy gets to carry him from one room to the next, and then we sit and cuddle the next hour and a bit away in the post opp room.  Quieter all around, a lot less crying.  More juice a couple halves of popsicles, a shot of Advil before the long ride home, and by 3:30pm, we’re sprung.

On the way out of town, we stop at the pet store and pick out three fish to bring home with us.  We wanted to get home soon, as J would be able to have more Tylenol by 5:30pm if he needed it.  Lo and behold by the time we were home, if he wasn’t routinely talking more and happy to see his brother and sister and show them the fish he picked out for them.  “Here’s some medicine J.”  “I don’t want it.  I don’t need it.”  That’s awesome.  We were hoping to skip the next dosage of Advil, but if he doesn’t need Tylenol either, that’s great.  We’ll wait and give it to him at bed time.  So the fish have names now.  Jasper has named his “Terry”, Daphnie has named hers “Pearl”, and Clayton has name his “Yellow”.  Note all three fish are your general run of the mill ($0.19) GOLD fish.

 And so, the kids are in bed, mom and dad breathe the last big sigh of relief on a long day.  Thank you Jasper for being as strong and as brave as you are.  As I tuck him in, I tell him, “I hope this is the hardest thing you ever have to do” but a knife in my stomach tells that it’s probably not about to be.

Life is hard.  Parenting is hard.  Watching your kids have to go through something like this is excruciating.  God give me strength.  I almost let you and Jasper and mommy down this day.  I’m going to need a lot more if we ever have to do something like this again.  Amen.
Thank you to all of you who prayed, thought of and cared about Jasper and our family through the surgery and the entire ordeal as we continue.
Stayed tuned for the next story:  “First treatment”  Gosh I hope that day goes easier than this one…………!!

Moving ahead!!

Port insertion Surgery has been expedited to Wednesday Sept 28 @ 11am.  Treatments will be able to begin the following week, October 5th.
Hopefully Jasper will take the process of surgery okay and recovery period will be minimal.  The child life specialist has agreed to meet us pre-op and play with Jasper for a while as well as go into the OR with him until he is asleep.  I hope that's enough....  I'm nervous and worried that he'll be upset but hopeful that he'll surprise me and be a "super dude".  We practised on 'George' (his doll that the child life specialist gave him) last night, put the gas mask on him till he was asleep and gave him the IV, and then inserted his own special port, then stitched him up.

I think Jasper will be okay with it, because he is okay with having a port like Isaac :-)  Please keep Jasper in your thoughts and prayers while he undergoes surgery tomorrow and treatments to follow.  I'm looking very forward to seeing the positive changes in Jasper, that I'm this therapy will give him.
God bless.

Nothing major.  Were in London yesterday.  Met with 'Terry' the Child Life specialists.  His job is to play with the kids.  Give them something positive at the hospital as opposed to just doctors and nurses doing all the poking and proding.  Not saying doctors and nurses aren't positive.
He went through the process of surgery and showed Jasper a port that had been inserted in his puppet's chest already.  He sent us home with some props and tools used in the surgery so Jasper can play with them and become more familiar with them before it's his turn.

We also had a baseline check in with a dentist there in the hospital.  He was very nice and friendly as he only deals with children.  He says Jasper mouth, teeth, gums, jaws etc are perfectly normal.
Still no word on having surgery moved up, but we're still trying.
Take care everyone.

The Canadian MPS Society - Smiles

The Canadian MPS Society - Smiles

Status update on Jasper

Our most recent trip to London was yesterday (Sept 7) in which we had a follup appt in the Metabolic clinic just to make sure all Jasper's baseline checks (height, weight, GAG level etc) were done and up to date before we start treatment.  We we also fortunate enough to have our pre-opp with the anesthesioligist done as well.
Port-a-cath insertion surgery is scheduled for Oct 19th, but we're hoping our team is able to get that pushed up as they won't start ERT on Jasper until the port surgery is done.
At this point, it looks like Wednesdays will be our weekly trip to London for treatments.

All the best to all.
The Mores

Jasper's Story

Jasper Dennisun More was born, January 9, 2009, a healthy 10 lb 3 oz baby boy.  We were so happy to bring home our beautiful boy who would complete our family.  As a newborn baby Jasper was usually a happy and content baby who slept well and was a joy to have around.

Around the age of 3 months we went for a routine check-up with our family doctor  and questioned what turned out to be a bi-lateral hernia, meaning and inguinal hernia on both sides.  We were referred to a pediatric surgeon in London who would repair the hernias.  At the same time, she mentioned, Jasper also had an umbilical hernia which they wouldn’t do anything about right now.  (This is the reason his cousins have dubbed him “The KING of OUTIES”)

Following surgery to repair the hernias, we assumed everything was great, we had our healthy baby boy back.  About 3 months later, we noticed a small bump on his lower spine would appear when he would bend over.  We questioned our family doctor at our next appointment , and he referred us to Dr Carey, a Paediatric Orthopaedic Surgeon, also in London.  At this appointment Jasper had some x-rays taken and we were told he had what was believed to be lumbar kyphosis.  He suggested we wait it out for a period of 6 months in hopes that with growth and time it would be corrected as his bones continued to grow and mature.

After a long 6 months of waiting, we did not see any change in the appearance of Jasper’s spine and we also noticed he had started to develop a deformed rib cage. The bottom of his ribs seemed to flare out and his chest had started to protrude slightly.  On our return visit to the orthopaedic specialist, we pointed out these changes, and Dr Carey ordered more x-rays.  Upon studying the newest set of x-rays we were told that “something just isn’t quite right with his bones.”  We were told all his bones were present and they appeared to be the right size, but the formation was not quite what it should be.  It was then that we were referred to a Genetisist in the hopes of detecting what was going on.

Approximately 6 weeks later we were introduced to Dr Goobie.  After asking us many questions and doing a thorough exam of Jasper she ordered both blood and urine tests.  When the results of these were received we were called to London to meet with Dr Goobie and this is when our world crashed.  Never in our wildest imagination did we expect to receive such devastating news about our beautiful little boy.

In April of 2011, the blood and urine tests confirmed that Jasper had Mucopolysacharides Disease (MPS).   We continued to question how something so rare and serious could affect our family.  Through much discussion and explanation she assured us it was.  The GAG levels in his urine were proof that his body wasn’t breaking down and disposing of waste the way it should.  Instead, it had already begun to store itself in his liver, spleen and bone structure.

There are various types of MPS and the next step in Jasper’s journey was to determine which specific type of the disease we were dealing with.  We were told that type I and type II were the most common and this is what they believed Jasper had.  Another blood sample was taken in hopes of determining the type.  After a long couple of weeks waiting for the blood test results, we were called to return to London for yet another blood sample.  The first results came back showing negative on type I, type II and type VII as well.  Dr Goobie was surprised as she felt very certain he would have had one of these forms of the disease.  At this point in time, we also agree for a skin biopsy to be done which takes much longer for results to arrive, but will be 100% conclusive.  In the meantime they continue to assure us that he has MPS and we begin to research the other types they are now testing for. 

Two more long weeks of waiting and worrying and we are told the results of the blood work are pointing to type VI but still inconclusive, we must wait at least 4 more weeks for the skin biopsy results.  Upon hearing that it appears to be type VI we are ironically happy.  The major difference with type VI is that it does not affect the brain and cognitive function and we see this as such a huge positive.

After waiting four more long weeks, we arrive in London for an appointment with Dr Goobie and we now meet the newest member of Jasper’s medical team, Dr. Prasad, Metabollic Specialist.  It is at this appointment we learn of the results of the mapping of Jasper’s DNA that they had started a few weeks back.  It has been confirmed that he has type VI and we review what this means and where we go from here. 

We learn that Jasper is only the 8^th known case of MPS VI in Canada (there are about 70 in the United States and 1100 worldwide) but we also learn that there is a treatment available.  Enzyme Replacement Therapy (ERT) using the American made drug Naglazyme.  We learn the drug is not recognized in Canada at this point in time and it is very expensive to the cost of $300,000 to $1,000,000.00 per patient per year.  We become aware at how difficult it may become to convince the Ontario Ministry of Health to fund treatment for Jasper.

At this point in time we learn of an amazing family in Campbellford, Ontario who has gone through an MPS VI diagnosis and successfully advocated to receive funding for treatment.  From the moment we contacted them, Andrew and Ellen McFadyen were a true blessing to our family.  Parents to Isaac who is 7 years old and has MPS VI and Gabe who is 5 they offered emotional support, advice, kindness, encouragement and assistance in obtaining funding for Jasper.  They have founded The Isaac Foundation, a registered charity, devoting all proceeds toward research into MPS type VI exclusively.  Isaac has been receiving ERT, funded by the Ministry of Health in Ontario for approx 4 years now.  He was the first in Ontario to seek and be approved for funding.

Our journey brings us to August 2011 when all the paperwork is filed to apply for funding.  There were so many steps to be followed, the claim had to be completed and accompanied by letters written by experts in the field, sent to our insurance company for review and to the Ontario Ministry of Health.  Initially we were denied and we started the process of appeal and advocation.  With much help and support from family, friends and The Isaac Foundation our application for funding was approved.  When we heard this news we were ecstatic!  A huge weight had been lifted from our shoulders.  Our little boy has been given the chance that every child should have – the chance to grow up.

WELCOME

Welcome to the More family Blog.
Here we will try to post photos, updates etc on all things to do with our family and as the blog title states, "Adventures".
Including a story and continuous updates on Jasper and his fight against MPS VI.
Please keep us bookmarked and check back often for updates.  I'm hoping this will alleviate mass email messages to family and friends every time there is news or updates.

Take care everyone.