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.