Welcome to Coleson's blog. We've created it to keep friends and family updated on the journey we are about to embark on.. We appreciate your support and words of hope and encouragement during this time when it matters most.

Thursday, May 26, 2016

New Surgery, New Plans, New Attitude

HOne of the hardest parts about this process is the complete loss of control over the well being of your child.  You get hit with this overwhelming information, just enough to fill you with anxiety but not enough to put all the plans in place or put you at ease.  Once you have all the information you start to take comfort in the "game plan".  I feel like that is where I am at today. 

We met with the neurosurgeon yesterday.  There was no denying once he threw up the pictures of the MRI that the cyst exists and is big.  Even to my untrained eye I knew immediately where it was and that it was big (I'm sure you will see it too in the picture below).  He explained the root cause of the cyst was due to a malformation of the skull which creates a "pooling" of spinal fluid in the base of his skull.  This in turn creates and imbalance of fluid flow along the spinal chord and brain.  Think of it as a river, and his skull in creating somewhat of a dam which affects the rest of the flow of the river (aka his spinal chord) which causes other "pools" to form along it.  Fortunately it is only the 1 and there are not others.  Bone malformation is one of the most common Hurler traits and is the reason for the hip surgery.  Hurler Syndrome is a "storage" disorder meaning substance (called gag) that should get processed and expelled by the body rather does not and instead stays in the body in places where there is room, mostly the joints.  Because this gag material is sitting in places where bones would normally develop, it causes the bones to develop abnormally.  It is a total body affect but each Hurler kid's bones develop different depending on where most of the gag goes in the body and in Coleson's case it had a big affect on the skull. 

It is big enough that they need to address it.  To fix it, they have to break down the dam so to speak.  So next Tuesday, the 31st, he will have surgery to remove some bone at the base of the skull  which will allow the fluid to stop pooling in the skull which naturally (over the coarse of a year) should reduce/eliminate the pooling in the spinal chord.  It is too dangerous to do surgery on the cyst itself as that would be require surgery on the spinal chord.  We will be back at the University of Minnesota for this surgery so back to our normal stomping grounds.  I asked if this surgery could be combined with a new port placement and was happy at least to hear that it could be and in fact is scheduled with the same general surgeon who put in in his central line 3 years ago.  It does mean another 6 hr. surgery and 4 night stay in the hospital but at least he will walk out of the hospital.  We have some follow up appts and MRIs over the next year but we should be able to plan the hip surgery for next summer. 

Though I was hoping he would not have any more "Major" surgeries after the hip surgery, I guess, technically, this isn't after the hip surgery.  It is unexpected and a risky procedure itself, but it allows Coleson another year to comprehend the hip surgery which may not be a bad thing.  It does mean another traumatic and scary experience for Coleson however and though I was hoping as he got older, he would just "get used" to all the pokes, prods, medicines, IVs, X-rays, EKGs, MRIs, echos, blood pressures, pulse ox..... you get my point, I think he is getting more afraid of them.  We will have to take extra care in tackling each procedure as simple and nonintrusive as it may seem to be. 

So we have some re-arranging of plans left to do but it also allows us to get home around mid-July instead of at the end of July so that we can get settled before school starts on August 1st. I do believe that everything happens for a reason, so while I still don't know the reason why kids are born with such difficulties, I do believe we were not yet ready for the hip surgery and needed another year to prepare.

After the appt. yesterday, we all went to Mall of America and spent 6 hrs. riding rides.  Paul and I even got to ride the adult rides for once since Grandma was with us watching the kids.  Peyton is still not big enough to ride those yet but maybe, just maybe by next year she will be.  She can't wait.  Coleson too loves the excitement of the stomach turning rides and unfortunately will have quite a few more years before he will be big enough to ride them. 

So think of last Tuesday as a trial run for this Tuesday and next year.  The outpouring of support has really kept me going these past couple of days and I cannot thank you all enough for that. 
The white is the pooling fluid and the "dam" where the skull meets the chord.