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.

Friday, April 26, 2013

Long Day of Surgery

All smiles before surgery!
Today marks the first day of Coleson's treatment plan.  It is the day we've been waiting for since we received the diagnosis but a tough day none the less.  He is having 6 procedures today and will be under aesthesia for about 6 hrs.  So if there is a day we need thoughts and prayers, it's today.

He first is having a ERG - Electro Retina Gram - measuring the electrical response of his eyes and then an eye pressure exam.  Then he gets his Hickman port and G-Tube put in.  He also has another MRI and his first intretecal injection (to the spinal cord to get the enzyme to the brain).   Then he has to have his ear tubes replaced.  The left one is clogged due to the thick waxy fluid build up that is common in MPS kids. 

Yesterday we saw the PT/OT/ENT... QRST (just kidding) and Pulmonologist.  As far as his fine and gross motor skills he is right on track with a normal 18mo. old, however we know his progress will be halted by chemo/BMT so we need to start therapies and working with him pre-BMT to ensure he gains as much as possible beforehand.  Also the Pulmonologist said his lungs/breathing/respitory system seems strong though she did inform us of the eventual risk of Restrictive Lung Disease which is due to the smaller rib cage that will prevent him from taking deep breaths as he grows.  So he may not be able to run a marathon or exert himself like a healthy kid could...but perhaps he will follow daddy and be a golfer or horseman! 

The worst news we received yesterday was from the ENT who informed us of the clogged tube and also did some audio tests on him.  He has mild to moderate hearing loss in both ears and recommended we get the sedated ABR test to be certain the degree of loss.  We tried to schedule it for today since he was going to be sedated any way but they couldn't fit it in with the already very full day of surgeries/tests.  Since we don't want to sedate him more than necessary we will probably wait until we return in June to have it done.  She also recommended a sleep study to see if he has sleep apnea - another common MPS sypmtom.  As many of you know, he wakes up frequently in the night still..which means so do I.  Also as many of you know he has had hearing issues since he was born and I have not been happy with his ENT since it took 17 months to convince him to do the tubes in the first place.  I really feel let down that he did not take his hearing issues as seriously as he should and if he did, we may have corrected any hearing issues within the first months of his life.  However, I will try not to dwell on this for too long.

Everyone here is so nice and they really care for the familes.  In fact we were delayed at the ENT, missing our consult with the surgeon so the surgeon actually came over to the ENT's office to meet with us.  It is even in a different building! We also met another mother (not an MPS mother) in the OT's office who recognized us the other day at the cardiologist.  She is a manager at Bruegger's Bagels and gave us a free breakfast today.  This is the first breakfast I've had all week!  This is where I'm typing at the moment since Coleson won't be out until 2:30 this afternoon. 

We skyped with Peyton again last night and you can tell now that she is starting to miss us, especially her baby brother.  He was sleeping during our video call but she got a kick out of seeing him none the less.  At one point we thought she was close to crying but we reassured her that we were going to be home soon.  She is counting down the days with smiley faces on the calendar.  She seems so grown up.  She has told us all about her days playing with friends (it was Avery and Jocee last night) and what a good girl she has been.  I was her age when my mother was dying of cancer and finally did so soon after I turned 4.  I know she will have some memories of this time even later in life and so I get particularly emotional when it comes to the impact on her.  But I know she is a strong little girl and will be the best big sister that Coleson can have.    Here is a picture of our princess (princess Belle this day).

Princess Belle going to see Beauty and the Beast at Gammage Theater

We will be staying overnight in the hospital and he will have his first Enzyme Replacement Therapy tomorrow.  Another long day but again a day we've been looking forward to halt futher progress of this disorder.  Then we will be coming home... albeit temporarily.  We will be returning for the long haul in June perfect timing to miss the Arizona Summer and enjoy a Minnesota summer.. albeit from inside the hospital room. 

I will try to update again after he is out of surgeries!