Ahh - Home Sweet Home!

This kid never ceases to amaze me. 

We got home last night and he was just a real champ. We dosed some oxycodone before he went to bed, and I thought for sure he would be up with pain but much to my surprise, I was the one that was up all night checking on him, as he slept like a baby through the night.

Today we were flooded with visits from friends and families bearing food and gifts. Cannon loved all the attention and I think I caught him hamming it up a bit at times where he'd moan a little until his cousin came in and he'd perk up and ask "Grab a remote, we can play Need to Speed!" Hmmmm.

We were surprised by the Green Room team by Cannon's favorite meal: spaghetti and meatballs! (Note: Pasquales meatballs rock!) He ate his biggest meal yet, had his first bath and is now resting comfortably. His goofy personality is even starting to every so slowly come back! Here he is concentrating on his meatball dinner.

Anyway he's really handling the pain fabulously. He does have tears when it really hurts him and the medicine works wonders, making him comfy for a nap or able to walk downstairs for a quick visit. His incision seriously amazes me. I have never seen anything like it. It's healing beautifully and I swear the surgeon that did his stitches must do intricate needlepoint. Amazing work!

But I'm amazed at how he continues to push himself. He has great self discipline and even does exercises on his own. We strategically put the Xbox and PS3 into different rooms to force him to walk around a bit and he quickly took the bait!

In the meantime, all 18 kids that visited today (minus the adults) took turns riding scooters and bikes and doing laps around the block, taking the dogs for walks, and keeping each other busy doing old fashioned things like making slingshots out of sticks, string and tape (ha!) and going to the park.

Also a big THANK YOU to everyone who has dropped gifts on our doorstep and have come by to say "hi" - the kids (and I) really appreciate it and we are really overwhelmed by the incredible outpouring of support and love. 

Day Two Was Tough - But It Could Always Be Worse

There's no real way to sugar coat things now -- Thursday was tough. During the surgery, doctors had to manipulate the muscles in the neck, pulling them way over to the sides to access the base of the skull and spinal column. If you're like me, those muscles are tight and get pretty sore even during a deep tissue massage. So I can only imagine the pain that he's in right now. On top of that, he has a sore throat and a huge blister on his tongue from the ventilator, which makes eating difficult. He's still on some heavy duty pain meds including Valium, oxycodone, Tylenol and Toradol. Despite this, the poor guy was moaning all night long.

I was totally proud of him yesterday for pushing through the pain and trying his best to do what the doctors and nurses asked him to do. And whoever decided to make liquid Valium taste like black licorice? Seriously? What kid likes black licorice?!

So the goal was to get him up and moving, which he did like a champ, and to get him moving his neck -- which is NOT easy. We were joking that he could master "The Robot".


But one day, one step at a time, he's still in a lot of pain and really struggling to even lie comfortably in bed. He really appreciates all of the comments and especially the videos from his friends and family. It is really the only thing that will make him crack a smile.

He met with the physical therapist who walked him through some exercises, with the help of a little morphine.

He was even able to walk down the hall and even did a flight of stairs!

While Cannon is pushing through the pain and really making us proud, we are so thankful because it can always be worse. There's nothing more sobering than being in a hospital surrounded by sick children with cancer, and other life threatening diseases. So while we will share Cannon's experiences, please know that all in all we are truly thankful for a boy who is healthy overall and may even get to go home today, unlike many children here, may never leave this hospital.
Thank you for all your prayers and well wishes. We hope to leave today (Friday) to get back to Mountain Lakes for the weekend (fingers crossed)!

Out of Surgery - Recovering!

After 3+ hours of surgery, Cannon is now in the ICU resting comfortably.



Cannon was in GREAT spirits this morning. He ate up until the last possible minute the night before (Fritos, Doritoes and a bag of M&Ms at 10:59pm before the 11pm deadline) and some water. He slept great and that probably contributed to his good mood. We arrived at the hospital at 9am for tests. Then he had his "giggle juice" (aka Valium) -- which seemed to work within 5 minutes... here's a quick video to show you.

http://instagram.com/p/luwcAmBSP7/#

Surgery started at around noon and was supposed to last 90 minutes. The nurse came out to inform us he went to sleep easily and that his vital signs were good. However, at the 90 minute mark, the nurse came out and said it would be another 90 minutes.

There was an important variable in his surgery -- Cannon had a syrinx -- or a pocket of cerebral spinal fluid -- that was clearly visible on the MRI. This photo below (while not Cannon) reminds me of what Cannon's MRI looked like. In the Before picture, there is a white sryinx which is a fluid-filled neurological cavity within the spinal cord that shows that the fluid is not flowing down his spine normally. This is why they wanted to do the decompression surgery, to relieve this pressure and help the fluid start to flow regularly again.

There are two ways to relieve the pressure. The first way is a craniectomy is to remove the bone causing it (in this case - the base of the skull has tonsils that can become malformed and put pressure on his spinal column). Sometimes this works alone -- and the removal of the bone allows the fluid to start flowing freely. Other times it can take a bit more to relieve the pressure, and doctors will have to open up the dura, or the thick membrane that is the outermost of the three layers of the meninges that surround the brain and spinal cord.

With Cannon's case, there was a slight complication. Once the surgery began, doctors realized that the elevated pressure had caused Cannon's anatomy to change - and hair sized veins were the size of fingers which forced things around a bit so that normal veins and arteries were not where they normally should be. The enlarged veins were also troublesome because if not handled well, he could have lost a lot of blood. The pediatric neurosurgeon that conducted the surgery actually said when he saw what he was dealing with, he called in 3 other neurosurgeons to consult. He also told a story about how he had seen a case presented by a leading surgeon at a medical meeting where another child had a bone disease and also had enlarged veins and in his words, "the results were not good." (not sure what that means but it did not sound good to me!).

So the doctors decided they had to play it safe and just remove the bone. They did see some initial signs that the pressure was lessening and fluid was flowing better and decided to watch and wait. We will have a follow up MRI in a few months to ensure the syrinx is under control (or shrinks) and that the pressure improves. He said there are other ways they can go about lessening pressure in the future, should things not get better over time.

He has been resting comfortably, although he got sick from the anesthesia after surgery. He continues to keep up his positive attitude in between his snoozes.

The nurses and everyone at CHOP have been amazing. Asking lots of questions about HPP, very engaged in his treatment/therapy. Doctors willingly give out emails and are so helpful. We have had a great team every step of the way. Which brings me to the surgeons who sutured up his incision. I'll try to take a better photo tomorrow but I cannot tell you how incredible it looks. There must be hundreds of tiny sutures, it's like artwork. No staples or anything! The reason there are no bandages is because they look at the incision site to see if there's any evidence of pressure or swelling, which at this point, there is not.

Thank you again for all your thoughts, prayers, notes, texts, Facebook posts, visits! We could feel the love and the positive energy and it helped us get through a very tough day.

More to come tomorrow!
XO

We're baaacckkk - what we know about chiari and HPP

Hi Everyone! It's been a long time -- in fact the last post was in August 2013 which as you can see above that Cannon was tested for optic nerve pressure as part of his clinical trial for his enzyme replacement therapy.

During that day - that test you see above - they detected elevated optic nerve pressure, which can indicate pressure on the brain. Cannon has craniosynostosis, a skull abnormality that results from premature fusion of the soft spots in a baby's brain before the brain is fully developed. We have been watching for pressure since Cannon was a baby and we thought we were out of the woods, but during his last checkup we saw the elevated pressure and had an MRI.

The MRI showed no issues with the craniosynostosis, but instead uncovered a different skull abnormality called a chiari malformation. Basically the craniosynostosis can cause the back of the skull to push down lower than normal, causing the back of the base of the skull to push down and inward, putting pressure on the top of the spinal column. Not only was there pressure, but evidence of a large sryinx, which is a pocket of spinal fluid that indicates that fluid is not flowing normally down the spine. The pediatric neurologist that made the diagnosis was shocked that Cannon did not have any headaches, tingling in his limbs or other neurological issues. I will see if I can dig up the images of his chiari and sryinx. It was one of the few images that I've seen (with an untrained eye) on an x-ray where it was clear what the issue was.


The recommendation: a chiari decompression surgery, which is basically a craniotomy, or a removal of the bone that is causing the issue. A second opinion at Children's Hospital of Philadelphia (CHOP) yielded the same conclusion/recommendation.

Interestingly, chiari malformations are uncommon but both surgeons we saw averaged approximately three decompression surgeries per month!  

So tomorrow we head to the neurosurgery of CHOP in the Woods Building for our surgery with an arrival time of 9am. The only variable in the surgery is how invasive the procedure will have to be in order to relieve the pressure. Best case scenario: they remove the bone and the pressure is relieved and the fluid flows freely. Otherwise, if the ultrasound shows the fluid is not flowing, they need to go deeper, cutting through the dura (casing surrounding the spinal column and brain) to further relieve pressure. We are hoping for the best case, obviously!

We will keep you posted on his progress. As for tonight we had his favorite dinner, shrimp scampi and two brownies (minus a la mode).

He actually asked me if I could do an intro to send a message to everyone -- so here is a link to it. More to come tomorrow.

http://instagram.com/p/ltIxmLhSMq/