Home, sweet home!

Someone is excited to go home.

Even the neurologist was impressed. The past 24 hours we saw a remarkable turnaround. He was sick, in pain, crying and immobile. Today, he was up and walking, playing games, moving his head around. It was like a different kid. 

Cannon has read (or been read) every single one of your messages, played the videos and enjoyed the photos. When he came home, he had a full envelope of handmade cards from his classmates - we went through each one. Some of them made him laugh really hard, others were done with such care and love, and others were super creative and so impressive. We can't express how much we appreciate it all. The car ride home was a little rough. Those PA/NJ highways aren't exactly the smoothest ride but he managed a smile knowing he was headed home!






The recovery continues. His incision pretty looks good but he is still on a lot of medications (there are like 10). 


Since he had a patch sewn in, he needs to really take it easy and give it time to heal. That means no running around, no roughhousing... it will be a long 6 months of letting things heal.

The hospital is a very sobering experience and being there made us realize how lucky we are. Thank you for all of your continued support and love. We felt it all along the way. 

Bacon, please!

This morning didn't start out so great. Lots of pain, complaining, and tears. But as the day has progressed we have seen a lot of progress!

First he's getting out of bed and even walking a bit... followed by a 3 hour nap.

Then... we thought food might be a possibility. I have no idea what it is about post surgery with this boy, but he could eat POUNDS of bacon. I ordered two orders and he was like, "That's IT?" Wolfed it down and then ate a good size plate of pasta with butter, a chocolate milk, and seems to be holding it down.

If we continue on this path, there's a slight chance we could break the world record and come home tomorrow! Originally the doctor said we should expect Sunday or Saturday best case scenario but today we actually heard the word FRIDAY.  Someone is doing a happy dance!

More to come later on - with pics!

Tough night but hanging tight

We had a small setback last night as there was some fluid leaking from Cannon's incision which required them to give him an extra couple of stitches. That didn't go over so well with the little guy. After that, he actually slept pretty well thanks to the morphine and other pain meds. 

Right now, it's intense neck pain and headaches. If you feel the back of your neck - those two muscles that support the head - those had to be pulled way over to the side by his shoulders to reach the spine and back of the skull, so you can imagine how sore he is. Add to that the pressure of the fluid changes and he is pretty uncomfortable.

I can't thank everyone enough for the messages! Cannon had notes from Paris, Lebanon, Canada and throughout the US. He thinks it is so cool to see how people are praying for him and sending their positive thoughts. That is the one thing that seems to really engage him to get his mind off the pain so thank you!

He still is cracking jokes between the tears. Last night - he held out his finger with the oxygen sensor and said "ET phone home." We got a kick out of that.

More updates soon!

Great news!

All of your prayers and well wishes must have worked! We had a great post-op report!

Dr. Heuer came into the waiting room and told us the surgery went much smoother than he had anticipated. Last time he had surgery, you might remember that the doctors were concerned because his arteries were enlarged and this forced them to be more conservative in the procedure as he lost a lot of blood. This time - because he grew - and pressure has somewhat improved - those arteries had moved and presented no issues! So doctors were easily able to get to the area of concern, remove the scar tissues that had built up causing more pressure from the previous craniectomy. They were also able to open up the dura, which we hope will relieve pressure even more.

We are in the pediatric ICU (PICU) and he was awake when we arrived. His pain seems under control but as the meds we wearing off, we have our moments. He has a morphine pump and is still taking Valium to help with muscle spasms.

His incision looks great - the doctors here must have aced their needlepoint classes as I have never seen such small, intricate stitches.

The best part is his spirit has not been brought down at all by this. He still had all his wits about him -- and his sense of humor. Apparently during the surgery they removed his lower hospital pants. He did not like this at all! When we asked him how he was post-surgery, all he could say was "Where are my pants?" And when the nurse said, "ok anything I can get you?" He said, "Yes, pants!"

And... his hunger has already returned which really shocked us all (even the nurses and doctors!)

For now, the pain is at bay and he is sleeping peacefully. Cannon loves receiving messages so feel free to send videos or texts to me at 908-377-8700. I will read them to him and show him when he is up and feeling better. Thank you again for all your prayers!

The wait

I have never been good at waiting. And this has to be the worst possible wait.

The nurse just came out and told us that he's still being prepped. He went to sleep like a baby and they said he was "high as a kite" from the "giggle juice" (Valium).  I'm guessing the procedure will start around 9am - and then will take 2-3 hours. That is one..... long...... wait......

We are here in the waiting room. I can't grasp how some families are eating, smiling, laughing, listening to music. I sit with my computer and a box of tissues - oscillating between tears and gratitude and comfort from all of the texts and posts. I can't tell you how much we appreciate your love and prayers. We all feel it and it makes such a huge difference.  Thank you.

Cannon, conversely, has nerves of steel. He was as cool as a cucumber last night. Slept well, stole the covers, told me all the things he wants for his "post surgery gift", downed a rack of ribs, 2 Shirley Temples and even took a dip in the pool. He now has new motivation to get home as the proud owner of a new Playstation 4. It feels like an early Christmas to him -- and that makes me happy.

And the nurse just came over again to tell us he's doing ok - things are moving very slow. They just put in an A-line (arterial catheter) to monitor his blood pressure real time.

In the meantime, we sit and wait.

Ignorance is bliss...

Cannon has been doing so terrific that sometimes it's easy - and wonderful - to forget he has HPP. But just when I think I can take a deep breath, the rare metabolic bone condition rears its ugly head to remind me of the severity of this terrible disease.

A few years Cannon had surgery to relieve a skull malformation called a chiari malformation. You can read more about it in my post from March of 2013. In a nutshell, Cannon's brain extends beyond the base of his skull into his spinal column. The pressure caused by this condition has caused a large pocket of fluid - called a syrinx - to form in his spinal column, preventing the spinal fluid from flowing easily up and down his spine. While he has no current symptoms aside from a headache here and there, neurologists fear that without surgical intervention, he could have other neurological symptoms (including loss of feeling or use of parts of his body).

The first surgery was a chiari decompression surgery where brain surgeons removed a piece of bone from the base of his skull with hopes that this would help relieve the pressure and improve the flow of spinal fluid. Unfortunately, because the pressure was so high, they had to be very careful and conservative with this procedure, as Cannon had some arteries and veins that were supposed to be the size of hairs that were the size of a sausage. We are very blessed to have skilled surgeons at CHOP who knew that this put him at risk and made this decision on the spot in the OR the last time around. I could tell by the look on the surgeon's face the last time that it was not an easy surgery for him.

Cannon has since had followup MRIs to monitor the syrinx, which unfortunately has actually not decreased, but possible even increased, in size. Because of the continued neurological risks, we are going back for a second round of surgery - this time likely more invasive. An anatomical MRI seems to indicate that the veins and arteries that were swollen the first time have shrunken in size, making an intradural decompression surgery more feasible. The "dura" is the sheath that encases the spinal column. This time, surgeons will cut through this sheath to hopefully relieve the pressure within the spinal column that is causing the syrinx. Of course, I'm explaining this in the most basic of terms, but that is what we are dealing with.

Cannon had his pre-op appointment today - he did will for his vital signs, bloodwork and testing. Now, we wait until Tuesday. Please keep Cannon in your prayers and pray for his surgeons who will be performing this very intense procedure. We are comforted knowing that these surgeons are so incredibly skilled and talented. The last time we met with Dr. Heuer, he left our appointment to perform spinal bifida repair on an unborn baby in utero. Just incredible and amazing! He will be joined with the Head of Neurosurgery for this procedure, Dr. Storm.

More to come! Thank you everyone. <3

Clinical Trial Check-In - Month 54!

Where has the time gone? 54 months have passed since we started this blog when Cannon first entered the clinical trial for asfotase alfa, the enzyme replacement therapy (ERT) for hypophosphatasia. Every 6 months or so we've been traveling to Pittsburgh to the wonderful Children's Hospital there for a clinical trial check-in where Cannon sees a slew of specialists including endocrinologists, radiologists, ophthalmologists, physical therapists and geneticists as they examine him for his progress on the experimental drug.

This time was the first time I finally had the chance to meet Nadene Henderson, our Site Coordinator. She has been a tremendous help getting all of our appointments scheduled and helping us to get to and from Pittsburgh answering any and all questions along the way.

Today we hit some milestones. My "little" boy weighed in at 81 lbs! He is growing like crazy too. I got a little teary when Dr. Vockley got to the "short stature" box and said he officially had to circle "resolved" because he has hit the 25th percentile. Talk about prayers answered! Here's a clip where Dr. Vockley is examining Cannon and comments on his sclera.

Cannon grew 131.7 cm from 129.59 cm in the last 4 months! I thought he had been growing before my eyes... and he has been!

AND he gained 10 kilos which isn't surprising with my big eater.

Once we signed the consents, he had his blood drawn and it was time for breakfast: cornflakes with chocolate milk (gag). He was super excited for his new concoction and passed the time entertaining all the nurses with his silly jokes, videos and bringing them up to speed on the latest cool video games and Minecraft tricks.

From there we went for a kidney ultrasound. Luckily, we didn't have to have the full game of x-rays this visit, which usually is somewhat time consuming.

From there we went to our good friend Chris in physical therapy. Chris has watched Cannon grow before his eyes and has seen him go from a long jump of just a few inches to today's personal record BREAKING 4 FEET!!! WOW.  CRAZY. This kid went from a 4 inch long jump to 4 FEET in 4 YEARS. He went from 35" to 48" in just the past 4 months!

Now on to eyes, Cannon's least favorite part of the day with a dilated eye exam to look at his optic nerve. We have been concerned about his optic nerve since he had his craniectomy in March 2014. We still need to have another MRI before he's in the clear as his 6 month post-op showed that his syrinx was still pretty pronounced, which wasn't the greatest news. We continue to pray and be hopeful that we see some good progress at the 1 year mark.

The ophthalmologist examined Cannon and said that his optic nerve looked good. He still has some minor calcification on the surface of his eye but that was consistent with what we have seen before and they don't seem too concerned about it.

Overall a great visit and we are headed to the airport to hopefully catch an earlier flight home!