Surgery countdown is on...

Well, we had another busy day today though not as long as yesterday. Kahlial didn't love the pre-op chores so much... checkup with neurosurgery, pulmonary testing, chest xray, final halo fitting (not as "impressive and viking-esque" as yesterday without the rods and sheepskin coming out an extra 5 inches everywhere). Kahlial wasn't impressed with it during either fitting, repeating "out, out, out!"

He's actually only gets upset momentarily while being messed with, otherwise he's saying "thank you" and blowing all the nurses and docs kisses. He actually loved his opthalmalogy exam, telling the doc what all the 3-D pictures and the items on the screen were, then getting Percy & James (Thomas the Train characters) stickers for reward. I always anticipate him trying to take them all when they offer up the whole basket of stickers, but he very careful sorts through looking for what he wants, Thomas the Train is always highest on the list these days.

Well, just got official word... we're first case! Can't believe I just put an exclamation point after a sentence about our son going to surgery. Yet, I'm happy to get in and get the surgeon while he's fresh and hasn't drank too much coffee yet. So we've got about a sixteen hour countdown with pre-op beginning at 0730 and surgery approximately 0900.

We're planning on taking Kial down to the Holiday Workshop downstairs here at the RMcDH. They are providing all the children here with five gifts each (not just patients, but brothers and sisters too), then two gifts per adult. Pretty amazing! I'm very impressed with all they do here. They're offering a magic show later too, but I think we're going to choose to stay in our room to cuddle, snuggle, and play with our guy.

Oh, one other thing... Just had to share our amazement as Kahlial just told us every letter of the title on the book Eric is reading. He amazes us constantly, one day counting to 12, next day telling us all of his primary colors, but this one takes the cake! He had our jaws dropping to the floor this time. Here's hoping he amazes us every day with his seamless surgery and recovery.

Pre-op Euphoria!

Here's Kahlial in his medicated happy state prior to his CT... so cheesy and too funny! He was rambling on about Thomas the Train and all his friends and could pretty much care less that he was being rolled away from us. We had a slew of appts today straight through from 8-5pm. Kahlial did so great. We were totally shocked when he actually opened his mouth, stuck out his tongue, and said "Ahhh".... twice, with two different docs!

Anyhow, things went well. Saw Dr Rappaport, a pediatrician with specialty in medically complex children at the end of the day... Great guy, great sense of humor, great note to end our day. We'll be keeping an eye on a couple of Kial's labs throughout his stay though, as he is apparently a bit anemic and neutropenic at present. We meet with the neurosurgeon, pulmonologist, and opthalmalogist tomorrow.

Kahlial did get fitted for his halo today. We'll do another fitting after they trim down the lining and rods tomorrow. It looks so "viking-esque." You'll see tomorrow (I'll try and remember to take a pic this time and post for tomorrow). One person made the comment that it was "really light".... Sure, light to a 125 to 200 lb adult. Yet, 4-5 lbs placed on the torso of a 20 lb little boy is different all together. I'm sure he'll totally surprise us with how well and how quickly he adapts, or so we hope.

Apparently, Dr Mackenzie, the orthopedic surgeon performing the fusion will have just two cases on Wednesday. Still won't know until tomorrow if we'll be first or not... Stay tuned. Almost forgot the best news of the day - They are estimating under 4 hours for Kahlal's procedure (much better than the 6-8 originally discussed.

Flight and Arrival in Delaware

Well, Kahlial got his wings! We had a really excellent flight crew for the second half of our flight and the captain offered Kahlial to come check out the cockpit. It was great and they loved him... He's such a little ham! In total, he didn't sleep but about the last 30 minutes of our flight. That, and all else considered, he was really a champ on the planes. Didn't even have to break out the candy and earplugs for those around us. It seemed like it really clicked for him what was going on, that he was "flying in the air on Jeremy the plane" (Thomas the Train character).

Well we're very happy that weather wasn't an issue in getting here. A nice little tailwind actually got us into Philly about a half-an-hour early. Eric was pretty excited to see the stadium all lit up (Army vs. Navy game). He tried hard to talk us into a detour, but we continued onto our "home away from home," The Ronald McDonald House. We arrived just in time for dinner, prepared nightly by volunteers(nice not to have to worry about that one).

I spent time talking with the lovely mother of a little girl with another form of dwarfism, metatropic dysplasia, three years old, and adorable! She is affected by severe spinal kyphosis, scoliosis, and related respiratory issues. She's already had five spinal surgeries in Indiana. She's now here to have the "hardware" place during those surgeries removed and immobilized in a halo by Dr Mackenzie (our surgeon too)... apparently the same day as Kahlial, Wednesday. This is both good news and bad news for us. Since Dr M apparently scheduled one or two other surgeries on "our day," I am assuming he's thinking that Kahlial's surgery will not take the 6-8 hours we were originally told. However, we were selfishly hoping to be the only, and therefore, first surgery of the day... we'll see.

Day two at the house was relatively uneventful and wonderfully lazy just loving, cuddling, and playing with Kahlial. The house has much to offer with great play spaces, train tracks, and a slide that Kahlial fearlessly enjoyed (as Mama held her breath). We took Kahlial to the "treasure chest" where he picked out a nice and shiny new red SUV. He's been strutting around the house, and I do mean STRUTTING with some seriously cute attitude (attitude is rarely cute, but this was adorable). People are eating him up around here, again, such a ham!

Well, I'm going to finish organizing Kahlial's Medical Records a bit in preparation for a slew of appointments tomorrow: Surgical Services, Pre-admission testing and labs, Halo fitting, Physical Therapy, CT under general anesthesia, and finish with a doc who sees "medically complicated children" for a general exam and review of systems. Boy, it's starting to feel very real. Sets in when you see him running around everywhere so happy with everyone telling you how fast he is, knowing he's going to come to a major halt here real soon.

Giving Thanks!

This Thanksgiving we are particularly grateful for our smily, silly, snuggly little boy, for the strength our family derives from each other, for a surgical team we believe in, for medical advancement and a procedure that can help protect our son, and for the love and support of our friends and family. Thank you! We wish you all health and happiness this holiday season and always.

Surgery...How did we get here?

Kahlial met Ronald McDonald back in July when we traveled to A.I. duPont Children's Hospital in DE (where the specialists "live") where an MRI was performed to check for cervical spine instability. This is a common issue for those with Kial's condition, Spondyloepiphyseal Dysplasia Congenita (SEDc), and one that can cause obvious problems if not corrected. The MRI did show instability, particularly in flexion (chin to chest position), and resulting "signaling" or injury of the spinal cord. Kahlial has what is referred to as an os odontoideum, a condition in which the dens (tip of C2) is separated from the axis body (body of C2), as well as ligamental laxitiy. This mobility of the dens allows for unwanted atlantoaxial motion (slipping of C1 on C2) and may lead to impingement of the upper cervical cord or vertebral artery.

Dr Mackenzie, orthopedic surgeon and head of the Medical Advisory Board for Little People of America (LPA), recommended a decompression of C1 (removal of the posterior portion of the first vertebrae) and fusion of C2 to the occiput (back of the skull) using a bone graft from Kial's hip, along with two titanium cables in order to prevent continued damage to the cord and potentially life-threatening injury. Kial has worn a cervical collar (seen in all the pictures since July) to help maintain stability and prevent injury until surgery, scheduled for December 16th in DE. We are now getting ready to "upgrade" to a halo and vest device that he will wear post-surgery for 2-6 months (we anticipate 3 months), then back into the neck brace full-time for 6 months.

It's going to be quite a year.... Already has been I suppose. We've wrapped our brains around this surgery about as much as we can. We knew that this procedure was highly likely for Kahlial. We just didn't expect it so soon. However, we've found a number of reasons why doing it early will be beneficial: hopefully won't remember much, won't interfere with schooling, will be able to be a bit more of a rough and tumble little boy earlier.

So off we go.... We'll be flying out on December 12th. Stay tuned for updates regarding pre-surgery appts and diagnostics, surgery on the 16th, and the recovery to follow. Please send positive thoughts and energy Kahlial's way. Thank you!