Wes has been a busy little guy. He absolutely loves school. He woke Sunday morning asking, if I would get him dressed for school. He led the costume parade as Superman. Yes, this is very fitting for such a strong little guy. He is definitely my little super hero.
Past few weeks have been pretty busy. Wes had his ear tubes replaced. He saw the little tiger hospital gown and had a melt down. We were very thankful for the hospital music therapy. He loved playing the instruments and singing.
During his surgery, he had some breathing difficulty. Dr. Becker came out and sat down. I knew this was not good. She and the anesthesiologist are very concerned with his airway. His airway started to collapse and he nearly needed to be intubated, but the surgery is short and the anesthesiologist worked with him. She of course feels that there needs to be a plan for Wes that results in him having a safe airway. Until that can be accomplished she still recommends a trach. His breathing did improve after his tonsillectomy, but has again become concerning. Nothing like before, but then not like it should be.
Two trips to Gainseville in to weeks. First to see the pulmonologist, then the craniofacial team and have a sleep study. Soon heading back for a full cardiology work up to see if his obstructive apnea has done any damage to his heart. That is the biggest concern, permanent heart and lung damage. Damage beyond what I can even wrap my mind around. Dr Curry just says Wes has come so far to let this…
Thursday morning we saw the craniofacial team at Shands. Before I even started sharing Wes’ recent tube surgery, Dr. Seagle expressed how complex Wes’ case is and the fact that he needs some major surgery. The information about his tube surgery was just confirmation to this fact. I asked specifically about his experience with these specific type surgeries, which this team does not see very often, one case a year. This is probably why no one has offered a plan for Wes until now. Six months ago, I put the pressure on for a plan. I got nothing! The surgery is huge, more involved than his original neurosurgery.
Why does it take so long to be heard? I was not speaking alone; I had Wes’ pediatrician and local ENT behind me the entire time. Wes was 7 months old, when his breathing became an issue. Nearly 3 years later and 3 plastic surgeons, to finally get a validated plan.

Each year a very special mother pulls together an amazing fundraising event, Alexa's Appeal Dinner, for CCA, Children's Cranofacial Association. Her mission is not only to raise funds for an organization who touches so many, she is also dedicated to educate other's of the various craniofacial syndromes that affect so many and their journeys. Knowledge is key in understanding.
Denise creates and shares at this event each year that I personally love to watch. Yes, I cry through the first one or two views, because the videos are so connected to our journey and powerful. These are the children and families I have watched grow, turned to for support, advice...These are very special children who have touched my heart and continue to inspire me. There is one very special little guy in this video that warms my heart and inspires me daily. Anytime someone asks how I do this, I know they have not seen the depths of his smile.
If you watch this video you will see unimaginable strength and beauty that lies beyond a face. When you have time to sit and let your heart melt, here is a link to this years video http://www.photodex.com/sharing/viewalbum.html?bm=175895
This is Alex's Appeal home page http://www.alexasappeal.com/index.html

Such a nice feeling, being home. Friday night, we came home to a sick boy. Brandon has been fighting high fevers and strep throat while we were away. Yesterday since he was still spiking a high fever, we decided to have him tested for the flu. He tested negative and will start a new antibiotic today. After a year of strep and tonsillitis it is definitely time to say good-bye to his tonsils.
The last two days in Chicago were informative, yet still left us with many unanswered questions. There was good news concerning the report of the two new encephaloceles. Dr. Bowman does not exactly agree with this report, instead she feels that the areas that the radiologist is calling encephaloceles are diseased sinuses. She can not completely rule out the encephalocele diagnosis, but for now would just continue to monitor this area regardless. She feels that they may have over called this as a precaution not to miss anything. Understandable with Wes being under a microscope and he does have a rather “unique” unknown head.
With this positive thought came another concern. Wes head is very unique from front to back, which may be causing some pressure/compression on his brain stem and the flow of spinal fluid around his brain stem and spinal cord. Not that this is easily understood, it does help to have this as a reference for myself. He has platybasia, tonsillar ectopia, hypoplastic cluivus, and a very unique layout... The brainstem compression is the reason she originally placed restrictions on Wes' activities, no trampolines, contact sports, gymnastics... This area was briefly mentioned initially, as an area that would have no prefect fix or surgery, if it became an issue. At that point there were no immediate concerns. Her description always begins with Wes has a very “unique” head.
The portion of the appointment that caught me off guard was her feeling that Wes needs to be taught as a visually impaired child who needs to learn braille. She does not feel that Wes vision will improve and will likely get worse. I did not ask if this is what she anticipates or where she feels he presently is visually, I was shocked. We discussed his vision in depth as she did an assessment of his vision and development at the beginning of the appointment, but after reviewing the MRI she shared this thought. He does get therapy, but is presently not learning braille. Those of you who know Wes, know how well he does visually. The smaller the little guys are the more intrigued he is.
The pituitary cyst that was not seen on this report was again identified. Dr. Bowman compared last years’ scan and she feels it is unchanged.
Dr. Bowman would like to see Wes back in 6 months for another MRI and CT scan. He will have extension and flexion during these scans. She will look at his brain stem, neck, and again the sinus in question at that time.
We also followed up with the plastic surgeon who did Wes' initial palate repair. Dr. Vicari felt that Wes would benefit from a nasoendoscopic evaluation, regardless of where we have surgery for his VPI. Apparently one of Wes' original encephaloceles was bulging where his palate should be hitting for closure, which could have caused muscle defects. The ENT, Dr. Rasttater, who preformed the nasoendscopic was a bit concerned when he looked into Wes' airway. He has begun to snore lately, but nothing significant. I have seen extreme and Wes' breathing has never been completely peaceful, so it is difficult for me to judge. He would like a sleep study before deciding on a palate surgery.
With Wes' anatomy the ENT is also concerned about how his jugular veins run, which would be a concern with his VPI surgery, also a rubbery nodule in his neck. He will look at both during his next MRI and will schedule a broncoscopy while he is sedated.
Beyond all the medical info, Wes’ nasoendoscopy was quite an experience for all involved. Basically the Dr. Rasttater inserted a scope with a camera on the end. Wes is then suppose to say specific sounds, apple, baby, pepper, suzy…instead Wes cried and scream. Eventually when it looked as though all hope was lost, the speech therapist just went with him and had Wes start yelling, “take it out” then she was able to squeeze in a few of those key words. The results showed that Wes’ palate and pharyngeal walls (throat) are not moving enough to close of his nose.
For now there remain many questions, as I imagine will be the case for a long time. Now I face the challenge of balancing and not letting these questions and fears over run today.

Day one in Chicago.  It is amazing we travel this far and still possibly not where we need to be. 
Main concern at this time is Wes' speech, which I believe is clear to everyone that he will need another surgery.  Symposium last year, Dr. Williams said a videoflouroscopy and nasoendoscopy were needed to best navigate a plan for VPI (Velopharyngeal insufficiency). At Shands Wes had a videoflouroscopy. Here they have ordered a nasoendoscopy for Friday. We will get a report with their plan after.
It also seems to be the consensus that during this surgery, Wes collapsed nostril will be addressed. Not fully repaired, as this comes years from now.
After this surgery, the plan becomes much more complicated. As if surgery is not difficult enough. This plan will include surgery to address his flat nasal bridge, surgery and orthodontics for retruded midface and mallocculsion, possible hypertelorism (wide set eyes). This plan will become more detailed as he grows and develops and will be a process over many years.
The biggest complication seems to be that each issue and surgery is not independent. Everything is intertwined and completely involved, if that makes sense. As Dr. Vicari explained that his encephalocele sat in the area where his soft palate should hit to close off his nasal passage as he talks, I started to realize how everything fits or tangles together.
It seems that we have been able to piece together care for Wes, yet have not found the complete team. I honestly do respect when a doctor offers or suggests the opinions of others, which is where we are now. Dr. Vicari suggested and encouraged us to seek a second opinion concerning surgery for Wes’ hypertelorism with an expert team. He plans to get a list together of doctors who specialize in this specific type of surgery. Options seem to range from New York to Seattle with one possibility in Miami.
So today we walk out of an appointment, not unlike many other appointments with some questions answered, some still to be discovered, and new ones weighing heavily on our hearts and minds. For now we are heading to playroom and then the Zoo. Life was meant to be lived, those who know me and my guys know we love to have fun.

Wes is a big school boy! He is now an old pro with one whole week behind him. First day, no tears, second and third day were screaming and being torn from me, and the fourth day was the hardest on me as he walked to his little seat holding my hand trying to be a big boy with his little tear filled eyes and trembling bottom lip. Yes, that was the day that tore my heart apart. Tomorrow morning brings a brand new week. He is doing great. It is nice to able to peak in on him occasionally, but there are some anxieties. It was hard bringing Wes to someone new, hoping and trusting that she will give him what he needs, take care of him, and know just how special my baby boy is. Of course he has already turned on the charm and is surely to win the hearts of Mrs. Noble, Ms. Debbie, and Ms. Erin.
We were able to squeeze in a little fun before beginning school with Thing 1, 2, and 3 at Universal Studios in Orlando.
Hard to believe it has been a year since our last trip to Chicago with our pint size pirate. Next week, we will be making the trip once again. Before beginning school, Wes had a MRI at Shands, which has brought many questions. There was a time when each fever, temper tantrum, unusual eye movement, change in breathing caused me overwhelming concern. I felt as though I was always waiting, worrying about what lie around the corner. Slowly I have stepped away from that place and found some comfort with our new normal, only to be snatched back with the shock of change. I expected his MRI to be much like the one last year with some concerning issues, but nothing new. This is not what we got.
Before anyone asks, we don’t know what it means and can not explain anything. We have a report, but only a doctor can tell us what that means. At this point anything we think is only speculation. Most likely, if others were to read the MRI, it would be like reading foreign language. Of the issues noted, the most disturbing was that Wes now has two new encephaloceles in his frontal sinus. Both containing brain and showing some damage.
For now we await responses from Wes’ neurologist, endocrinologist, and ophthalmologist for answers on the other issues. The neurosurgeon would like to see the actual scans and will discuss these with us next week. So here we are waiting…

Lots to update…We recently had a craniofacial team meeting at Shands. I pressed for an idea of what they expect as a plan for Wes long term. Most of the time, we only get the immediate need to know information. Of course those of you who know me, know I need to know everything. I also totally understand there are no concrete plans. At this point I also understand much of the medical lingo.
Here is the plan with a strong emphasis that there is nothing definite. Wes will need some surgeries in the future, I want him to have a team who can explain this plan with confidence after much experience. 5 to 7 years old, he may have surgery to bring his eyes closer together, build a nasal bridge, and address his midface retrusion. This may be staged, which would involve multiple surgeries and will also involve many surgeons. There are many questions to be answered before proceeding with any of these surgeries. Before kindergarten, scar revision and minor work to his nose. Around 9 years old, a bone graft from his hip to his gumline. His teenage years will follow with surgeries to revise.
During this appointment we discussed a gene mutation, MTHFR, with the genetic counselor. Turned out there was some information in his chart that had not been discussed. Follow up did not happen as it should have. It looks like this month we are going to be able to pursue a more definite genetic diagnosis. Although some may not understand this, I want to know his diagnosis. We continue to get these “isolated” diagnoses without the entire view. This was again apparent with Wes’ last x-ray to investigate a bulge on his back. Now he adds another doctor to the list to monitor scoliosis. Waiting for results and getting these surprises are heart and mind wrenching. A genetic diagnosis would give us so much information and beneficial connections.
It appears that the most pressing and immediate issue is Wes’ speech. He does have nasal emission, which means surgery to address Velopharyngeal insufficiency. He is doing well with speech therapy, but if his palate is not closing appropriately there will sounds he will not be able to make and he will continue to have nasally speech. It breaks my heart to see him get frustrated when people are not able to understand what he is saying.
He is having a videoflorscopy that I thought would determine whether he needed surgery. Instead they were ready to schedule surgery for June 25th, because it is obvious that he needs surgery. Yes, head spinning quick! It will not be happening that quickly, since Wes’ neurologist has ordered a two day EEG.
A few weeks ago, Wes had a very scary episode that appeared to be a seizure. He was running around, eating cake, and acting completely like a typical 3 year old. We went out to eat and all of the sudden his whole mode changed. First he wanted me to hold him, suddenly felt fevered, breathing changed, and then seemed very unlike himself. We quickly headed to the house. In the rear view mirror, I watched his eyes begin to flutter then roll back, rapid breathing changes, and nonresponsive. As I was unbuckling him, he began vomiting. Immediately after, he was again responsive, yet very agitated and clinging to me. After a quick bath, he went sound to sleep. The next day, Dr Curry checked him out and all was fine. Same week we headed to the neurologist. She was concerned about the way Wes stares at the ceiling and this episode, which prompted a two day EEG to monitor him for seizure activity. Concerned to see how this is going to work. Two days of his little head covered with probes and then wrapped securely with a monitor on his back and hooked to a camera when possible.
June 23rd and 24th are back to Shands for a videoflorscopy, endocrinologist, genetics, and ophthalmologist. June 26th starts the two day EEG with no results for weeks. July 16th was scheduled to be neurosurgeon appointment, but he needs clearance from the neurologist, which will be delayed now. July Wes will also be going to speech camp in Panama City Beach.
In between running to appointments, we plan on enjoying an active summer and a potty training adventure. I plan on enjoying my boys.

Thank you to everyone who made a generous donation of pop tabs possible for the Ronald McDonald House. As you can see the donation filled up an entire donation house. We were told they raise around $6000 each year for the house in Gainseville just from pop tabs. This simple donation helps make a difference for many children and families during a very difficult time. The Ronald McDonald House has provided us with so much more than just a place to rest. Keep saving your tabs. We plan on making a donation with each visit to Gainseville.