Saturday, September 19, 2009

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
This is Alex's Appeal home page

Sunday, September 13, 2009

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.

Wednesday, September 09, 2009

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.