Meet CASE!

This amazing boy was born 12-21-10 at a whopping 2 pounds, 13 ounces due to an unknown syndrome and Inner-Uterine-Growth-Restriction. His doctors didn't expect him to live but living life to the fullest is what he does!!!!
This Is his story:
-Case was born with a multi-cystic, dysplastic non-functioning left kidney (that has since shriveled up) and fluid pockets on his right.
-He is currently J tube and TPN dependent and fed 24 hours a day intervieniouly and straight into his intestistines due to swallowing issues, poor motility and intestinal issues.
-He has suffered from Crainiosynostosis (Sagittal), Chairi Malformation a tetered spinal all corrected via surgery hopefully to never return......
-He was born with an ASD, VSD, PFO and several "normal variants of the heart that are currently stable.
-He suffers from Failure to Thrive and extreme short stature.
-He started growth hormone therapy in March, 20015. He gets daily shots and they are WORKING!
-About a year ago, he passed out in a hypoglycemic shock with sugars below 20. He was quickly stabilized being we were at the hospital (thank God)....He still suffers from severe Hypoglycemia and can not go without nutrition for more than two hours, even at night.... He has home health nurses at night and while at school.
-Case also has very severe GERD on top of the motility issues and lack of function in his gut. He was unable to tolerate night feeds and is now on TPN and Lippids through a central line in his chest. He takes several medications to help but nothing seems to completely work....
-Due to lack of growth and intestinal failure he had his central line placed 1-20-15 (this is a semi-perminante IV in the major vein by his heart).
-On top of all of this Case was born with many birth defects and congenital anomalies. We hope to some day have a "name" to go with what ever syndrome he suffers.....but for know, we are blessed to have our boy with us.

Sunday, November 2, 2014

Our Cranio Journey.

Our Crainio Journey. 


Steep a cup of tea and get comfortable because this is gonna be a long one!
I've never really documented Case's journey from diagnosis of Sagittal Craniosynostosis  to the Crainio Vault Remodeling. In part, the struggles of the journey were still fresh in my mind and I wanted to wait awhile for the results of a full scull repair to be apparent. 
Let's start in May of 2013. For a while, Case's Geneticist thought he had a suture (every baby is born with small gaps in their scull that close as they grow older) or two that had prematurely fused causing his brain to be crowded and his head to grow wrong. She requested a CT scan and for us to be seen after the scan by the Crainio-Facial clinic at Children's. He was already a patient at the clinic due to his sub-mucus cleft pallet and other Cranio-facial anomalies. The CT scan showed that the top suture on his head was indeed closed meaning Case had Saggital Crainiosynostis. The first Dr we saw does not do Crainio Vault Repairs but her face was strewn with concern. His head was misshapen and his brain was growing wrong.....that sent us into an immediate whirlwind of VEPs ( a visual test used to detect inner cranial pressure and other symptoms of Craniosynostosis), eye exams to check for swollen optic nerves, etc. 
Case was then scheduled to see who we will call Dr. X in July of that year. Dr. X said he needed a Cranio Vault Repair within the next few months. His concerns were that there wasn't enough fluid around his brain, the frequent headaches, etc. 
So, there we were, planing for a major scull surgery around October! This was going to increase my son's quality of life. It was one answer to the many questions his very complicated life holds. I called the blood bank to prepare to donate blood for after the intense repair that would cause a loss of a lot of blood. I called the scheduler, made babysitting plans for his three sisters, planed mentally and physically for what would be the most invasive surgery of his life.....I was ready! In about August or so I got a call. I thought I'd be getting the final date of the repair, instead I was told Dr. X was leaving the facility and we needed to make an appointment with Dr. Y.
"Okay." I told myself....."It's okay. I'm sure Dr Y feels the same way and we'll move forward as planed. 
So, we went back to the same hospital and saw Dr Y. I was wrong. Dr. Y did not agree Case needed the repair unless he showed solid signs of increased cranial pressure. Headaches, vomiting, irritability could all be side effects of other things Case had going on. After all, we have an "incredibly complicated son". 
I was trying to process all of this. We went from having a huge surgery in the fall to possibly never having the craino vault repair....wow. 
I was obviously befuddled. Dr. Y handled me courteously. I told him I wanted Case's Neuorsurgeon consulted. Case has a great Neurosurgeon that knows Case, his complications and needs. Dr Y agreed and called me a few days later. 
The decision was the same. Case is complicated. "There's no hard evidence he needs the surgery", he said. "Case is too complicated. We have to be sure the surgery is necessary..... No pressures are showing up on the VEP, his optic nerves look good. We will just continue following him and if pressures come up, we'll talk surgery then." The Neurosuren also said Case is complicated and we can't be sure the headaches, etc. we're from the Crainiosynosis so, he too wanted to wait and that's when I felt like my heart fell out of my chest. I was out of controll. All I could do was trust God and the Drs and go on with life.....That. Was. So. Hard. 
In the back of my mind I knew if Dr X just would have stayed a little longer.....this would all be better and we'd be "on the other side" of the repair. But, I knew Dr. Y was/is a very good Dr and that him and the Neurosurgeon were doing what they believed was in Case's best interest....
Okay, let's skip to the spring of 2014. After meeting with an amazing Neurologist and a slightly "off" VEP it was decided that Case would have an Invasive, Innercrainial pressure monitor placed in his head to see what pressure was really there.... The end of April, he was admitted to the PICU at Children's. They sedated him, drilled a hole in his scull and placed a pressure monitor on a sunny Tuesday morning. We stayed there and pressures were monitored. At this point, he was having around 12 headaches a week, still vomiting at night, was way behind in speach and growth. His pressure spiked some during the invasive testing but not as much as we expected and only before a headache....Wednesday, they still weren't sure he had enough pressure to operate. "If he weren't so complicated, they go ahead and do the repair" but they were taking every precaution necessary to make sure Case was not submitted to unnecessary pain, etc for not enough reason.....That's when Dr. Z comes in....Dr Z is another plastic surgeon that saw a need for the surgery and thought we should go ahead. He consulted Case's Nurosurgeon and they agreed they would go ahead and do a full Crainio Vault Remolding Friday! Holy. Freaking. Cow......it was going to happen and I was ready. I prayed we were right. I prayed the posible "reasons he'd never leave the operating table" wouldn't happen. I prayed he wouldn't bleed too much, get an infection, etc. 
Surgery was set for that Friday and the is what they would do:
But a zig-zag incision from ear to ear. Pull his skin and fatty tissue back then take out his scull in sections. They'd then cut it "like a blooming onion" and put it back using surgical paist, chips of his own bone and dissolvable plates and screws to secure the repair. 
I handed him over and can say, I wasn't scared. I knew this needed done and I felt peace. I'll tell you when I cried.....when I saw him all bandages up in his PICU bed after surgery. It. Was. Done. We wre on the other side and it felt good! A weight was lifted off my shoulders. No more wondering, no more pleading, no more pushing... now....just to sit back and hope it was the right choice.

Fast forward to now. We celebrate his 6 month crainio anniversary today,  November 2. I have no regrets. He's gained 7 pounds (because he can swallow better and is eating more by mouth), his speach and over-all development has DRASTICALLY increased. His headaches are GONE! The pressure was so severe, it caused his brain to decend below his scull (Chairi Malformation 9mm). Well, that's GONE! The only thing that still exists still is night vomits. We still don't know why they happen but, I know, some day, with the help of Case's AMAZING medical team and God's grace we'll know why they happen and he will get relief. 
In a nut shell, our crainio journey was hard. Confusing. Exhausting. More so, than the NICU and maybe even the Genitic journey....but it's over and we are blessed.❤️
A few days before the Crainio Vault repair. 





6 months post-op.