Ethan’s Brain Surgery

My son Ethan is having surgery February 7th. We are having to travel to NYC due to the complexity of his condition.I want to explain his diagnosis and a brief history for better understanding. This is a just a brief summary of his history and current state, although there are many more details.

He has a Chiari malformation 1 which is his brain extending 12mm out of his skull and into his spinal canal. Additionally he has a retroflexed odontoid which is essentially the top of the spine bent backwards. His is so severe that it is kinking his brainstem. So he has brainstem compression coming from both sides. This has caused something called Central sleep apnea which is the brain not sending the signal to breath while he’s sleeping. The compression on his brainstem is also completely blocking cerebral spinal fluid (CSF) from entering the brain on the back side and obstructing CSF flow substantially on the front side. He also has Empty Sella which is where CSF is being forced into the space where his pituitary gland sits, flattening his pituitary now completely. It is currently impacting pituitary function. (Hormone levels). This is also seen with chronic high intracranial pressure.

Last year he had some slight elevation of his optic disc with no visual changes. He was also on an NG tube for 6 months due to inability to swallow even his own saliva, amongst other neurological symptoms. He had multiple blood tests coming back positive for things outside of his Chiari which made his case a little more confusing. Due to the complexity of his condition, it was recommended we go to Weill Cornell for their expertise.

They recommended surgery last year but come time surgery to be scheduled after new MRI, he was starting to eat again and neurological symptoms were slowly fading.. We asked to wait and monitor and continue to treat inflammation since he seemed to be doing better. We were given that option however with close monitoring of his optic nerve, apnea, and frequent follow ups with his neuros. We were told he eventually would need surgery however.

He seemed okay through the summer until some symptoms started to come back. New neurological symptoms we hadn’t seen before. By November it was loss of sensation, pupillary dysfunction, new onset aniscoria and droopy eyelid. He started to have sensitivity to his head that would send him into episodes that started out light but would eventually turn into slurred speech, imbalance, nausea, paralyzing pain, screaming and unable to walk. We saw one of his pupils was constricting and dilating without change of light. We took him to the ER where they simply ruled out a brain bleed but mentioned some signs of swelling, however not deemed an emergency due to current mental state. We were advised to see ophthalmologist next day where he now had significant decline in vision in one eye, bilateral optic disc swelling, and some damage to cranial nerves. Sent back to the hospital where MRV also showed traverse sinus stenosis which is a narrowed drainage pathway for CSF to leave the brain. So the flow of CSF into the brain is severely obstructed and the flow out of the brain is now severely obstructed causing fluid build up in the brain causing high intracranial pressure. Here we are years after intermittent severe changes in our son, now learning that his Chiari along with triggers such as biometric pressures etc have been causing intermittent ICP. We were told to get to NYC for urgent surgery until neuro optho said the swelling had gone down however pressure is still not normal.

Because it has already started affecting his optic nerve, they said he will go blind the next raise in intracranial pressure. We are free to now come in early February as we requested Ethan able to celebrate his 6th birthday before his procedure as well as have time to plan and get things in order to go up there. His symptoms also decreased with lower pressure so we felt comfortable requesting February date.This has been nothing short of exhausting and stressful but we are praying this will end his hospital stays, doctors visits, tests, etc and keep him from sustaining any more damage to his cranial nerves and optic nerve. There is a chance that the ICP may continue following surgery and they plan to put a bolt monitor in his skull after procedure to monitor pressures along with short term diamox. Depending on how this goes he may need additional surgeries. We are also unsure of whether they will have to shave down or remove some of his C1, C2 along with a fusion until they open him up or possibly down the road some. We are praying for the best outcome but going into this somewhat blind as to what all additionally they may need to do. We fully and completely trust his surgeon however and know he is in the best and most capable hands.

It has been a long road of ups and downs for Ethan and he has learned to be so brave in situations where he shouldn’t have to be. I have personally witnessed him do terrifying tests and endure so much pain and teach himself to breath through it. He is amazing and shouldn’t have to suffer through this anymore.

My wonderful family and friends have asked us if we had a go fund me and have already been so kind and generous to us in this difficult time. We are beyond thankful for any and all help or prayers that his surgery go smoothly and solves the ICP without further intervention. That my son won’t have to endure anymore suffering and able to just be a kid. Thank you to everyone supporting us and helping us get Ethan the help he needs. There are no words to express our gratitude and are blessed from last year and now to have so much support and love coming our way. Thank you from the bottom of my heart for helping my son and our family navigate this financially, emotionally, and spiritually. This has been very difficult due to many obstacles and all aspects. We appreciate all of you more than you could understand.