Update: Connor will likely need a second surgery. The NS says the hind brain is still very tight in the back. We go to NY in April with new testing results to find out if/when surgery will be scheduled.
Connor has started having issues with choking, fatigue, and bad headaches. His symptoms are more "typical" Chiari symptoms now. Whenever he coughs or sneezes he says his head hurts in the back, or he just cries out in pain. His hands and feet keep "falling asleep". I have had to pick him up from school frequently over the last month for headaches. His Cerebellar symptoms of balance and speech issues are still greatly improved since surgery.
At 6 years-old, Connor has frequent headaches, fatigue, leg pain, ringing in the ears, weakness, and his words "tingling" in his legs and arms. If he pushes himself to hard during the day, then he wakes up screaming at night. It usually takes at least 20 minutes of crying and Tylenol before we can get him back to sleep. He can't run around with other children his age, though he tries with all his might. He always lags behind and pays for it later. But he has a great time anyway. He's incredibly shy, but tends to be more social when his brother is there to warm things up for him.
At 11 months Connor still wasn't crawling, or even putting any weight on his legs. We took him to neurology where they declared Hypotonia and hyperflexibility and told us to continue PT. He started walking and we continued on. When Connor was 4 his grandmother asked why he always tilts his head to the side. We had stopped noticing, after all, the neurologist declared him fine. And having two older special needs children we didn't have a good grasp of "normal". This led down the road of occupational and physical therapy. We were shocked with how far behind Connor was in everything, some things he only ranked at 12 months. The first PT we tried just told us to get him to a neurologist and referred us out to someone more equipped for neurological issues. They ordered an MRI and found A Chiari 1 Malformation, at last answers. Nope.
Chiari 1 malformation is seen when the cerebellar tonsils are down past the foremen magnum. The brain is literally coming out of the skull and into the spinal column. In Connor's case 10 mm below and part of his medulla oblongata is down as well, this blocks the flow of Spinal fluid. The problem is there is not a lot of research on the effects of this condition. The old school thought is that it just caused headaches and choking, unless there is a syrinx, which thankfully Connor does not have. The newer research, as shown on the NIH and mayo websites, that there are more symptoms then previously thought. This isn't the Drs fault, it takes time for research to get out there. Connor is showing these symptoms. He also has the top of his spine pointed towards his brain stem. This greatly reduces the space for fluid flow.
Why New York? I'm not willing to wait until he starts choking on his own spit, or develops a cyst in his spinal cord that's caused permanent damage. Would you? The pediatric neurosurgeons who are up on the latest research for Chiari is a small group. Then the Neurosurgeons who also address the Spine issues makes it smaller. Now you have to find a neurosurgeon who will check and use proper technique in the instances of Connective tissue disorders. This led to 1 answer.
We were able to get Connor into the doctor September 2015, thanks to some very generous people. He found extreme issues with balance and coordination and other signs of Cerebellum injury. He also noted that he has a Retroflexed Odontoid, which means his top vertebrae points towards his brain stem, this turns his brain stem into a sandwich. The Doctor ordered further testing and we found that, unfortunately, Connor does need to have surgery. He has delayed swallowing, luckily he protects his airway, and while he doesn't have enough of to qualify for a diagnosis of sleep apnea, he is having episode of central sleep apnea and Hypopnea. With Chiari these could stay where they are at but more than likely they will get worse and it can change quickly. The Doctor asked us to keep a journal of his bad days to watch out for the quick decline. Right now we are hoping we don't have to schedule until Summer, but we will be discussing timing with his Dr this week. A new symptoms that has emerged is some problems accademically. He's very smart but having problems with the basic A,B,C's, while doing fine in higher thinking concepts.
In November 2016 Connor had his Decrompression and split duraplasty in New York.
The surgery has helped Connor's balance tremendously. He can stand on one foot for 5 seconds without holding onto anything now. His other foot he can do about 2 seconds. He is ticklish now, he never was before. Hot food actually burns his mouth now. His stutter disappeared for awhile. It's back a little bit, but nowhere near where it was. The new swallow study shows surgery helped his swallow delay and he isn't pooling fluids as much as before surgery.
Unfortunately, Connor has started having problems with choking and almost daily headaches. The newest MRI shows his brain is still very tight and we will likely need another surgery soon.
Because Connor has Von Willebrands type 2M, he will require at least 5 days in the hospital to get medication and watch for bleeding complications, as opposed to the usual 3 days. The Neurosurgeon wants him in NY at least 10 days after surgery so he is close in the most dangerous timeframe. This puts us in New York at least 2 weeks. If he has instability with the RO he would require another much more complex surgery.
I know this is long, but this is Connor's story. Please help my sweet, cuddly, smart as can, be 6 year-old, get the surgery he needs.