Hi and welcome to my site that goes through my battle with a rare malformation called Arnold Chiari Malformation, Type 1. In the rest of this website i will refer to it as ACM.
So you may ask, Who can have this malformation. Anybody can have it, some people have had it their whole lives and don't ever know they have it because they have never had severe enough symptoms.
How do you get it? Most people have had it since birth, i say most because it has not been proven that everybody is born with it, but it is more than likely the case. Certain things can bring on the symptoms, for example: Epidurals, Trauma to the head or neck such as a fall or a car accident, or a rough roller coaster ride are just a few of the things that can bring on the symptoms.
Just What exactly is it? Basically it is where your skull is too small, or how i like to put it is I have too many brains to fit in my skull. What happens is the cerebellum, which is the bottom section of the brain, is getting squished and is below the bone in the base of the head called the posterior fossa. And in most all cases a part of the brain called the cerebellar tonsils is herniated and way too low. Just like your tonsils in your throat these tonsils have no known purpose.
So you may ask just how do you correct this problem. The only way to correct this problem is with surgery. They make an incision in the back of your head down to your neck. They take out a piece of the posterior fossa (the bottom piece of skull) and they open the thing that surrouds the brain and the spinal fluid called the dura. Then they shave off the back part of the first 2 or 3 vertebrate depending on the severity of the situation. The reason they do this surgery is to help decompress the brain because it is so squashed the cerebullum puts pressure on the brain stem and can cause a number of symptoms.
So just what are the symptoms? The main symptoms are severe headaches at the base of the head and neck pain. Others symptoms are not limited to but include: Dizziness, shortness of breath, heart palpitations, depression, vertigo, drop attacks, numbness and tingling feeling, loss of feeling and movement in the extremities of the body, it can cause paralysis, fatigue, cough attacks, Irritable bowel syndrome, difficulties in walking to where you need the aid of a walker, popping in the ears, blurred vision, double vision, and balance and coordination problems.
Now it's time to hear my story with ACM.
I've always been clumsy all my life and never been able to go up and down stairs without difficulty from dizziness. I can remember ever since i was in high school i would go to school come home from work by 6, eat dinner and go to sleep. I never thought it was all that unnormal.
Then 2002 rolled around. In february my chest had been really hurting me so i went to see my doctor. He listened to my chest didn't do a chest x-ray or anything and said i had pleurisy and put me on ibuprofen, he said pleurisy is just an inflammation of the chest wall. I didn't think he was thorough enough when a month later i was still having problems, so i went to a different doctor in the practice, at this point i was coughing and had times where i thought i was having a heart attack the pain was so unbearable, at this point i was only 19 so i was scared to death. I saw this doctor and had a chest xray and lots of bloodwork he said i had walking pneumonia and was on antibiotics for 6 weeks trying to get rid of it.
Then in May, i started suffering severe depression and had suicide attempts at cutting my wrists. I started seeing a psychiatrist who put me on anti-depressants and anti-psychotics and diagnosed me as being manic-depressive. I continued seeing him then in June i went to an amusement park and got drug on a roller coaster, it was really rough. Then i started having severe headaches, nausea, and a staggering walk. So my doctor ordered an MRI and more blood tests.
In July, I had the MRI on a monday afternoon. Tuesday afternoon my doctor called me back and told me i had a condition called arnold chiari malformation. He didn't know much about it but told me i was born with it and it was a rare condition and I needed to see a neurologist. The first thing i did was look it up online. I found out all about it and scared myself even more. Then i tried getting into a neurologist and it was going to be 6 weeks so i got on a cancellation list so i could get in if somebody cancelled. I got in about 2 weeks later. In this time i was leaving work early probably 3 times a week.
I went to the neurologist and he told me he wanted to try medication, then he did his neurological exam. I couldn't walk in a straight line or stand still without swaying. When i touched his finger then my nose i kept on poking myself in the eye. He told me i needed to see a neurosurgeon.
So a week later i see the first neurosurgeon. He took a look at the medications i was on and automatically assumed i was a nutcase. He told me i had this my whole life so why would it start bugging me now, and i could get steroid shots in my neck for the pain. I had read online and decided to get a second opinion.
A friend of mine knew a doctor at OU medical center that was the head of neurosurgery, but when i called to make an appt it was going to be 2 months. So i emailed all the surgeons there explaining what was going on. Then the neurosurgeon i wanted in the first place, Dr. Francel, called me one saturday morning and i had an appt that thursday. He told me my tonsils were herniated 5-7 mm and i needed surgery.
2 weeks later on August 20th i had surgery. When i woke up i felt like i had been hit by a truck, but when they cut through 7 layers of muscle in your neck, cut open your skull, and take out part of your first 2 vertebrate your gonna be sore. I spent 7 days in Presbyterian Hospital. Then i came home really sore. Now I am off of the anti-psychotics and still on anti-depressants but it hasn't been a month quite yet since surgery. I am doing great. Pre-op my symptoms included, severe headaches (i was on 80 mg of loratab a day), horrible neck pain (was on 32 mg of zanaflex a day), popping in the ears, double vision, depression, numbness on the left side of my body, severe fatigue, popping ears, chest pain,severe irritable bowel syndrome, low blood pressure, and balance and coordination problems. Now that I'm post op the only thing I'm struggling with is neck tenderness which is from the surgery and I'm still healing and popping in the ears. I have a blocked eaustachian tube so I'm taking a decongestant for that.
While surgery was scary it was the best decision i could have made and I am glad that i made it. Below you can find some pictures of me 4 days post-op.
Here is my incision, it took 17 sutures and is around 8 inches long, at 4 days post op.
This is 4 days post of also, aside from the hospital gown you would never know i just had surgery, except for the look on my face says I'm not super happy.
These are all the gifts i received while in the hospital.
Well now were on to chapter 2 of the story
In January of 2003 I started having really bad headaches again. My balance problems never did improve, but they started to worsen so i returned to Dr. Francel. He did another MRI and referred me to a neurologist. Well to his surprise there were problems with the MRI. I still wasn't getting good flow to the back of my brain stem and there was a syrinx present starting at c5 and going down the length of my spine. So the plan was to either to a syringosubarachnoid shunt (a tube going from the syrinx to my chest to drain the syrinx) or another decompression in hoping that it would take care of the headaches and the syrinx. So we got a 3D cat scan for a better picture. After the cat scan we decided on the 2nd decompression.
I had the 2nd decompression on April 1, 2003. I had a posterior fossa decompression with a laminectomy of c1 c2 and c3 with duraplasty. They used 25 sutures compared to 17 sutures the first time I woke up after surgery and couldn't feel my right shoulder, arm, or hand. I'm still in physical therapy twice a week to work on that and to work on my neck. I was in the hospital a total of 7 days. I continue to have problems and they seem to be progressing. Right now i don't know what the next step is but i will keep you posted. Here's some pictures from the surgery.
This is 4 days post off, 25 sutures.
This one is compared to a $20 and i didn't even get to keep the cash!
This is 6 weeks post op