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I live with a degenerative and progressive brain condition called an Arnold Chiari Malformation. My Thursday Thirteen for today was inspired by Thom, who didn't realize that I had had brain surgery and dug around here to find out why. Here's a list of 13 facts about ACM.
- Arnold Chiari Malformation is the medical name for what I like to call a brain fart. In a nutshell, people who have ACM have brains that are too big to fit properly inside their skulls. Although I like to think this is just because we're so doggone smart, medical evidence does not support this hypothesis. Because the brain doesn't fit like it should, the tonsils (foramen magnum) hangs down, outside the cavity of the skull, and into the spinal canal.
- ACM is a real pain in the butt. . .er, head! The spinal canal is made just big enough to hold the spinal cord so when a protruding piece of brain scoots down in there, life can get really complicated. Things like killer headaches, dizziness, nausea, vertigo, tingling sensations, difficulty swallowing, double vision, difficulty hearing, balance issues, and insomnia are just a few of the symptoms that ACM triggers and that ACM patients deal with on a daily basis.
- Size does not matter with ACM. Some people have very small protrusions and suffer considerable pain, while others may have a larger protrusion and never have a symptom. In my case, a chunk measuring 8 mm -- not really big, but definitely not small -- sits in my spinal canal. If it hadn't been for the fact that I suddenly began having headaches so painful that it hurt to breathe, I would have never known that I had a problem.
- What comes out, can't go back in. If the brain breaks free of the dura mater, the protective membrane that surrounds it, it can never be put back inside. The solution to this problem is surgery. To allieviate the pressure that this piece of brain puts on the spinal cord, decompression surgery, typically composed of three parts (craniectomy, laminectomy, and duraplasty), is the most widely recognized treatment.
- If undiagnosed and treated, ACM can cause paralysis or death. Dr. G, Medical Examiner, part of the Discovery Health Network's programming, had an episode about a young man whose ACM was undiagnosed and who died because the brain protrusion pinched his spinal cord.
- No known cause or cure. Although many physicians believe that ACM is a hereditory problem, there is no definitive proof of it. Nobody in my family has the problem except me, but Julie Carter, a Chiari activist, and all three of her daughters have it. If you're a fan of Extreme Makeover: Home Edition, you might be familiar with the Carter Family; they received a home makeover from ABC a couple of years ago.
- Classified as a rare disorder by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). What this means is that ACM affects less than 200,000 people in the U.S. population. Ok, so this just means that I'm special -- I can live with that!
- I'm a Zipperhead! What's a Zipperhead, you want to know? That's the nickname that Chiari patients call a decompressed Chiarian. After the surgery, the back of your head looks like its been closed with a zipper, and that's where the nickname comes from.
- Getting a diagnosis is not easy. Because ACM triggers so many different and seemingly unrelated symptoms, most ACM patients don't get the proper diagnosis for many years. This was my case. The problem was clearly present in my early teens, but I was shuffled back and forth from the ENT to the family doctor to a gastroenterologist. Back in those days, the MRI was not available and since it is the now the way that most people are diagnosed, I can't blame any of the doctors who treated my symptoms without getting to their root cause. I just had to wait until technology caught up with them.
- Decompression surgery can alleviate some of the symptoms, but it doesn't solve the problem. Because of #4, surgery doesn't solve the problem; however, it did help to control the symptoms better. After I had surgery, the headaches occurred less frequently, which is good. I still have problems with double-vision, my balance is so bad that my neurologist joked how it's a good thing that I don't drink because I would never pass the standard police drunk test (heel-to-toe walking a straight line), my depth perception is so off that I can't see well enough to walk down a flight of stairs or even a curb without falling, and I have complex-partial seizures that come from the right parietal lobe of my brain. The remaining symptoms may sound bad, but I manage to live a relatively "normal" life with them.
- ACM is more common in women than in men. I like to think that this is just because we're naturally smarter, so our brains are naturally bigger! It's not likely that the medical community supports my findings, though.
- Roseanne Cash, daughter of country music legend Johnny Cash, is also a Zipperhead. She had decompression surgery in December of 2007. Maybe it was ACM that was causing her "Seven Year Ache." Ok, that was a very bad joke!
- ACM is an invisible illness. Because people can't see the piece of brain that hangs out in the spinal canal, they often misjudge a patient's reaction to the symptoms. Our society is trained to look for visible symptoms of an illness -- watery eyes and sneezing for allergies, red bumps for chicken pox, coughing and a stuffy nose for a cold, etc. -- and when none are present, judgment of the patients who suffer from invisible illnesses tends to be rather harsh. Perhaps because my invisible illness involves The Brain, that most sacred of internal organs (muscle just doesn't sound as dramatic here, sorry), I am treated far more kindly than someone who has Lupus, for example. This is just plain wrong!
If you'd like to join in on the Thursday Thirteen fun, click here.