WTF is Chiari?

That’s a damn good question! Hi everyone, my name is Ella, and I was born with a somewhat rare brain condition called Chiari type I malformation. Unless you’ve known someone personally affected by it, there’s a good chance you’ve never heard of this problem. As my brain surgery date looms ahead of me, I’ve decided to start this blog to record my physical and mental experiences as I battle this almost unheard of condition.

I’ll be blogging about all kinds of things, from detailed definitions as to what Chiari is, my life experiences so far dealing with it, my mental state as I approach surgery, sharing the things that get me through the day and pictures of my MRIs, scars and recovery. In this post, though, I’d like to give a brief outline of what a Chiari malformation is, the symptoms I have personally presented with and how they have disabled me and what course of treatment I am looking towards. I’ll try to keep it brief, but I have a tendency to ramble!

To begin with, Chiari type I is a brain malformation where the cerebellar tonsils (ie. the very bottom of the brain) is displaced downwards and kind of wedged in the foramen magnum (ie a gap in the base of the skull). This pressure at the back of the brain obstructs the flow of spinal fluid and causes all kinds of nasty symptoms from headaches to paralysis.

Chiari symptoms seem to always be a bit jumbled, which causes a bit of difficulty in diagnosis. Many people are lucky enough to get no symptoms at all and can easily live out their lives never being bothered by their malformations, but the rest of us get varying degrees and a wide range of sometimes debilitating symptoms. I’m certainly not going to go into detail about all of them here, but I’ll list the ones I personally suffer from:

  • Chronic headaches
  • Pressure headaches and loss of vision (occurs after activities like laughing, sneezing, coughing or standing up too quickly)
  • Dizziness
  • Fatigue
  • Spontaneous and short bouts of synaesthesia (not unpleasant but certainly weird)
  • Neck and shoulder pain
  • Hyper sensitivity to sound (which for me is my most disabling problem, as I present with symptoms very much like a temporal lobe seizure when things get only moderately noisy)
  • Nausea and vomiting
  • Muscle weakness around the arms and shoulders
  • Problems with concentration and memory
  • Tingling, numbness, pain and short term loss of movement in hands and arms

So far I’ve been treating my Chiari symptoms by using mild pain killers, which have little to no effect most of the time, restricting my physical activity and wearing earplugs while in public places to prevent seizure-like occurrences. Sadly, over the last few years I’ve found that these measures are not working well enough to give me a good quality of life and the independence I desire. For a long time, I could not leave the house alone and would occasionally even need at home supervision. I’ve spent weeks at a time confined to my bed, I can not seek a job and can not support myself or the people I care about. Obviously, more drastic measures are required.

The treatment for a symptomatic Chiari such as this one is, unfortunately, surgery. It involves removing pieces of the skull and first vertebrae (ie the first neck bone) while opening the membrane around the brain and grafting it with an artificial patch. All of this is supposed to reduce the compression around the back of the brain and help to restore spinal fluid flow.

I’ve been battling with the prospect of this surgery for years now. It’s always been seen as an inevitable possibility if my symptoms did not reduce, however due to the large amount of seemingly inept neurologists in this city, we had certainly found no one I’d be willing to hand my brain’s wellbeing over to. All of that changed a few months ago when we went to see a neurologist who specialises in Chiari malformation who ran a few more (correct) tests and then gave us some real detailed information about what to expect from the surgery. After a few more months of umming and ahhing about it, I finally decided on getting it done, and a date was booked.

Now we play the waiting game, and I can tell you now that I’m such a bizarre mix of ecstatic excitement about the idea of this disability ending, and sheer mortal terror about the fact that it’s my brain we’re talking about putting knives and drills near and the fact that I’ve been told that the recovery will be considerably painful (and of course, as with all brain surgery, there is the possibility of complications or even worsening my initial condition). Here’s to the challenge, though! I can safely say that this experience so far has taught me more about myself than any other I’ve been through. Surprisingly, if someone said “I could make it so that you never had Chiari”, I’d probably turn down the offer. I’ve learned a lot from it, I will undoubtedly learn a lot more in the months to come, but I am ready for it to end now.

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