The End of the Beginning

So, I've described the beginning of the story in the previous two (agonizingly long) posts. The problem with this story, for me, is that it doesn't really seem to go anywhere after a while. The details dissolve into tedium-- Things happen, mysteries arise and are brought to bear, characters are introduced and cast aside, crises are diffused... But all these elements are present in any story. Still, there is an amount of summary needed in order to bring the story up to date.

What I didn't realize in "The Beginning," is that my trip down the river was a baptism of sorts. My journey through the pouring rain was passing time as I drew closer to a world that, some few years later, I have come to know too intimately. I have learned the language, the reasoning, the inadequacies of this new world, through immersion.

Soon after my return from the canoe excursion, I sought medical advice. I saw a young GP who was fresh out of residency and had no clue what to do with me. He referred me to a neurologist along with copies of my (normal) brain MRI, normal labs, etc. The neurologist ordered more tests, all of them normal. Over two months had passed since the canoe excursion, and I was feeling awful.

Running out of ideas, the neurologist at last ordered a lumbar puncture. This was scheduled to be done under fluoroscopy at the hospital next door. To this day I am not sure why, but I was not at all afraid. The radiologist made note of my calm manner as he scrubbed my back with betadine. He was quick with the needle, and I suddenly felt a spray of lukewarm fluid over my back. Then the manometer was attached. "That's the highest pressure I've seen in a long time! No wonder you're feeling like shit. This should make you feel a lot better," he said, and drained about 30 cc's of crystal-clear cerebrospinal fluid. I felt like a fog had been lifted from me when he was done. It was incredible, and I was giddy with relief.

It so happened that I had heard of this condition before. They called it pseudotumor cerebri, or idiopathic intracranial hypertension. It wasn't supposed to happen to me, though-- It was a disease of morbidly obese, middle-aged women. Not thin, healthy teenagers. Happen it did, however. Before I left the hospital that day, the radiologist warned me that the local doctors might not be so willing to accept this diagnosis, and that I might have problems getting help. Little did I know, then, how right he was.

I would eventually have to travel by plane to a large teaching hospital to get an official diagnosis and treatment. The doctors in my hometown would not diagnose me because I didn't fit "the profile" for the disorder, though repeated spinal taps showed consistently elevated CSF pressure.

After many months and many medications, I was still in agony and barely functioning. I had a VP shunt placed, and revised. Then there was the LP shunt, and a revision. After all this, still I knew something wasn't right. My head ached terribly, I was still falling, vomiting, seeing trails behind moving objects. Then there it was-- the answer right in front of me, apparent even in the first MRI done nearly three years earlier. A congenital malformation of the skull, Chiari I malformation.

With the Chiari diagnosis, I had to start all over. A new hospital in a new city, with a new neurosurgeon. Which is where I stand today, having exhausted most of my options. This is why I say the story never seems to go anywhere, though I know my doctors are giving me all they can. There is no cure, and my body seems to have fallen to pieces over the course of a few short years. There is always something wrong, and I can barely dare to hope that someday it might be different, that the story might have some closure despite the permanent marks it's left on me and those I love.

I've been reminded repeatedly in different ways that I will never be normal; In that way, too, the story doesn't move. The goal is, in the words of my surgeon, "to be normal enough." Something that's hard to do in this new world where the only norm seems to be abnormality.

None of this has really been edited, so don't shoot me for the tediousness of it or for any mistakes... I hope I didn't bore anyone to death.


Blogger Dreaming again said...

wow!What degree of Chiari do you have?

My son has ACM type 1. I need to bring it up at his next neuro appt, we can't get his migraines under control and at 13, we may be looking at Chiari headaches instead of migraines. We were 'lucky' to find the chiari at age 8 months of age, quite by accident.

His last MRI came back without the chiari on the report, but he showed it to the neuro and said "Is this the chiari thingy?" I thought it was pretty sad that a 10 year old saw on an MRI what a radiologist missed! (especially a 10 year old that was developmentally delayed and couldn't read!!!!)

Benjamin's last MRI still did not show compression with his chiari, while the chiari went down quite a ways ... there was room around it. The pediatric neuro said that will more than likely change as he goes through adolescence ... I dread it for him. But , at least we know that he has it, it will be first suspect, not last.

Have you done, are you considering the decompression surgery? Have you checked into the hospital in NY that specializes in Chiari (I have forgotten the name, if you haven't heard of them, I can find out for you, just email me at PeggikayeE@aol.com)

Benjamin had Bicoronal craniosynostosis which is what caused us to do the ct scan, which showed a portion of the frontal lobe had not fully developed (which the neuro hadn't looked at the films before walking into the exam room and saw the malformed brain for the first time in front of us, it was one of 3 times that I was to see a doctor visibly pale in front of me)
so they ordered the MRI to confirm the bi frontal lobe atrophy and found the Arnold Chiari Malformation Type 1. I'm thinking 8 mm ... but it might be 5 mm ...my brain is not functioning tonight.

As far as normal in abnormalities, when my oldest son was diagnosed with tourette's syndrome and OCD and was having trouble understanding what was different about him, I had him read some stuff on it. While reading some letters from other mothers about it ...he looked up and yelled "I'm NORMAL IN MY ABNORMALITIES!!"
From that moment on, his self esteem took a turn for the better.

Good luck! Keep me posted on what's going on. I so admire you pushing through all this to go to med school!

12:13 AM  
Blogger bronwyn said...

Wow, what a very small world it is!

My ACM1 was initially fairly small but significant in that it decreased CSF flow all around it. Then, later, the shunt in my lumbar spine drained too much fluid, basically creating a vacuum effect from below-- which lengthened the malformation.

I had the decompression (with duraplasty and all that other stuff) in December. On my pre-op MRI, the herniation was at least 12mm, though my neurosurgeon didn't give me an exact number so I'm not sure. His attitude (and i agree) is that the length of the herniation doesn't really tell you much about the patient's problems.

The NY hospital- Oi. My opinion of them, after extensive research, is that they're good people. They have good intentions. But they do too much surgery. Of course they have helped some people, but I believe they often do *more* surgery on a given individual than is necessary. Rather than just a decompression, they go for all kinds of plates and nuts and bolts. They do a TON of craniocervical fusions on ACM1 patients.
One of the surgeons is a member of (and posts to) a very active ACM web group that a large number of his patients also participate in... This is treading the tightrope above a sea of ethical problems. I have also seen him on a couple of occasions "flame" unhappy patients who post to the group. He posted confidential information about one woman without her knowledge or consent. The other case would require a lot more explanation, but his actions were reprehensible.

Sorry to go on at such length about that. Many, many Chiari patients who are online will tell you that NY is "the ONLY" place to go. This is totally untrue. There is a handful of highly skilled and qualified ACM experts to choose from.

In your case, there is one particular surgeon who comes to mind... He is a pediatric specialist whose primary research interest is craniosynostosis. He is also considered one of the best for Chiari, etc, etc. If you are interested for future reference, drop me an email and I will give you names of a few good peds neurosurgeons- God forbid you ever need to use the information! bronwynwinters .AT. hotmail Dot com.

Jeez.. Sorry so long. Thanks much for the comment, and I do hope things are going a bit better for you...

2:11 AM  
Blogger Dreaming again said...

When I first joined the world wide web, first thing I did was join some ACM groups ... didn't take me long to unsub ... as much as I'd like the information and support, I just don't think that surgery is automatic in all cases!

In a child where you can see the herniation and see that there is no compression to DEcompress ...and headaches & dizziness are only symptoms ...is brain surgery (because face it, that's really what we're talking about) necessary?

And all the groups seem sooo militantly strong on you MUST do this!
Do I think it's in his future, probably so, but I'm not doing it before we have clear evidence we need it.

Had he been diagnosed with the craniosynostosis (you know, that's harder to type than myasthenia gravis!) just 2 years earlier, he would have been caught up in the automatic surgical repair of all craniosynosistosis. He was one of the first in our area to 'wait and see' and we didn't ... we had several times that we wondered if we'd made the right decicion, and it kept coming back to haunt us about every 3 years ... but now, at 13, his skull ALMOST looks normal, you'd have to know cranio/facial stuff to know the problems ... and no long lasting complications. (unless, his sinus stuff really IS a result of the lack of correction which is mixed opinions ...half of the docs say yes, half say no)

I've read some of the NY group, and learned a lot. I hadn't realized they were operate on everyone mentality ... I have a problem with that mentality to begin with ... so thanks for the info. I'll be emailing you for the pediatric stuff.

thanks! And I'm finding this all interesting read ...

1:49 PM  

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