Archive for September, 2008

LOVE

All 3 have ACM and Syringomyelia

All 3 have ACM and Syringomyelia

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LIPSTICK

Validation is hard to find when it comes to Chiari- and for the life of me, I’ll never understand why, when considering that the most fine-tuned organ in the body is being compromised. One would think that compression of the cerebellar tonsils and brainstem, coupled with adhesions from a lifetime of friction between the two and the restriction of cerebral spinal fluid flow would cause some very serious neurological problems and dysautonomia. Pardon the pun, but this seems a no brainer to me. How and why do doctors dismiss this? I think a large part of it is egos protecting ignorance. If they dismiss what they do not know or understand, they won’t look foolish when having to admit they’re unsure. Rather, they pretend to know all about it and feign expertise by confidently proclaiming that they would surely recognize Chiari -and of course, none of us have it.
Such games are dangerous and perpetuate our suffering. Sometimes forever.
If doctors took the time to understand chiari malformations, they would learn that there is a HUGE array of symptoms associated with it, for fairly simple reasons. We are, after all, all built slightly different. While human anatomy is mainly the same, it’s all in different shapes and sizes. If one person with a large foramen magnum has a large chiari, he will likely be less symptomatic than someone with a smaller foramen magnum and any degree of tonsillar herniation. Blocked space is bloced space, it matters not the size of what is plugging up the hole. What matters is the blockage and the compression. This is simple stuff, people.

For some reason, it would seem that the majority of physicians (of all specialties) were taught the very basic symptoms of chiari at one time or another (during residencies or in med school). Those signs and symptoms seem to to consist of “crushing sub-occipital headaches, nystagmus and dizziness”. Most of the time, chiari is not considered even when a patient presents with those maladies. That is sad in and of itself, but what is sadder still is that those symptoms are but a fraction of reality.

I’ve heard specialists say that they have patients who present with “temporal lobe seizure like episodes, migraine headaches and vision complaints” who they say have “incidental, asymptomatic Chiari Malformation”. Excuse me??? Am I the only one who sees the stupidity in that? Why woudn’t a neurosurgeon attribute those complaints to a symptomatic chiari malformation!? Because the patient doesn’t have nystagmus or the “classic sub-occiptal headaches”?? Morons.
If a patient has an “asymptomatic chiari”, they should be symptom free maintaing the same happy-homeostasis of “normal” people!!!!!!!!!!!
Good gawwwwd, I hate dismissive, egotistical, arrogant, ignorant doctors!

I watched a patient with “asymptomatic chiari” present with sudden heart palpitations and the inability to walk without falling down. I’m no neurosurgeon but wouldn’t you think the BRAINSTEM COMPRESSION may be the culprit?? MAYBE!!??? Nah.. she was a rule-out acute heart attack and sent on her merry way- sideways.

Now, I’m not a chiari freak who attributes every hangnail to ACM. I actually have no use for people like that, but let’s be smart. If your BRAIN is hanging out of your SKULL (that happy protective place), something is WRONG. Hello!? It’s your BRAIN. Sometimes i’m pretty sure that doctors think we have a spare one. Your brain is supposed to be INSIDE your skull- that’s the point of your skull. There is a HOLE at the base of your skull so that your spinal cord and cerebral spinal fluid can have a nice pathway to your brain. If you plug-up that hole- problems are sure to follow.

Am I nuts? Yes, but that’s beside the point. This is very simple stuff. If any other body part or organ was displaced, not a soul would dismiss it. If you walked around with your tongue hanging out of your face since birth, surely someone would say.. “hey, that thing belongs in your mouth” and a doctor would eventually fix it and attribute your social alienation to the obvious. We’re talking about our BRAIN, damnit. Give messing with it, the credit it deserves!!

Let’s talk about another thing… I look completely normal (or so people tell me). This does my condition no justice. Why is it that I must skip the shower and wear no make-up to my doctors appointment for them to take me seriously? Why must I look like shit if my neurological system is fubared? Don’t dismiss me anymore because of my lipstick, pal!
Do NOT judge how people feel by how they look on the outside- It’s childish.

Now let’s talk about radiologists..
I have had dozens of MRIS. My children have had dozens of MRIs. As you can see by our films, our Chiaris are hardly subtle (note the part of the brain that NOT IN THE SKULL WHERE IT SHOULD BE). We’ve all had at least one report say “normal MRI of the brain” and/or “incidental chiari”. Oh , really? I bet their brains are on the inside! (or maybe not).

I would like to ask them to define “incidental” for me. If you do not know my symptoms and you’ve never examined me, who are you to tell me that your findings are incidental? If you found a malignant tumor in my head when my chief complaint was headache “incidental”? If you’re reading a chest xray to rule out pneumonia and find a massive tumor, do you call that incidental? With each MRI I fill out what my complaints are. Guess what? If I write visual disturbances and headaches and you find a chiari malformation while ruling out MS or a mass… GUESS WHAT? If I don’t have a mass or MS, my chiari is no longer “incidental”. It’s a clinical finding. Say “eureka!!!” and pat yourself on the back.
Attaboy.

/rant off

SOME PORK FOR ME

We need Federal Grant money to supplement the generous private donations to ASAP.org.

In 2008, our Congress authorized $17.2 billion in Federal Grant appropriations. It’s time they share the pork with the zipper heads.

Please contact me if you’re willing to help me lobby. I have no experience, but I’m willing to learn.

We need research!!!!!!!!!!!! Last week!

MY 3 BEAUTIFUL CHILDREN

I had 3 children during my pre-diagnosis days.
My youngest (Grace) was 4 when I had my surgery. Upon my diagnosis, I became very concerned about whether Chiari is genetic. I was told that there is only a very small percentage of familial incidence; less than 12%, with a then 50% chance of that gene ever becoming functional. I was told that there was no need to test my children unless they showed very obvious signs of ACM.

They were wrong. I should have had them tested sooner. All 3 of my children have varying degrees of Arnold Chiari Malformation, Syringomyelia, retroflexed odontoid, basilar invagination, Spina Bifida Oocculta and Ehlers Danlos Syndrome.

I will tell each of their stories during the next week.

Too little is understood about the genetics behind these conditions. The 8-12% familial incident rate needs to be looked at more closely. I would bet my titanium cranial plate that this number is hugely underestimated. While I know 8-12% is the actual percentage found among patient populations, I challenge how hard they’re actually looking at their patient’s families. All too often I hear symptomatic patients referred to as having “incidental asymptomatic Chiari” simply because doctors don’t understand the wide array of symptoms associated with Chiari.

AN INTRODUCTION TO MY DROOPY BRAINS

My diagnosis of Arnold Chiari Malformation during the fall of 2004 was both a nightmare and a blessing. After just turning 32, my life was becoming even less manageable than before, which was a very sad feat in itself.

I’d literally spent my entire life wondering what was wrong with me. As a child I tried as hard as I could to be organized, like my friends were. Their clean, neat desks and backpacks used to amaze me. Why couldn’t I do the same thing? Why was simple tasking so hard for me? Why would I read the same page 5 times before I’d even notice? Even then, the words would go in, but miss the processing needed to comprehend and retain it. I would try to speak a word, but forget it before it would hit my lips. My brain knew what I was trying to say, but my mouth just wouldn’t cooperate. It’s almost like a disconnect. I can see and hear the words in my head- they just won’t come out. I carried around enormous guilt, assuming I was simply lazier than my friends. Maybe I was just not as smart as they were? What never made sense was -why on some days I was sharp as a tack, full of wit, savvy and remarkable organization skill, while most other days I couldn‘t assemble a 10 piece puzzle if it would‘ve saved my life.

This dreaded condition named Chiari is made so much more torturous by its teasing ways- It’ll allow you to know your full potential just long enough to yank it out from under you with the blink of an eye, or a literal sneeze. My very own brain feels violated by my own brain.

I would have similar problems with my vision. I could and couldn’t see all at the same time. Sound crazy? I swear it’s true. I couldn’t explain it as a child (which was maddening), but all I knew was that my vision was very “wrong”. I could see, but my brain just didn’t process what it saw. I knew that my eyeballs were working, but I couldn’t explain that, and regardless, I still couldn’t see.

Of course, back then, I couldn’t articulate these thoughts. I just knew something was very different about me and it hurt and scared me. Aside from these torturous cognitive problems, I was also very ill. I was the kid who never felt well as a teen. I had headaches and neck pain, that was so common I took it for “normal”. I know that sounds odd, but I just accepted that anytime I wore a necklace or halter top, I would pay for it with a headache that could kill a horse. People with congenital Arnold Chiari Malformation don’t really know “normal”. All we have are our good days, bad days and known triggers. A painless, astute and coordinated day is a trifecta akin to scoring a real unicorn from your birthday candle wish. It just doesn’t happen, no matter how hard we wish it.

I spent my late teens and twenties chronically ill with severe fatigue, generalized malaise, dizziness, headaches. I went to dozens of different doctors and specialists from age 15 on. I was told I had everything from mono, to chronic fatigue syndrome, to “you’re too tired (no kidding!) take a nap”, to the typical PMS label, and of course, the standard “you have anxiety and depression”. I don’t know a person with Chiari who hasn’t been issued a mental health diagnosis first. It’s infuriating, insulting and prophetically causes you to actually begin feeling crazy! It’s for this reason I started my story out by saying that my diagnosis was also a blessing. I finally got validation that something was indeed wrong! I wasn’t crazy.

I eventually had an MRI of my brain (thanks to a doctor who finally took the time to hear me). I had several strange symptoms that couldn’t be ignored. I was still producing milk 3 years after I stopped breast-feeding my youngest child. This is more abnormal than it sounds. Your milk should stop production shortly after you stop nursing. I was also having drop attacks, where my legs would just collapse without warning. My MRI was to rule out a pituitary tumor and/or MS. The MRI found neither. It did, however, find a venous angioma that required a consult with a neurosurgeon. To this neurosurgeon’s credit, he took a very thorough history from me and was fascinated by my symptoms. After looking at the MRI films, he concluded that the venous angioma was “nothing”, but I had something called a “Chiari Malformation”. He went on to tell me that it really wasn’t a problem because it wasn’t very long (5-6mm). He dismissed it and sent me off to a neurologist to rule-out “temporal lobe seizures’. I’d like to note that my tonsilar herniation was very apparent, yet it was never so much as mentioned by the radiologist in his report. I went home and looked up what a chiari malformation was. I was floored. They could have named this condition after me. Its symptoms included almost every complaint I’ve had for 20 years! I called the neurosurgeon and asked him why he wasn’t more impressed with his find since I had so many signs and symptoms of ACM. He told me that Chiari categorically was not my problem because I wasn’t herniated enough. The ignorance in the neurosurgical community regarding ACM is absolutely astounding. I explained that I even had (in my possession) medical records that dated back to when I was 11 years old complaining of dozens of the symptoms of ACM. He still dismissed me. I knew I needed to do more of my own research then. I also called the MRI radiologist and asked him why he ignored my brain falling out of my skull. He asked me to bring the films back in so he could look, and all he said was that it was an oversight. He then wrote an addendum to his report. How much more flip can a person be about someone’s brain!????

After countless hours scouring the Internet, I found The Chiari Institute. I was blessed to find out that this world renowned treatment center was 10 minutes away from my home.

I ultimately had a posterior fossa decompression with duraplasty 4 months after diagnosis on February 18, 2005. It took 2 months to get my family on board with the idea that I needed this surgery. I literally would have given up one of my limbs for any chance of regaining my cognitive function. I was actually herniated more than twice what the MRI showed. My vertebral arteries were imbedded and my brainstem kinked.

I’ve since had about a 60% improvement in my symptoms. I no longer have drop attacks and my eyes and brain finally work in conjunction. My cognitive function is still not what I hoped for, but I am very pleased that I tried. .I have no regrets about my surgery- other than I wish I had it 20 years sooner- Which brings me to my children…

Help is needed seeking Federal Grant money to supplement the generous private donations to ASAP.org.
In 2008, our Congress authorized $17.2 billion in Federal Grant appropriations. It’s time they share the pork with the zipper heads.