Notebook

Chiari Malformation Type I

What is Chiari Malformation?

Chiari Malformation (Arnold-Chiari) is a serious neurological disorder where the bottom part of the brain, the cerebellum, descends out of the skull and crowds the spinal cord, putting pressure on both the brain and spine and causing many symptoms.

Symptoms

The most common symptom of Chiari malformation is a headache, which begins at the back of the head (neck) and radiates upward. The pain is often made worse or can be brought on by coughing, sneezing or straining. These activities are known as valsalva maneuvers.

Visual problems such as nystagmus (involuntary eye movements), double or blurred vision may occur. Balance difficulties, vertigo and dizziness also may be present. Some people may have cranial nerve compression. This can result in apnea (cessation of breathing), gagging, swallowing difficulties, facial numbness or syncope (temporary loss of consciousness).

Patients may have muscle weakness, particularly in the upper extremities, coordination problems, and gait abnormalities. Imaging of the spine may reveal a fluid collection inside of the spinal cord, known as a syrinx. Some individuals may have hydrocephalus, a buildup of fluid in the ventricles of the brain.

Treatment

The first step after diagnosis is to consult with a neurosurgeon who has experience treating and managing this disorder. Be aware that you may need to travel and you may wish to consult with more than one specialist.If symptoms are mild and not progressing, your doctor may recommend conservative management. Supportive care such as headache and pain management, physical therapy or a reduction in activities can help manage symptoms.An operation may be recommended. This is referred to as a posterior fossa decompression. The surgeon makes more room in the back of the head by removing small pieces of the skull bones. This reduces compression of the brain stem and allows the tonsils to move back into their natural position. The specific surgical techniques will vary among surgeons; no consensus yet exists on the best variation on this surgical procedure.

The Importance Of Awareness

One of the major obstacles that Chiari and syringomyelia patients face is that it seems like no one has heard of the conditions. Once thought to be rare, MRI's have dramatically increased estimates of the number of people with Chiari (~300,000 in the US). This lack of awareness creates an additional burden for patients as they try to explain to family members, friends, employers, etc. what they are going through. In addition, the lack of general awareness has translated into a lack of research dollars from the US government. Diseases which affect about the same number of people receive millions of dollars of federal support each and every year.It's time to raise our voices, raise our profile, and make people aware.

Life with someone who has Chiari...

When you look at me, I don't look ill..... I might have a pained expression in my face which may appear to be angry. I'm not angry, I am in pain and I am trying to hide it from you the best I can.

If I am quiet or in a non-talkative mood, don't assume that I am in a bad mood, I am merely trying to come to terms with what I can no longer achieve, and can no longer do all that I want to do, and that it is very frustrating for me.

I might be exhausted from dealing with the pain and discomfort, or trying my hardest to do the things I once did on a daily basis.

Don't take for granted the things you did today, because tomorrow those things may not be that easy.

Don't pity me, I can do that very well for myself at times and I do try my hardest not to sink deeper in to it.

Don't compare me to other people with "similar" pain issues, everyone has their own problems, some worse than mine, some not so. Some people have managed to come to terms with it, I just haven't yet. Pain is different in all people. Not even two Chiarians have the same symptoms, although we do share a common bond.

I know it could be a lot worse, and I am grateful that it isn't, I really am, but it doesn't make me feel less pain or feel any better. In fact sometimes it makes me feel down that there are people that are worse off than me. I just can't imagine.

Just because I am in pain, doesn't mean that I can do something about it. Tylenol, Ibuprofen, or Aleve - they aren't a cure all. Some medications don't even work, or they give me bad side effects.

Don't tell me what I can and can't do, don't label me as disabled. I do want to try and do the things that I used to do, but if I can't, then at least I have tried and proved that to myself. Don't get angry with me for trying over and over........ I will get the message eventually.

Life with someone who has Chiari...Con't

Don't assume that I am angry or upset with you......sometimes we make mistakes and lash out at others. I am frustrated with myself. I don't mean it, I might have missed the fact that I upset you. Don't ignore it, tell me so.....but do it nicely please, lets not start an argument.

Don't think that just because I can do something today, that I can do it again straight away, or can do it tomorrow or the day after. My symptoms can change daily, my energy levels and patience can be sapped by the simplest of tasks one day, and appear easy to do the next.

Try and treat me "normal", I will respect that and will be very grateful. Try and encourage me to do "normal" as much as possible - Well as "normal" as I was a year ago!

Don't make exceptions for me, I will do that myself.

Don't be upset if I cancel long standing plans at the last minute - I want to be there, and I am trying to be there but sometimes I can't and don't have the energy to.

Learn about my condition and it will be easier for you to either help me when you can, or be aware that sometimes I do need help and haven't asked for it, or that I really don't mean to be rude or ungrateful. I am very grateful, I NEED friends now more than I ever have. You will know why I react in the seemingly wrong way sometimes........it will help you stop feeling like you have upset me..you haven't.

Don't be afraid to ask me how I am......I won't always give you bad news, so don't ask expecting it. But sometimes I might - so don't ask if you don't want to listen.

Help me to see my behaviour through your own eyes - but take a step back first and try and see it through my own.

Don't think that I am just in pain and that's the end of it. There's a reason that the pain is there, and I really don't have any control over it.

Life with someone who has Chiari...Con't

Don't be afraid to give me a hug or a little encouragement, we all need that sometimes. If I reciprocate or give you a smile back it means that I appreciate it, If I reply with a mean face...I still do appreciate it..very much, there's just something else going on inside my head.

Don't be sad that you cannot take my pain away, don't feel helpless because you cannot help me, what's the point in us both feeling frustrated and useless? Most of the time, I can't do anything about the pain myself.

If I tell you that I cannot do something, please don't get upset with me. I just really am not able to do it. Just "listen" to me, that's all I ask.

If you ask me to do something and awhile later it still isn't done...it isn't because I forgot (ok sometimes I do) and it's not because I'm lazy(but I can be), it's just because I don't have any energy left. Sometimes all I can manage to do is to sit around or sleep, and it doesn't mean that I expect you to do everything for me. It just means that I can't do as much as I may be expected to or as much as I used to do. I will get around to it. Tomorrow I may feel better.

My life has changed drastically and not for the better. Please don't expect yours not to change if you are involved in mine.

Know that I still love you, I might shout, I might yell, I might complain, I might get frustrated easily and lose my patience quickly. I am very grateful of the help and support, just don't make me beg for it.

I have a lot to come to terms with, I have a lot to face up to, it takes time to do so and even longer to accept. Believe me in the last year these are two things I have had to learn for myself.

Frustration can bring out the worst in all of us - I don't do it on purpose.

And If you are standing behind me in the checkouts - Please don't talk amongst yourselves about the scar down the back of my head/neck and take guesses as to how it might have happened. My ears work perfectly well and I can hear you. If you want to know, ask me. I can't hide it and don't want to.

Life with someone who has Chiari...Con't

I am not always miserable. I don't always have bad days. But if it's a "Chiari day", then just understand, even if it's a silent understanding - I will thank you, even if it's a silent thank you.

Know that I do understand that even though I may be the one with Chiari, I understand it affects you, and hurts you too.

And know that someday, once I learn to live with it, life will be easier....AKA I will be easier to live with(maybe).

My Journey....

On February 23, 2009 I had brain decompression surgery to try and alleviate the symptoms that I was dealing with on a daily basis.

The procedure that was performed by my Neurosurgeon was a Sub-Occipital Craniectomy, Duraplasty with Bovine patch, c-1 laminectomy and titanium implant. In layman's terms...a piece of my skull was removed to make room for my brain, the Dura over my brain was cut open and a patch made of cow heart was put in its place, also to make room for my brain. The backside of my first cervical vertebrae was shaved off since my brain came down into my spinal column that far, and a titanium plate was screwed to my skull to cover where the piece was removed. I had a total of 28 stitches.

I was in surgery for about 4 hours, and in recovery(PACU) for 2 hours. I was then put in the Neurological Intensive Care Unit(NICU) for 24 hours. I was finally moved to a regular room where I stayed for 2 days and was released.

My incision is from the middle of my head down approximately 8 inches.

I was off work for 3 months while I recovered.

My Journey Con't...

Together We Can Find A Cure

This presentation was made with help of the following:

Conquer ChiariConquer Chiari is our informal name, our goal, and our website. Chiari and syringomyelia are complicated diseases, which require complex solutions. No single action will beat them, but the combined efforts of many people on many fronts will. For far too long these diseases have destroyed people's lives, its time to fight back.

Education, awareness, and research are our weapons.

Compassion and conviction are our guides.

Strength, courage, and determination will sustain us.

We will win. http://www.conquerchiari.org/index.htm

ASAP(The American Syringomyelia & Chiari Alliance Project, Inc.) Providing Research, Education and Support Since 1988

The American Syringomyelia & Chiari Alliance Project, Inc. (ASAP) is a non-profit, 501(c)(3) donor- supported organization founded in1988 by Barbara and Don White. ASAP began as a grass roots organization in the Whites spare bedroom due to the frustration they encountered when Barbara was diagnosed with Chiari malformation and syringomyelia. Since then, ASAP has grown tremendously but our fundamental goals of research, education and support remain the same.http://www.asap.org/

Misty CamaraDiagnosed with Chiari Malformation Type I 1/14/2009

Proud Zipperhead since 2/23/2009

Any questions please feel free to e-mail me at: mlc11407@comcast.net

Looking for fellow Chiarians in and around the Western PA area to start a support group.