Knowledge is Power - Where I go to talk to others about Chiari Malformation

I have joined some facebook groups like Chiari Kids and Chiari Friends in Oklahoma.  Also there is a really big group on yahoo called The World of ACM Association Support Group and if you join that one you'll also want to join the WACAM Parents yahoo group as well.

There is a lot of knowledge out there about Chiari....it just isn't in the same place!  Some of it is from doctors and books but most of it is the parents and people living with it every day.  Real life knowledge is the best.  I personally didn't go into the groups looking for friends (maybe I have Asperger's too).  I joined because I am a researcher at heart.  I love having a problem and conquering the problem if I can.  I do believe if it isn't right down the street it's at my fingertips on the keyboard of the big wide web out there.  I also believe that I will be traveling to see a specialist for this for my children hundreds if not over a thousand miles away!

Other good sites are:

http://asap.org/index.php/disorders/chiari-malformation/
http://www.conquerchiari.org/index.html
http://csfinfo.org/


I'm sure others will come along at some point and post their favorite support groups for CM out there.  Please do - knowledge is power!

Syringomyelia - A word I have learned to say as "Syrinx"

Here's the facts:

Syringomyelia (sih-ring-go-my-E-lee-uh) is the development of a fluid-filled cyst (syrinx) within your spinal cord. Over time, the cyst may enlarge, damaging your spinal cord and causing pain, weakness and stiffness, among other symptoms.

Syringomyelia has several possible causes, though the majority of syringomyelia cases are associated with Chiari malformation, a condition in which brain tissue protrudes into your spinal canal. Other causes of syringomyelia include spinal cord tumors, spinal cord injuries and damage caused by inflammation around your spinal cord.

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a cyst forms within the spinal cord.  This cyst, called a syrinx, expands and elongates over time, destroying a portion of the spinal cord from its center and expanding outward.  As a syrinx widens it compresses and injures nerve fibers that carry information from the brain to the extremities.  Damage to the spinal cord often leads to progressive weakness in the arms and legs, stiffness in the back, shoulders, arms, or legs, and chronic, severe pain.  Other symptoms may include headaches, a loss of the ability to feel extremes of hot or cold (especially in the hands), and loss of bladder and other functions.  Each individual experiences a different combination of symptoms depending on where in the spinal cord the syrinx forms and how far it extends.  Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining.

Many disorders share the early symptoms of syringomyelia, which also can occur in association with other conditions.  Estimates of the number of Americans with syringomyelia vary widely, but a conservative estimate is that about 40,000 people in the United States are affected, with symptoms usually beginning in young adulthood.  Some cases of syringomyelia occur in more than one family member, although this is rare.

Those are the definitional facts (I make up words sometimes when I feel it fits so definitional is my new word for the day).  I am very worried that my 4 year old has not just one syrinx but several.  I have posted her syrinx image before but here it is again:

See those "white marbles" ?  The ones in the neck are huge of course, but there are smaller ones going on down her spinal cord.  Well, that's syringomyelia.  Now for Olivia, I know the pressure in her spinal cord and head have to be an issue because she talks about "the worm in her head knocking" and "her neck beeping".  So, I plan to post more about how you go about treating this stuff...but for now it has to wait.

References:

http://www.mayoclinic.com/health/syringomyelia/DS01127

http://www.ninds.nih.gov/disorders/syringomyelia/detail_syringomyelia.htm

What the heck is Chiari Malformation anyway?

So, what the heck is Chiari Malformation anyway?  This is the question that must be answered!  Chiari Malformation (CM) is basically when your brain is too big for your skull.  Yep really.  Well, here is the technical meaning of Chiari:

Chiari malformation (kee-AH-ree mal-for-MAY-shun) is a condition in which brain tissue extends into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward.

Chiari malformation type I develops as the skull and brain are growing. As a result, signs and symptoms may not occur until late childhood or adulthood. The most common pediatric form, called Chiari malformation type II, is present at birth (congenital).

Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.

CMs may develop when the bony space is smaller than normal, causing the cerebellum and brain stem to be pushed downward into the foramen magnum and into the upper spinal canal. The resulting pressure on the cerebellum and brain stem may affect functions controlled by these areas and block the flow of cerebrospinal fluid (CSF)— the clear liquid that surrounds and cushions the brain and spinal cord—to and from the brain.

So basically it looks like this:  



So, a mere millimeters can cause a lifetime of problems!  Now if you notice in the picture above showing the Chiari 1 Malformation, it shows the CM and also shows the syringomyelia or "syrinx" for short.  (Yes syrinx is spelled that way.)

So here is what my two kids chiari looks like up close:

Picture from:  http://www.chiariinstitute.com/chiari_malformation.html
References:
http://www.mayoclinic.com/health/chiari-malformation/DS00839
http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm

In the beginning with Chiari Malformation

So, after several attempts and understanding the complaints of my just turned 6 year old daughter Abrianna, we finally learned she has Chiari Malformation 1.

Here are a list of what she was complaining about:

When she was 3 to 4 years old she kept on and on about her stomach.  She also had a lot of swallowing issues with reflux, not as an infant, but actually turning 3.  We did everything possible to get to the bottom of her stomach issues.  We had every test imaginable on her stomach and intestines.  Even a surgical scoping procedure that gave us no answers.  Unfortunately, a few weeks after the scoping she was freaking out at 2am saying her butt hurt and I looked at it and saw about 100 pinworms crawling out.  Yes, shutter and shake at the thought of pinworms crawling out of your baby's butt, especially in the wee hours of the morning.  The medication we thought caused her an awful reaction of severe I mean SEVERE headache and stomach pains....now I think it was the irritation of her fits from the situation.  The stress or something.  Fast forward to last summer 2012 where she would have constant nose bleeds and headaches.  We thought they were related went to the doctor and the ENT.  Nothing again.  NO answers.  Headaches turned into all over body symptoms from her saying things like "I know I have an arm but I can't feel it" to neck pain.  The neck pain with headaches landed us in the neurology office and that sent us to the MRI.

I was so worried about sedation for the MRI for her (since she suffered hallucinations after sedation from the scoping procedure) that I put off the MRI and decided to just wait and see.  Well, that was in the fall and in March one morning Abrianna woke up and was unable to walk.  It was honestly one of the scariest moments for me with her.  She began sliding downhill fast.  It was headaches to weakness to nosebleeds to weakness to headaches to severe neckpain.

March 21, 2013 Abrianna had an MRI of the brain and C-Spine w/out contrast. The neurologist called us and said "Well the good news is that she does not have a tumor or anything in her brain causing these issues, however, she does have Chiari Malformation 1".  He went on to explain it over the phone the best he could and then advised us to avoid blogs and websites because the information would just scare us.  So, I did.  I actually know a father of 6 with Chiari and he functions just fine.  So I just wasn't worried at all.

Now, in the meantime, let us not forget precious Olivia, who is going to be 4 in a few days.  Well at the age of 2 she would tell me she had spiders crawling in her brain.  I thought she was talking about Sponge Bob or some cartoon, so I ignored it.  Then she would spend an entire year coming up to me throughout the day saying "my neck is beeping" "I'm not feeling well" "There is a worm in my head".  I took her to the doctor for earaches I was convinced she had and her ears were fine.  Finally, she began to wake up with headaches and I took her into the neurologist and told him she was waking up with headaches in the middle of the night and early mornings and he said "oh that is not good".  I thought perhaps she was mimicing her sister in complaints, so I didn't think anything would turn up.  So, on July 5th, 2013 we had Olivia's brain MRI with and without contrast.  She did perfectly, woke up a little grumpy from the anasthesia but otherwise perfect.  We learned the following week that she also had Chiari Malformation 1.  So, now I have two babies with Chiari Malformation that I know of.

We have two older children, Victoria 13 and Sarah 12.  Neither of them have been tested for Chiari but one does have Asperger's Syndrome and did suffer from seizures when she was smaller until 5 where she magically outgrew them.  The other has hyperflexible joints and fine motor issues.  A LOT of fine motor and gross motor issues.  Our plan is to have them tested for Chiari as well, but that will have to wait a bit.

So, Friday September 13, 2013 my to littles had their full spinal MRI's.  The following Monday we learned that Abrianna's MRI looked great minus the Chiari.

However, Olivia unfortunately has several Syrinxs going down her CSpine and Thoracic to the Lumbar area.  :(

So Olivia's actual MRI report states:

Identified again is a Chiari one hindbrain Malformation with cerebellar tonsils extending through the foramen magnum to reach the level of the posterior arch of C1.

A sepatated syrinx is identified extending from C4 inferiorly to T3.  The syrinx is sepatated, expands and thins the spinal cord occupying almost the entire spinal canal.  The syrinx measures approximately 5.5 cm in vertical deminsion and approximately 7mm on AP deminsion.  There is intermittent, slight prominence of the central canal in the thoracic spinal cord extending from T4-T5 to T6.  A small syrinx is identified in the thoracic spinal cord at T7 measuring approximately 7cm in vertical deminsion and approximately 3.5 cm in AP deminsion.  Two smaller syrinx are identified in the conus medullaris posterior to T-11-T12 and posterior to T12-L1.  

The Conus Medullaris terminates posterior to L1.  

No obvious evidence for segmentation anomaly is identified in the spine.  

Impression:  There is a Chiari one hindbrain malformation.  Multiple syrinx  are identified in the cervical and thoracic spinal cord as described above.