Texas - Surviving 16 hours plus stops

So we ended up leaving Wednesday October 9, 2013 after school for Texas.  Only taking the two Chiari kids on this trip we decided to ensure they would be B-U-S-Y with activities, food, movies and Innotabs.

I packed each child a kit that looked like this:

    

I also bought a 3 drawer organizer for the van at Target.  I find it easier to see in a drawer than dig through bags all the time.  I recommend adding a little Velcro to the drawers to keep them closed when you stop.  It works well.

Finally this is what we looked like ready right after we got gas and were on the highway headed to our destination.

 

   

Kids started with a movie                    Dad Driving               Mom- (don't you hate taking your own pic?)

Well, anyways we stopped for dinner about 2 hours after driving and ate.

Of course when you are driving you see all kinds of people, buildings, police and sometimes even a big fire!

After a bit we watched the sunset and both kids were fast asleep for the last hr to our midway Dallas.

 

We stayed the night and got up, grabbed breakfast and left early for the next 4hr drive on to Austin.  We stopped at 2hrs into the drive for lunch and arrived at the hotel about 2:30pm.  Fortunately we had a comfortable room ready and it looked great!~

      

Finally the morning came on October 11, 2013   

 

So, we didn't wait long at all before we went back.

 I let them take their goodie car bags in to play between the professionals  that came in the room to do vitals and history.  That really made it much better, because they were able to keep distracted from the amount of time it takes to wait.

We got there on time at 9:15am for our 9:30am appt. They came out to tell us right away that Dr. George had to go to a trama of a baby with pieces of skull in their brain and it would be 30 mins. (I was thinking 30 mins? That seems fast!). We started with the nurse and then the PA comes in and does history and does all the neuro tests. It was funny because apparently my 2 Chiari kids cannot "walk a tight rope". And my CM SM can't balance on right side well. Apparently that is common with CM people??? 

Well she no more than left and Dr. George appeared. So, I suppose you could say they definitely kept us company. Dr. George was great with the kids. He definitely does not want parents to "help" their kids answer a question. I made that mistake and he said "mom it's not a quiz you don't need to give her the answers"! I can't remember what the question was, but he definitely wanted to talk directly to the kids. He ran "racing" them down the hallway (my CM SM kid gallops when she runs. Always have and it looks like a skipping type of run). He then had us follow him to a different part of the office to view the MRI scans. He was very detailed in explaining to us that our 6yr old just looks like a CM and he couldn't see anything else on those scans (but I'm so irritated because we burned the MRI's and checked the images before sending and they were all there and somehow either the mail or their office staff messed up the scans so there were only 6 to 7 pics total for both of them!). I had the scans I have been posting on FB for ya'll to see on my phone and he said "text those to me". So I did. Well he said with the 6yr old he would go through the muscles and tendons in the neck NOT cut them away at all to work and remove some of her skull and cut into the outer dura only. He said a lot of surgeons make the mistake with kids of cuting the muscles and ligaments in the neck like they do on adults and trying to reattach them at the end and in kids that doesn't work. He explained the complications associated with the dura removal. Then he said the opposite for 4yr old CM SM. He said that he would go through the muscles etc. and that he would be opening her inner dura looking for blockages or tissue he may need to remove to see if that is causing the syrinxs. He showed us pics of a different patient before and after surgery that had a syrinx similar to our 4yr olds (not as big and hadn't stretched her cord as far and she was 13) but the shape was similar. It looked so much better but it was years later. He said maybe it would reduce in size about 50% but probably always be there . I asked about shunting her and he said no he does not want to do that but can't say he never would because sometimes circumstances require it but that it was usually from multiple failed surgeries with scar tissue that requires shunting. He said 3-5 days in the hospital and the surgeries would take 1.5 hrs. He said he only operates on 20-30% of the CM cases he gets. We talked about my 6yr old CM that is doing so well with modifications but he says that is "still" treating your child. He said that if you wait too long you can miss a window of opportunity to help prevent future symptoms. Like the modifications work right now. At some point they are not going to work anymore and when that day comes the pain she will feel will be unbearable. 
He said many times that he wasn't going into detail about all the things they look for in the surgery (I think he didn't want to overwhelm us). He didn't recommend doing both kids at the same time but was willing to do it. He said he feels each child deserves their own attention and it is hard to give each one that at the same time (recovery as a parent). We asked him about anterior issues causing the CM but he skirted around the response saying that relates to the CM 1.5 or 1.0 etc. but ultimately they all require the same attention of needing more room. So I believe that he feels that decompression is the treatment for all CM cases, however it could be that he didn't understand exactly what we were asking. I tried to clarify the question later during the discussion but he still went back to the decompression resolves the issues. 
They wanted better scans and then wanted to schedule their surgeries. I left telling them I wasn't quite ready to schedule the surgeries and that I would get them better scans. 
Dr. George seems very very skilled. He has very steady hands and was able to multi-task. When asked which child was more urgent for surgery (CM SM or CM for age) he said 4yr old was more compelling. OH that reminds me, when I mentioned Dr. Mapstone saying that they could outgrow the CM he looked at us with a funny disbelief look and said "no because at 6 their head is 95% adult size already. The brain is done growing and the skull too and the skull thickens on the outside not the inside and face as well with fat. At 4yr old about 80-85% done growing but that since 4yr old is 100% blocked surgery isn't an option with her. It is a must. 
I thought ya'll might like to know about our visit. I wanted to post it all asap so I didn't forget anything. We are still going for the TCI consult, but Dr. George is wayyyyyyyyyy better than the OK NS we saw.  

When we left we drove the whole way home without stopping.  We hoped they would fall asleep but stayed away the whole time until the last 70 miles lol.  They were good and I thank God for many things, but last night especially the TV in the car and

   STARBUCKS!  :)

Chiari and Retroflexed Odontoid and Basilar Invagination

So, now that I understand what Chiari is I am learning about Retroflexed Odontoid and Basilar Invagination.

Let's start with RETROFLEXED ONDONTOID; this is where the odontoid bone on the first vertabrea is bent backwards.

Here are pictures I got from  http://www.chiarisupport.org/photo/normalvschiari?xg_source=activity



Mostly what I can find is this is related to "instability" and that it "kinks" the brainstem.  I would love to see good comments on places to go to learn more about this.  I have spent a few days researching off and on and really can't find much out there except blogs with people's situation with this condition.  However, I can't really find a good medical site to help give a better reference.



BASILAR INVAGINATION:

   http://www.dizziness-and-balance.com/disorders/central/cerebellar/basilar%20invagination.htm

Basilar invagination is a craniocervical junction abnormality where the tip of the odontoid process projects above the foramen magnum. It may be congenital or acquired (also termed basilar impression) and is often associated with platybasia. There is stenosis of the foramen magnum and compression of the medulla oblongata resulting in neurological symptoms, obstructive hydrocephalus, syringomyelia or even death.

Ref:  http://radiopaedia.org/articles/basilar-invagination

Here is another definition I like:

This is a rare condition. To understand it, it is important to know how the spine works. The spine is made up of 33 bones, or vertebrae. These and the discs between them provide a passage for the spinal cord and nerves. The spinal cord itself connects the nerves of the body to the brain.

Basilar invagination occurs when the top of the second vertebrae moves upward. It can cause the opening in the skull where the spinal cord passes through to the brain (the foramen magnum) to close. It also may press on the lower brainstem. The brainstem is a stalk-like part of the brain that connects the main portion of the brain to the spinal cord.

This is similar to Chiari malformation. That, however, usually is present at birth.

Ref: http://www.cedars-sinai.edu/Patients/Health-Conditions/Basilar-Invagination.aspx

So this is basically saying that here is a condition that is like Chiari only the spinal cord is going up into the brain!  Ouch.  That has to hurt right?





A really good reference was here http://www.northshorelij.com/cushing-neuroscience-institute/for-healthcare-professionals/case-studies-june-2013-AD


 

Before surgery                                        After Surgery

Free Falling and......

Well, along with Chiari Malformation and Syrinx, I am also discovering that there are other things I must now research.

1.  Tethered Spinal Cord and Occult Tethered Spinal Cord
2.  CSF Malabsorbtion
3.  Scoliosis
4.  Retroflexed Ondontoid
5.  Basilar Invagination
6.  Ehlers-danlos syndrome (EDS)

Perhaps other things.  I am so shocked this is all related!  I mean the most important thing as a parent when your child is ill is to find the "boo boo" then "apply the care where and as needed".

I'm learning this is a lifelong condition that requires tons of medical monitoring and care.  I have learned that my child with the syrinx issues going all down her spinal canal could become paralyzed or die from this in a worst case scenerio.  I have learned that surgery is not a cure.  I have learned that I have to try.

I have been literally pushed off a cliff and I am learning to fly.  I am free-falling in mid air and trusting that God will catch me or let me land perfectly.

We have ONE LIFE here that God gives us.  I'm letting God direct my perfect path.  Things are falling into place that ONLY God could do.

What I'm doing while I the ride?  I'm educating myself and other people with headaches and weird symptoms to never give up on finding their answers.  I am learning about diet and food (currently trying to gradually ease into a Paleo diet.

I'm driving to Austin next week to see our first Neurosurgeon to find out what his plan would be then I'm coming back home for a few days then I'm driving to New York to meet another specialist to find out what their plan is.  Then I'm comparing.  I even learned of another Neurosurgeon in Miami and Chicago.  Maybe I'll go see them too?  I do know when God tells me I'm going to hear Loud and Clear what he wants me to do and Matt and I will be in agreement.

So, please, just let go and free fall and trust God in your situation.  It is so liberating!

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.