Last Day of School a thank you to the staff

So, my first grader is having her last day of school today.  Fortunately, her oldest sister was able to go with her to help out.  They had some bouncy houses and she cannot do those with Chiari.

I am so thankful for all the staff at her school, but especially nurse Paula and her First Grade Teacher Mrs. Y--k.  In fact, Abri wants to be a first grade teacher when she grows up!  She told us short people make great teachers.  She is excited about her yearbook and I think she is going to greatly enjoy seeing all the familiar faces from school of other kids and staff.

I want to apologize to the teacher she gets next year.  This is because we are currently and will probably continue to spoil her rotten all summer!  :)

Abri has lost her first upper big tooth.  The other one is going to be gone soon too.  My toothless sweetie is getting so big.

I look at her and think about all the good days she has without headaches and neck pain and swallowing issues and leg weakness.  Then she has breakthrough moments that are severe for her and I know we are doing the right thing.

Here is what Abri's Chiari looks like right now:

Here is a picture from the chiari institutes site:

Abri does not have the syringomyelia (unlike her 4yr old sister Olivia), and that is another reason to do the surgery.  We never want her to get it either.

It is hard to tell that Abri has Chiari because she seems fine so often.  Here is what happens when her neck is flexed and extended:

Do you see how that "Chiari" which is really the "tonsils" of the cerebellum get pinched in there?  See how her brainstem gets "knocked around"?  That's what causes all her weird symptoms all over her body!  I cannot imagine how crappy that would make me feel all the time every time I moved my neck around!  I think Abri's beautiful smile is what keeps us from remembering that herniation is restricting her CSF flow.

As this month of May ends, we look toward a surgery that is getting closer.  I am writing a lot about this now, so that in the future when my kids or others ask me how I did it, I'll be able to remember!  Some days seem to run together.  Keeping ourselves totally occupied with business is the only way to avoid obsession about this situation.

Today, Olivia was dragging her feet on the carpet.  Her legs were hurting again and last night Abri was crying over the inside of her head feeling too full she will say.  She says it is like a balloon.  I worry some about Abri and Olivia's head pressure, but I know that God has it all in his hands.  Somehow, hearing these complaints helps me to know that we are doing the right thing, and I think that is how God comforts us in a strange way.

We cannot wait to get past this surgery and DEFEAT THE DEVIL AND CHIARI ALL AT THE SAME TIME!  What a testimony we are going to have!!

Thank you for all the people praying for us.  We love you all.

More on Instability, Syrinxs/syringomyelia and surgery in 30 days

So, we took Olivia to get her CT scan done.  She was NOT happy to be going :(

Dr. George ordered the scan in order to check her anatomy for "instability".  He did not specifically say anything about the retroflexed ondontoid that I have wondered from the "hill" on her MRI.  I'll be asking him about that at our next visit, scheduled the day before surgery.

Olivia did GREAT for the CT.  NO SEDATION!! That's a first.  I wish the health insurance companies would just pay for the scans all at once instead of having to go 4 different times!  Here is a picture of her during the scan:

                                                              So brave with Daddy

Abrianna is scheduled for a craniectomy with great hope that she doesn't need anything else, but we are having to give consent for more things.  I guess when you have a surgery like this you have to consent to let the Neurosurgeon do whatever it takes during surgery. 

Olivia is scheduled for a craniectomy, duraplasty, and laminectomy in order to get her Chiari Malformation Type 1 and Syringomyelia under control.   I have requested that they harvest Olivia's own skin for the dural patch rather than use a foreign patch that could cause complications if her body rejected it.

Both surgeries are scheduled for 90 mins each.  I am thinking that Abrianna's should be faster should they only have to do the 1 thing. 

 For those who are new to the blog and havn't seen the pictures posted of what the MRI looks like currently for Olivia:

Her Syrinxs are very large.  So large, they saw them on the CT scan.  Here is what Olivia's CT scans looks like:

3D is so cool Right?  Anyways........According to Dr. George, Olivia's bone anatomy looks great and he is not worried about instability.  I am going to specifically ask him about retroflexed ondontoid at pre-op appointment the day before surgery.  Praise the Lord for good news finally!  But I looked through the images on the CT scan several times and I could sort of see the syrinx when I adjusted the brightness but I definitely had to know what to look for because I really can't see it on the images at all!

That makes me even more concerned for her because of what she has gone through.  I remember when she was 3 she would say all the time "I'm not feeling good" and "My neck is beeping".  :(

Well, both girls are scheduled for surgery on June 20th in Austin TX with Dr. George.  It is extremely stressful. 

I'm looking forward to having this fixed and moving past this.  Every year I say "I just have to make it to Christmas this year".  Currently, I just wan to get through July 4th.  That is two weeks from surgery and Independence Day.  I am going to celebrate July 4th as our independence from Chiari.  Everyone says that surgery is NOT a cure and I know that.  However, knowing that we are working to get rid of the syringomyelia is independence.  Working towards reduction of neck pain and headaches for Abrianna as well is independence.  Freedom from disease.

I am being told this is going to be harder on me than my children.  I sure hope so.  Currently, that is definitely the case.  Can't even look at them without my heart sinking in, with a sharp pain that immediately jumps into my throat.  I swallow away the tears so that I do not worry them.  Sometimes they look at me and say "what's wrong mom?"  and I just tell them "I have a swallow stuck in my throat". 

Amazing that your kids can see right through you right?

Well, that is the update for now. Here's a pic of my Chiari warriors with their sisters.  :) 



Back from Austin TX and making progress with Dr. George

Well, it was definitely a big trip.  We left Tulsa at 2:30pm and we didn't get to check into our hotel until 1:30AM!!!  Yes, really.  Apparently, President Obama, President Clinton, and President Bush were visiting FT. Hood, so Texas decided to shut down the highway 36 miles from where we needed to be in our hotel.  We knew we were going to get in late, about 11pm but not like that.  Thank goodness we were able to "backwoods" off the highway and follow country roads to get around.  A 50 miles detour got us there.  I feel sorry for the rest of the people that probably slept in their cars there!

So, we went to see the doctor.  We were there on time, but his office is crazy busy.  He was in surgery that morning and was two hours late.  But his staff is wonderful and we waited with good company.  I was able to meet a lady I had been chatting with on Facebook who also has two boys dealing with neuro issues.  One has a large arachnoid cyst and the other has chiari. 

Dr. George came into the room.  He had a tag-a-long Resident doctor with him.  He advised that Abrianna will have a wonderful outcome after the decompression.  She has what he called "classic" chiari and tussive headaches.  He explained tussive headaches are not just from coughing and it is from the vessels dialating with activity due to blood pressure increasing.  So, since there is no room in her head for brain vessels to expand, when they do bad headache that subsides quickly as bp goes down.  She has been very stable with accomodations and meds.  However, he said 98% chance getting off meds with surgery.  It is important we do it while she is this young because each time these headaches come on more "damage" is done and the surgery wouldn't be as successful in getting rid of pain symptoms.  As for other symptoms associated with Chiari, he talked about "spectrum" symptoms and the goal for this is to remove the headache pain. 

On to Olivia.  She is very complicated.  He is going to plan to do decompression with duraplasty because of the syrinxs.  Then he is going to remove part of her c1 called lanectomy.  However, before he would schedule surgery he is getting a 3d CT scan of her head and neck.  If the bones do not look right, he is worried about instability caused from the bone removal, so she would need to have fusion as well.  He is not worried about tethered cord because she has sacral dysgenesis type 2 which is a mostly truncated sacrum, where the filum terminale doesn't really form so there is nothing to tether.

So, I am praying so hard that she does not need fusion.  Dr. George did say that if she looks boarderline he is not going to fuse and if she needed it later, he would do a 2nd surgery.  He believes in least invasive methods. 

I couldn't believe how strong I was!  Seriously had many pictures in my mind of trying to hold it together.  Maybe the tiredness from lack of sleep had something to do with it?  But I felt like I was in the best place possible for this. 

Our CT will be done this week and it is a stat order with results due by 24hrs.  It is a 10 min test and it stinks that they have to sedate for it.  I think it is to keep her from moving during it. 

I'll post the results when I get them.

Changed our Minds on the Neurosurgeon

So, we loved the Dallas Neurosurgeon a lot.  However, the MRI testing and follow-up conversations with his office have caused us to re-evaluate our decisions.  So, we are actually going to use a Neurosurgeon in Austin TX Dr. George.  To us, he seems to have the best reputation and skill for handling such a hard thing as Chiari Malformation and Syringomyelia.  Dr. George is participating in studies for Chiari and he is also considered a Neurotube specialist. 

In reading about Chairi Malformation and Syringomyelia, we know it is often associated with other disorders such as:

Hydrocephalus
Spina Bifida
Syringomyelia
Hydromyelia
Tethered Cord Syndrome
Spinal Curvature such as Scoliosis
Various bone growth and hereditary genetic disorders

For me, right after Olivia our 4yr old was born, the pediatrician that was on call (not her normal one) pointed out that she had a hemangionma that is located in the small of her back at the very base of her spinal cord.  At the time it looked like a bright red birthmark about the size of a quarter.  It was extremely noticeable.  He said she appeared perfect except he was worried about her spinal cord being "tethered" because that is the place they do NOT want to see a birthmark. 

Olivia had an ultra-sound when she was about 6-8weeks old and they cleared her of any issues.  Now I know that an ultra-sound is not the test of choice for confirmation of a tethered spinal cord.  It should have been an MRI.  Some people still think the ultrasound would have been ok.  Hopefully there is more data about it now out there.

Olivia's CSF study revealed that her cerebellar tonsils are also touching the brainstem.  I have been reading about deformative stress.  I am pray this isn't the case with her, but her scans do look like retroflexed odontoid.  We will see what the NS says. 

Abrianna our 6yr old, her study stated it was abnormal and the impression was that it was normal.  The Neurologist said you cannot be abnormal and normal.  We still can't get clarification from Dallas Children's on this report.  I have found that when I question a radiology report, I do not get very far with it.

I think the important thing is to focus on April 11.  That is the day we will get all the information and schedule surgery for June.  Pray for us.  I'll post in a week or less about what the Neurosurgeon says.

Valentine's Day News....not so lovely turns into blessings

So, its Valentine's day.  The new office is open and everything is looking like it will be nice.  The weather is good and I'm "working" with my hubby.  All is going well until I get a frantic message from my realtor to call her right away on my house for sale.  I'm excited thinking "yay finally maybe we sold it!".  So, I called the realtor and she says "your house is flooded, I'm going there now, that is all I know".  Uh....what?  Yep.  Apparently, the upstairs instant hot water tank decided not to work anymore and a valve malfunctioned, causing a massive flood in the entire house.  Remember I just said UPSTAIRS tank? 

Here are some pics of what it looked like:

                                                     This was my laundry room

                                                 This was my laundry room ceiling

                                                     This is where the water collecting in the hallway ceiling

                                                            This was my hallway wall

                                                                different hall wall view

                                                               Over the bathtub it was a ceiling

                                                         different bathroom ceiling view

                                                              ceiling full of water

                                                         This is NOT texture.  IT's peeling paint.

                                                          Pretty huh?

                                                          No more wall behind the bathtub either

                                                            Only the heat air return vent lasted!

                                               Wall damage

                                                     Water heater leak. You can't even tell!

                                                Water heater hose fitting leaking.

                                                 The cold water side was fine.  The hot water broke.  Sheesh!

So, I hired a water restoration company and carpet company to clean up the house.  The carpet cleaning company was able to save the carpet but not the padding.  Apparently the water was STANDING 6 inches throughout the house.  The walls in the house sucked up the water like a "wick".  So, everything looked and sounded horrible. 

Enter the water restoration company!  OK I do not know how they do it, but they make your house look totally normal.  No mold, no messed up walls (except they remove the walls that are not saveable).  They drill holls every 5 inches or so along the bottom of the walls and then they use thermal imaging cameras, water detection equipment, dehumidifiers and fans.  They did this for about 9 days in the home. 

In the meantime, I contacted my insurance company.  This loss IS NOT COVERED.  Yep, I feel robbed but I'm still working on this, but man.  The house was vacant and I purchased vacant house insurance policy.  However, this isn't one of the perils that are covered.

I called the city utility department to see when the leak started.  They said they sent me a letter the night before because our usage seemed a little high.  They sent the letter after they sent TWO city workers to read the meter manually (which you have to go through the yard and at least drive over the soaking wet street).  So, apparently the water coming out of the house and running through the yard and down the street wasn't enough to tip them off to the problem.  This is because the work order said check the meter reading.  Not sure when it is important enough to shut off the water.  The city claims that they do not call homeowners when they suspect a leak.  However, other citizens there have told me that is not true and that the city called them after filling a pool.  The city advised only the homeowner can shut off their water. 

The realtor who was supposed to be checking on my home said she hadn't been in there in over 10 days.  The people that found it were a serious couple apparently taking a second look so that they could make an offer on the home.  That deal did not work out (obviously).

I have paid about $4,000 so far just to get it dry.  I am estimating the repairs to total about $15-20K total (including the drying). 

I am selling my new van to pay for the repairs.  :(  Very sad.

 However, I have through God's direction decided to allow a family that we have known for several years move into our home and buy it from us.  First off they have endured many trials in life.  They always lend a helping hand to people and even money.  Their own home has not survived well after several floods in their basement, a gas leak, a fire, a poor repair to the fire where the home was re-repaired, and an earthquake that has caused the home to sink in on itself.  So many problems with their home has left it in dis-repair.  They can't sleep in their bedrooms during cold nights because the living area is the only room to stay warm enough (60 degrees brrr).  Also, the home has some safety concerns and after living there and doing numerous repairs for 10 years, they just couldn't get ahead.  They have to walk away from their home and have been looking for a while for another home.  Since they are a family of 8, there are not many options for them.  They do not have good credit and could never qualify for a decent loan.  So, we are working out them moving in to our home, actually scratch that, their new home.  It is a fresh start for all of us. 

Ideally, in our heads we wanted to walk away from that area with a clean slate, however, this challenge is something hard to overcome.  This family has been to this house so many times, with our kids growing up together, that it is definitely going to be an easy transition for them.  They will have no repairs (new roof, new heat and air, new water tank, new walls, new flooring, etc.).  Literally, nothing to worry about for repairs (beyond an occasional toilet backup after kids clog it).  :)  I told my friend that I felt like she has blessed many people in her own way over the years, giving what she could, and I watch her trust God so much and sometimes, it seemed like he didn't come through for her.  Deep down, I feel like God wanted me to do this for them before now, however, I think we all had to be ready for this transition. 

So, we are working on getting our agreement in writing and making this work for our families.  God does turn evil into good.  Just wish he didn't have to use so many floods! :)

CSF Flow Study part 1

We spent President's Day getting our CSF Flow studies done at Dallas Children's Hospital.  The flow studies are actually "videos" and so I can only post pics of them.

Olivia's Brain report:

FINDINGS: (for the Brain)

The sagittal images shows the corpus callosum is normally formed.

The cerebellar tonsils extend 5 mm below the level of the foramen magnum. The CSF flow images show paucity of CSF flow along the dorsal aspect of the foramen magnum along with abnormal neural movement in 4 out of 30 phases of the cardiac cycle. The
dynamic images show downward and anterior movement of the cerebellar tonsils and brainstem. The ventral aspect of the brainstem abuts the dorsal aspect of the dens on the dynamic images.

The optic chiasm, optic tracts, and optic nerves are normal. There is a normal hyperintense neurohypophysis within the sella turcica.

Axial images show normal ventricular size and configuration. There are no pathologic extracerebral fluid collections. There is normal signal intensity in cerebrum, basal ganglia, thalamus, brain stem and cerebellum. There is no restricted diffusion.
There is age-appropriate myelination pattern. There is no cortical dysplasia.

Normal signal voids are seen within the intracranial vascular structures. The paranasal sinuses are clear. The mastoid air cells are clear. The orbital contents are normal.   

IMPRESSION:

Chiari I malformation with abnormal CSF flow dynamics.
Please refer to separate dictated report for details of the syrinx in the upper cervical spine.


Olivia's Spine Report:

FINDINGS: (spine)

Alignment: There is normal alignment of the spine. The vertebral body heights and intervertebral disc spaces are normal.

Marrow: Marrow signal is normal.

Cord: The conus terminates at L1 vertebral body level. There is normal appearance of the filum terminale. There is an expansile multiloculated syrinx within the cervicothoracic region extending from C4-C5 intervertebral disc space level inferiorly to
T2-T3 intervertebral disc space level. This measures 8mm in its maximum diameter. There is an expansile syrinx at T7 level as well as within the conus. There is also a filar cyst, just inferior to the conus.

The cerebellar tonsils are low lying. There is normal signal in the spinal cord.
Soft tissues: The prevertebral and paraspinous soft tissues are normal. The vertebral artery flow voids are patent. There is no hydronephrosis. 

IMPRESSION:
Multilevel expansile syrinx within the spinal cord. This appears stable, in comparison to outside MRI dated 9/13/2013.

So, basically, Olivia's CSF doesn't appear to being flowing right and her ondontoid is touching part of the brainstem.  A friend had me start studying "Deformative Stress".  It appears to be related to EDS somehow.  The other thing is that it appears the syrinx's in Olivia's spinal cord are "stable".  I would have preferred them to say "resolving".  Here is where I take a huge SIGH.

Here are some pics of Olivia's newest MRI.  The first picture shows how the ondontoid appears to be pushing in on the brainstem.  The second pic shows the area of concern circled in purple.

 

 

                                               This picture shows the "Filar Cyst"

                                                   This picture shows the syrinx in the thoracic area that is large (black spot in the lower part of the picture)

                                          This picture shows the other syrinxs that don't look any different. The Chiari is also very apparent. 

 

Now here is the report for Abrianna's Flow study:

 

FINDINGS:

The sagittal images shows the corpus callosum is normally formed.

The cerebellar tonsils extend 5 mm below the level of the foramen magnum. There is paucity of CSF flow along the dorsal aspect of the foramen magnum along with abnormal neural movement in 5 out of the 30 phases of the cardiac cycle. The dynamic images
show downward and anterior movement of the cerebellar tonsils along with minimal movement of the brainstem and no impingement on the subarachnoid spaces by the neural tissue is.

The optic chiasm, optic tracts, and optic nerves are normal. There is a normal hyperintense neurohypophysis within the sella turcica.

Axial images show normal ventricular size and configuration. There are no pathologic extracerebral fluid collections. There is normal signal intensity in cerebrum, basal ganglia, thalamus, brain stem and cerebellum. There is no restricted diffusion.
There is no cortical dysplasia. There is age-appropriate migration pattern.

Normal signal voids are seen within the intracranial vascular structures. The paranasal sinuses are clear. The mastoid air cells are clear. The orbital contents are normal.  

 

IMPRESSION:

Low lying cerebellar tonsils with normal CSF flow dynamics.

 

 

So, reading this report she has "abnormal" flow in at least 5 out of the 30 cycles, but they said she had normal flow.  They also diagnosed this as "low lying cerebellar tonsils" WITH "normal CSF flow dynamics."  So, I called the neurosurgeon's office and spoke with his assistant.  I advised that I do not know how you can have abnormal flow and tonsils beyond the measurement for chiari 1 and be "normal".  I sure hope she is, however, I don't know how Olivia's report gives her the same measurements, 1 less abnormal flow and gets her the Chiari diagnosis with abnormal flow.

 

I just simply feel that it is ridiculous that we pay for an MRI and can't even get a correct report.  So far with MRI's I have learned three things:

 

1.  CLEARLY understand how the anastesiologist will be knocking out your child and discuss prior successful anesthetic events.  For instance, in Oklahoma City and Dallas they use gas to start sedation so they can insert the child's IV without it being so tramatic. 

 

2.  Make sure they put your kid in STRAIGHT in the MRI so that you don't end up with a crazy rabbit trail worried that your child has scoliosis on top of your other worries.

 

3.  Ensure that you get your MRI disks IMMEDIATELY and take your laptop to ensure the images are readable right then and ask for at least 2 copies of the disks.  Also, call and get a copy of your MRI report a few days after your test.   More than likely, you will get the report LONG before your ordering doctor will realize they have one to read.

 

I'll repost a follow-up when we learn if Abri's report will be re-done.

 

 





 

Dallas, TX - finally found our NeuroSurgeon

Well, we have made the decision to go with Dr. Swift at Children's in Dallas, TX. 

Here is his bio:

Dale Swift, M.D.
Dr. Dale Swift was born in Lorain, Ohio. He attended the College of Wooster in Wooster, Ohio, where he graduated Phi Beta Kappa in 1980. He earned his M.D. at Case Western Reserve University School of Medicine in Cleveland and was invited to join Alpha Omega Alpha, the national honor medical society.

Dr. Swift completed his surgical internship and residency in neurological surgery at Columbia Presbyterian Hospital in New York City. He completed a fellowship in pediatric neurosurgery at Children's Hospital of Pittsburgh in 1992.

Since 1992, he has been an attending neurosurgeon at Children's Medical Center Dallas, Medical City Dallas Hospital and Texas Scottish Rite Hospital for Children. He is currently associate clinical professor of neurosurgery at The University of Texas Southwestern Medical School. Dr. Swift is certified by the American Board of Neurological Surgery and the American Board of Pediatric Neurological Surgery.

Dr. Swift's research interests include the pathophysiology of Chiari malformations, neuroendoscopy in children and arachnoid cysts. He participates frequently in international pediatric neurosurgical training, most recently in Bangalore, India, under the auspices of the International Society of Pediatric Neurosurgery.

Here is a publication that he was involved with:

 Suboccipital decompression during posterior cranial vault remodeling for selected cases of Chiari malformations associated with craniosynostosis.
Scott, W. W.,Fearon, J. A.,Swift, D. M.,Sacco, D. J. 

Here is the facebook site.  He is the doctor that is the 2nd one from the left with a big smile :)

 https://www.facebook.com/Neurosurgeonsforchildren

This is a tv episode where Dr. Swift is featured.  

 http://www.youtube.com/watch?v=R3myAl7xsJQ&feature=share 

 http://www.youtube.com/watch?v=NNrB_pwym34&feature=share

This features Dr. Swift and his family 

 http://www.youtube.com/watch?v=2wEfVPjWSUI&feature=share

So, anyways you get the idea.  There is a lot of info that we covered but the plan is:

New MRI's that measure the CSF flow called CINE that take place in Feb for both girls.  

Olivia will have surgery in March 2014.  She will have decompression, a dura patch and something done to her C1 spine, which I'll post more about as I understand that more.  She is also probably going to get cauterization of the cerebellar tonsils to shrink them back up.  

For now, I'm trying to cope and get through the nights.  Days are good.  Begin alone or trying to sleep without worry is almost impossible.  I just pray a lot.  

Hugs for all my fans that are going through this and who pray for us :)

 






  

Cerebellar Tonsillar Ectopia

So just found out another kid has Chiari.  Well, actually they labeled it Cerebellar Tonsillar Ectopia.  I have read it is because it doesn't meet the measurment guidlines for true Chiari.  But, I think that looking at the images

That it looks like anything BUT Chiari!  I mean it has a classic shape.  So, I am just going to be praying that this is going to remain stable.

Why did we get her MRI done? Well, honestly because for years we have known something wasn't "right".  The fine motor issues are getting better, but at 12 you should be able to open packages and door knobs!  Things like handwriting in school, super slow.  Gross motor issues have now got a new name of hypotonia.  So, yea, I think the Chiari has affected her over time for sure.  This is more proof to me that the size of the herniation doesn't matter.  My 4yr old's herniation isn't that bad at all, yet she has significant (actually the docs call it "compelling") syringomyelia. 

The plan for now?  Physical therapy to increase muscle strength, and working out at the gym.  Weight gain is a concern with the limited activity that goes on due to being tired easily. 

The goal is to live a great life, a healthy life, and a full of God life.  :)