Friday, September 26, 2008


The story of a beautiful little girl named Grace.
As I mentioned in earlier posts, I was concerned for my children after my ACM diagnosis in 2004. I was told that there was only a very slim chance of them also having it. My doctors told me to only have them tested if they showed very apparent signs of Chiari. All 3 kids had vague symptoms over the years, but nothing debilitating. Grace would have an occasional headache and complain of strange pains here and there, but nothing very worrisome.

Grace successfully potty trained at age 2. 6 months later, she began having accidents at night. I assume that this just happens now and then, but it would go away. After it went on for months, I was told that it could be behavioral.
This went on (and off) until she was 4. At age 4 she began getting UTIs and kidney infections. She was hospitalized twice in a short period of time. During her hospitalizations they did ultrasounds, which always came back normal.
A few months later, her infection recurred and she was put on prophylactic anti-biotics for 6 months.

After my decompression surgery in 2005, I would float in and out of Chiari support forums and I'd try to stay updated with the latest findings in the Chiari world.
One day in 2006, I read about a new theory regarding Chiari and Tethered Cord Syndrome. Initially, I blew it off because I was never told I had Tethered Cord.
A short time later, I read up on tethered cord and was astonished by its signs and symptoms. My son Mike and daughter Grace had many overt symptoms of TCS. I went on to read about how it can cause many bladder and kidney issues. The bells in my head starting going off.

After Grace completed her 6 month round of antibiotics, she was scheduled for a repeat ultrasound of the kidneys and bladder. This was done a year after her last.
This time, the ultrasound showed startling results. Over the year, Grace's bladder became enormously dilated. Her bladder was holding over twice (300+ cc) what it should at her age and she didn't even feel it. After she voided, they repeated the ultrasound and she maintained 100+cc residual urine in her bladder- this was even after I went into the bathroom with her and told her to make sure she pee'd every last drop out that she could feel. The ultrasound also showed right-sided hydronephrosis.

So, in the course of a single year, Gracie ended up with a neurogenic, dilated bladder and hydronephrosis. It was at this point I told our pediatrician that she needed to be scanned for Chiari and tethered cord. The doctor looked at me like I was NUTS. After many months of discussing this with family (who'd been through a lot that year thanks to a car accident I had and subsequent cervical fusion surgery)- it was decided that we'd have her scanned after the new year (2008). I will bite my tongue (or I should say tie my fingers) about how I felt about that ridiculous decision to wait- It's moot now, because the wait occurred.

Grace and Mike both underwent complete brain and spinal MRI scans including x-rays under IV sedation (propofol -great stuff!) on 2/6/08. Grace went first and I stayed by her side for the whole thing. Once it was over, she recovered in a small room off to the side. While I sat with her, I was handed the films they just completed and her CD-rom. Being the NEED-TO-KNOW-NOW type of person that I am- I looked right away.
My heart sank to my feet and I'm pretty sure that some invisible man snap-kicked me in the gut. Grace had a very apparent Chiari malformation, which was no surprise to me. What did surprise me?........ The GINORMOUS syringes in her cervical and thoracic cord! The syrinx in her cervical cord was actually bulging it was so wide. I cried my eyes out. The dumbass MRI techs came in to see why I was crying. I showed one of them the syrinx and he just shrugged and said he didn't think it was a big deal. I'm used to Chiari being dismissed by the ignorant, but a professional minimizing the seriousness of THE HOLE in my daughter's spinal cord made me spit fire. How can people be so &^%$ing stupid!? I mean, really!?? Syringomyelia is degenerative, progressive and without halting it, can ultimately paralyze or kill her. If left, will cause permanent spinal cord damage. I really hate stupid people.
I shot the tech a look that could kill and he got the point and backed off quickly.

Michael's MRIs showed no surprises (thank God) as far as I could see. Once they were both fully recovered and awake, we went home and sat in strange silence.
They were both scheduled to go to the Chiari Institute a few days later.

Grace saw Dr.Kula and Dr.Bolognese that day. I can't say that I was surprised by what their findings were, but I was surprised by how quickly surgery was scheduled.
Grace had tethered cord, spina bifida occulta and Ehlers Danlos Syndrome along with her Chiari and Syringomyelia diagnoses. She needed decompression surgery and section filum terminal surgery. There was a few days of discussion about which should happen first. it was decided that she'd have the SFT first, in the hopes that her cerebellar tonsils would ascend once she was no longer tethered.

On February 21, 2008 Grace had her surgery. I cannot tell you how frightened everyone was. She was given Versed (REALLY GOOD STUFF!) before surgery to help ease her anxieties about the IV. I was allowed to go into the O.R. with her, which was comforting for both of us- until-... she entered the second stage of anesthesia. I was warned about it beforehand, but I guess I ignored them because I was not prepared for what happened. Her eyes rolled into the back of her head and her body shook like she was convulsing. 4 of us had to hold her down. I don't think I've ever lived through anything worse than that, particularly since it was then that I was escorted out.

Her surgery was 4 hours long- the longest 4 hours of my life. Dr.Milhorat finally emerged with excellent news. All went perfectly. I was finally able to breathe again.

Mike's surgery was the following day. I'll write about the details of his story on another day. He and Grace shared the same ICU room. their beds were foot to foot.
As crazy as it must sound to have both surgeries done at the same time, it really wasn't. It made it easier on everyone (especially the kids). They supported one another and competed in who would get up first, eat first etc..
The hardest part of it was having one child in ICU while another was being rolled into the O.R.. I'm pretty sure that I aged 10 years on that day.

Grace healed well, but continued to have symptoms. She had headaches, foot drag, continued (almost nightly) incontinence, chronic tummy aches, dysautonomia, sweating and chronic nausea. 2 months post-op she had repeat MRIs. The good news was that her cerebellar tonsils ascended (a lot.. almost 6mm) but she was still blocked because she was originally herniated over 10mm- If you do the math, you'll see that 6mm wouldn't cut it, but it was a nice try. She needed decompression surgery.

We waited until the 6 month post-op mark to do the decompression surgery in hope that her tonsils would continue to ascend. No such luck.
On August 6th Grace had her decompression surgery. The summer leading up to it was awful. It was just weeks worth of torturous anxiety. I wish I didn't know it was coming for all that time. We told her a few days before the surgery at the advise of the wonderful people at Child Life at North Shore University hospital. She took the news well because she felt so awful. She was he bravest little girl I ever saw. The morning of her surgery (at 4:30 am) she woke up with a smile- no tears whatsoever.

Again, she was given Versed (did I mention how awesome that stuff is?). I walked her into the OR again, and thankfully she didn't have the same reaction to the anesthesia she did the last time. I was told the surgery would be 6-8 hours and that we wouldn't be getting any updates until it was over, which is why what happened next scared me to death.
I spent a good hour+ crying my eyes out. And when i say crying, I mean crying from my gut. i was so scared. I finally surrendered and took 2 Xanax that my PCP gave me for this ugly day. it helped and I finally calmed down. About 20 minutes later, i was sitting there and heard my family's name being discussed behind me. I turned around to see a nurse coming toward me with a grim look on her face. My heart almost stopped. She saw my fear and said that everything was okay, then took her hand out from behind her back. She handed me a gallon sized baggie FULL of Gracie's beautiful golden hair. I threw-up in my mouth. That threw me into another hour of tears and fears. The hours went by like days. Her surgery was 6 1/2 hours long but it felt like eternity.

She looked fantastic in PACU. It was amazing how good she looked. She was smiling and happy and had complete pain control. She was quickly moved to PICU where she spent the remainder of the day dozing off. She woke up the next morning and literally hopped out of bed. we were all amazed. She was 18 hours post-op and she was bouncing around like nothing happened. She ate a huge breakfast, took a walk... then reality set in.. The next for days were nothing like the first. She was grumpy, pukey and downright mean. I never saw her like that in her entire life.

She was discharged on day 5 and has recovered beautifully since.
She still suffers from dizziness, nighttime incontinence and nausea, but we're hoping that time will heal those pains.

Grace and Michael were each granted a wish through the Make-A-Wish Foundation. Now that the surgeries are finally over, she can set her sights on something fun.
Something tells me that she'll be looking to meet Cinderella :)

1 comment:

Dee Hall said...

I have been following your blog since you started it. I want you to know that you are a heroic mom! Your children are so lucky to have a Mom that is always fighting for the best care. I also go to TCI. Im going in on Tuesday the 30 for CCF. I have already had ICT,SFT, and ACDF in the last 4 months. I was originally scheduled for PFD w/CCF but my hernation shrunk from 7.0 to 2.2. I do have retroflexed odontoid w/medullar kinking. That is why I still have to have the CCF. Good luck to you and your family. You are in my thoughts and prayers.

Be Well