A year ago yesterday, we sat in Dr. Keating's office and listened as our world came to a screeching halt. A year later, that weekend, that day, that moment, is still fresh and reading the words that I wrote a year ago still makes me teary eyed. I doubt there will ever come a day when I can read that entry or any of the entries leading up to and after surgery without tears. But that's O.K. Remembering is necessary - not easy but necessary.
So much has changed in a year and yet so much has remained the same. As we prepare for our next visit with Dr. Keating and start the MRI process again, reading my entry from May 1, 2011, reminds me that we can handle the really hard things in life as a family. We can walk through the bad together and get to the good.
Victoria and I marked the one year anniversay of being told that she needed brain surgery by participating in the Walk for Babies. When I signed up for the walk months and months and months ago, I didn't make the connection to the dates.
Not once during or after the walk did Victoria complain about leg pain. I was so shocked that she walked three miles without mentioning leg pain that I asked her if her legs were bothering her last night or had bothered her during the day. The answer was a resounding no. A year ago leg pain was a constant for her. There were no activities that she could do without mentioning the pain in her legs. She was even skipping dance recitals due to leg pain. To see her do the Walk for Babies this year without pain is amazing.
Below is blog post that I wrote on May 1, 2011, telling the world that our beautiful little girl was going to need brain surgery.
I don't know how to start this post - but then again I don't know to handle the situation we are in. With Chiari I Malformation and cranial cervical instability and herniated disks - brain and or back surgery has been a possibility looming on our event horizon for three or four years now. We found out on Friday that living with a possibility is completely different than living with a definite reality.
What started out as a yearly trip to Annapolis to see Dr. Keating turned into one of those moments in time when you face a life defining moment. Dr. Keating told us that Victoria was going to be last because we had a great deal to discuss. I didn't think too much about the statement. Victoria always goes last because she has a Chiari I malformation and we always have a bit more to discuss with her. So we got through the boys check-ups without any major issues (I will update on Alex and Nick later). Throughout the boys exams, Dr. Keating was asking us about various issues caused by Chiari and Victoria. I have been seeing a bit more swallowing issues with her, but we have been dealing with swallowing issues with Nick for so many years, that Victoria having difficulty swallowing just didn't send up red flags. All I can say is hindsight is 50/50.
After the boys were done, we went into Dr. Keating's office to view Victoria's scans. He pointed out two major issues and I could feel my heart stopping with each word. We have been doing this for years and even though the kids haven't been candidates for surgery I have kept up with the problems and issues caused by Chiari, syringomyelia, tethered cord etc., etc., etc. So when Dr. Keating pointed out the syrinx on Victoria's MRI my world stopped spinning.
A syrinx is a fluid filled sac inside the spinal cord. Victoria only agreed to participate in a cervical spine MRI and a brain MRI prior to our appointment (and that cost me 6 new Barbie dolls!). We know that she has a syrink that begins at C-6 (the 6th cervical vertebrae) and continues to T-2 (the second thoracic vertebrae). There are 7 cervical vertebra so this isn't a huge syrinx. However, we did see portions of either a second syrinx or a continuation of this one in the thoracic area. We will know more once we get her thoracic and lumbar MRIs done. The syrinx she has is taking up 50% of her spinal column. This means there is pressure being placed on her spine and the longer the pressure continues to be placed on the spine the more neurological damage is done.
But her syrinx isn't even the worst news - it gets better. Victoria has a 20 mm herniation of her cerebellar tonsils. This means her cerebellar tonsils have descended out of her skull and now reside in her spinal column. We knew this last year. What we found out this year was that her brain stem has also descended out of the skull into her spinal column and appears to be a bit stressed from the pressure and location. This is why Dr. Keating was asking if we had noticed any changes in her breathing when she sleeps (i.e. any periods of apnea) and if she was having increased swallowing issues (major red flag is an inability to swallow fluids - while she has had an increase in swallowing issues, including swallowing fluids it isn't an extremely frequent occurrence and she can still swallow liquids).
The tears started for me with the syrinx and continued almost through the remainder of the appointment. These weren't the racking sobs that came later these were just the tears that I couldn't stop no matter how hard I tried. Thankfully, Steven was able to ask questions. I once had a friend tell me that no matter how upset you are when you are in a situation like that and your child is involved, you remember every little thing. It took about 2 hours, but I did recall the majority of the appointment in ridiculous detail. Like I said the world literally stopped spinning for me and I'm sure for Steven as well. We have more questions that we will be sending to him via email and we will be having follow ups sooner rather than later.
So, Dr. Keating wants to do surgery this summer. We will be scheduling a date for surgery once I work through everything that I need to with my office. Right now July is looking like the most promising month to schedule surgery. Part of me is terrified to wait that long with the brain stem issues, so we are also looking at a possible June date. As silly as it sounds, I want her to be able to participate in her Kindergarten graduation.
Dr. Keating wants to go with the most conservative and least invasive approach first. This means he won't be cutting the dura (which is the covering of the brain). He will go in remove a portion of her skull and C-1 vertebrae to give her brain more room. What he is hoping will happen is that her cerebellar tonsils and her brain stem will return to where they belong. If they don't, he will then shave the dura to provide even more room. One major complication is that she has Ehlers Danlos Syndrome and she is more prone to a Cerebral Spinal Fluid leak if the dura is cut or possibly even shaved. The Ehlers Danlos Syndrome throws in quite a few unknowns.
The other hard pill to swallow is that there is no cure for Chiari Malformation and Syringomyelia. Believe it or not brain surgery is a management tool for Chiari Malformation. We are hoping that she only needs to be decompressed once, that her syrinx will shrink and never return and that she will become asymptomatic for the rest of her life. As a friend said when I told her about Victoria's surgery - pray for a miracle. I will pray for a miracle and for safe passage for Victoria through this chapter of her young life.
Right now we are working through what needs to be done prior to surgery. Some of the routine doctors visits that we had scheduled will now become a bit more complicated. We are working through schedules and planning how we are going to be handling things including how to tell Victoria. No, we have not told her yet. If anyone has any advice on how to do this please feel free to share. I don't know where or how to begin. Victoria was indignant when we told her that she needed an MRI because her brain was in the wrong place. Her response was "Mommy, my brain is perfect!" Yes, Victoria it is perfect now, it is up to us to get it back to it's perfect location.
I don't have the luxury of forgetting how things were before surgery. Chiari has no cure. Brain surgery is merely is treatment option to alleviate symptoms. We know this - it is a fact of life that will be with us forever or at least until a cure is found for Chiari. Looking back helps me to stay focused. It helps me to see even on the bad days that Victoria has come a long way from just a year ago. Looking back helps me to move forward. Life has no guarantees but I life does provide victories and moments to celebrate.
As I look back on where we were a year ago and where we are now, I am amazed and humbled. I know that my daughter has an inner strength that will get her through her life. I know that life is not going to be easy for any of our kids but together we will get through the hard parts and we will celebrate the victories and the moments of joy. I know that Chiari and EDS will not define who our children are ... Alex, Nick and Victoria will define who they are and what they do with their lives ... Chiari and EDS will present issues and obstacles but they won't define our children they will simply make them better at being who they are.
Congratulations Victoria! I know we will do the Walk for Babies again next year and have even more to celebrate.