Update: Ashley's MRI & Surgery

I'll just start from the beginning on this, so everything is understood, this is the situation with Ashley

As some of you know, she had an MRI of her head and neck done just as a baseline so if she developed future problems that we would know where she was at this age in comparison. I finally (after 3 months and several phone calls to the pediatric orthopedic Dr's office finally got a response about her results. Since they had not contacted us we had figured there was nothing to worry about, but we wanted the results anyway.
The doctor finally called me back a few weeks ago and hit us with the news that Ashley had "Foramen Magnum Stenosis" and a "Syrinx" also known as syringomyelia and she needed to go see a neurosurgeon to determine how bad it is and what our next move should be.

So we got the earliest appointment we could with the neurosurgeon and we went yesterday to see what her opinion on everything is. We had done a lot of research on this prior to the appt and we were expecting the Dr. to tell us that it was only a minor issue and it was something we could monitor until symptoms started to show.

To explain what these are in the simplest terms is: The foramen magnum is the circular shaped bone at the base of the skull that connects all your top vertebre (spine) to your skull. Running through that hole is your spinal cord. your spinal cord connects all the way to your brain and down to your lower back and controls movement.

Around your spinal cord there is cerebral spinal fluid. This fluid runs all around your spinal cord and up around the brain and back down and flows constantly and cushions your spinal cord and brain . Kind of like the way your blood runs through your heart as our life force, the cerebral spinal fluid is the same for your spinal cord.

The problem that a lot of achondroplasics (people with Ashley's form of dwarfism) have is that the hole (foramen magnum) is not circular shaped, it is more like an old fashioned key hole and can be shaped differently because of the way their bones grow differently than ours.

The problem with having that hole smaller is that it can cause not enough room for the spinal cord essentially "pinching" it and of course leaves little room for the cerebral spinal fluid to flow. This is the problem Ashley has. This normally does not cause problems until achondroplastics are into their 40's and 50's and can cause pain and numbing in extremities because of nerve damage. Ashley is in the 1 out of 1000 people who have developed this at 2 1/2 years old.

She is too young to tell us if anything hurts and if it does, since she was born with it she wouldn't know any better because it would be normal for her. Our only way of really knowing if there is a problem is by the MRI pictures.

Now...... The "Syrinx" to give the exact definition is: A syrinx is a fluid-filled cavity within the spinal cord (syringomyelia) or brain stem (syringobulbia). Predisposing factors include craniocervical junction abnormalities, (foramen magnum stenosis in Ashley's case) ,spinal cord trauma, and spinal cord tumors. Treatment includes correction of the cause and surgical procedures to drain the syrinx or otherwise open CSF flow.

So that last bit was really medical words, so in our terms a "syrinx" is essentially a cyst inside the spinal cord. It is formed when the cerebral spinal fluid is restricted and cannot flow properly and has to end up somewhere, and in Ashley's case, it is starting to build up within her spinal cord.

The problem with a syrinx is that over time the fluid build up causes damage to the spinal cord because there is only so much space that it has and each part of it serves a purpose. So the fluid being in there causes pressure and essentially damages the spinal cord which then leads to poor function and numbness etc......

So, now that we have out, here is Ashley's issue. They cannot just go in a drain the syrinx with a needle or shunt because it is not the main problem. The problem is the tightening around her spinal cord that is causing it to begin with, therefore the tightening around her spinal cord (foramen magnum stenosis) is the problem that needs to be addressed.

Ashley not only has the tightening, but it is so bad that is has causing what they call "high points" or "distress" on her spinal cord at the base of her skull before the issue of the syrinx. If you look at the MRI, you can actually see white spots within her spinal cord that cover almost 70% across in one spot that is actual tissue damage. There is a little bit of spinal fluid moving on one side, but at the back there is very little and her spinal cord is literally being pinched by the bone.

The doctor told us the prognosis for Ashley is definitely having the decompression surgery done and that Ashley's condition is in her words "serious, it needs to be done ASAP, she would not even wait until November because with as mobile as Ashley is if she were to be in a car accident or even fall and hit her head hard enough it could paralize her"

The procedure for relieving the pressure off the spinal cord is called "foramen magnum decompression surgery"
Basically the long and short of it is they go in with whatever tools they use (that I don't even want to imagine) and cut the bone (foramen magnum) around her spinal cord so it has room and is no longer being pinched. It is essentially neurosurgery.

So......our baseline MRI just to check things out turns to neurosurgery consultation to indefinite surgery for Ashley, and she has an opening in her schedule for Monday. Mike and I were very much taken back about all this because we were expecting her to tell us, it's ok, lets just monitor it, instead we get, this news and the thought of neurosurgery in a few days.

I called the surgery appointment scheduler and even though the Dr said she could do it on the 5th or 12th, the surgery scheduler said the Dr doesn't even do surgery on those days, so apparently she moved whatever else she normally does to fit Ashley in so soon. Well, she just called this morning and said she could not get a time slot for the Dr, to use the hospital on Monday and it will have to wait until the 12th of October. So that gives us a couple more weeks to do more research.

We are very pulled between jumping head first into this, getting more info, a second opinion, a second MRI to see if God has healed her, and really seeing if this is really necessary. From what the specialist said it is something that is inevitable and her question to us was, "how long do you want to wait, and if you do, what will the damage be" The spinal cord does not fix itself, damage is damage, so how far do we want to research this and drag it our before we make a decision.

She is scheduled for surgery on the 12th of October. In the mean time, I am going to call every neurosurgeon I can find and try to get another opinion before then and talk to as many pediatric neurosurgeons on the Little People of America website I can find to get their opinion.

Well, we did even more research last night and we have prayed about it and we understand the urgency that the doctor is telling us, but there is also information stating that sometimes these can spontaneously start working great themselves and that some doctors can be too quick to do the surgery which itself is not without different complications. There is s lot of info I am finding on the Little People of America site and have tons more to do as well as talk to different doctors.

We need God's wisdom at this point and want to do whatever is in His will for us, because we know whatever happens it is in HIS PLAN, and timing, not ours. We are leaving it is His hands and we will be doing as much research as we can before the 12th and keep everyone updated. We would prefer to have another MRI, but then we are torn between putting her under sedation for the MRI to get the same results, worse, more urgency or a healing. We know that He can heal her if it His Will, but we also know that sometimes His Will is not ours. We just are praying to keep stable heads and make the right choice for her.

In case you're wondering who this doctor is, she is the Chief Pediatric Neurosurgeon at Beaumont, graduated from Harvard has several years at both U of M and Univ of California specializing in neurosurgery and is board certified and has a ton of accredidations, she is one of the top in her field and knows her stuff, but we also know God heals and at this point we only want His Will to be done for His purpose. I am thankful they could not get us into the hospital on Monday, that is our first sign, let Him work in His time. We will continue to do updates to keep everyone in the loop