Benefit for "Give Reilly a Lift" Cause

We are running a Special Benefit This weekend at http://lilashleyscloset.etsy.com
50% off proceeds from ALL HAIR FLOWERS will be donated to Reilly's family to help raise money for a much needed van for Reilly.
More Info http://reillyslift.blogspot.com/
http://lilashleyscloset.blogspot.com/
Please join us in this cause at Facebook Causes "Give Reilly a Lift" to help this wonderful family.

She's Back:o)

Well, Ashley is just about back to normal. She has learned to slow down and it has helped a lot, but definitely something she had to learn herself. We kept telling her a few days after surgery to slow down when she walked, but she would get excited and try to start running like any normal 2 yr old, only to fall, and say "owies" There have been a LOT of kissing boo-boos these last couple weeks.

When she was in the hospital, it must have taken them a few tries to get a good vein because she has a mark on every foot and hand. If we ask her if she has a boo-boo, she holds both hands up & sometimes her feet to be kissed. Her demands are getting less & less though now that she's on the mend.

One thing that has changed a lot since her surgery is her kyphosis, it seems to have virtually disappeared. We're not sure if it's because she has been walking for a few months now or if the extra room in her spine in helping, we'll see what the surgeon says on her follow up. Speaking of, that's actually next week, should be sooner, but her Dr. is really booked up. We expect that she will have a good report. Here are some pictures of Ashley's day. She was in such a good mood in the morning. Since she could have clear liquids, jello worked, so we had home made jello for breakfast, so it wouldn't be so hard on her during the rest of the day to fast. She fell asleep in the car ride to the hospital as but as soon as she realized where she was that happy mood vanished as she snuggled her Daddy.
We were with her as soon as she started waking up, this is our first glimpse of her in recovery.



So here she is later that evening, I'll need to fix my date/time on my camera because this is one of the cheezy smiles she threw us just a few hours later.

Ashley's decompression surgery went great!

Over all we have to say what a relief and miracle this whole situation was, God is Good!!! The surgery got started almost 2 hours late. They waited to take her blood and cross check it until after she was relaxed in the anesthesia to make it easier on everyone. She did very good, but knew something was up when she opened her eyes when we got there.

We had woke her up earlier than usual in hopes she would sleep through the fasting and not be so hungry which worked well, but when she saw the blue uniforms, her eyes got real big and she wouldn't take her eyes off every nurse that walked in the pre-op room. She had a little oral medication to relax her so when we had to hand her off, there wasn't a lot of fear like she has had before.

They came and updated us 3 times through out her surgery letting us know each step pf the way where it had progressed, then the Dr. came out. Mike asked her if she found what she was expecting to and she said " yes, everything and more, she was a mess"

Instead of a "pinching" that were were previously under the impression of the Dr said that the bone of her foramen magnum was actually "digging" into her spinal cord. She had to remove a section of bone nearly 3" around. We though that was a lot, but she said if she didn't becuase Ashley is still in growing stages, that it would have grown back over the area.

She also had to remove her C1 vertebre (the top bone in the spine under the foramen magnum). She said there was also a part of her skull that had formed a point where the bones meet at the base of her skull that had actually pierced the Dura (the "skin" around the brain between the skull and brain) Thsi was causing pressure on her brain and said there was a lot of bulging in her Sinus (basically the blood supply of the brain) When she shaved that point off the bone it relaxed immediately and she sewed up the hole that the piece of bone had pierced through.

So overall, it was worse than we had expected, but truly a miracle that she had even got to the point she was without any obvious symptoms. Dr Gilmer -Hill did a wonderful job and said that she is much safer now. Although there will always be the risk of damage as anyone with achondroplasia, she is not placing her on any "no can do" lists. She said, ballet, dance, even gymnastics would be ok, but no contact football, which I doubt she'll be doing anyway being that she is quite the little lady:o)

We were right there when she started to wake up and she signed Daddy and said Mama even though she was confused. She moved her arms and legs upon request, started signing more and ate some crackers and juice while enjoying little einstiens. It was amazing how alert she became after just a few hours. She even thew us some big cheesy smiles. I'll have to wait until tomorrow to upload them as Mike has the camera to catch more of her tonight.

Another early day tomorrow thank you so much to everyone for checking in on us all and sending your prayers and thoughts. We will continue to give updates on her progress.

Today's surgery..Thank you all for your prayers

Well, we're off to the hospital soon. Ashley's surgery is scheduled for 2pm today. She had a breakfast of home made apple juice jello. We tried to get her to drink some chicken broth for more nutrients but she wasn't having any of that! We should have opted for the pedialite. Thank you to everyone who has send kind words and prayers our way. We are praying for the best. Mike and I have it seems emotionally had to shut down a bit and cannot think about the what if's. We are trusting in The Lord that He watches over our little Ashley and being thankful for the peace of mind. We are so thankful that Ashley is too young to understand what is going on right now, and we are taking the burden and thoughts from her. I couldn't imagine being a grown adult and knowing this had to be done, thank God for youthful innocence!

Surgery update

ok, well, this has been an eventful last few days. I had called pretty much every ped neuro in my state as well as every specialist in head, neck & neuro on the LPA website to no avail. Nobody wants to give an opinion without seeing her in person and the soonest appointment I could get with anyone was the 29th which is too late. It's really hard to get past the receptionists and referral offices to get to a nurse even, let alone the actual Dr.....very frustrating!

I was tickled pink when I got a call back from Dr. Carson's office yesterday and they referred me to Dr. Jallo who actually does the decompression surgeries. She said they could not help me there but he may be able to. The secretary at Dr. Jallo's office out of John Hopkins said if I forwarded the pics to her she would forward them to him which I did yesterday morning, but have not heard back from yet. But even better news since then.

Thank you so much to the ladies who have taken time to give prayers, advice and help with research. Alex, once again I cannot express my gratitude to you for helping me get in touch with Peggy and Dr.Pauli. I just got off the phone with Peggy again and she and Dr. Pauli looked over Ashley's MRI photos and said they completely concur with my ped neurosurgeon said. Dr. Pauli's response to hearing Ashley's upcoming surgery is on Monday was "good" because they feel the same amount of urgency that our Dr. has. So as much as we don't want to put her through this, it is definitive that it needs to be done as soon as possible.

I asked about another MRI and Peggy said "absolutely not" so no such luck on waiting to see if results show the syrinx is stable or not. It is covering such a large part of her spinal cord that it is imperative it is done now.

The one thing that confuses me is when I had first looked at the MRI's I thought the white spot near her foramen magnum was the syrinx, but our Dr. said it was "high points" distressed" points. Peggy said that white spot is actually her syrinx, so I'm confused about that. I'm going to look again at the photos and see if I may have mis-understood, but either way, surgery is a-go for Monday the 12th, only 6 days.

Poor Ashley has no Idea what is coming:o( She is so happy and lighthearted I just hate to have her go through this, but the risks are far greater if she doesn't. The scary thing is, these tests were done over 4 months ago and they are telling us do it immediately, we're so thankful something hasn't happened already and I'm quite a bit ticked off at the hospital for not sending the results to my Dr. to begin with.

We should have never had to chase down results that were this serious!!! Somebody really dropped the ball on that one, but on the other side, God has his timing for everything and obviously it was meant to be found now. We can at least be thankful we had the MRI done when we did.

From now on, I will not let regular or even specialist Drs tell me NOT to get a test done on Ashley that I feel is needed. This should have been done 2 years ago and my ped-ortho, neurologist and pediatrician all advised us not to because the sedation could be more damaging that what they would find. That will NOT be happening again!

We know that no matter what Ashley is in God's hands and we trust that He will watch over the surgeon's mind and hands and fix our little Ashley. In the mean time, we need to get our heads together to figure out what preparations need to be made.
****** New update*** Our neurosurgeon just called, it's 8pm, that's impressive, and she explained things again and I mentioned our concern about the fact that the MRIs were taken 4 months ago and that it could be worse, and her exact words were" it could not have gotten any worse in this time or she would already be paralized" so that confirms the urgency , but also gives us peace of mind that it has not gotten worse. But yowza, to think her spinal cord is this close to something so detrimental reminds us again, the miracles God truly can work because she is still here and walking around like there is nothing wrong.

These are Ashley's actual MRI photos, they show the areas of concern except for the syrinx, that is kind of hard to spot in comparison to the stenosis and "high point" a.k.a "distress" within the spinal cord. The syrinx is actually a thin skinny line that runs down her spinal cord beginning between C2 & C3.

The area that is having the pressure is right up there under the foramen magnum by C1 vertebre.

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