The next step

It has been a long time since I have updated the blog.......I guess we have been a little busy!  I have been constantly thinking of things to add, but haven't had a chance to actually write them down!

Rachel is doing so well in so many ways and we are so thankful to have her as a part of our family.  I can't imagine not having her with us.

She loves to hang with her brothers! 

 
And she loves loves loves to play in the water.  Every day she asks to go out to play in the pool.

On the more serious side of things, yesterday we had an appointment with Rachel's neurosurgeon, Dr. Mapstone.  This was our first appointment with him after having an MRI of her spine and brain, and a CT of her thoracic spine (all of which was done 3 weeks ago....).  They never called with the results (which they said they would) and the first thing Dr. Mapstone said when we sat down with him was that her situation was too complicated to discuss over the phone (Ahhhhh!).

So, basically he talked about four different problems she has.

The first is that she has enlarged ventricles in her brain (due to spinal fluid not draining properly), but that everything seems stable.  He described it like a clogged bathtub that lets some water out, where the water going in and water going out has reached equilibrium, but the water never drains all the way out.  So, he does not believe we need to address that issue right now.  He basically said that as long as she doesn't show any symptoms of pressure building up (headaches, learning difficulties, etc.), then no intervention is necessary.

Second, she might have a chiari malformation (this is not confirmed, just suspected; he said the images weren't clear enough for a definitive diagnosis; but this could be the reason for the spinal fluid not draining properly).   This is basically a problem with the way the brain is connected to the spinal column; the brain is sitting down in that funnel too low.  Or it is being pulled down.  (When I looked up chiari malformation, there were different types; the doctor did not tell us what type it might be).

The third issue deals with the location of the scar on her back.  (Actually, this is two separate issues; one being bone related, which we learned about a couple months ago from x-rays and meeting with a pediatric orthopedic surgeon, the other being spinal cord related, which we just learned about).  This is in the thoracic (upper back) region.  The MRI showed that, at that location, her spinal cord divides into two, then it comes back together again below it.  (I have no words for this.........other than I believe God has protected her.)  Dr. Mapstone believes that her spinal cord might be tethered or "hung up" with scar tissue or adhesions in this area.  Surgery is required to release the tether.  Without surgery, there is a 50% likelihood that she will suffer permanent nerve damage as she grows (due to the spinal cord stretching rather than moving freely).

The fourth issue is that her spinal cord also seems to be tethered at the lower end of her spine.  This also will require surgery.  The dangers of not addressing this problem are the same as above.

So we have both surgeries planned for Wednesday, July 30.  Dr. Mapstone will release the tethered cord, both locations, in one day.  He will start with releasing the lower portion (which he said is the easier of the two), then as long as everything is going well, release the thoracic portion as well.  (There is a possibility that Problems 1 and 2 might be due to the spinal cord pulling down at the base of her neck, due to the tethering.  If this is the case, then the surgeries might alleviate those problems.)  The combined surgeries will last about 3 hours, and she will be in ICU for 1 day and then probably 3 days in a hospital room.  She will have to stay on her back or stomach for the first 48 hours.  (Then, at home, she is not supposed to be overly active for 4 weeks......hmmmm).

So this feels like a bigger deal than we were hoping for.....although we had discussed all of these possibilities.  It just seemed that because she is doing so well, that I was hoping there would need to be only minimal intervention, if any.  And, it does seem difficult for her to have to endure this surgery, when she is doing so well physically and developmentally; but, Dr. Mapstone said that if we do not do anything, she will progressively get much worse, maybe even being wheelchair bound eventually.

We still don't know exactly what the scar on her back is from.  He said maybe they just removed some cartilage, or fatty tissue, or maybe it was a repaired meningocele (which was the diagnosis given by the orphanage physician), we just don't know.   

We are so thankful that we got her when we did -- a year later and she might have had nerve damage that was permanent (or more damage, we still do not know if she is going to have ongoing deficits).

We are so thankful for her!  She is so endearing and charming.  I am praying the hospital staff gets to see that side of her personality, not just the mad, "stop restraining me" side.

I have also gone from being a little mad at the orphanage because they did not seem to have fed her well enough, to now being very thankful that she is still so small.  Growing is what would make this tethered cord problem get worse, so God was protecting her by keeping her small. 

We are thankful we are at a location where the care she needs is only 1 hour away (and at a hospital campus where I used to work).   The travel part does not seem hard.  And I feel that it is a good children's hospital.

So I am still processing all this and will probably have a few freak-out moments over the next month, but I am mostly thankful.

Sarah