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Hey gang,

**I started typing this, got to the results of today’s meetings and realized how long this will be.  You will need a few minutes to get to the end, but this one is really worth reading in its entirety.  **

At least we weren’t told, “Well, I’ve never seen this before.”  That is a phrase that has become all to familiar lately, but after the past two days here in NYC, the theme has changed.

Yesterday, Thursday 28th, started very early.  We knew to expect the unexpected with regards to traffic, so we were out by 6:00 am.  The weather was calling for a clear but cold morning.  About the time we hit the George Washington Bridge, it was snowing harder than I have seen in quite a while.  The only reason I point this out first, is because we had told Kelsey we were going to NY to see snow; the weather report was calling for no snow; we got 2-3 inches before she was sedated.  A great way to begin the morning. We also had a very funny thing happen on the way, but you can’t tell Nicole I was the one that told you this.  You know she is tired, her mind has been totally fixed on Kelsey and not much else for a long time. Before we left, she grabbed a Starbucks chilled coffee from the refrigerator, one of those in the glass bottles with the twist top.  We had driven about 3 miles from the house when I heard her take the wrapper off the lid, pop the seal on the lid THEN shake it up!!  Her only words, “Really?”  We are driving our cousins Porsche Cayan, very nice car, and she tries to shake a full chilled coffee with the lid OFF.  I told her that it wasn’t funny then, but in a few days it will be hilarious.  Anyway, moving on………

Our biggest fears have been when we have had to put her under to do anything, tests or surgeries.  The anesthesiologist was aware of this and took great care of her.  We did have to use gas to initially get her down, which was horrifying for her and very traumatic for Nicole.  We (she and I both) keep seeing Kelsey’s face under that huge mask, screaming for us to hold her.  About 40 seconds was all that lasted, but it was long 40 seconds.  Anyway, without doing a total play by play of the entire morning, I will say that she did very well while sedated, her respiration and heart rates stayed up where they needed to be.  The staff was so caring that they rescheduled a couple of people to move Kelsey up to do her CT while she was still out.  In total, she had 4 MRI’s, 2 CT’s and 9 X-rays all from 10 am to 2 pm.  That was a lot for one little person.

This morning was an early start also.  We had to be on Long Island by 9 am, but with traffic who knows, so we are out once again by 6 am.  This time we take Emily, our 12 yr cousin, to be Kelsey’s entertainment for the day.  What a wonderful girl she is, she treats Kelsy just like her own sister.  Our first appointment was with Dr. Bolognese, the surgeon.  He was a very nice man with a warm and welcoming disposition.  We could tell after about 10 minutes of talking with him, that he had studied Kelsey’s file.  The file, by the way, is literally 6″ thick, we saw it on his desk.  Dr. Bolognese’s greatest amount of concern is not centered around the tethered cord, which we had thought and somewhat hoped for.  Rather his concern is with what he is calling a Basilar Impression.  If you remember a few months, maybe longer, ago I mentioned something that Dr. Feldstien had seen, an abnormal bone growth from the anterior, front, side of the spinal column.  He made us aware of this after Kelsey’s second surgery, but did not know if anything needed to be done, just that it is something that doesn’t appear right.  The geneticist from Baltimore made the same comment, that something is there, she is just not sure what it is.  We have been told this several times, but no one has ever offered a solution.  Dr. Bolognese explained it by saying that Kelsey’s spinal cord is angled to sharply towards the front of her skull.  The cord should be straight as it enters the brain, at least in this particular region.  The bone that everyone has called an abnormal growth actually is supposed to be there, it has a name but I can’t remember it.  The problem is that the bone is too large along with the brain stem/spinal column making too sharp of an angle anteriorly.   What she is experiencing is a compression, very similar to what led to the first two surgeries, expect on the front side of the stem versus the back.  We were also told that her case of EDS, which has been confirmed now by two specialist, will exacerbate this problem in two ways.  #1, with the bone rubbing on the stem, scar tissue is being formed and will cause excess pressure on the stem.  #2.  With the removal of a portion of her skull, a portion of C1 and C2 vertebrae, the EDS is causing an exaggerated form of cranial instability.  Wanna very crude definition?  She’s kind a like a bobble head doll, a very smart, kind, funny, energetic bobble head.  There was no way for the first surgeon to know the surgeries would contribute to this.  Those surgeries absolutely had to be done, we had no other choice.  If, maybe, if we had known of the EDS before the first surgery, things may have been different, maybe.  There is a world of uncertainty around this and it was way beyond the point to debate whether or not the right things have been done. Dr. Feldstien knew he was making the right call in ’06 and ’08, we knew he was making the right call and Dr. Bolognese confirmed today that he made the right call.  He was very complimentary of Dr. F, stating that his work was recognizable in the MRI as conservative, with the utmost concern for the child.

Of all the areas of the brain stem to be compressed, her’s is the area that controls the heart rate and the involuntary control of your breathing, the medulla.  At night, if she is positioned in a certain way, she can cause the bone to press into the brain stem and short out her breathing or heart beat. During the day, any sudden shock downward or upward could cause the short circuit.  One way to think of it is like this:   if you have ever tried to reseat a hammer head on a handle, you do not tap the head, you hold the hammer straight up and tap the bottom of the handle on something, causing the head to force itself down on the handle.  Same principal here.  He also explained why she can ride in a car for 20-30 minutes with no problem, but 1 hour or longer really drains her.  The simple repetitive motion of her head bobbing as we drive will cause a repetitive  tapping of the bone into the brain stem.  Sit on the couch, watch a movie and let someone lightly punch you in the stomach for the entire movie, tell me how you feel after 2 hours.  That is what she is feeling, except that light “punching” is effecting her heart and lungs. This is Dr. Bolognese hypothesis and now we are going to prove or disprove to determine our next course of action.

For the next 3-4 months, an arbitrary time frame, Kelsey will  have to wear a jacket for at least 3 hours a day and a collar in the car.  The jacket will be attached to a collar that will slightly lift her head to alleviate the pressure.  In the car, she will wear the collar, very similar to a whiplash collar, to keep her head from bobbing excessively.  We were also told that anytime she is not wearing the jacket/collar, and she comes to us saying, “Listen to my heart” we should lift her head slightly off of her neck to see if that corrects the problem.  The hypothesis is that we will see a great improvement in her breathing, her heart rate stability, her lack of energy during the day, her reduced body temperature and all the other symptoms we have been seeing.

If we find that the collar/jacket is helping, Kelsey will willing accept wearing it, she will be feeling better than she has in a long time.  If the collar/jacket works, then we have proven Dr. B’s hypothesis to be true.  If he is right, then this means that Kelsey will be in store for what will be the most extensive surgery she has has yet.  The brain stem will need to be moved away from the bone causing the problem and lifted out of the way so that no matter her position, no pressure will be placed on it.  When he goes in for the surgery, he will find one of two things.  Either the bone and stem have been fused together or there is ample room to add distance between the two.  I forget what happens if they are found to be fused together, I just know it’s not good, so let’s pray that is not the case.  There are two different ways he can access the site of the brain stem that is being effected.  Either from the back, through the initial surgical site, which would be recommended, or by going in through her mouth, opening the back of the throat and accessing the site from here.  Just thinking about that is hard to fathom.

As for the tethered cord.  The lumbar MRI showed no sign to TC.  While he feels she may not have it at all, the signs point more toward her having occult TC.  Due to the EDS, there could likely be very thin membranes connecting the spinal cord to the tail bone, but these membranes are so thin they are not visible by MRI.  His suggestion is that we wait for the next growth spurt to see if she has any leg and back pain, loses control of her bladder and has any balance problems.  At this point, we will do a urodynamics test to check for voluntary bladder control and an MRI with her face down, which has never been done.  This should really identify if she has a tethered cord.  Nicole and I were both very pleased that Dr. B did not immediately suggest surgery.  We have read claims and heard others say that he is too quick to operate.  I went into the meeting a little defensive, ready to demand a second opinion (from whom I do not know),but I quickly became less defensive as he explained his process.  He has concern for Kels and has no desire to operate unless it is absolutely necessary.

I have left a few details out, but after all this I think you have been given all you can handle.  Additionally, I was up at 3am Wed morning and 5 am the last two, I’m a little beat.  For those of you who called me after 4 pm yesterday, I was asleep and stayed that way until 5 am today, sorry I missed you. I will be back on here to put up a few more details maybe tomorrow, but for now, that is what we’ve learned.

If I had to make a specific prayer request, as I am often asked to do, it would be that we continue to seek God in this.  It is easy for us to say that we have found the right doctor, or the right prognosis, but we are really just wanting this to all be over.  I also asked that you continue to pray for Kelsey’s immediate and deliberate healing.  We have at least 3 months before any decisions will be made and I know miracles can be done in way less time than that.  I also asked that you pray that she accepts the devices we will be putting on her in good way.  She is a tough, independent kid and is not going to like being held back by anything.

Robert, Kerry, Pastor Wesley thanks for the help getting us to New York.  Shannon and Dave, thank you for being the generous and gracious hosts you have always been.  Scott and Ashley, thank you for doing what you did.  Kaki, thanks for taking the time to stay with Callie and Cam.  Everyone that I have not mention, do not think I have forgotten who you are,  I haven’t, you have made a difficult time more manageable.

In Christ,

A.

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