I'm just a little bit...

FRUSTRATED. 

It feels as if we are at a stand still... and all I want is just a little relief for my baby. 

I'll start by very briefly explaining the new drug I mentioned in my last post.  It's called G-CSF (that's actually the classification).  It is used to stimulate the production of granulocytes (a type of white blood cell) in patients undergoing a certain therapy that will lower your white blood cell counts.  This medication is used to prevent infection and fevers caused by chemotherapy.  I know of it as being used before the Bone Marrow Transplants in EB kids.  If I understand correctly, it's used about 5 days prior to starting the transplant.  Some EB patients that took this prior to their transplants started feeling better within the next day- their mouth healing, skin clearing up, etc.  But then the transplant began after the 5 days...

Now there is no data on this drug being used just to help stimulate "healing" in the mouth, esophagus, or skin.  It's never been used in this way before.  Now most of you know that some of the children that made it through the transplants are doing okay, but some of them are worse than before the transplants.  Most every one is different.  One of the transplant patients started the G-CSF strictly to "see what it would do," and had some good results within a few days, but then had to stop it due to a secondary infection.  

And if you know me... you know that I want this drug like yesterday.  I mean, what do we have to lose, right?  My baby's time clock is ticking...

So another super EB mommy got me in touch with this doctor and I practically begged him to let us try it on Tripp.  He was so nice.  I felt like he really cared and he was really willing to help us.  The problem is that this is brand new and no one knows short term or long term side effects of using this drug in this way.  AND he doesn't think the drug will do anything as long as Tripp is on steroids (which he's been on for about 2 years now).  And the steroids are difficult to taper off when you've been on them for so long.  But he told us that if I could do a viral swab in his mouth (and make sure the herpes virus wasn't lingering around in there again), try and taper his steroids down to about 2 mls, and draw some baseline labs on him... then we could talk about it.  So between me, Dr. Defusco, and our SUPER great and wonderful home health nurse, Kati Corso- we got the swab done and the labs drawn.  And I had already begun tapering his steroids.  

Viral swabs were negative (I guess that's one good thing), but his breathing starting getting wheezing when I started tapering his steroids, and his blood work was all over the place.  Electrolytes were up and down and crazy numbers... and he was more anemic than any blood work has shown before.  Go figure.  My excitement turned to disappointment really fast.  

Dr. D thinks I may have tapered his steroids too fast.  Which I probably did in the excitement of actually being able to try a drug that could make him feel a little better.  But doing that is bad- and it was a wake up call for both of us when we saw his labs.  I didn't think I tapered too fast.  I went down about a ml a day and then stayed at 3 mls for 2 days and then went to 2 mls.  Which is something that I actually do about once every 2 months (but I usually stay at around 3 mls).  I don't know... we aren't actually sure what made his blood work so out of whack, but we are going to try and draw new labs tomorrow to make sure these were accurate.  Which means more poking on sweet Tripp... ugh. 

SO... this of course means that this drug gets put on hold until we can get him stable enough to be able to try it (I'm assuming).  It's just frustrating.  This drug is no where near a "cure," I'm very aware... but if it can just help even his mouth to feel a little bit better (his mouth is just horrible right now)... I'm going to fight for it.  Like an angry momma tiger.  Grrr...

And what's my next frustration, you might ask? :)

Bath time.

Like I said in my previous post, I've been having to heavily sedate my little bubba at bath time.  I hate this.  The sedative I use drops his blood pressure and he trembles and his little teeth chatter.  And as yucky as I bet it makes him feel, it usually only lasts for about half of the dressing change.  Usually either the first half or the last half... never the whole time.  And then lately, after he's finished he's been wanting to play because I guess he feels better afterwards... but he's to sedated to really even stand up.  And that just makes me feel horrible because usually after bath was his most favorite time to play because he felt so good.

SO.

We tried bath tonight with no sedative.  Just the pain medicine and the anxiety medicine (not for mommy, for Tripp:).  He tried to flip over about 3 times when I first laid him down, but then we were able to talk him out of it and calm him down enough to let us unbandage him.  And bath was actually okay.  It was almost back to how it was before he was really sick.  He still cried, of course, but not to the point where it was unbearable.  

And then, of course, when we finished- he felt horrible and had no interest in playing.  

Really? 

I just don't know what the answer is anymore.  Everything is trial and error, but it's like nothing ever works the same way twice.  His poor little body must be so messed up from so many different medicines.  It's probably just plain confused.  I wish we could do to Tripp what my grandpa used to say about himself... "Why don't y'all just take me off all this medicine and see what happens?

That's not a bad idea, Paw Paw.... I might try it. 

On a brighter note, we did receive Tripp's song and an autographed picture from the one and only Elmo:)

I just made a simple little iMovie with two pictures because that's the only way I knew how to share the audio file that was sent.  

He sent us a whole CD of songs, but this version of "Tripp's world" was the only one I was told I could share! 

And what Elmo says, goes!:)

Tripp loves hearing his name in the song. 

Much love to you, Elmo... much love. 

I'll keep you guys posted on what happens with the G-CSF.  I'm hopeful.  I'm just not getting too excited about it because I know it's only a temporary fix if (any fix at all).  But like I said, if it can give this baby just a little bit of relief, it's worth it.  

I'm so emotionally exhausted seeing him in pain... being the one causing him the pain by doing what I have to do to keep him as clean and healthy as possible.  It's heart wrenching.  It really is. 

But moments like this are what keep me going...

This is occasionally what happens when I say, "Smile for Nanny!"

I love this little ham.

Love,