Always something...

So, Emily had her gj-tube changed back in March and ever since we have had troubles with her stoma hole (the hole that goes into her belly).  First it was really raw from the leaking and then it started widening.  I was in contact with our wound care nurse the entire time and for once actually feel like I have done what I can (though I am sure there is always something more I could have done right).  We went to see her last week and she and the surgeon blame the lack of healing on Emily's nutrition.  They must think I am purposefully starving her!  It was tremendously frustrating to not have them understand Emily's condition.  So, I emailed our contact at NIH last night and sent pictures this morning.  Thank goodness they are quick to reply.  Here is what NIH had to say," We wanted to both confirm, and reiterate, this isn’t because of a nutrition issue.

 

She [the NIH doctor] had the suggestion, when you go/communicate back with the surgeon, to remind him that Emily has a progeriod syndrome, to look at photos of that to familiarize himself.  She thought using that medical association will key him/her in that there is a disorder of adipose tissue (lack thereof), and will give him/her a patient population point of reference.

 

We are thinking....this is us without of course tremendous G-tube experience, that it may need to be surgically closed, and a new ostomy created.  It seems significant enough of a hole now, and the granulation of the skin around the site is really quite mature.  It is hard for that to close on its own much more.

 

I just wanted to relay Dr. Brown’s suggestion to tie in that her form of lipodystrophy is from the progeriod region of mutations.  As you know, she doesn’t have progeria, but her skin/fat is more similar to that condition, and may help the pediatric surgeon identify more with what she has, and realize it isn’t a lack of nutrition at all."

It is so disheartening to think that we will very possibly have to do a revision on Emily's g-tube (remember we already did one after the initial placement).  That means they will close up the hole surgically and create a new one.  And most likely it won't be just one hole, but two, a separate hole for her stomach and for her intestine.  I very vividly remember the first time 9 years ago it was placed and what trauma that was, and I really really don't want to repeat that.  At the moment we wait to hear from the local wound care nurse as she will confer with the surgeon and hopefully get us in to see someone sooner.  We don't want surgery, but we sure don't want this to become an emergency situation.  And nights are always the darkest, but last night I was beyond frustrated and thought to myself, that this just cannot be the way it ends.  Getting nutrition into her body has got to be easier than this.  I will update when I know more.