An Unexpected and Unexplainable Surprise

We met with Dr. Roy yesterday, Hannah’s ENT. He is one of those doctors that you just feel a trust with, someone that is going to tell it to you straight, but also willing to actively “ride the ride” with us. Dr. B., our pediatrician, searched him out for us after our first ENT wanted us to proactively trach Hannah back when she was 11 months old because of her laryngomalacia and her prognosis.

We starting seeing Dr. Roy back in September of 2009, and yesterday was the third visit with him.   He noticed pretty significant laryngomalacia and some vocal cord weakness when he did his first laryngoscope (camera down Hannah’s nose while she was awake) as well as moderate reflux.   Back then, he heard the stridor sounds Hannah made when she was upset and the breathing issues when she was upset.  The trach was always in the picture, but we were able to walk away from the two previous visits with “We will review where we are in two months.”

Then we had Hannah’s multiple gagging/breathing episodes, two of which landed us with 911 at the ER.  I thought for sure we were close to having to make the trach decision.

We stopped solids and put Hannah on a liquid diet.  Not a single gagging episode in the 6 weeks that we started that.  Not even a minor one!

So when Dr. Roy came in yesterday, Hannah yelled at him.  He actually loved hearing that because he had never heard a strong vocal from her before.  We explained that she does now yell and has a stronger cry these days.  He also thought she looked really good too, which she does.

He did another laryngoscope today, and Hannah was a trooper.  This was her 5th time having that dang camera put down her nose into her throat.  But the results were GREAT!   He was so pleasantly shocked and surprised that Hannah’s laryngomalacia has significantly IMPROVED since our last visit plus her vocal cords were much more active — the vocal cord weakness has IMPROVED as well!  Her reflux is still there but definitely looks better.

We never expected an improvement in Hannah’s symptoms, especially the ones that are so closely tied to her Gaucher’s disease.  But it happened.  Her laryngomalacia and her vocal cord weakness have definitely IMPROVED.  Say it with me, IMPROVED!!  I love saying that!!!

We all agreed that we would rather have to deal with a gastric tube in feeding Hannah if it comes to that than having to deal with a tracheotomy.  For whatever reason, a G-tube just does not seem anywhere near as drastic as a trach.  Dr. Roy also agreed that a G-tube is much easier and safer to deal with than a trach.  But we are not at a G-tube yet, as long as she keeps her weight and keeps growing, we are going to stick to bottle feeding.

So all of the breathing/gagging/feeding issues we have been dealing with for the past many months have been all related to her hypersensitive defensive gag reflex, a neuromuscular nGD issue.  But no laryngospasms, no breathing issues, nothing else.  We have dodged this bullet for now (I know it may happen in the future, but for now I’m rejoicing!).

IMPROVEMENT!!  We drove him from this appointment, and I had a HUGE smile on my face the whole way home.  It was just so unexpected.  It wasn’t that I was expecting it to be much worse (though I was expecting some worsening), but to have it significantly improve?  WOW!!!