First of all, Quinn is finally gaining weight! She was placed on this super high calorie formula and she is gaining weight on it! She weighed in at 37lb! which is 2 lb more than what she was even last week! Now, before I actually take into account that it was a true weight gain, i must consider that some of this is likely just formula weight as Quinn's stomach is being dumb again today and it's pretty bloated. And 2 lb in one weeks seems incredibly unreal to me. However... we KNOW she is gaining weight as she looks a bit chunkier so in reality, she IS gaining weight off of 30ml/hr of formula for 16 hours a day. Woo hoo!!!
We wanted to address a few things... first of all, our options and Quinn's quality of life. since Quinn is gaining weight on this newer formula, we know now that she is getting adequate nutrition in her stomach and we can feed her at a slow rate, one in which she tolerates, fairly well. However, Quinn's motility all throughout still sucks! We give her so many *stool softeners* she's on fiber supplements as well and high dose of reglan. This gives her some relief, but she still exhibits daily symptoms of stomach upset, such as the "frog" that likes to "hop in my throat" (Quinn's way of saying retching)... pretty much every morning. She still exhibits stomach pains daily, though it is not at all severe 90% of the time, however it is still daily and her stomach does still bloat up, severity depends on the days when her motility is stupid, which is at least a few times a week (one of those days being today and yesterday). He does not feel that she needs a pyloroplasty now as anatomically, he said that she is ok.
Based on the pain, the daily retching, and the wonderful pictures and videos I was able to present to him, and a little of her clinical presentation... AND the fact that we are likely looking at a mitochondrial disease (funny as soon as I mentioned possible mito he said... OH! well that would explain her! lol) he said the Roux En Y J-tube is not at all a bad option for her and one that he would recommend. For one, with neuro-degenerative diseases, it is likely she will need this for a very long time if not the rest of her life, and he'd be doing a muscle biopsy at the same time.
OR, we could do nothing and keep doing what we are doing currently for as long as possible, which is surprisingly working, however she does not like it much because she is basically unable to eat much more than icechips and teensy tinsy bits of puree throughout the day. And this falls into a Quality of Life type of deal. If she gets the Jtube, our goal would be how it was when the GJ tube actually stayed in place and that would be jtube feedings at night and small portions of regular oral food throughout the day. And for some reason, when we had the GJ tube in for a week, she did wonderful on this! she had nice color, and was really active, however we aren't really sure how much real benefit she got from this since it never stayed in for longer than a week.
Here is MY thinking (and my mom's), i don't want to do the j-tube if she is just going to keep having the same issues as she is currently having. For instance, if it is her motility all throughout that she is having a hard time with, how would a J-tube help her?
Now, I read all the time about children with GJ tubes who are able to vent through the ferral bag, yet are simultaneously getting fed through the Jtube. Is there a way to do this with food? Like, we KNOW food... regular people food is the real issue here. It's just so darn hard for her to digest. But liquids are a little easier. If i were to feed her say... a toddler type of meal, one that typically would cause her issues, would it be able to come out through the ferral bag so she can get the satisfaction of actually eating, but not have to deal with the painful side-effects of it?
We also talked about bowel management. They are really in favor of Cinci. I think personally, her bowels are being well managed here. We have a wonderful GI doc and the docs in the office all agreed with me that he is a good GI. They discussed adding Senna into her daily medication regimen, and it's a route we wil llikely go either way.
It came to my attention yesterday that I am likely not giving her enough fluids throughout the day to allow the stool softeners we give her daily to work (since she poops super hard poop that cuts her booty). This totally sucks because that means I am going to have to tweak a few things to see if she can handle the extra volume of fluids and still gain weight. This is something that we can work on while we are waiting for the surgery approval. Plus, we are going to be adding Senna into her daily regimen. If we can find something that works for her and keeps the pain/bloating/retching to a minimum, we will likely hold off on the j-tube surgery for as long as possible. After yesterday, today she even woke with a bloated belly, although no pain yet. She looks pregnant again. <--- we had been able to keep her belly down recently so this belly thing sucks.
So, it's a lot to think about, obviously, if/when they do the surgery, they will be taking a muscle biopsy as well, and they are planning on taking a biopsy on her abdomen if there is enough muscle, since that is where the surgery will be taking place
I got Q a preschool game... excellent for waiting rooms and sick days
This is just from her 30ml/hr of formula on for about 2.5 hours plus maybe about 2 ounces of water (icechips) She gets humongous if I try to feed her.
