Will These Doctors Make Up Their Minds?!?

First and foremost, Quinn is doing AMAZING!!!! Absolutely amazing!!! We have her on the jtube feeds at night and are feeding her teeny tiny portions of soft food throughout the day. very, very small portions. Though I know she wants to eat like we do, she understands this new schedule and is doing remarkably well with it. not to mention that she is not as bloated and so probably knows she is feeling well. She LOOKS like she is feeling well. she isn't as pale and tired all the time. She has so much more energy and she is thinking more clearly and talking a whole lot more. It is truly amazing the difference in how she is now and before. And she is requiring less oxygen! So all this is wonderful news. I, on the other hand just want to get rid of all the tubes. So i'm still anticipating our next GI appointment to talk aboutthis surgery option... or if it IS an option. Though we are trying to keep her weight up, i don't think we are actually increasing it. I think we are sustaining because she still looks super skinny. i can see all her bones.

So today, I got into my mailbox and had a CCS paperwork package. CCS is a secondary insurance for medically fragile children. Previously, she was put on it due to her CSID, which was misdiagnosed. Well, we were still on it for GI issues. Now after San Diego discharged her from their clinic, stating that they couldn't find a mitochondrial disorder, I figured that was the last we would hear from them. WELL>>> this package consisted of paperwork for her NEW diagnosis. And wouldn't you know it? Her diagnosis is Mitochondrial Metabolism Disorder. Now, the thing that confuses the crap out of me is how on earth she carries this diagnosis now and that the San Diego Children's Hospital diagnosed her? he SPECIFICALLY told me to take my daughter home and enjoy her and that they coudln't find a mitochondrial deletion and so she couldn't be diagnosed with it. I'm so lost and confused now! I just don't understand this...

I know that CCS requires evidence of the diagnosis in order for it to be actually diagnosed through CCS. They are actually really strict on that. So, what evidence do THEY have that she has Mito? I was told they had no evidence to confirm the diagnosis.

I'ma call San Diego on monday. but I doubt I'll get any information what so ever. CCS requires me to make follow ups through San Diego for this diangosis, but last time we were there, they told us we didn't have to come back. I seriously don't know what to do now...

"Nothing in this world that is worth having comes easy." I heard this saying today and it hit me kinda hard. I often wonder why the Better Being would give me a child like Quinn, one who loves to eat, but whose stomach simply does not allow it. One who must go through so much pain and suffering. why would He let me watch her endure so much torture? Why would he make me have to walk this road alone for so long with her? "Nothing in this world that is worth having comes easy."

Quinn is tolerating her J-tube feedings much better. She's lost a lot of weight and looks really skinny. I'm trying to bulk her up. Though we are still dealing with distension... yes, STILL, it seems much better if I only give her very teeny tiny portions throughout the day of oral feeds. She's being a trooper about it. Here's some pictures.

We Are Home!

Ok, so basically, Quinn had her j-tube replaced today and she did great. She had a little bit of a rougher time coming out of anesthesia and had a few apneas, but she just needed oxygen till she fully woke up and she was fine for the rest of the time. They started feeding her a few hours after the procedure to see if she would tolerate. She did ok. slight bloat, but she was good and playing around and it was obvious, she didn't need to be there. I THOUGHT it was too soon to send her home if they were looking at seeing how she tolerates. IMO, 3 hours is just not enough time to see if she is tolerating a feeding at 50ML an hour. She did ok, but I swear to God, and i didn't feed her a darn thing! They discharged us, we started driving the 1.5 hours home, she fell asleep in the car and woke up and she was super distended again! WTF!?!  we JUST get discharged and she decides to bloat up AFTER? not during? I promise, I fed her nothing from the time she got discharged to the time she got distended. The only difference is that she is not in pain from this, so i suppose it's a good thing, but doesn't that just piss you off? pisses me off. Anyway, I'm just dealing with it the way we always do and will continue to unless this becomes another emergency. Until then iv'e got a little work to do...

Ok, basically, Quinn's jejunum/duodenum goes from the side of her stomach and goes upward, kinda like a hook and then heads downward. This obviously makes for an interesting placement of the Jtube. It was already dislodged after just 4 days of it being in. Could this be a vicious cycle? It's very likely. The docs said they would probably replace it a couple times before they think of other options for feeding her. So my question was could this anatomy of her stomach be contributing to her severe DGE? The answer was, yes, it more than likely plays a role in this.




Basically, a normal child's stomach will empty when it is about 1/2 way full. Quinn's emties when food hits the top of her stomach, so old food can be sitting there for a while, not to mention that it just simply takes a LONG time to get there. THEN. it has to go UPWARD instead of DOWNWARD so gravity in this situation is NOT her friend. This explains why I was seeing some formula leak back into her stomach. So... why are we not discussing fixing this? that was my main question. And the answer was that I would have a very difficult time finding a surgeon willing to fix this problem. She said that the operation in itself is complicated and has its own set of risks and that it's not exactly a garunteed fix. She feels that Quinn already has slow motility anyway and so this operation has a high liklihood of not benefiting her. As with any operation, i'm sure there are risks, but i disagree. I asked her what kind of complications can come with this? Quinn already has a g/jtube, that is obviously complicated in itself as far as placement goes. Not only that, but she is not without problems with her gut, as we have known for a while now. Her stomach gets so distended that it has caused her ribs to fan upward, permanently, this squishes her lungs which makes her require oxygen. The hypoxemia in itself is suspected to cause her seizures. Who's to say that if we can fix this, all her problems will disappear? Well, I think there are enough problems that are possibly associated with her stomach distension to at least give it a try. What's the worst that will happen? aside from a complication in the OR... I would think that if she makes it through surgery, the only thing that could be bad is if the surgeyr just didnt' work and we are stuck back to where we are now... a slow degeneration of her stomach... back on the g/jtube, back to oxygen. BUT if the suregery DOES work, she could get rid of her gtube and her jtube and her oxygen and even possibly her seizure meds!



I know that Quinn has a lot of problems that point to a mitochondrial disorder. she is highly suspected of having this disease, however, she has had so much bloodwork for mitochondrial DNA and other diseases that resemble mito and all of her bloodwork has come back clean. We have NO answer for why she has seizures, NO answer for why her stomach seems to be degenerating and just NO answers for why she is the way she is. So this leads me to believe that if she has a problem in her stomach, such as this one that could potentially be fixed, we MAY be able to make her better.



So, after discussing this a little further with the doc, she said that it might be a good idea to get a surgeon consult. Which we are planning to do this. We are to see her normal GI doc in a couple weeks then we can discuss this surgeon consult with him. My ENTIRE family wants to fix her and if there is a possibility to do this, we are going to find out!



So now i'm curious, dear friends. what is your opinion on this?
 
Anyway, it's good to be home. Quinn is happy and i'm ready to get some sleep.

Today's Story

Hey guys. Just a small update. Quinn is doing much better. They gave her this stuff called Golytley to help her poop and clean her system out. It's been pumped through her stomach all day today and she did fine with it. They did another KUB to check her bowels again and we were suppose to be able to start up tube feedings if her bowels were better. And of course, she still has to be NPO because now the J-tube is dislodged. So she is suppose to go in to have another procedure tomorrow under General Anesthesia... AGAIN to have it replaced. THEN hopefully we can get her going again. i'm kinda scared because if general slows the motility, then well, we are going to restart this cycle? Not cool. She's been without any kind of food for 4 days now. Poor kiddo. But surprisingly, she's ok with it. i'm concerned though because with her pooping like that and no food on her, she's loosing weight quickly. We gotta get this kiddo eating again.

So the information that I was told today was that Quinn had her procedure for her J-tube, everything went well, but it slowed her motility... a series of unfortunate events... for a child that already has slow motility, this could be bad, but generally it's ok. but THEN she got an infection on top of it, which also messes up the gut. So she was just bad luck all around. This caused her to get a bowel obstruction, which caused her stomach to become distended which squished her lungs and caused her to go into respiratory distress. That's the story. Hopefully, we won't get a repeat of this tomorrow.

Today's update

Quinn had a KUB done (not sure what that is) but it think it was an xray to see if she had an obstruction. it is confirmed, Quinn has a bowel obstruction. She's been off all feeds for hmmm I dunno, about two days now i think. so her belly is getting a rest. They are starting her on this stuff that is suppose to clean out her system. They will be starting it tonight along with some pain meds through her IV. Hopefully that stuff will work immediately so we can get her system cleared. Next, they will do another KUB and make sure that her bowels are cleared. After that, i do believe that we can start her feeds.

Hopefully this will be tomorrow! that would be nice :) PLEASE PLEASE pray that this is what she needed and that her stomach will handle her feedings so we can go home! I don't know what would happen if she doesn't tolerate feedings. we had the dietician come in today and tell me that my daughter is very skinny and off the charts for BMI. honestly, I dont' see why this is a problem because she is growing. She is tall. in like the 90th % for height and she is 35lb. a little below the 50th% for weight. But her height/weight ratio is off the charts low. And they are comparing her growth from all the other times she's been inpatient. They said that it's a significant decrease in BMI. i'm trying to sugar coat it and tell them my family is tall cuz we are and that she hasn't had any food, because she hasn't. but, yeah not sure how that went over. I think as long as she is growing, she's good. either growing tall or wide or both, i'd think growing is growing and that's a good thing.

Honestly, i hate being in the hospital. I just want to go home. I'm tired, I'm all traveled out and I hate the high's and low's the waits and sees. Adn how we NEVER have a definite discharge date. LoL! I was telling Joe today that I wish I could just travel forward and peek into the next few days so i could come back to myself and let myself know what to expect. But, that's impossible. It would be nice though.

anyway, Quinn is doing good thus far. I hope that she's doing good because she's doing good and not because she isn't eating, if you know what I mean.

Quinn is In the Hospital

After Quinn got her J-tube on Wednesday, things have kind of gone downhill from there. She came out of General Anesthesia just fine and we were sent home hours after the procedure. I tried to hook her up to her J-feeds that same night, but she was in a lot of pain. The pain continued throughout the night, i was giving her tylenol round the clock and she was just miserable and crying and crying. i thought for sure something couldnt' be right. So the next day, we took her back in to the doctor and he checked her tube placement, which seemed to be alright. He did say that she had  ALOT of air throughout her system and that she simply needed to fart and poop and she would be ok. He prescribed her tylenol codiene and glycerine to help her and instructed us to give her laxatives. So, we did that. fast forward to Friday and she still had little relief. Even the tylenol Codiene was hardly helpful.

Last night, my dad was watching her for me. I went out with a bunch of my friends. Good thing I was the DD! My dad called me at 5am and said that I needed to meet him at the ER because Quinn was not doing well at all. He said the she was doubled over in severe pain from her stomach, that it was rock hard and that she was not breathing very well at all.

I met him at the ER and Quinn was in respiratory distress!!! It was so scary! Her belly was swollen (though not has bloated as we have previously seen) But it was rock hard and so painful. She was wretching and gagging like crazy. She was in bad shape. The ER wasted no time and she was taken back immediately and started on a ton of steroids, zofran, oxygen and lines were immediately put in. She was put on a cool mist for a few hours and we were able to finally get her breathing under control. Thankfully it's not in her lungs, but all airways. I'm not sure why her airways are causing her problems, but the staff said it was caused from her intubation on Wednesday.

They took some more X-rays and confirmed that she has a lot of air throughout her ENTIRE GI system and that she had some stool in her large intestine. They called the childrens hospital and they transferred her and admitted her. They did a CT scan of her abdomen. The GI doc came to talk to me and said that Quinn's motility is really bad right now. She's hardly moving anything through. It's all just stuck in there. They connected her tube to a suction for a few hours and she has been NPO for almost 24 hours now. She's lost about 1-2 pounds. Thankfully, after some rest, meds, and oxygen, she is doing much better and is completely stable. They were actually talking of moving her to the ICU, but she's doing better now so she's on the main floor. WHEW!

The doc says that the new J-tube doesn't appear to be the problem. It seems to be in place and nothing seems to be perforated. We think this problem was probably inevitable, unfortunately. She has a small fever that comes and goes on its own and her blood works shows increased white blood cell count. The doc says her body is working really hard right now.

for now, as far as i know, the plan is to continue the IV antibiotics for the next couple days, get rid of the infection and try and get her to poop. Once we accomplish that, we can slowly start her J-feeds back up to see if this problem crops up again. The doc said she will likely put her on TPN depending on how the next couple days go and if she can sustain a little bit of weight.

All this just came on so fast! I didn't expect it! She was SO sick Saturday morning. she's doing much better now, but she doesn't even want to get out of bed. She is guarding the crap out of her stomach it's so sore! she really is such a trooper though!

Now I need to get to bed. I've been up for almost 36 hours now i think... I duno maybe only 24, i lost count. I'll update As I can.

GI doctor's update

Hey guys. We saw our GI doctor. Quinn is LONG LONG LONG overdue for a g-tube change. Her extensions are slipping and everytime we remove the extensions, because her belly is so distended usually, we get squirted with her nasty stomach contents. Meds leak out when I push them through.. yeah it's definitely time. But this time, the doctor is going to give her a g/j Tube. The J-tube is a tube that bypasses the stomach and goes directly into her intestines. this will be the way we tube feed her from now on. Since her stomach empties at such a slow rate, and she LOVES to eat by mouth, the formula with some solids throughout the day still causes her some serious distension. So we are going to try this out and hope it works. The only bad thing about this is that she has sleep apnea and is on oxygen, therefore, she has to be put out for this, meaning general anesthesia. This could go bad, but she did great last year for her MRI, so hopefully, we get the same results this time. She is bigger now and her breathing mechanism is more developed so it should be fine.

We also discussed the results of our latest labs... the one that completely ruled out MNGIE. Which lands us back to square one... obviously there is something going on with her, but what, we just don't know. Will we ever find the answer? Yes and Possibly are becoming a small pin light in this dank cave of  the undiagnosed. Quinn is happy. I know it seems strange, but for the most part, she is pretty healthy. We are able to sustain her growth and keep infections at bay easily right now. Her seizure medications have been working and the oxygen is keeping her very stable. She is does not seem to be in constant pain or agony and she adapts very well! So, we aren't really going to pursue anything right now. I don't really want to. I know i should go and see this Dr. Boles guy in CHLA, but I'm tired and exhausted. I get so down in the dumps and discouraged when we see a new specialist and they try to find out what's wrong, only to find everything normal. And on top of that, i just want her to be a kid. i'm tired of her being a pin cushion and if I have the choice (which I do right now), I'd rather spare her from the pain and suffering. So, we are treating the symptoms and continuing on.

The GI doctor said the fact that she has a high lactic acid level leads him to believe that that Quinn is suffering from some kind of mitochondrial dysfunction. She also has classic symptoms. However, there isn't much we can do since all her bloodwork for the mutations have all been normal.

As for me, yes, frustrating knowing that my daughter doesn't seem to be getting better and we won't know if she will or will not.. but i'm happy because my baby is happy. And she is thriving and alive and has the mind of the average 4.5 year old (which by the way seems like a mix of both our future teen years AND our past terrible two's) YIKES! she's so stinkin cute though!

Thank you for reading!