So, if you can believe it, we are still at the hospital...

Quinn's line was continuing to grow a bug called micrococcus, which from my research is a very resistent bug. We were treating with rocephin and vancomyacin before we found out what bug it was. Thankfully the micrococcus is actually susceptible to vancomyacin. Unfortunately for us, however, Quinn's cultures remained positive and after 5 - 6 days on the antibiotics, it was decided to pull the line, which was actually a good thing because after the line was pulled, it was noticeably swollen. The dressings had covered up the swelling. she hasn't had a peripheral culture taken since the night she first started with fevers... Monday night... but the line was obviously not clearing from the bug. Clinically, Quinn is doing great! It's hard to be in the hospital when your child is obviously sick, but even harder when your child is feeling well... and Quinn has been feeling excellent the past few days. She hasn't had any fevers, she's tolerating her feeds (aside from the GI doc who thought she should get oral feeds and that mirilax would fix her problems... a whole different story that I just don't feel like getting into... it failed, of course and I am getting really sick and tired of having to prove that Quinn PHYSICALLY can't handle enough oral feeds to actually sustain nutrition.) anyway, aside from that off and on stomach pain, she's doing marvelous and I just want to take her home. She has been getting elavil as a daily med and donnitol as an "as needed" med (in which she has needed at least once or twice a day, but that's ok, better than tpn imo) and donnitol works wonders ! for her. She looks great. But the cultures are still positive for micrococcus.

So, after we pulled the line, they had to place a peripheral IV in, this was today... unfortunately, her peripheral veins are not tolerating the vancomyacin at all. Her IV area is getting swollen, red and there is an area that puffs up like a big HUGE bug bite, but 20 minutes after the vancomyacin is turned off, the area returns to normal. The IV is not infiltrated and we can still get blood return, so we determined that her peripheral IV's are just not liking the Vancomyacin. So this afternoon, she did not get her full dose. we had to turn it off half way into the infusion, and I need to...

I have ot get off now maybe i will finish this later...

basically we are here till at least monday.

Still here!

And we probably will be  for a little while longer. Quinn has been tolerating full strength at 30ml/hr for the past couple days, but now she's back to feeling lots of pain with feeds. We are treating her pain and stopping feeds for an hour when it gets bad then restarting. She also hasn't pooped in a few days...

She's been battling fevers off and on too, up to 103.

Her line is positive for gram negative. They have been increasing the vancomyacin and rocephin dosages every day. just sucks. Aside from Monday, clinically she isn't doing terrible and so far, the infection has not spread into her whole body, so this is good news. Unfortunately, we probably won't be home for at least a few more days. : ( We have been here for just about 2 weeks and this is putting a HUGE crunch on my financial situation. I am going to look into TANF to see if they can help me temporarily as rent is going to be a major blow this month, especially considering that I no longer have any help from the X boyfriend. Everything that is going on right now is just so stressful... the past few months have been difficult, but add a break up into the mix and then a long hospital stay... I'm just not prepared for anymore bad news right now. And i just want to be home. So does Quinn. she is starting to get depressed i think. she's crying for her family every night. She misses them all.

I really hope we are out by Easter. The church is putting on a little show and Quinn was suppose to be in it. she's been practicing. The good thing is, my dad is coming here with the video camera so we can record her doing the show and they are going to play it on the big screen at church so she can "be" there. I think that's the sweetest thing... this is just in case we don't get out by then. But, i was basically told not to expect discharge for a few days at least. ugh!

We WERE gonna go home today.... BUT...

Quinn got a fever...

I went home last night cuz i ran out of clean clothes and so I needed to take care of a few things. She did so well over the weekend and feeds were being tolerated much better so we were gearing up to head home today. So, imagine my surprise this morning when I walked into Quinn's hospital room with her face looking all swollen and a PERIPHERAL IV in her ARM and tpn and fluids running into it and then antibiotics hooked up to her PICC line... Wow... so i got the nurse and found out that she has been having fevers all night (nothing greater than 102) and they won't run tpn or fluids in the PICC line because they are suspecting infection. Lovely. So, she's already lost one Peripheral IV and Quinn has been feeling really icky all day today and she's puked passed the fundo and since they cut off her mirilax yesterday (due to watery stools) she hasn't pooped yet since then. So... we won't be going home today, or probably the next few days since it takes 3 days to grow cultures. My guess is though that this is something else that has been brewing (my hope) because she has had a cough for a week now adn they told me not to worry about it.

So anyway, we are still here, but despite her setback, she is tolerating 25ml/hr at half strength. Not sure what kind of effect this infection will have on her motility though but i'm hoping it won't be bad and shut her gut down again because that would just suck because we have already been here for 10 days working on her feeds and that means, mama doesn't get to pay the bills. Radical. My thought though is... at least this happened before they sent us home instead of after.

Ugh. Feeding Nightmare!

So, we are still in the hospital. Quinn is still not really tolerating her feeds, however she's getting there i think. she's doing a lot better. The type of reaction she was having was very scary, but we have given her some more pain meds and a muscle relaxer or antispasm or maybe it's the same.. and that is like... the magic pill! She's no longer having the same reaction to the feeds, however she is still having a little bit of pain and some very obvious distension. She becomes distended in like a lumpy type fashion... these lumps are the areas that her motility tends to be a bit sluggish. It's rather frustrating... and she is very consistently having distension and pain on the side of her stomach that her J tube is. She is still on 5ml/hr of 1/2 strength Vivoenx and pedialyte and she does well for a number of hours, but then things start accumulating and she starts complaining of pain again.

Yesterday... i think it was... the doc was telling me that we could go home on TPN if the nurses would come train me and i said... ummmm.... no.... i don't think that's a good idea. See, i believe that Quinn CAN come home on feeds, we just need to find the right combination of meds and a tolerable rate for her. Yes, i do know that we aren't near that just yet, but I was being hopeful. After today, I'm less than hopeful since she is still accumulating the feeds, however, we have yet to get the new tube placed and I am really hoping that could be just what she needs. I just want to avoid home TPN for as long as possible. I know she has mitochondrial disease and I understand what this means ith her stomach and where this is going, but my kid still has bowel sounds (not always and sometimes they are far from active) but they are still there most of the time. TPN scares the living crap out of me. and i just don't think I can handle the extra stresses of it. When Monday rolls around, and we haven't gotten farther than where we are right now, i might rethink the home TPN thing, but i'd much rather keep pushing.

Yesterday was a terrible day for me... not for Quinn, she had a good day, but we had an awful nurse who did not listen to my concerns and was just totally clueless on how to properly care for Quinn! I was so aggravated by her. Eventually, she made me so pissed and as I was taking Quinn for a walk, I bumped into Quinn's GI doc, this surprised me and he could see that I was visibly quite upset and so he asked me what as going on... and that's all it took to overflow the dam! I was leaking FAUCETS out of my eyes and was just so stressed out and frustrated and I just couldn't take it anymore. I was literally a sobbing mess, trying to explain to him what had happened! What a great doctor lol! He totally understood! And today we had a discussion about what took place and why I was so upset. And rightfully so! Basically what had happened is : after an already very stressful day with her and her getting mad at me because she needed an order for EVERYTHING! even when all the other nurses were doing the RIGHT things the day before and her basically arguing with me over every little detail of Quinn's care... I told her to stop pushing oral feeds into her stomach (cuz she felt that Quinn should be eating and drinking... yes a kid who has a J tube should be taking stuff by mouth into her stomach... hmmm)... she was pushing more stuff into her stomach and I told her that I thought she should stop pushing stuff into her tummy because she was obviously distended and she looks at me and says... what are you talking about mom, she's not distended and she starts pushing on Quinn's stomach and Quinn's wincing in pain. And she walked out the room. WOW!! HOW COULD YOU POSSIBLY BE THIS BLIND!!! So i grabbed the camera and took pictures of Quinn's stomach (which I will post here when we get home) and my roommates were there through all this too (who were also having issues with this one nurse) and they also couldn't believe she said that. OBVIOUSLY, SHE WAS DISTENDED!!! So anyway, long story short, she felt like i was being ridiculous, that my child (who was seriously doubled over in pain and crying and laying on my lap) was not in any true pain and that's what really set me off, so obviously, i'm pissed and walking out and the first person who happened to ask me what as wrong was her GI doctor and that just threw me over the edge and i couldn't hold it in anymore. I think i cried for a good 30 minutes on the phone with my mom. Thankfully, today has been a MUCH better day. Yes, i did complain to this nurse's boss.

Yesterday also, she had a blood pressure of 60/40 and she needed 2 IV boluses to get her up to her norm. (she tends to run low anything over 80/40 is acceptible for Quinn). She is also having very fluctuating temps requring either heating pads or clothes off... however most have been normal and i'm certain this is due to dysautonomia.

Please pray that we can get Quinn's Vivonex up to a tolerable rate. If this doesn't work out, we will be discussing home TPN on monday as we really just need to get home or Quinn and I won't have a place to live much longer.

Quick Update

Well, feeds are not going as planned. Every single time we try to feed her, she gets very uncomfortable and becomes in a lot of pain. Even on just 5ml/hr she becomes very sick: pale, lethargic, nauseous, her hr climbs, her breathing gets wonky, her o2 sats drop, and it's all written on her eyes. She looks very visibly sick. I am not comfortable wit hthis reaction. After about 30 minutes of her crying and begging me to stop feeding her, we just have to turn the feed off. The reaction she is having to the feeds is very strange to me, especially considering that she's in pain off just a few drips into her stomach. and the symptoms leads me to believe that this might be autonomic in nature.

Quinn's surgeon won't be on till friday and her GI doc wants to talk to him about placing a special jejunostomy tube into her J stoma to sort of bypass some of the intestines that are definitely not working well and are causing a significant amount of pain. the new tube however won't get here until Monday and if she is not tolerating feeds, she will have to go home on tpn. Well, her reaction to being fed is a bit scary considering that it LOOKS like she is having the same symptoms that she had when she got very sick in November. So in other words, i just don't feel comfortable taking her home with those kind of symptoms. Also, we spoke with her GI doc a lot about Quinn's overall care plan and he just really thinks she needs to be seen at CHLA, where her specialists are.. I have more to that story... but in short, i think he's feeling defeated and her issues are no longer GI issues, they are due to her mitochondrial disease and he is unsure of how to treat her properly. He's a great doc and he was totally honest with me and he's doing his very best and i know he is. We really like him a lot. But I think we might need to be transferred out to CHLA so she can be treated by the specialists and if today goes just the same as yesterday, I will ask for the transfer and i don't think i will get any resistance.

It just sucks. Anyway, that was the quick update.

Back in the hospital

Yeah, after our appointment, we basically came home, spent the night and turned around the next morning and took Quinn to Loma Linda. We have been having a problem for the past week (now week and a half) with her stomach pain. It hurts her so bad. She was screaming while pushing meds, having scream fits at night, having scream fits during the day... tummy pain was just bad and had been going on long enough for me to not be ok with. Plus shew as pushing tons of green bile and her home nurse said that she had very little bowel sounds where her J portion was. Plus, it just kept swelling up (like it normally does) but with the pain and everything else, I just felt she needed some more help. So I called up her GI doc and he recommended we go to the ER. We did and her X ray showed some issue in her small intestine, and she had a big ball of stool blocking the exit plus she has very minimal, almost non-existent bowel sounds in her small intestine, right where she has been in serious pain.

So they admitted her on Friday and started a clean out with golytely, which at first failed miserably. She was started on TPN too. Through the night and this morning the pain was unbearable. she was just screaming, the golytely was just sitting there and not doing anything for her except accumulating. She was hard and distended and in tons of pain so we had to cut it off for a few hours. we restarted it later today at a much, much slower rate and she's starting to poop finally and the pain isn't so bad. WHEW! But she doesn't look very happy.

We are watching her closely because the pain she is in right now is very similar to her pain in November right after the surgery she had... though this probably isn't teh same situation, it still has me on edge. She also started up with a significant cough and green boogies today, but no fevers so htat's a good thing. One thing that has been just awesome is her o2 sats have been in the 100% off oxygen! We have been talking about weaning her from her o2. she's even been doing much better in school during the times she is able to come off for activities.

so, we have had a night nurse for the past two nights, it was one of the nurses who cared for Quinn in November, when she was really sick. She was the nurse on the night that Quinn was Hallucinating pretty bad. It was funny cuz she was talking to me about that hospital stay (seriously, all teh normal nurses know Quinn because of that stay, they usually NEVER have a patient crash so fast and so hard like Quinn did, that all the nurses involved that day, i'm sure she left a chunk of an impression) So anyway, she was telling me that she remembers caring for her and she was talking to other nurses about how she just didn't think she was so well that time in November. Like, she could tell that Quinn was very, very sick, much sicker than the stupid resident who was taking care of her made her out to be. (seriously, he made me feel like a looney bin.) Anyway, she said when she came on one day to check on Quinn's progress and she heard about how Quinn got moved to the ICU, she said that she knew it was going to happen. Quinn should have never been moved to the general peds floor, she should have been in ICU or the step down unit after that surgery, or even a few hours after that surgery when it was obvious that she wasn't recovering well. And she was telling me about how that docling was blaming her hallucinations on the pain meds, even though quinn was still in a GREAT DEAL of pain and about how there's just no way she could have gotten taht sick because of anxiety (like what the resident was saying). We all think Quinn taught taht resident a valuable lesson. So, ew talked a lot about that night and I told her what had happened and what I thought of the piece of work docling. She said that she would have NEVER knew that Quinn could look so much better, even though we know she's in pain and in hosptial, that's more like the kids on that floor are suppose to look anyway. She commented on how much better she looks. It's nice that we have had her over and over again because she is very aware of Quinn's baseline and how she is when things aren't going so well. I hope they don't switch her around again.

so anyway, we are just waiting on tomorrow to come so we can discuss a more thorough plan. her normal GI doc will be on service (how lucky is that)!

Our Day At CHLA

We left early... like at 5am. We were attempting to do 1 of 2 things... beat morning traffic and arrive early, or be able to survive morning traffic and arrive just in time. Luckily, we beat the traffic and arrived early, which was ok because there was just a lot of stuff to unpack from the car, plus Q had to go pee and that's just not a quick thing to do with her. We still arrived an hour early. The staff was all just so generous and sweet... definitely a different demeanor than what we normally get at Loma Linda... Q was exhausted! the minute we got into the hospital and she peed, she just conked out! 

 

 

 

Unfortunately, we had to wait a while before we were able to see the Dr. but once we were able to see him, he spent an hour and a half with us! I didn't even have to give him a detailed summary of her life... he had already done his homework and the thing that impressed me the most was how accurate he was. Before we really got into detail about just Quinn, he discussed with me what he thought the problem was. He said that she has dysautonomia, which would explain why she has good and bad days, why her blood sugars can be so up and down so fast and why she had all kinds of just whacky stuffs going on with her body that are just unexplainable. Her ataxia was one major thing... those times when she is just exceptionally ataxic and there's no real reason as to why, it's completely autonomic in nature. He said he rapid heart rate, her blood sugars, her breathing, her stomach motility... basically everything can all be attributed to dysautonomia. As he is sitting here telling me all this, I'm thinking to myself, he's going to tell me, it's not mito. LoL! So, after he goes into great detail explaining how the dysautonomia affects Quinn and what it means for her, he then told me that her dysautonomia is caused by her mitochondrial disease. So yes, she absolutely has mitochondrial disease. 

He then went into great detail about how the mitochondria work in our bodies and how it is not working very well for Quinn. He had answers about what happened to her in November and he told me that it will NOT happen again so long as the doctors follow his protocols on how to treat Quinn's disease. 

 

For all the doctors that have ever told me that Quinn was just a "medical mystery" , Dr. B told me that he treats 20 patients that present EXACTLY like Quinn... PRECISELY! this actually gave me goosebumps! Quinn is not the only one! 

About MNGIE... I just wish San Diego could have gone into greater detail on these results with me. Quinn has only 1 mutated allele... and it was sent off to two different labs to examine the gene... both labs were only able to identify 1 mutated allele. If there were two or if the results differed from each other, he would be more inclined to say this is the exact form of mito that Quinn has, however, it looks like she is just a carrier. However he also told me that that's not to say that this mutation is NOT the one causing her problems, currently it is just not recognized as disease causing, and there are very few patients who even HAVE this mutated gene... so basically, Quinn is DOCUMENTED! He believes the mutated gene is likely a PIECE to the puzzle, but is not THE puzzle.... we want to find THE puzzle. 

 

We then went over her current treatment plan and what we are going to do to tweak it. He said that her Periactin can actually be doubled if that doesn't help her pain (he calls it abdominal headaches), then he wants her to try amyltryptaline or something like that... Also, her carnitine is going to be tripled. He is adding CoQ10 to her meds as well. He feels that since Q is on continuous feeds, cornstarch is actually probably more harmful for her than beneficial, the reason is that it is more stuff to shove into her gut and is known to cause problems in slow GI systems. He recommends discontinuing that treatment. 

As far as the PICC line... unfortunately, he didn't give us much hope that she would be able to get off it anytime soon, if at all. The reason being is that her pseudo episodes seem to be severe enough to warrent either frequent hospitalizations or some form of IV nutrition/hydration. Plus, she is chronically dehydrated already without it, she's on such a low rate of tube feeds, that we would have to push a lot more feeds. Right now, he wants her on D10 IV fluids if she needs to have her feeds turned off for more than 3-4 hours and she needs to be on TPN if she doesn't get feeds for 24 hours or more. The reason for this is because of her autonomic crashes and he doesn't want a repeat of what happened to her in November. He said that Quinn is showing that she needs a constant form of nutrition in her body in order to avoid potentially life-threatening risks. And as scary as that sounds, i COMPLETELY agree with that! However... i'm not ready to admit defeat right now. i still feel that TPN is off the radar. as much as it's being thrown around right now, I do not feel that it is vital to her well-being at this moment in time. I'd rather save her liver and continue with the hydration. I DO however like the idea of TPN if she has to be off tube feeds for more than 24 hours. (but that would be temporary and done in a hospital setting). He is going to send her GI doc a calculation of what ingredients/mixture or whatever to be given in the event she needs TPN. 

 

The plan: there's some new testing that he wants Quinn to have... it's called mito nucleum and mito DX. Neither of which are covered by insurance... one of them costs 3000 dollars, the other costs 16,000! We have decided we are going to have a fundraiser to raise money for the 3000 dollar test. This test might identify the TRUE disease causing gene in Quinn. Dr. B feels that since there is a lack of maternal symptoms, Quinn's issues are likely in the nuclear DNA. Given how affected she is, he strongly feels that we will find the gene, however of course, he wouldn't give me any promises. There's a small chance that if we paid for the testing, CCS might reimburse us, it's a small chance though. I'm going to get started quickly to try and raise money for both the pulse ox and the testing. I think we can do it! 

We are also to get a "quantitative urine organic acids" test done. this is done through her urine and it can only be done when sick. We can do this at home! The only bad thing about this is, Quinn's immune system is phenomenal! She is rarely if ever sick with true illness... it's normally bowel issues, which is truly serious, obviously, but not infectious. So this will be difficult, but i have faith that we can get this done. 

 

Before we left, Quinn had a ToN of vials of blood drawn to test her nutritional status, her coq10 levels, carnitine levels, a CBC and probably some more cuz there was a lot of blood. the coolest thing is, she got to have her blood drawn throug hher PICC line! and boy, they were so sweet! they gave her a book about a boy and his PICC line and they gave her a baby to play with and do an IV on. she was a trooper! i was honestly touched by the kindness we received while we were there from all the staff we encountered. It was amazing. 

Dr. B recommends that we bring Quinn to his hospital in the event that she needs hospitalization. I LOVE her GI doctor though... he is going to write out a protocol letter for Quinn and I am hopeful that it will be enough, however if we run into issues (which i dont think we will because all her doctors seem to be on the same page and we have all been on edge waiting for this appointment), I will go the CHLA. 

 

I am to email him in 1-2 weeks to get the dictation from our appointment (if i don't already receive it by then), I am to email him in 2-3 weeks for the results of her blood tests, and I am to email him in 3 months with an update to see how the new treatments are working for her... and to update on any new developments. 

Oh, one other thing, he gave me a list of meds that she ABSOLUTELY CANNOT HAVE and he wants me to monitor her ketones... I am able to get a prescription for that! ...

 

One thing that he was unfortunately unable to help me with, is getting her probiotics  and pulse ox covered. :( that makes me so sad. 

Oh and he also thinks oxygen can be toxic if given too much for her... without a pulse ox to monitor her regularly and especially at night, i will not be taking her off it, however if we can get a pulse ox, I will be able to monitor her more closely and she will likely be able to have more time off the o2, until then, she is still o2 dependant and he was completely alright with that. He told me to do whatever the pulm doctor recommends. 

 

Wow I think that's enough. I'm pooped! we get to follow up wiht him in 6 months I do believe. 

To make light of a sad situation, I got to meet up with a fellow mito mama. Kris and Kylee go to CHLA often. Kylee is 7 and has a separate J tube just like Quinn. Kylee was back in the hospital again for another bowel clean out. her motility is so poor she's been in the hospital every month to clean out her system. Poor girl. She seemed to be in good spirits though, I hope she gets cleaned out sooner rather than later. she's a cute little girl and her mama seemed so nice too.