It's been a long time

In many ways, Quinn is doing very well. She got that newer longer J tube put in and we saw an IMMEDIATE turn around in her pain and discomfort. I am so very happy with that. If she had continued on for much longer in the amount of pain she was in, I'm not sure if I would have continued to let her suffer through feeds, so thank God for the small things! We are so grateful for her wonderful GI doc who, despite the protest from the head of the GI dept., went ahead and placed the new tube. I am forever grateful.

Quinn hasn't really gained any weight, but she hasn't really lost any either. We are still bouncing around at 30-35ml/hr. any more than that seems to cause pain and bloating. I know her motility is incredibly slow, but I have also learned to accept that this is just our normal. And eventually, there will probably be a new normal. Quinn constantly has all sorts of different colors coming out of her tube. If she isn't producing dark green bile, she's usually producing brown or reversing her milky vivonex formula into her stomach. In the past, I would have probably brought her in on numerous occasions due to the symptoms associated with the dark green and reverse motility, however, I know what the alternative is, and TPN is simply not an option for us right now. Plus, I don't want to be hounded over and over again about her need for a permanent line. I'm just not prepared for it and I don't know if I could say no under pressure. So far, both Quinn and I are coping well with our current normal. Since the new tube, her ketones have been nil, her blood sugars are much more stable, she seems slightly more alert and interactive and she just seems overall a little better.

Since I'm able to calm down a little on her GI issues, i can't help but focus on her issues with her right side. Yet again, we continue to see problems with her right sided weakness. I think that because she has been moving around more lately, it appears to be more prominent than it has in months. I've gotten very tired of seeing her fall multiple times, all due to her right leg simply giving out or just general weakness and it being very sluggish. She even gets frustrated. We've decided to entertain the idea of putting the AFO back on her right leg in hopes to both conserve energy and decrease her falls. She falls hard too and sometimes, she can't even break her fall with her hands. sometimes she just splats right on the ground from a standing position... no given warning. So orders have been put through for an evaluation and insurance approval. I am hoping to have the brace by the time school starts.

It's a short update. A lot has been going on, but I'll spare everyone the boring details and just post a ton of pictures! enjoy :)

In June, I met up with a couple of Southern California Mito Mamas and their children. Well, we kinda already knew them... Cheryl i've been friends with for almost 2 years and Kris i met at CHLA when we were there for our mito appointment. It was wonderful to see all our mito kids together. I am really looking forward to another meet up. in the future. 

  Oh yeah, Quinn also lost her first tooth!

 

This is from her somehow falling into the corner of the counter.... thankfully she missed her eye. 

  

This is 4th of July! so the picture right underneath, i just thought it was funny and had to post it. I was trying to take a pic of us three and it didn't work out well, but i got this funny face of Q. 

I also enrolled Quinn in swimming lessons. I can tell it's really hard for her, but she is not a quitter. She's amazing. 

And the one below is the most recent picture of my sweet girl. 

After literally giving up and thinking that she would never learn it, Quinn surprised me and my sister one day but all of a sudden swinging all by herself! I was so proud of her! this was taken yesterday! 

New Tube On Thursday

Hey guys. First off, sorry I haven't been on. Been going through kind of a funk lately and I haven't felt much like blogging. Not to mention that I lost my USB to my phone so i can't really put pictures on the blog and I know how EVERYONE just LOVES to see pictures! And i love showing off my sweet girl too. She's been doing pretty well without her PICC line. We have quite a bit of new interventions, but they are all going quite well. She's on Vivonex 30cal/oz. I tried decreasing the cals to see if i can increase the rate, but every time i increase the rate past 30ml/hr, she gets into even more pain and areas of her intestines start to distend and it's just very obvious that she cannot tolerate a rate past that for more than a 12 hours. I am also putting pedialyte into her feeds to keep her electrolytes up as 30ml/hr is just a very small amount to try and hydrate her. It's working. Still, even at a rate of 30, she tends to accumulate feeds in a portion of her intestines just past the J tube. I believe feeds are accumulating in the Y portion of the surgery and this causes an extreme amount of pain for her. And so on Thursday, she is going to go back in and have a new longer J tube put in in an attempt to bypass this area of the intestines that is obviously troublesome. They are also going to be doing a dye study to see if there's any excess scar tissue or any other reason that the feeds would be accumulating in that area. I am sincerely hoping that it's just bad motility in that part as otherwise, she would likely need additional surgery and that just doesn't go over well for her.

Lately, we've been dealing with lots of tummy pain and thick thick dark green bile. She had a fever of 102 over thew eekend, but today she's back down to 100.8, i'm hoping the fever is gone by tomorrow and I think it will be because she is acting herself again. Aside from the terrible tummy, things are ok. It breaks my heart to see her deal with the pain though as she is in pain far too often from feedings. Remember, this and the bulging and the slow motility was very much the reason why the docs wanted her home on TPN. We will avoid this for as long as possible. She is holding on to a very healthy weight of 40lb. and aside from the pain and periods of very slow motility, she is doing well on this. We are learning to cope with a new normal and I have had to learn to try and deal with it. Quinn too. This very thick dark green bile drainage along with the severe pain is an indication that the motility has slowed significantly latelyl, which is likely due to the fever or the slow motility caused the fever, who knows honestly, but normally, i'd have taken her in at this point. However because we KNOW she has this issue and we KNOW the outcome, I'm keeping her home so we can deal with it here. We have urine dipsticks and we've been testing her ktones and specific gravity to ensure she's adequately hydrated, she's on round the clock pain killers and when i need to, i stop the feeds and run gatorade with protein and sugar and pedialyte added in and this is what's keeping her home and thankfully, it's working :) So please hope that the new tube placement goes well and that they don't find anything that might need "fixing".

Here's a video of the bulges.

We've Been Home

Sorry I haven't updated, but normally, when I don't update, you can assume everything turned out well. And it did. We ended up pulling the line last Saturday and all cultures were negative by Tuesday morning and so we were able to go home. Children with mito are much more at risk for line infections than children without mito and so we opted (against the advise of the GI doc, who made it very clear) not to place another central line. We came home last Tuesday (a week ago). I am so grateful to be home! so is Quinn.

She is on a new medication called Amytriptaline, which was causing some psychotic side effects. the med is used for pain control, but she was suffering from just about every symptom on the side-effects list. We were told that most side effects disappear after one month of usage and she's been on it for one month and we are just now starting to see some of the psychotic symptoms disappear, thankfully! I don't think she's on a very high dose though and she is still in pain, so hopefully when we increase the dose, she won't have the same side effects.

she had a really good week. we have her on the Vivonex formula and we came home at a rate of 30ml/hr and adding 250cc of pedialyte a day into her feed bag to help account for the fluid loss. We have been trying to increase her rate to a rate that can both nourish and hydrate her and so far, we have her at a rate of 37ml/hr during the day and staying at 30ml/hr at night. Unfortunately, yesterday was a very rough day for the tummy and she wash aving a lot of pain and bloating and had blood sugars in the 60s. i had to put her on straight pedialyte with sugar. I have to run this a bit faster to try and avoid the hypoglycemia so i was running it at 40cc/hr and at night, she started to get really distended. Her motility is being really buggy. But i think ti's jsut from all the excitement of being back at home. Plus, we had the twin's birthday party this weekend and she was really active and running around and jumping int he bounce house. And then yesterday was her first day back at school. so she's been pretty active. Just stinks that so much activity is healthy for you, but makes her tummy act really mean. At least it's still moving and I can giveh er a medication to help take the pain away for a little bit.

I have also come to terms with the fact that no matter what I do and how hard I try to avoid it, Quinn is ALWAYS going to have her good days, weeks, and months and she's ALWAYS going to have bad days, weeks and months. her GI motility is just a piece of work and I have learned to accept that she will have a bigger belly than other children, she will have more pain and require more pain meds, she eventually be smaller than other children. I learned to screw the calories and give her a better quality of life. Yes, calories are important, but sometimes, when the pain is too bad and the tummy is too big, I just need to cut off her feeds and give her a break and run pedialyte with sugar instead ot keep her sugars up.I never wanted to do this before because I never wanted her to lose the calories. Unfortunately, she's tolerating such a small rate of formula, I've always felt that pushing for the calories was really important, when in reality, she just needs a boost of hydration and sugar and time to let her belly start working again. We need to avoid TPN as I have been told many, many times before and see in far too much, the side effect of TPN is death.  the line infections that TPN causes are so serious and very life-threatening. Plus, it destroys the liver. The ONLY way that I will EVER agree to send Quinn home on any kind of TPN is if there's nothing moving in her GI system and she is skin and bones and totally malnourished. As long as she has some sort of motility. Just as with her stomach. i know her stomach motility sucks so bad. It's terrible! But my kid, she loves to eat by mouth. Right now, her stomach can still move stuff into her small intestine. Yes, it is a very long process and what she takes in orally is a fraction of what a child her age should be taking in orally to sustain life, but since the stomach still works and I can at least drain some of it if it's not moving fast enough, as long as her stomach will allow it, she will still eat by mouth. it's a small amount and mostly in pureed form, but it's something. Same with ehr J tube. Quinn's small intestines really do suck. you can actually see lumps throughout her small intestine when her motility is being slow of where her motility is more sluggish. It's like a traffic jam in certain areas. It's the strangest thing to actually see her tummy all lumpy, but hey... as long as stuff is still moving, yes slow, but as long as it's still moving, she's going to remain fed through the J tube. it's going to take a lot of work, but I HAVE to do this, for Quinn. TPN is not what I want for her. It's something I will avoid for as long as possible.

Also, while in the hospital Quinn was evaluated for her swallow and she is back to having a terrible tongue thrust and stage delay. So we were referred for speech therapy again for feeding. She's having a hard time swallowing, but her swallow is safe. It's just very difficult for her to get her food down into her stomach. Like she tries very hard looks like she always gets stuff stuck in her throat when eating and she's gagging on it and stuff. Anyway, so that's something new to add to her list of things to do. She's had feeding therapy before, for very much the same reasons when she was a baby, however she hasn't needed it since she was 2.5 years old. It helps. she always pockets food, but she's ALWAYS done that... never something we have been able to break. But also, she pushes forks and spoons under her tongue instead of cupping her tongue like a bowl, I dont' know why she can't do this. She also can't stick her tongue out to lick her upper lip. It's weird. But we will figure out what exactly is going on.

Anyway, here are some pictures.

Ok, so I lost my USB cord to my phone and I had to go through photobucket, but photobucket is no longer easy to work with and it's slow so here's clickable thumbnails. sorry it has to be like that.

How Quinn was when we first got in, not how she was in ER, because somehow, she was fine in the ER lol! She was feeling really icky and tummy was so painful.



Look at this picture, you would never know that there was a screaming 5 year old in the  bed next to her, the florescent lights all on and she had been and was being moved and poked and prodded by nursing students and nurses. She slept through it all. 



This is how her stomach was looking after about 10 hours of 10ml/hr of 1/2 strength vivonex. Her tummy just was not working very well. It was firm and lumpy in the areas that were sluggish.



 This is what her tummy is suppose to look like! This was a good tummy day and we were almost at full strength, i think she was on 20ml/hr or something. She was having a good day.




And this is what her tummy looked like when that nurse fed her that tray of food and tried telling me that she was not at all distended. I snapped a picture right after she walked out of the room. this was when i had a break down moment and was literally a sobbing mess trying to tell this doctor what had just happened.



This is what her hand was looking like when she was getting vancomyacin in her peripheral iv. It actually got worse than this and had to be cut off and ran continuously over the course of 2 hours. 



And here are some pictures of our bounce house fun. I just love the look of complete happiness in her face. 

continuation of last post...

So, about the vancomyacin, they called her reaction 'red man's syndrome' and the way we are getting around this is giving her the doses on a very slow IV drip. And that's definitely working. Just takes two hours to infuse the antibiotics.

and about the PICC line, I am REALLY havin a hard time deciding if a central line is still the best thing for her and as of right now, my decision to place another central line is still no. Yesterday it was a definite no.. and definitely against another PICC line that's for sure, but the GI today asked me to strongly reconsider placing a permanent central line into her chest. Her reason... Quinn has many bouts of pseudo obstructions that would require some form of hydration/nutrition and for the most part, we can handle these at home, but we have been handling them with IV hydration at home. She thinks we will be in the hospital even more if we don't get the central line in. All the GI docs here feel strongly for her need to have a permanent line placed. I emailed Dr. B (mito doc) and basically, he didn't give me much of an answer... just told me that in mito kids, central lines have a very high infection rate in mito kids and that it's best not to place one if it's not absolutely necessary. So, is Quinn having a central line absolutely necessary? What makes it absolutely necessary for her? That is a HUGE debate in my head. I'm leaning a lot towards... no, it's not absolutely necessary. So i need to battle with myself what would keep us in the hospital more... possible line infections, or pseudo episodes. And both the two weigh evenly in my mind. So for right now, I am saying no to a central line.

One other thing that we need to address is Quinn's swallowing problems. She won't drink. She is choking on all her drinks. and last night, when i gave her some chocolate, both my aunt and the nurse got to witness how difficult it was for Quinn to swallow. It has been mentioned to possibly do a swallow study tomorrow to try and see if she has a safe swallow. I think her swallow is safe, but i know she's also having a difficult time swallowing. She will however eat ice chips. and everytime i try to give he a drink of water, she either refuses and says she is going to choke on it, or she drinks it and ends up choking. So... that's a new issue. She has had issues swallowing in the past, but it got better.

so for the line placement, i still think the best thing to do here is to take her home without a central line and keep trying to push better fluid consumption and increase the rate of her feeds to a level that can both nourish her, and hydrate her. It's a fine line, but I do feel that it is our best option as of right now. If she runs into another episode where she absolutely needs hospital, we are going to go to CHLA, where her mito doc is.

Anyway, was jut told that if they don't put a central line in tomorrow, we will be here till middle of next week if we can continue getting clean cultures. *PLEASE CULTURES STAY CLEAN!!!* we've been here for 2 weeks and 2 days. so ready to go home... oh and my mom told me that my dogs ran away. and i really don't know how I am going to be able to pay my rent and car payment this month... this is a major stressor right now ugh!

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!