Got out on Monday

We were able to break out on Monday. I'll always go to Loma Linda. I think the residents and doctors communicate very well (minus that stay in November, but I partially feel that that stay is the reason we have 0 problems anymore). Our regular GI was able to give the attending her history and that despite how she looks, she has a lot going on in the inside. I love our GI doctor. He's never short of amazing.

So, we spent all week decompressing her. She was getting long-cath enemas every 12 hours, which were doing absolutely nothing for her. She wasn't even pushing out the flush that was flushing her colon. Everything was just staying up in her butt. She literally had no movement in her colon at all. So after 2 days of this complete failure, we had to stop doing the enemas. After a few days of total gut rest, we tried to start some oral food of clear liquid and clamp her drains. As soon as we started a little bit of oral food, Quinn's stomach started to get very distended again. The GI doctor saw her the morning before we started oral liquids and the evening after our trial and when he saw her he was like, "she wasn't this distended this morning!". Nope, she wasn't. So, he got a syringe and an extension and started venting her belly and A LOT came out. So, his conclusion: He feels that a lot of the air that gets trapped in her small and large bowel are not just from pseudo episodes, but also from her stomach not working very much at all. It accumulates food and air. Basically, she has a pretty nasty case of gastroparesis. So basically, she is only allowed to have a liquid diet by mouth, and only for her own pleasure. We have to keep her drain opened 24/7. So when she eats her clear liquids, it just goes right into the drainage bag. First, we did a trial to be sure that this is what she needed. Well, it worked! Quinn has had the flattest belly for the longest period of time that i have ever seen. We were also able to increase her rate in her J tube from 30 to 40 and this gives her a good level of hydration and nutrition. we spent a week in.

During that week, Quinn was having episodes... I'd call them mini-episodes of just a hiccup of gut shut downs. She would drain a lot of green bile. Her belly would randomly get distended and then go down again and she also wasn't pooping. Speaking of not pooping, she still hasn't since we left hte hospital. Unfortunately, if we were to keep Quinn in the hospital for all of her mini pseudo obstruction episodes, we would never leave. One of the things the doctors kept telling me when I'd express my concerns for the minor things (stuff we deal with at home all the time), they all just told me that unfortunately, Quinn has a classic case of pseudo obstruction. There is no other treatment for it. Sometimes she works, and sometimes she doesn't. And that has been the case in the course of her hospital stay as well. We need to just deal with the minor and keep bringing her in for the major. They also said that I was doing all the right things I needed to be doing at home. Made me feel better.


It's HARD! and it's WORK to keep this kid hooked up to all this crap, monitor ketones every night, monitor fluid output vs input. Blood sugars, to keep all her lines from getting tangled all the time. Despite all the attachments that come with her, the most difficult thing that both her and I have had to deal with is the fact that she can no longer eat. It's hard to eat in front of her! she literally eye balls my food. and she gets very sad. I think she's starting to understand. I know she feels better and i know she knows she feels better, but its' so hard. She LOVES to eat. I enjoyed giving her even just tiny amounts of food by mouth to satisfy her. Now it's just liquids and lollipops. Poor kid. I feel so bad for her :( I don't understand why she has to go through this. and I don't understand why my kid can't just be one of those who hates to eat. This would be so much easier if she did. I never quite understood how a kid can have such a terrible case of gastroparesis, yet still be so incredibly hungry all the time. That stumps the doctors too, and unfortunately, there are no meds that she can take to suppress appetite.

Anyway, she has a cardiology appointment coming up. For a second opinion. I feel she needs to be monitored in cardio regularly. I think that her heart just has a huge demand. Every time we run into trouble, her heart rate is usually the first sign that we have. Plus, she complains of chest pains accompanied with a high heart rate a lot! most of the time, it's because her blood sugars are low, but sometimes, it's just a random occurrence, like this morning. I'm not sure if they can even do anything about it, but I'd like to double check... again to ensure that her heart is healthy because I just have a strong suspicion that her last cardiologist was very dismissive with her high heart rates. I think she at least needs to be followed yearly to ensure there are no changes. Her heart murmur is audible again too... though I am certain that it is benign.

Quinn also had an IEP for school. I can't believe it! she's starting Kindergarten!!! my kid is going to be so big! Ok, so i have heard of people having a lot of problems in IEP's and making sure their children's needs are properly met. Her school psychologist totally set everything up for her! I walked into the IEP expecting to fight for an aide for her, but no. I didn't have to fight for anything. Quinn qualifies for OI (Other Impaired). She gets a 1:1 aide the whole time she is at school to help her. Plus, she gets to have the school bus pick her up and drop her off right in front of my house! Quinn will be going to school in her chair. She has a lot of attachments. In school, she will need to be on her feeding pump, her drainage bag,  and her oxygen pretty much the whole time. She needs to have the blood sugars monitored and her o2 sats for the times when she is able to come off the oxygen. She also needs someone to help her with the drainage bag (decompressing the air, emptying the bag when it gets full and putting it in and out of the fanny pack). We got a fanny pack for her drain, but she can't take the drain off her chair and put it in there and this aide is suppose to help her with all that stuff. I feel so comfortable with Quinn being at school! she's going to have a great time! I just hope I can keep her from stinking. Her formula smells bad. I don't know how to contain the stench. I'm going to have to rig something up for her within the next two months to try and conceal it because I don't want her being made fun of.

That bile just accumulated within a few hours.

Back In

Yep back in the hospital. So, a lot has been going on since I last posted. Over the past month, Q has just kinda fallen into a very, very slow downhill spiral. First of all, we know Quinn has issues with her gut. they are typically on and off. She can have two very bad days motility-wise, in which her stomach is blowing up like a balloon and she's just off, and then go back to her baseline. We can sometimes have weeks of no bad days, but this past month, she had way more bad days than good days and it slowly caught up with her into what we are currently dealing with. She also is no longer fully potty trained. She's having so many accidents her in pants that there's just no point to putting her in panties anymore :( Sad thing is that she knows she had an accident and she will tell me and get all embarrassed, but she just doesn't seem to be able to control it anymore. ugh! it's frustrating because she's 5.5 years old.

So, last week, I started to notice that Q's tummy wasn't really going down. I took her to the pediatrician who also said that yes, she is distended, but she's acting totally fine. so we did an Xray and the next day the pedi called me to tell me that she needed to be seen at the hospital. Her liver was enlarged and her stomach was greatly distended with gas and fluid. So, since her doctors all think that she should be see at CHLA, where her mito doctor is, we thought that it would be a good idea to just go down there since I kinda knew where this was headed. It was a tough decision because she was still acting very normally and she was not spilling any ketones or anything like that. So, we drove all the way down there, which is about 2.5-3 hours away! we get in there and the docs thought she looked well. Mind you, i KNEW she looked well, but I also knew where she was headed. I gave them her histoyr and told them her pattern in this and that I thought it was a good idea to nip it in the butt before it got worse than what it is right now. The took another Xray, which also showed the same distension as the one from the day before, but they took all her blood and all her bloodwork was normal. Because her blood work was normal, they felt that I was doing fine with her at home and that there was nothing they could do for her that i couldn't or wasn't already at home and they sent us home, with a diagnosis of pseudoobstruction!!! I was shocked. Loma Linda would have admitted right away given her history and two consecutive Xrays indicating a pseudo obstruction. She was smiling and her labs were normal which is why they sent us home. I don't blame them, but I really thought that hospital was more familiar with how Mito kids were and that I shouldn't have to wait till it's an emergency to bring her in. Well, apparently i was wrong and totally wasted my time going out there. Not only that, but I KNEW where this was headed! I KNEW my child was entering the "danger zone" and just had a feeling that this wasn't the end of this fiasco.

So, I did my best to care for her at home over the weekend, checked her ketones, she was still doing remarkably well considering taht her stomach just kept blowing up like a balloon. She wasn't in very much pain at all, I could still hear bowel sounds. The problem was that I had her at 6 caps of mirilax a day, senna and generlac and had given her an enema and she was still getting bigger and bigger and she was not really pooping anything that I would consider an actual bowel movement. She had been excessively hungry and excessively thirsty. Her blood sugar for some reaosn was high. and she hadn't peed all of yesterday.

So, yesterday, I decided to take her to the local ER for another Xray and to tget their opinion. Due to how well she was doing, i was certain her labs weren't going to be abnormal. But when the ytook her Xray they were like... she really needs to go into the hospital. Her Xray, which was alreayd pretty bad had gotten significantly worse. They diagnosed her with a small bowel obstruction, which im certain is pseudo. She was also hypoglycemic and her heart rate was in the 140s.

So, we are decompressing her entire body. Draining her J, her G and her booty. I'll keep you guys updated. I think this will be a short stay. She is acting fine so I'm assuming we won't have to worry about any shennanigans she might want to pull while we are here.

What a great summer so far! Overall, Quinn is doing quite well. She does still have her ups and downs. At times I feel she is borderline in need of the hospital, but we are working things out here at home, which is all I ever wanted. Those crazy doctors who told me that without her IV line, she would end up back in the hospital within a month. BUAHAHAHA!!! Ideally, I'm sure she'd do better with a line, but not for the consequences at this point in time.

We went to Sea World this summer! and Quinn had a blast! She's been talking about it so much lately and she wants to go back there soon! Maybe next summer, kiddo. I am so happy we were able to take her. Quinn also lost another tooth this summer, and again, we couldn't find it! So she had to write another note to the Tooth Fairy about how she lost her tooth... literally.

My little dog, Jezi got attacked by a coyote and survived! It was sooo scary! My dog Jezi is actually Quinn's dog Jezi. Those two are like two peas in a pod. She loves that little dog. Jezi is a chihuahua mix with a wiener dog (chihuweenie). So she's little. I was at my mom's house taking care of my sister's children and I had my dogs over there in the backyard. sometime right after the sun went down, my older dog, Jenna started barking hysterically. I let her bark for about 5 minutes (shame on me). I guess i just thought she was being over-dramatic and barking at someone walking by maybe. Well, she wouldn't stop barking and she was barking like a crazy dog so i went out there to see what was going on. She just kept barking and running from me to the middle of the yard and back again. I could tell she was telling me something. Then all of a sudden from the blackness of the night, Jezi comes shooting right past me and into the house all super fast. I was like.... ummm WHAT JUST HAPPENED? So I picked her up and she was grunting and breathing all weird and she was in shock. I examined her and she had drool ALL over her back and two puncture wounds on the sides of her and scratches all over her little legs. The only logical conclusion that we could come up with was that the coyote actually jumped the fence, picked up Jezi and tried carrying her back over the fence, but actually dropped her and she was able to escape. My poor little girl. She ended up on antibiotics for her wounds and had a broken rib. But that was it. She was lucky! Now we call her a warrior and she thinks she is a badass because she escaped the Jaws of a coyote.

I have been looking into buying a house. What I truly want is a place for Quinn to call home. I grew up in the Military and so I moved around a lot. I have no roots. I have no ties to any particular place in the Country. I have no strong relationships with much of anyone. I get jealous of friends and family who can drive around an area and say, "I grew up here." "I remember when this used to be." and etc... I want that for Quinn. I want her to have a home. I want her to develop life-long friendships. I want her to be able to reminisce about the past with people who were actually in her past to share that time with. I just don't have that and I want that for Quinn. There are a lot of things keeping me living where I am right now. I am not sure of programs like the one that Quinn and I are on in any other part of the Country. This program allows me to stay home and care for Quinn while being able to live a life of our own. If it weren't for the program we are on, Quinn would have to have a nurse and I would be working a lot to make ends meet. I wouldn't be able to be there for her if she goes inpatient as often as I am. I think she would be a lot sicker than she currently is as well. So, I choose not to move out of state. I also would like to move closer to the hospitals so that way I can just go home at night instead of having to worry about a hotel or sleeping on an uncomfortable bed. so that way I don't have to drive 2+ hours to get clothes and stuff. The problem with moving closer to hospitals is that the cost of living is extremely high. I couldn't afford it. I've checked into it and it's just too much. The place that I am currently living is the lowest cost of living here in California. I thought about moving further North, because I would really like to have grass and maybe a pond or lake nearby. right now we live in the desert, but I don't know of any good hospitals up in Northern California that could take good care of Quinn. Financially, best bet is to stay here. I think i could be happy here, but this is definitely not the predicament I ever expected to be in at this point in my life. I wanted to be well on my way to a nice career. Making at least a decent living and have a healthy child. My dreams seem so far away from me. I'm sure I will make it all work somehow.

Please pray for another mito warrior, Brent. He has had an incredibly difficult time and lately is really struggling in the hospital with a possible HLH flare up. Hang in there guys and keep fighting, Brent.

photo uploader is being really stupid for some reason. 

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.