A little easier

Life is.. a little easier I guess. Everyone who told me in a week that the PICC line care would be easy like the G and J tube care, you are so right. It's easy as pie now and I'm so quick at it! wewt! Last week was pretty difficult though with her PICC line. AS you guys know, we had an issue with the Kink in the line, but over last weekend, I had noticed a lot of blood accumulating under her dressings, and it was getting worse every day. it semed that the line was just moving in and out everytime her arm moved, which was causing some bleeding that eventually got to a point that I wasn't comfortable with, so on Monday, she, again had toh ave another dressing change and we were working hard to figure out how to secure her line. Her nurse said that it was protruding quite a bit (in other words, her body is trying to push it out of her), but we did what we could and are going to salvage this line for as long as possible. I have pictures, but can't find the USB for my phone so i'll have to post them in my next entry.

And in case you didn't know, dressing changes are complete torture. and this has to happen no less than once a week, so having to change the dressings 3 times within 7 days was just not exactly comforting. Poor baby screams so much through the changes.

I couldn't figure out for the longest time how on earth Quinn's PICC line was helping her pain in the stomach. I mean, yes i'm sure it was helping her with the hydration and everyting, but I figured that since we were actually trying to INCREASE the feeds, the pain would still be about the same. Well, I finally came to the conclusion that draining her G 24/7 (or at least near that) is helping her pain tremendously. Before, i wouldn't drain her G all the time, only during periods of extreme distention (usually like 3 times a week), but since getting her PICC, i'm able to hydrate her and replace fluids if need be, therefore she is continuously venting and draining her stomach, which has actually caused her pain to be decreased by a crap ton! She blows up the ferrall bag like a balloon no less than 3 times a day, but man, the pain is so minimal. I am hoping there is a way that we can doing this without the PICC. the hydration is obviously essential, but since i've narrowed down the culprit for her pain or at least the majority of it, maybe we can work towards some kind of supplements getting added to the J feeds to hopefully keep her at the hydration level she needs to be at in order to remove the PICC line. Her stomach motility is still crap. She still gets a ton of pressure build up to the point of blowing mic-key button valves, leaking around her J and now a new development, blowing out her meds and her extension (which was hopefully a 1-time-thing, cuz that was just so strange). But her entire motility of her stomach is just the pits... and her small intestine... and her colon. Gosh! i really hate that.

In other news, Quinn is feeling much better, the point of annoying the crap out of me all day long with her questions and energy levels. I just don't know what to do with it. I mean i'm so happy that she has so much more spunk now, but she's getting on my nerves. I just want someone to come and take her for an entire day... some one i trust, someone who i won't feel guilty for leaving her with them for 24 hours, someone who doesn't mind if i use them just to veg out on my couch for a whole day... hrmmm that won't happen, but it would be so nice!

April 7th is the BIG DAY we get to see Dr. B in CHLA.. this is the MITO doc. My mom was talking to someone in her dad's hospital... eerrrrttt... let me stop right there and just ask for prayers for my grandpa, he was given a 1 % chance of survival a couple of days ago. He suffered a heart attack, collapsed on the floor, was given CPR, was shocked and everything and intubated and my mom was making the decision to pull the plug when he made a miraculous recovery. He still needs prayers but he is awake, and talking and the nurses and doctors said it was just a miracle. There's obviously something more he needs to do in this life before he goes on.

Anyway.... in this hospital, my mom was of course, talking about Quinn and she came across a resident who was training with a bunch of Mito patients who are in clinical trials for bone marrow transplants! He said the kids who were severely affected and unable to even walk didn't have very good outcomes, but the children who were able to walk and talk, though still obviously affected by their disease, basically children like Quinn, if they gave the bone marrow transplants early, he said that there were a lot of improvements! This makes me feel really good about them possibly coming up with a treatment soon! I can't wait to talk to Dr. B because her neuro was talking about how he thinks she can possibly enroll in some clinical trials! that would be amazing if she could enroll in a clinical trial that would help her! I would be so happy!

Pictures and Update

Oh boy. this week has been so incredibly busy. It's been insane. The weekend we got home wasn't exactly a "relax" weekend. I was having a hard time dealing with this PICC line, i created a nice clean area and bought a chair just for Quinn's "access" area. my mom came over to help me organize all the fun stuff that came with having a PICC line... on top of all the other accessories she gets for having two feeding tubes, drainage bags and oxygen. Needless to say, we have one spot for quick grabs and a whole other area for bulk items. Accessing her line was first a very daunting task. I was so afraid to mess up and I actually did once! she has two lumens, so two lines that need to be flushed and I was working on one and cleaning it up and I accidentally flushed the other one without making sure it was sterile. Then she got an incredibly minor fever 100.6... so that had me on edge for like a day, but all is ok. As far as flushing her line, it was very difficult to flush! I was bending syringes trying to get it to flush. It was just soooo hard! I knew it was going to be a little resistant because she's a peds patient and has smaller cathedars and smaller veins, but I KNEW it shouldn't be so hard to flush, so I was really anxious to see what the nurse had to say about that at her dressing change.

She has been having a very difficult time making it to the bathroom, this has been going on for about 3 weeks. She's been having diarrhea and also she is peeing her pants. I had to start putting her back in diapers. It's quite stressful because when I ask her if she knew she went, she says no. Plus, as far as her poop goes, it just comes out of her bottom. she's on 3 caps of mirilax a day, so we went ahead and decreased it to 1.5 caps, but the problem is, now she doesn't have any meaningful bowel movements. They are all just like... small amounts of watery stool. If i remove the mirilax all together, she simply doesn't go. so we are working to re-potty train her and her bowel regimen just has to be on a day to day basis.

On Wednesday, Quinn had an appointment with her pediatrician, it was just basically a checkup after the hospital and to update her on all the things Q has been going through and to let her know that she got a PICC line and to file some paperwork. She was just so sad to see that Quinn's stomach has been declining so badly. She knows when it gets bad because i typically take her there before anything else unless it's a serious issue and needs to be seen in the ER, so she's seen Q at her worst when it comes to her stomach so i think it's no surprise to her that she ended up on a central line. but still, they love Quinn and she seemed so sad that she couldn't help fix her. We are all very anxiously waiting on her appointment with the mito doctor in CHLA. this will be April 7th!!! so close!

That same day, she had her dressing changed, all the way down the hill.... and it was complete TORTURE! My poor kid. she is so brave. She was crying so much, but smiling in between the tears and trying so hard not to hurt. no wonder why this stuff hurts her so bad, she has such sensitive skin that she developed bleeding pressure wounds, just from the dressing being on the skin. I'ts not an allergic reaction, it's literally just a pressure wound and any kind of dressing is going to be hard on her skin because it's any kind of tape that will hurt her. It's just from the pressure of it being on her skin. The BioPatch they put in the middle also caused a pressure wound around the cathedar site. poor thing. Both the nurses who changed her dressings this week commented on her very sensitive skin. And she has to have this done every week (well she had it done twice this week). Anyway, at first, before the nurse picked up the dressings, she tried flushing her line and i was telling her that i didn't think it hsould be that hard to flush and she said that it absolutely was extremely difficult to flush and was going on and on saying that we needed to go back to the hospital to get the PICC line looked at and to possibly need it to be flushed with stuff called TA. So anyway, she tore off the dressings and we found the root of the problem... Quinn has a HUGE kink in her line close to the cathetar site, under the BioPatch. Well, that would explain it all, so nix on the going to the hospital thing, now the issue is how to alleviate the kink. The kink was so bad and had been kinked for so long that it developed a weak spot, and is at risk for being kinked again. Not good news. so we maneuvered the dressings and BioPatch accordingly and wow... wow wow wow... I can't believe how much EASIER it is to flush!!!!

On Thursday, Quinn had an appointment to go and see her GI doctor. We were both amazed to see the scale read 40lb! Quinn gained 2 lbs since leaving the hospital, which is great! she also looks much more plump. Ok, i know what some of you may be thinking, Q is at a healthy weight... and in essence, that's right. If some of you follow... They have been talking about Q's nutritional status for a long time. If you look at just her weight, you would think to see a healthy looking kiddo, but Q's BMI is off. Before she got her J tube put in, she was off the charts low for BMI and the nutritionist at hte hospital kept telling me that she was malnourished... well duh! that's why we are doing J tube. Despite having an excessively high caloric intake due to the tube feeds, she still struggles with appropriate weight gain, she fluctuates on the scale significantly. her appearance with clothes on can be deceiving since she is at a good height, and her belly is at least a little distended most people would think that was ok. Now, you take off my kid's clothes, she is literally SKIN AND BONES... especially back in November, when seh got her J tube placed.

September ? ish

A Few Weeks Ago

 

Her poor bad belly right before that last hospitalization

 

She looked very malnourished. BUT... weight is not the reason she got her PICC line in the first place. At first, the GI did talk to me about the TPN, but I told him that I felt her weight was ok,  and she was climbing on the BMI charts and I don't want to destroy her liver prematurely. But she does need some help with some extra hydration. the rate at which we feed her is just not enough to cut it.

anyway, back to the appointment... He gave us some prevacid to help reduce the acid in her stomach...and we know she is refluxing again, so that should help. And he also prescribed us this powdery substance to put around her J tube stoma to protect the skin from breaking down from the bile that tends to leak out around the stoma.

Did I ever mention how absolutely AMAZING her GI doc is. We LOVE him! I want to give him something in appreciation for how he goes about treating my daughter. He is very affectionate with her, he doesn't like to hurt her, he never ever rushes us, he thoroughly answers our questions and he is very knowledgable! I just feel so blessed to have found him, in the midst of all the craziness that has gone on with doctors and Quinn, he is always the one to go above and beyond to sort stuff out for us! I feel like he really works very hard. I want to do something to show him how much I appreciate him. Have any suggestions?

Last but not least, Quinn finally got approved for the home nursing who will come out once a week for PICC line care and dressing changes. She came out today and tortured my kid some more with another dressing change. She's really a very sweet lady and used to work in the NICU when Quinn was there! She took a long history and I really enjoyed our conversation. she was also very good with Quinn. She also offered to try and help us get her a night time pulse ox for her!

We also had a meeting with the school nurses to discuss Quinn's care plan for going back to school. I too am just thrilled with the care she receives at school and how careful they are with Quinn. I love the nursing staff (who yes, can sometimes take a long time to get back with me but this time they didn't and it was cool), but they have been the same nurses working with me and Quinn since Q was 3. So it's really cool to have them around because they kinda know how difficult things have gotten for her and are aware of her health status. she does get to go back to school! there are a lot of guidelines, but her teacher is just great and is really good with Quinn and incredibly thorough. The nurses also told me that they would pay for a stroller with swivel wheels to help her with her oxygen and also offered their help and resources to help get her a pulse ox! I just can't believe I don't have any reservations with sending ehr back to school on a PICC line and having her sugars checked while there too. I trust them so much and feel like she is in good hands! so rare to get such wonderful feelings about the school staff.

We are also in the process of starting her transition into Kindergarten. I will be meeting with her school principal and school psychologist on Monday. NERVOUS!

Wow that was a lot to update but we also had an incredibly busy week! time to go put the kid to bed.

Enjoy the pix and vids. these are from the last hospitalization.

Q sweats pretty bad when her tummy isn't being nice.

On a Bad Day At The Hospital

 

 Every they asked her to give them a finger for sugar checks...

 She Gave them the Bird.

Bath Time on a "feel good" day

 

Uncle Dan and Aunt Tonya Visit

 

Home Again

 

Q sings this a lot. she calls it her "perfect song"

 

Defeated

I have been having a hard time lately. I know to some, it may seem small, but I have been thrown some hard blows emotionally and man, I'm not afraid to admit the toll it's taken on me. Quinn and her health have been taking up all my thoughts lately. Especially lately. Sometimes i look at her, and she just looks so pathetic. Her eyes are so dark, her face so pale. She's so frail.

I read about children like her, children worse than her, children who are still with us fighting, and children who have lost the battle with Mito. Around this time last year, I would have never thought we would be here, in this situation. If you would have asked me last year, I would have been in complete denial. Even though I knew in my hearts of hearts that she had a mitochondrial disease, I STILL wouldn't claim for her to have had one. I STILL wouldn't allow anyone to believe that it's truly what she had. I'd find reasons as to why she couldn't have it... her development was a bigger one. And then i would also get pissed off at specialists who would tell me that there's no way she could have it, because of (insert the dumbest reasons here). I wanted to meet other children to compare her with so that way I could feel better about what IT is. About HOW she is. I saw the progression on other blogs, what their children were going through, how much worse off they were than my child. And I never would have imagined that months later, i'd watch mine fighting to live in the PICU. how strange this world is, how daunting this disease, how drastically ever-changing.

Today, as i was looking at my kid, my kid whose disease is no longer masked by a very healthy appearance, I found my thoughts in the darkest of places. Places I truly don't want them to go. how long might I have with her, how many more Christmases? How many more birthdays? Family can ask the toughest things. I am bombarded with questions that I just have no answers to. Will she get better? Can't they just do something to make her stomach work? How long does she have to live? I can't answer these. It tears me up inside just hearing these questions. how am I suppose to know the answers to these? I look at her and I'm just so mad that things have come to this. It sounds so strange, but I get so pissed off at everyone.

I'm not stupid. I know that the chances of her gaining back any kind of gut function that she has lost is not very promising. What's hardest is hearing the same doctors who have told me for years and years that there was nothing wrong with my daughter, that she was just going to get better... those same doctors now tell me that there's nothing more we can do, except to treat the symptoms as they appear. I am told that there's nothing more we can do to move her stomach forward. there's no magic pill. there's no surgery that will contract her stomach muscles. there's nothing we can do. 

Just a few weeks ago, I totally broke down in front of Quinn's teacher. She was asking me how Quinn's neuro appointment went and I just told her that Q was diagnosed with Mito and I told her that I had known she had it for so long, but now we know for sure and I just started BAWLING my eyes out in front of her! It was terrible and embarassing!

dealing with this PICC line makes me feel completely defeated. It was a decision that came on so fast. It wasn't like the J tube, where we had months and months to decipher if this was the right decision for her. it was a... you aren't going home without a PICC line kind of situation... she NEEDS help. Her stomach needs help because sometimes, it's just not able to do its job. I still feel defeated. Seeing her line in her arm makes me want to cry. I just feel so bad for Quinn and all that she has to go through, but guess what? It's like, no sweat to her. She's the most amazing kid and today, she had the most amazing day. It was the first day in a very long time that she was with very minimal pain.

I know this blog was all out there. I'm feeling kinda scatter-brained. i am just feeling... defeated.

We are Home

We made it home today. It's been busy. Quinn got her PICC line in yesterday and she did great through it. she had no issues with her blood pressure or breathing. so that's all good news.

We were trained on a new glucometer and need to check her often adn when she is starting to look low. She's also now on carnitor and periactin and we give her cornstarch twice a day to keep her sugars in check.

As for the PICC, she's in a chronic state of dehydration. At first we discussed doing the TPN for her, but I think she just needs the hydration for right now as she is not low on her weight. Her stomach is crazy bad though. i don't know what to do about that and we were told not to expect it ot get much better... but there's always hope. We are hoping to avoid TPN and trying like mad to get her on a good plan to keep pain and bloating to a minimum. This isn't working too well as she's still in a lot of pain. today, she cried herself to sleep because of the pain. I'm suppose to cut off her feeds if she is in a lot of pain and her stomach is looking very distended, I then infuse her with her IV fluids to avoid dehydration. She also gets a maintenance of IV fluids every single day.

The PICC line care is incredibly overwhelming. There's actually so much I want to say, so much i want to write about right now, but I am just so exhausted and trying so hard to get a grasp on everything that is happening. I'm so extremely worried about line infections. I am incredibly terrified actually. And i'm so afraid of doing something wrong... like forgettting to remove all the air out of the syringe, or accidentally leaving an air bubble in the IV line. I'm so worried. what if she destoys it on accident? I am going to spend this week trying to get into a good routine and habit for her. Thank god she is not accessed all the time. I don't know how some moms do this as I am freaking out and pretty worried something will go wrong.

Decisions to be made

So, We are still in the hospital. Quinn had an endocrinology work up and  there were no real abnormalities found in her bloodwork except that her cortisol level was found to be low. The endo feels like the level was drawn 3 hours too early and so she wants it redrawn today (which is what they did). So we should get those results this afternoon. if the levels are found to be low again, she will need to be treated for it and it would explain her low blood sugars. As well as her low blood pressure and a couple of other weird "Quinn" symptoms.

Quinn is funny. Our first 36 hours here, Q's tummy was fine, actually. but, since she follows her schedule pretty much right on, I KNEW she was going to be ok on that day. It's just the way her tummy works. I called it and i was right. So on monday, she had a bad tummy day. she was puking, pushing out bile, her tummy was big and bloated everything was painful. given her bad day, it was still not one of the worst "bad" days we have seen, but her docs got to have a piece of what i'm going through. i showed the nurse who was taking care of her on her "good day" her "bad day" tummy and she was so surprised. Because she hadn't looked like that the day before.

so, Quinn's GI doc is the one that is on her team this week which makes a WORLD of a difference because he really cares about Quinn. He also knows all the crazy stuff we have tried to keep her tummy working. We discussed a lot of stuff. He really wants her on TPN at home, or he wants us to try it because he thinks it will help her through the bad tummy days. After posting on my trusty P2P board and asking family and some FB friends, I thought it would be more beneficial to have her on just IV fluids to push her through her rough patches instead of TPN. The problem with TPN is that it needs to be run continuously and scheduled... so i can't just start it up if she is having a bad day. Plus, labs need to be continuously monitored. I just don't think Quinn is "that bad" yet to need tpn. She's growing on her J feeds. Granted, it's very painful for her, but it's still working. she's not underweight. She just has a lot of issues digesting her foods. So I think the better choice here would be to go with IV fluids at home and I can just run them if she is getting distended.

Typically, if Q is getting distended pretty bad, i would cut off her feeds because she just has so many issues with it. However, cutting off her feeds can cause her to go into a hypoglycemic crisis (as we have already learned). Even running pedialyte isn't cutting it for her sugars. She just doesn't absorb much in her tummy during the "bad" days. Not to mention that despite having plenty to drink, she is remaining dehydrated. For instance, yesterday, and the day before that, she only peed a maxiumum of four times for those two days combined. And the pee was obviously dehydration pee (pretty brown). So she needed IV fluids. Despite having had plenty to drink and being on her normal at-home regimen. Our Doc told us not to plan on going home without a PICC line. so, we feel that having a PICC line to get her through tummy issues will be very helpful. Not to mention that we can hang D10 if she is having hypoglycemic attacks.

Ok, back to endocrinology. Wouldn't you know it?... Quinn's blood sugars have been very stable since we've been here (except in ER). We didn't do a fasting test. But yesterday when endo came in and talked to me, she said that we know Q has lows. she's had them in the hospital before and obviously when i brought her to the ER. But she doesn't believe the highs we are seeing are true highs because she believed we would have seen at least some highs by now. She has always been lower than 120 in all the pricks they have done. So she felt that she's not having true highs. plus, her A1C levels came back REALLY GOOD!!! This was the most happy news I have heard in a long time. She told me that she can tell me for sure that Quinn does NOT have diabetes. WHEW!!! i was so happy to hear this. So, instead she gave us a new glucometer to have at home. We need to check her 6-8 times a day, plus she needs to be checked at school. She is likely going low a lot more often than we think she is. for quinn, her low is 70. anything under 70, we were told to treat her. Now this part is really funny and just made me laugh my ass off! Last night, when they were checking the 8 o'clock glucose levels, she was found to be hyperglycemic. Recall in my previous sentances that she hasn't had any highs and the doc didn't believe that she was having true highs. I find this quite comical because this is the same kind of bull crap that she pulls on me at home! So the nurse had to page the doc to let them know that Q had a high reading. It's nice that they have kept us long enough for them to actually see her true colors. sometimes, I feel like a total lunatic when i bring her in and tell them that this is what i'm seeing and she pulls no such nonsense in the hospital. So, i'm excited that we are hopefully going to be able to start treating these things instead of being completely blown off. I think also that having this mito diagnosis truly helps. It's odd how that helps.

So, not sure what the plan is today. i'm hoping the Dr. B (our GI doc) will get the ball rolling and let us put the PICC in today and let us go home. That would be so nice. Q is not "sick sick" per se. So she's getting ansy being in the hospital. So am I. i can't afford the gas and I can't afford to feed myself and I can't afford her being in here at all. So I just want to get the hell home. I have a lot of stuff to take care of. I need to clean my house and find a spot where we will be diong PICC dressing changes and sterilize that whole area.

In Hospital

Again. currently battling sugar issues now. Suppose to have an endo consult tomorrow and likely some testing.

Q has been having major lows and major highs. I was unable to keep her very stable at home. her tummy was just humongous so one night i decided to discontinue her feeds and put her on just Pedialyte and by morning, she was in a hypoglycemic crisis. She was pale, cool, clammy, frantic and very shaky. It was quite scary (but also, nothing new). I gave her some juice and grapes and less than an hour later, her sugar was super high. It was decided to take her in and that's what I did. Despite the electrolyte drip and the feeds still going into her, she was found to be spilling ketones in her urine and still dehydrated (even though she got tons of pedialyte that night). Well, because Quinn is Quinn.. and this is just the way things are. she's been pretty stable since we arrived. She has had some slight abnormalities with her blood sugars, but nothing drastic like what we had been seeing for the past week. At the same time, she has been having minimal output from her G drainage and stomach seems to be working again. thankfully, they still want to do an endo consult and look into some possibilities for her drastic glucose changes. (thank God glucometer records readings!!!) And her GI doc is on the team tomorrow so I get to talk with him and let him know all the good stuff that's been going on. I REALLY hope it's NOT diabetes and i really don't think it is. I just want a plan. WTF am I suppose to do when her stomach is not moving things, she needs hydration and she needs sugars? I'm NOT taking her in every week for fluids. there's gotta be something more I can do for her that doesn't involve a central line. These episodes are brief, maybea couple days at most, but they are also frequent, so obviously, we just need a good solid plan. I hope I am able to convey that message tomorrow.

A new Chapter

Ugh! I have a feeling we have added another issue in the world of Quinn.  We've been knowing for some time now that Q suffers from some hypoglycemia issues. This is a result of her stomach motility. It's terrible. When she gets hypoglycemic, she has all tell tale signs. She gets very pale, she starts gagging and wretching and trying to puke. She has an altered level of conciousness. Anyway, it typically coincides with her days when her stomach is being stupid. On these days too however (which is at least a couple times a week when things are going good), her stomach is slow and causes a lot of problems. She gets very distended and is not at all comfortable. Plus, due to the pressure, her feeds start leaking out of her J stoma and juices leak out of her G stoma. sometimes, food reverses back into her stomach. So, to combat the pain and since crap is leaking anyway, we drain her in attempt to relieve the pain and release some of the pressure.

Last night was no different. Yesterday, she started to have a bad tummy. I knew we were due. if she has 2 days of decent movement, we are bound to end up with the third or fourth day begging for mercy. It started with diarrhea accidents. She was playing in the yard and brown stuff was all over her legs... yep diarrhea so crappy! She took two baths and we had tons of changes. Plus, her stomach was bloated and uncomfortable. by the end of the night, she was wimpering in pain begging for medicine, a hot pack on her tummy and venting. This is typical.. not necessarily the diarrhea but everything else is very typical. When I say that Quinn's stomach is being dumb again.. this is what i'm talking about, usually. So I hooked her up to her drainage and turned off her feeds for about an hour and turned them back on. this helps her at least go to sleep! So, in the morning, her drainage bag is filled with all this bile and I check her blood sugars and they are very high. last time was 239, this morning it was 276.

Not exactly sure what's going on here, but I know it's not really a good thing. She's rollercoastering on her blood sugars and ti's been a pattern since November, when her blood sugar issues were noted. On the other hand, I didn't check her sugars regularly until recently, so I can almost garuntee that she had these issues before. I just wasn't aware. At the same time, her stomach motility has gotten worse anyway. so, doesn't matter about the past. right NOW she's having some issues and I think they need to be addressed. My auntie has a son who has juvenile diabetes and she told me that the way Quinn's blood sugars are right now sound EXACTLY like that. She mentioned when her blood sugar is so high to check her ketones. I think they ahve stuff to check those at walgreens over the counter? Anyway, diabetes is believed to be linked to mitochondrial dysfunction. since she has mito, it's nto very surprising that as we see progression of the disease, soemthing like this would crop up. But i'm not a doctor and can't diagnose her. I am keeping a record of her blood sugars. they do in fact coincide with bad motility. either low or high. We have an appointment to see her GI doc on the 17th. i plan on talking with him about this.

This disease sucks. We also had another mito warrior who lost is battle last night. His name is Cooper Knight. I'm not familiar with his story totally, but I know he had to have an intestinal transplant a little while ago. Though I don't know this family or much of the story, I am so sad for the family. keep them in your prayers. This disease takes too many lives.