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.

Good times

Love my Feel Good Quinn! She's an amazing little cookie. Such a strong little girl. I hope we have more days like this! I love these days :)

Thinking Of You All

I know Quinn was just recently diagnosed, and seriously, everyone I know it the mito community basically told me that she had it and would be very surprised if she was never officially diagnosed. But somehow, I just never fully accepted this. I followed a lot of Mito warriors and cried many tears for them and their families, but something inside me was denying that I was actually walking their journey too. Now that we are official, I somehow feel that much closer to those fighting this disease every day.

Some families who need our prayers:

I'm so sad to say that Maggie has earned her wings. She was such a sweet and beautiful little girl! her family needs all the prayers they can get to help them through this very difficult time.

Samantha has been fighting in the PICU for two, almost 3 weeks now. She just had surgery to place a trach. Please pray for healing for her and that she can have no further complications during the rest of her hospital stay. Her parents are very eager to take her home again, but she still has a long recovery. She needs our prayers.

Sweet Eithene is always in our prayers too. She has been in the hospital for about an entire year now and is constantly suffering infections and now heart issues. Please keep the family in your prayers.

Mito just plain sucks.