oh where to begin???

Holy cow. Poor little neglected blog! I need to figure out how to get my facebook posts to update here, because i feel like the little random snippets off of FB would help explain why it is so long between updates.

Tonite I am tired. And kinda sick. I get it every time the weather changes, but today’s was particularly hard as it was just a cycle of pain, vomiting from the pain, vomiting up any meds i tried to take to stop the vomiting or the pain, etc. Pain meaning a headache from the outer recesses of hellish places, and i swear to you it was pushing my eyeball out of the socket. At least this is what it felt like. but that may or may not have actually been happening.

Anyways – nice spring break. Our fun family stay-cation to chicago started with kendall waking up tachypnic, tachycardic, and slightly febrile. Not wanting to disrupt our nicely planned vacation, we decided to not pack any of her monitors or O2. I also thought it might be a nice touch to not pack ANYTHING that a medically complex kid might possibly need to survive a nite away from home (syringes, meds, gauze pads, etc). It’s fun times! Knowing it was only for one nite though, i realized you just gotta let go and do the best with what you have (or don’t have)! i can say though that kendall is NOT a light traveller! We have two family trips in June/July that are probably going to require a small u-haul JUST for kendall stuffs!

So after surviving the nite, we wanted to take the girls to the Museum of Science and Industry downtown, mostly to go on the coal mine ride there. Now i am going to tell you a story about the coal mine ride. From the time i was, oh, I think in Jr. High (12 years old) and we first went on that ride on a field trip – i have been IN LOVE with that ride. I don’t know why. I love taking the elevator down below the museum, below the parking structure, down into the depths of the IL coal mines, making the machines start up, watching the gas lamp go out and kill the bird, etc etc. All of this was a REAL reality to me until my darling Father In Law decides one day to tell me “Terra, you DO realize that the elevator is just going down about 15 feet into the basement? It’s all just an exhibit.”  I was dumbfounded. i was 22 years old at the time and No, I did NOT realize it was only going down into the basement. He may as well have told me that Santa isn’t real and the Tooth Fairy doesn’t exist. To this day i am not sure that i really believe him because that ride is SO REAL!!!! (and i still love you Poppa Bob, even if you do burst my make-believe bubbles!)

So i really wanted to take the girls to see this. But the line was at least two miles long, and the babies were crank-o-matic just getting from the parking lot to the entrance. So we had to make a tough call to try to come back on a NOT spring break week, and without two babies in strollers. Instead we went to have a fun family lunch, and while we were there, we get a call from some dear friends inviting us to a nite of fun at a resort west of here. I’ll refer to this place as “BFE”. which means “berry far e-way” to the uninitiated, or any of my mother’s friends who are reading this who may be offended by my potty mouth. (my mommy makes me say that). Anyways – so we rush home, I finish up some work stuffs, we re-pack, and me and the olders (K1 and K2) start the drive out to BFE. We arrive in BFE to find NO CELL PHONE SIGNAL, so we can’t find our friends. At this BFE resort, there is an indoor waterpark that we THINK everyone is at, so the girls change into their swimsuits, and we trek through the bathrooms to go hang out while we find them. Long story short – we found them the girls swam till they were sick from chlorine exposure, and we went back to the condo for a nite of fun, hyperness, and lots of Twister games with friends. But back to the fun-ness of BFE.

on the way to BFE i hear a commercial that advertises it’s location as being “on your way to the Big Super-Walmart in Ottawa”!!!!! (which is a slightly larger version of BFE 20 miles up the road.) We pull in to BFE to find that they have a “Get your picture taken with our baby bear cub/tiger/other animal that may or may not turn rogue and rip your child’s face off while we capture the moment with a polaroid” exhibit. And NO KIDDING, they really had a for real baby bear cub IN A PACK-N-PLAY, waiting for you to get your child’s picture taken HOLDING this wild animal. I just do not understand this phenomenon….If God wanted us all to have black bears as pets to hold and cuddle and love it like a naughty pet, we’d all live in Alaska and have leathery skin. That may be just my opinion though. Anyways. The final straw to being in BFE and knowing it was when we walked into the bathroom/locker room and they had a HUGE sign on the wall that said “Please do not swim if you currently do or have had diarrhea in the past 24 hours. This especially applies to babies in diapers.”

I don’t even know what else to say about that. I mean really? People do this??? WHY would you even WANT to go swimming if you are having to run out of the pool every ten minutes to hit the pot? And if you are changing diapers like it’s your job cause your baby has the hershey squirts – perhaps you should consider keeping the baby in the hotel room and watching some cartoons. But i just kind of thought it was a given that you don’t go swimming when you have diarrhea. Apparently it’s not.

Anyways – we had a very fun nite with friends, and I was so happy that I got to be doing something fun with JUST the bigger girls for their spring break. All of that was shattered though at breakfast Tuesday morning when i open my phone to see a series of missed calls from Ben, right as he calls my friends cell phone and asks for me. I hear kendall screaming in the background and ben is asking where the old NG tubes are. NOT a good combo. Sure enough, she had pulled her GJ tube out. She used to pull her G-tube/button out with some regularity, and while it did involve a couple of ER trips to have an x-ray confirm placement, it was not really ever a HUGE emergency (except for the time in September that she ended up crashing really bad because of dehydration/blood sugar issues). But usually you just pin her down, make sure the stoma is open still, and jam the button back in (after you’ve checked the balloon to make sure it’s not leaking). Blow the balloon back up with a shot of water and there ya go. Good as new.

With this new tube – the part that you can see dangling out of her about ten inches, has a similar looking tube on the INSIDE that goes down into her intestines about a foot. So she had pulled a 3 cc balloon PLUS 12 inches of tubing out of her stoma. This happened because her balloon SHOULD have had closer to 5-7 ccs of water in it, holding it more firmly against her inner tummy wall. So clearly the greenish black stuff floating around in the balloon (once we could inspect it after she had pulled it out) must have somehow eaten a small hole through the plastic, caused it to have a slow leak, and out it came. Since i was still in BFE, Ben called a neighbor to come help hold a THERMOMETER in her stoma (because the main goal is to keep that stoma open SOMEHOW, with SOME kind of firm tube in place, so we can eventually replace the tube.) He couldn’t get a G-button to go back in, couldn’t find another kind of tubing up here in our medical cabinet/countertop area, was searching the basement for something, and nothing was really going in. So I told him it was time to take her to the ER (because, God love him, but if you’ve ever tried to un-do a lid that Ben has put on, or take something out of a container it is not supposed to fit into but Ben thought it should fit so he made it – you will know that if even HE can’t get a tube to go in the stoma, we must have issues). I flew as fast as I dared back up from BFE (an hour away) and met Ben in the ER, where they were as equally unsuccessful as Ben had been (but will still probably charge us a couple thousand dollars for “surgical procedures and supplies”, because they tried to place a BLADDER catheter into her stoma, and our poor nurse had to run up three flights of stairs to personally look for a more appropriate tube). So we thanked them, packed her up, went home so i could re-re-pack, pack kendall back up, and drive up to milwaukee for an appointment in IR (interventional radiology – basically it’s a “live view” X-ray. And yes Kendall does officially now glow in the dark from all the radiation she has received in her life.) Anyways, after finding the freeway i take to the hospital is GONE (they are trying to re-do it???), I manage to figure out the crazy detour and get her in to IR with fifteen minutes to spare before close! YESSS! I basically have to present a thesis statement to the nurse about why I think Kendall needs the sedation meds (are you kidding me? she has had random objects poked into a HOLE in her STOMACH for the last 7 hours, and now you have to manually DILATE her stoma and THEN place a whole new tube in it and you want to do all that while she is totally awake and aware???? Good luck! I’ll be down the street banging my head on the cement sidewalk to block out the sounds of her screams – call me when you’re done!) And luckily, the doctor agreed with my assessment of the situation.

now, when they set the legal drinking age at 21 years old, I am going to guess that they lined a bunch of kids up at random ages, gave them some sedation meds, and the age that it stopped being HILARIOUS to watch them at was probably 21. I am not encouraging sedating children (not all the time at least!). But I will say that seeing those meds hit Kendall’s system is one of the funniest things I have seen in a long time. She thought EVERYTHING was hysterical, and just had this totally goofy laugh going non-stop. She couldn’t figure out what to do with her binky and just kept holding it up to me and then laughing, and then laughing harder when i would laugh at her. It was all fun and games till they strapped her down to the table and moved the big machine over her head. She said “hi kitty!” to the x-ray, they yanked the tube we had gotten in, and the screams then ensued. They only had to use two sized dilators (vs. the five they had to use last time this happened), then they put the tube in, it got “stuck” going around the curve of her colon so they had to start over again, and this time they got it. We shot her full of some barium for good measure, watched it flow out into her intestines, and called it good. She was literally smiling and saying hi to random (non-existent) kitties within about forty-five seconds of getting out of the restraints and into her stroller, so I think the sedation was a GREAT call. I don’t know if she will need sedation before every tube change from here on out (GJ’s get changed out every 3 months as scheduled changes in IR. Unless you are Kendall. Then you set your own schedule which is more like every 4-5 weeks), but i can say that at least until we can “reason” with her a little more about how much quicker the procedure goes if you AREn’T squeezing your stomach so tight they can’t push the tube through, i think we may be looking at the sedation.

Anyways – we stayed the nite with my mom so i didn’t have to drive all the way home in rush hour traffic, only to turn around and drive back up to milwaukee the next morning for our regularly scheduled GI appt/followup. I didn’t really have any huge expectations for this appt, as I felt like things were mostly under control. BUT – in a huge ironical twist, because she had just pulled out her tube and i was able to show it to the nurse and say – what is this gross stuff growing on the inside of her tube? it was starting to clog up the tube and is IMPOSSIBLE to wash off – and she said she would have the doc look at it. He comes in and immediately wants to see the tube and says “uhhh, yeah that looks like yeast!” I encouraged him to smell inside the ziploc baggie and see if the beer factory smell emanating from it supported that theory and he said it certainly did! So what does this mean?

IT MEANS I WAS RIGHT!!!!!!! (not that i am gloating over this or anything).

I have been telling her docs for MONTHS now that I just don’t feel like oral abx are working on her. I question what is happening with other oral meds, but oral abx typically give us a couple days of good stomach functioning, then nothing. No diarrhea like you would expect when most babies are on heavy duty antibiotics, no real relief of symptoms (be they respiratory, ear infection, or UTI related), it is as if i am putting water through her tube. And these little yeasty dots confirm that!

She is believed to have what is called “Small Intestinal Bacterial Overgrowth” (SIBO). This is not a main diagnosis – rather, you can just add this onto the pile of other random diagnoses she has. This is a large piece of the puzzle though, in that it confirms that her motility (movement of food through the intestines) is really as crappy as we thought (sorry grandma and mom’s friends). Having yeast (and more than likely other fun bugs that should never see daylight but ARE supposed to help turn your food into poop lower in the intestines) coming out of her jejunal and stomach tubes, and indeed leaking out around her stoma and irritating the skin all over her tummy most of the time – means that when her lower intestines are shutting down, or moving VERRRY slowly, everything is kind of backing up, including that not good stuff. It is getting up to where there’s air, and more food, and other kinds of normal gut flora and it is just having a PARTY up in there, multiplying and wreaking all kinds of havoc on her stomach (most likely why we had to go to the J-tube in the first place). But most of all, when oral antibiotics are going into her stomach, they are encountering these “bad” bacteria, thinking THAT is what they were sent there to destroy, maybe controlling them for one or two days, and thereafter every dose is just consumed by the bad bacteria. Never reaching her bloodstream and attacking the REAL infection she is on them for in the first place.

i think that my initital reaction to this insight from Dr. N was to jump up and go “BOOO-YAHHHHHH!” I KNEW IT! I have had no way to quantify this concern to the docs, so they have blown me off for the most part, BUT NOT ANYMORE!!!!! I realize I am ridiculously giddy over what is still a not good diagnosis. But I’ll think about what it really means later. for now, there is a REASON, and for now, we think/hope that we can get it back under control. The plan as it stands right now is to let her system see what it can do on its own with this new tube. It may take three days, three weeks, or three months to see the yeast overgrowing the tube again. But when we see it, Kendall will have this tube pulled out, a new one put back in (hopefully the button this time since I convinced IR that 20 and a half lbs is actually VERY close to their random 22 lb mark for getting the buttons!), and she will start on a heavy heavy duty antibiotic into her gut and another one that will just sit in her tubes to keep them from letting the bacteria and yeast attach to them again. She will cycle through this antibiotic (Ampho-B – i am not sure how to spell the full name) for one week on, three weeks off, for at least a few months until we seem to have the bacteria under control and growing only in the place they should be growing. We will discuss whether a probiotic would be appropriate or helpful at that time. (Since I get asked that question a lot!) MOST probiotics work by putting MORE bacteria into the stomach/upper gut that get eradicated by antibiotics in otherwise healthy individuals. Kendall’s gut flora is already a little out of balance, and adding in MORE bacteria to a bacteria party is not something we want to just do willy-nilly. Trust me that I am the FIRST ONE to go for a more naturopathic approach to things with all my kids, and if a probiotic will be a better alternative than this big gun antibiotic, we will do it.

Anyways – it’s been a very very long week.

I am hopeful that we will get to still do our easter egg coloring, easter egg hunt, easter basket finding this weekend in spite of all the craziness. This will involve me getting a quick healing from this headache/pain so that i can fight the Good friday shopping crowds, and just a lot of other logistical issues to be worked out.

There is a lot more I wanted to /need to blog about. hopefully that will come tomorrow. Basically the jist of it is this – they believe they have narrowed down Kendall’s issues to a “probable” mitochondrial defect, but they do not know exactly WHAT defect she has (they have only identified about 50 out of what they presume to be thousands). They don’t feel like she has one of the more known to be very life-shortening ones (that is just their words, i am not sure what they mean in the greater scheme of mito defects), and do hold out hope that she will continue to grow and progress along a good curve. That is about as much hard and fast info as I could get out of the geneticist on the Monday we saw him inpatient.

Her paperwork/records at the hospital have her official diagnosis listed as “mitochondrial disease”. This is a change from the previous “MMA” (metabolic error). She still has a metabolic issue – her MMA levels are typically all over the map with no rhyme or reason. But since she doesn’t have any of the known genetic defects that cause MMA as a disease, they are attributing her MMA levels/issues to a bigger picture “mitochondrial defect/disease”.

This is still kind of sobering, and i readily admit that I do not have all the answers about this. We do know that we are in the process of gathering up all of her records to send to a mitochondrial disease specialist in Pittsburgh for review, waiting to hear if they think she should be seen by their mito team, and if so, making travel arrangements and hoping our insurance (which is based in Pittsburgh oddly enough), will cover the visit out of state. Many questions still in the air, but at least a few more arrows pointing us in the right direction hopefully.

 

With that, i am off to try to sleep off the rest of this horrible headache.

 

Sorry its been so long – I’ll try to be better over the next couple of weeks!

 

terra