Alternatively titled “Just in case you thought you were going to complain about being in the hospital again, your air conditioner will also concurrently bite the dust, and you will be grateful for the air conditioning your baby can receive in the hospital.”
But that wouldn’t fit in the title box.
So anyways – Kendall’s back in the Big House. She’s been doing a few of her little “off” things since last week, but nothing that her nurses or I could put our finger on. So we made it through Showcase (the girls’ dance exhibition for four straight nites) with no major medical issues. Then Monday morning we go to change her dressing like usual, and she FLIPS out. Like the three of us all needed a drink after we finally got things calmed down kind of flip out. As we took off her dressing, her arm instantly puffed up about two inches, and the screaming started. Her TPN nurse said we should not run anything else into the line until we could figure out what was going on with it, we started making calls to milwaukee and the nursing agency supervisors trying to figure out what to do, and eventually came up with the plan to bump her feeds up as much as could into her J-tube, and get a line study done Tuesday morning since we would already be up here for a regular appointment.
So Tuesday morning we wake up and I start the crazy run around getting stuff packed for the day, getting Kaylen ready to go to her friends birthday party, making sure we have snacks and all that kind of stuff – and I realize its kinda hot. Sure enough, the air con had stopped working again and it was already 80 degrees in the house. Which doesn’t seem that warm until you add in the like 200% humidity we have been dealing with, and then its kinda like sitting in a sauna at the gym. *sigh* So I called Ben who was in Pittsburgh for the week, he calls Mike, the most awesome friend/HVAC person anyone could ever ask for, and we hit the road. Where, about ten minutes into the trip, the CAR air con goes out. And by “goes out” I don’t just mean “lack of cold air”. I mean the sensor goes wacky and it thinks it has to HEAT THE CAR UP to 74 degrees or whatever is on the dial. EVEN IF I TURN THE WHOLE THING OFF. It seriously needs an old priest and a young priest and a silver bottle of holy water. Imagine driving in the hot sticky weather and holding a blow dryer against you the whole time. That’s kind of what the three hour trip up was like. Luckily the hot air only blows in the front, so Kendall was warm and flushed and starting to show signs of wear and tear (breathing hard) by the time we got up here, but she was mostly ok. (and for the record, the air con thing comes and goes in the car. It could be fine for two weeks, and then not work for one, but then randomly come back on – seriously possessed.)
My original plan had been to have the girls just come with me since it was going to be a quick line study and then just one appointment, but we stopped at my aunt’s house so Karissa could pee and we could get cooled off a little bit. She offered to call my cousins and have the kids come over to play, which just lit up the girls’ faces, so thankfully that worked out. I am sure they had FAR more fun there than they would have if they had been with me.
The line study actually went well, they saw no obvious holes or reasons why her arm would go stay-puft marshmallow man on us. (which, of course, I rolled my eyes at. Pushing a few cc’s of contrast into her arm slowly is FAR different than running her TPN, which chugs along at 3 ounces an hour. Plus its very irritating to the tissue vs the contrasty saline. Basically, it was like comparing apples to steaks. I was glad they saw nothing obvious, but none of the rest of her team nor myself felt like it was compelling enough to say for sure what was going on.) The fact of the matter is that this line is 15 weeks old, which, in itself is a small miracle. We have successfully treated through two infections in it, TPA’d (clot busted) it once, had it re-sewn into place, had it checked for placement twice, and have really never been able to use one of the lumens (it has two). It just seems like at some point, enough has got to be enough. It’s a lot to ask of that vein to hold up through the near constant coursing through of her TPN, antibiotics, extra fluids…And most picc’s aren’t designed to withstand that kind of long term use. (yes some do, and i know some last super long with zero problems – but that is obviously not what we were dealing with here.) If someone had been able to tell me unequivocally that turning on her TPN would be 100% ok and we wouldn’t just dump 40 ounces of tissue-burning fluid into her arm muscle, I’d have been fine with the whole thing. But no one could say that.
Our appointment with Genetics went about like I expected. Kendall’s latest round of testing came back “abnormal but not diagnostic”, which isn’t surprising since the stuff they were testing for is all completely random and doesn’t cover everything Kendall deals with. Her second muscle biopsy did come back confirming “mitochondrial myopathy, not genetically diagnostic” meaning that while something is effed up in her mitochondria, we still don’t know where or HOW they are messed up. Considering they have less than 15% (I think – I get the stats confused sometimes) of the mitochondrial genes mapped/known/discovered, this isn’t a huge surprise to anyone. They actually do have a couple more directions to go based on how this testing came back, and both of them actually KIND OF make sense – except that both of them combined still don’t really explain ALL of Kendall, and both of them combined also fall under “crap that happens to you when your mitochondria don’t work” (dysautonomia of non-Jewish origin, and ataxic migraine syndrome something or other). We aren’t moving forward with testing for either of those right at this moment mostly because the tests are still VERY new for them, and because neither of them would guarantee that any other treatment would help. So for now we just stick with “mito of uncategorized origin” as her “diagnosis”. Could have told you all of this WITHOUT having to sit down with the geneticist for two hours.
About the only thing worth mentioning that came out of this appointment was that the doctor was NOT happy with the 101.8 fever Kendall had while we were in there, nor the fact that she had been off TPN/fluids for over 24 hours at that point and wasn’t tolerating the J-feeds of sugary pedialyte we were replacing with. So he and our complex care nurse (who is at all of our appointments) went out to “conference” about the plan and came back in to tell me we had to head over to the ER for labs and cultures “just to be safe”. And that if the labs came back ok “we might be able to head home later that nite”. Which, as I came to later find out, was complete BS. They actually had called the ER and instructed them to admit us. But i think they knew if they said that I would have fought them tooth and nail, and possibly ended up not taking her. Which is probably only half true. I wasn’t confident that we were dealing with just a benign fever from being hot and cranky, and with all the line problems, I figured it was probably worth it to at least draw labs/cultures. So off to the ER we trucked, me still blithely thinking we’d be out of there in time to get a late dinner and be home in our own beds that nite.
After a couple hours in the ER when I asked the doc if they had labs back yet so we could start discharge, she actually laughed at me and said, they didn’t tell you they admitted you guys??? we’re working on a bed right now! Oh i was so ticked. But I did realize that it was probably the best decision, as by then Kendall was looking even punier, and within about fifteen minutes of that conversation, we got to see her metabolic crappiness at its finest. She had been getting a normal saline bolus because its the easiest to shovel in fast AND add antibiotics to. As her labs came back showing she was pretty hypoglycemic, they came in to hang D5, did another dexy (blood sugar check) and she was trending lower, so they ordered D10, and then she started kind of zoning out on us so they had to hand push dextrose boluses and call for the D10 STAT. Fun times. She remained mostly stable that whole time, and i think my own blood sugar was probably bottoming out by then (I’d only had a few bites of pasta salad and a coffee all day and this was about 9 pm) but either way – I never got freaked out. They got us up to our room shortly after all of this happened, and we once again had our awesome “room-coming” to our home away from home, complete with pretty pink blankets for Kendall, extra toothpaste for me, a bag full of little goodies for Kendall- just the kind of warm touches that our nurses always give us! Since I was not prepared in the LEAST to stay the nite (I had, as a superstitious precaution, put a tank top and a pair of underwear in a bag that i had left in the car, which was locked up into valet for the nite!) – I FINALLYYYYYY got a pair of hospital scrubs!!! And a cute little bathrobe that adds that “did she just walk out of the mental hospital” flair to any outfit. i love it. I want to wear it all the time. It goes well with my shoes that had broken completely the nite before in the ER and that I had used a whole roll of medical tape to stick back together, and was wearing my taped up shoes with the grippy socks you get from the hospital…so hot. And dangerous. I definitely looked and felt like P.Diddy.
ANYWHOO. We settled in for the nite, and I knew that if I got shaken awake in a few hours, we were in trouble, but that if we made it to the morning, I was probably right in saying she was just super dehydrated. We made it to morning. She actually behaved all nite, no crazy stunts, and my awesome dad was able to leave work after a meeting to come up here to relieve me so I could go gather up all my babies and get us all back in the same state (well, except for the sick one…) I got the big girls from my cousin, grabbed some food for myself finally, drove home as fast I could, stopping at the mall to get the director’s present for the big dance banquet that nite (I was in charge of the gift for Karissa’s team and of course what I needed to get was only at ONE STORE, but luckily it was on our way home). I just LOVE feeling like my life is an episode of “amazing race” and I’m constantly running (driving) from one location to the next trying to beat the clock. So we made it to my friends house to pick up Kaylen who clung to me like a baby monkey and just started sobbing. My kids break my heart sometimes. I can’t imagine how she must have felt, and and I know my friend did a GREAT job being a mommy to her and shielding her from the fact that Kendall was in the hospital again and instead turning the nite into a “fun sleepover” for her friends birthday, but oh my babies…all of them. It is so heartwrenching feeling torn between caring for the one who cannot be her own voice, laying pale and puny in the hospital crib, and feeling like my others babies must feel like I have abandoned them, favoring the one over them. And Ben in a whole other state…stab me. I just hate it. It never gets easier.
But – its been a good long run this summer of being together. Very few appointments for Kendall, and therapy mostly crammed into one day. And even now, as we still have clear cultures, and she isn’t “sick” – we’re still doing good. Waiting on a decision to be made yesterday with regard to her line status was definitely an exercise in FUBAR – I think at one point I counted five different plans that were on the table, changed, put back on the table, changed….In the end, we decided that doing an aggressive (understatement of the year) wean off of TPN right now (as in no more TPN, no more lines for hydration), while that would be everyone’s wish and hope, was not feasible. Since she is currently inpatient due to “dehydration with metabolic decompensation”, and that was just 24 hours off of TPN, logic would dictate that we probably wouldn’t get very far in a “TPN weaning trial”. Probably like the IL border. As in an hour away! So then the thought was back and forth between another PICC which would have to be threaded in through her right side (harder to do but not impossible) in order to give the vessels on this side a break, and because we aren’t positive that this vein is holding up real well anyways. In the end, I think one of her docs flipped a coin and it landed on the “broviac” side. Surgery then got involved and the discussions involved placing a line in a kid who STILL had a fever and was on three meds to treat a possible line infection (even though no one really thought she was infected). So we compromised, stopped the antibiotics, and put her on the OR schedule for Saturday morning.
which will mean a LONG day today of just sitting here, but we are doing good things still. I just had a good long talk with her dietician about goals and starting to wean her off of the TPN support, and am excited that we are FINALLY starting to make forward progress towards pushing her gut farther and harder. We are getting an actual fluid protocol put into place for the days when she just needs that little extra boost (to hopefully AVOID situations like this!!!) We are restarting some meds that have been stopped for various reasons, and addressing with an AWESOME attending (main) doctor some of the frustrations we have had regarding the continuum of care from the upper levels here. The big girls are with their wonderful Memaw and Poppa and are, I believe, on their way to a jellybean factory right now (my dad is trying to time the height of the sugar rush for the precise moment Ben should be getting them). Ben is on a flight home right now, and in a few hours we should all be together for a little bit. I think our air conditioner parts have been ordered so our house will soon be cooled off from its current high of 90 back to a more reasonable/breathable temp of ….well – whatever it is – it will be better than what it is right now!
And then HOPEFULLY if little missy behaves, we’ll be home together as a family tomorrow nite, in our cooled off house!
(but i’ll still probably be wearing my hospital bathrobe).
thanks for checking on us.
(or you can address me by my new hip-hop name, T-cup).