Goodbye Summer!

Holy hot day today. i was actually shocked to see the car temp gauge thingamajigger on the rearview mirror (you know – that thing) reading 95!!!!!! On Sept 21! Which I guess, is technically the last day of summer. It just seems a little late in the game to be THIS hot! but I will take it! i have LOVED how hot and humid and SUMMERY it was this summer. I hate winter. Well, I should say, I hate winters here in the cornfields where its FUH-REEEEZINNNGGG, and no snow to accompany it. Cold is pointless. anyways…

It was gorgeous weather and I wanted to bottle it up so I could open it on some gross freezing January day when I am sucking down steroids to keep my lungs open and shoveling tylenol into many small children to keep their own crappy colds at bay. (Isn’t that such an amazingly happy thought??? I know. Call me Pollyanna Sunshine.)

I feel like I have (as usual) 8934 things to do before this weekend, and all I want to do is go to sleep. But I am waiting up to give the final dose of meds to Kendall for tonite, and killing the time by blogging. And since I don’t have anything astounding to say other than – dang, I have a crapload of stuff to do and no energy to do it right now…I’ll ramble a bit. consider this your forewarning to hit the little red X up in the right upper corner of your screen! I promise my feelings won’t be hurt!

Anyways – I have wanted to do a post like this for a while  – so why not now in honor of mito awareness week? Now, to be honest, I am not sure how much true “mito education” this will be, because a lot of it I am still trying to figure out for myself. But here you go – a glimpse into what life is like for Kendall in the OTHER 22 hours of her day from what most of you see her in! Here is her bedtime routine: First, prepare all your supplies – mix formula, open new sterile bags, draw up meds (this pic is ALL of her daily meds, including her PICC flushes when she was on that). And that little group to the far right? The crappy IV meds we have to reconstitute – I say crappy only because of the insane amount of time it takes me (and now her nurse!!!) to prepare! They are actually good – all of them are – in that they really do make a huge difference in her functioning, every one of them.

Next, take a freshly bathed baby and wrestle her floppy little self into pj’s. These pics were taken a few months ago before we realized that she wasn’t actually absorbing any meds through her gut. She used to take a couple of her meds orally (sucking on the syringe by herself), but now that we NEED most of these to actually be absorbed/metabolized, they go through her J. So she gets just a couple ml’s of tylenol or gatorade in a syringe to keep her entertained while I do everything else that has to be done.

This all actually takes so much longer in real life that I have more pictures than words here! So – I’ll just do my usual rambly nonsense to fill in the gaps…

anyways – so the other pictures entail her modeling her long tubey (PEG-J), which is obviously now the much easier to handle G-J button (GJ = gastrojejunal, in through the stomach, down to the intestines). Her food/formula bag gets plugged into her J-port so her intestines get a “continuous slow drip” of formula all nite long. Did you know that your intestines do not expand like your stomach does? This is why you can’t “bolus”, or let food go in fast all at once, into a J-tube. Fun fact for the day. A “Farrell bag” (so named for it’s inventor I have heard) is hooked up to her G-port, which allows air/pressure/backflowed food (ie, reflux or vomit) to exit into the bag and hopefully not up through her fundo and into her lungs (since she is laying down at nite). Some nites we set the farrell to “drain” her stomach contents, meaning if she is having a bad tummy day (which can be anything from lots of bloating, to hiccuping/retching most of the day, to just not having had a bowel movement in a few days), we will help her tummy be able to relieve the pressure and hopefully get a little rest by allowing the vacuum action of the bag to pull anything out of her stomach that gets into it. Other days when she is doing ok, we set the farrell to “vent”, which means stuff will only come back into it if there is a LOT of backpressure or air being trapped. This all happens because her gut motility (movement/contracting action of the intestines) is VERY affected by the amount of energy she has in any given day.

She is also then hooked up to her pulse ox probe (we call it “pretty toe” because it lights up red, and because this is honestly the only way I have been able to come up with to get her to tolerate having the probe placed on her foot for hospital stays. she gets INSANELY sensory overloaded sometimes in the hospital, and since they usually want the probe on her to see what crazy heart things she is doing, she has to get used to having this heavy sticker on her foot.) Now normally, since we have learned to “read” her autonomic signs a little bit better, she doesn’t have to be hooked up to the monitor continuously unless she is getting sick. Back when i took these pics though, she was hooked up to the monitor almost 24/7 because this was when we were fighting a pneumonia from home. Notice the amount of tape I have to use to keep the stupid thing in place because she likes to try to pick it off and there is NOTHING like a low sat monitor to wake you up out of a beautiful sleep…(even if it IS only cause the probe has come off!)

and again, she was on the oxygen continuously when I took the pics, and now only uses it intermittently or continuous with illness. Today was one of those days she probably needed it, and now that I look at ALL that she was doing over the course of the day, I should have just TOLD the nurse to hook her up (she kept trying to tell me her sats/numbers were beautiful but they were not “kendall” beautiful, as evidenced by the fact that she was turning colors AND not sleeping all day – two big signs that she is going into autonomic dysfunction mode and needs a “reset” with the o2. So , ok, add that to the list of things I have to do when I get done blogging and go hook her up to stuff.

where was i…

Oh yeah. So she gets hooked up to all of that which leaves a hot trendy mess of wires. If you have ever freaked out over having a bumper pad or stuffed animal in your child’s crib for fear of choking/strangling – try putting your baby to bed with a cord actually PRE-WRAPPED around her head!!!! Yeah – good times! So – we have had to get creative and borrow ideas from many other great moms before us who deal with all of this and more at bedtime.  We gather all the cords up and pull them through the snaps of her pajama leg and voila – a much reduced strangle hazard. She still manages to get SO tangled up overnite…

anyways. So that’s bedtime for Kendall. Do not mistake the cute little buddha belly in the picture for “chubbiness” – i mean, yes, she is definitely finally getting some good meat on her bones, but more often than not her “chub” is painful gas from food that isn’t travelling anywhere in her slowed down system. So she cries for more food because food = comfort, except she doesn’t get that food = pain. Maybe she is now, a little bit. She didn’t want to eat much of anything after this last gastroparesis (the august hospitalizations), and when she did start eating, it was just mac and cheese (with very little “cheese” powder actually on the noodles). That has pretty much been the staple of her diet for almost 2 weeks now. She did have some “dipydipydipydipydip” (translation: guacamole) on sunday, so, you know, she’s getting SOME vegetable in…

Holy cow this post has gone in a whole other direction than what i started with…

oh well.

Anyways – i have other pics – of my OTHER beautiful babies. And a lot of existential talk about “worry” vs. “concern” for the future, and what that might look like for our family. But I might not get to that one this week. It’s probably only good to rip off so many scabs all at once. I don’t know how much emotional bleeding I can handle in one week. For right now, things are good. I will focus on that and hold on to that for right now, because really, that’s all we’re guaranteed. That’s all anyone is guaranteed.

and I’ll keep on keepin’ on. Cause that’s just how I roll.

gosh I should not end blog posts when i am this tired. i am sure this makes no sense!

 

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