Grab a cuppa…

whatever it is that gets you through long reads – cause contrary to my mother’s advice (don’t worry, the shock of this will wear off for her soon enough) – this one’s gonna be long. at least i think it is. it’s been a few days removed from the chaos of last weekend. I don’t think i’ve necessarily processed through it any more, but I am least a little more numbed to it.

first things first – she is doing good now. Today she was still just the slightest bit “off”. Unfortunately everyone is except for karissa. I DREAD thinking that something is about to hit our house. I know Kendall’s not ready for it, and I just don’t know if I am either! Ben leaves again on Monday for a long (time) and long (distance) trip again. When he drives, i consider that close, even if its down to podunk southern IL or up into packers territory. When he gets on a plane – that’s the tough weeks. Especially when 3 out of 4 may be fighting some random bug. BUT – hopefully that is just my overactive imagination and they are all just being abnormally brattacular and will be over it soon. but overall – she is much much improved from where we saw her this weekend.

the Iv fluid plumpness (water reltention) has worn off. Kidneys kicked back into high gear yesterday, and she is back to her normal bony self. It made me a little sad to see. i had hopes that maybe in some weird opposite world kendall way she had actually gained weight while being so sick. No such luck it appears. We have her official nutrition appointment on monday, so we’ll see what damage has been done then. I spoke with her nutritionist today and we both agreed that our goal was going to be that she has held her weight. IF she has managed to stay the same, it will be 2 months with no actual measurable gain. (of course she has gone up and down, but for her growth curve, no gain to plot). And while I do think her clothes are adorable – i would love to be able to put NEW outfits on her, things she’s grown INto. some of you last year were so foresightful to get her outfits that were sized 3-6 months that were for winter wear. Luckily – she MIGHT fit into them by this winter! she really doesn’t “look” that bad though, for the most part. If you see her, you don’t think – oh my gosh she’s so TINY! Well, ok some people do. It’s funny the disparity between the medical community (who all think she’s totally average for her age), and random people at grocery stores and whatnot who CAN NOT believe she is 10 months. They act like she’s a baby doll or something. And I guess compared to the 19 lb baby born in Indonesia today, she probably does seem tiny by comparison!

why did i get off on a size tangent again….

anyhoo.

I am avoiding the issue.

Which was this – I was very very very scared for her this weekend. Out of the five hospitalizations she has endured, i have only been scared of losing her three other times. The first was in the NICU, when I knew nothing, and had to learn to see my baby through all those wires and machines. I had never had a baby who needed monitoring before, let alone all of the interventions kendall had in place. I was scared that she would not be normal, scared to bond with a baby who i might lose. But i think i was scared because i just didn’t know any other emotion to be at that time. the second time i was scared was the nite of her first admision in february, with the RSV. When i woke up in the dawn’s half-light and saw how grey and limp she was, i knew she didn’t have long to hang on. I was scared that she would die because i had taken too long to pay attention to her signs. I was scared that she would not be able to breathe anymore, and i didn’t know what else they could do for her. the third time I have been scared was not even during a hospitalization. It was Maundy Thursday, the day before Good Friday. I actually typed out a gutwrenching blog entry, essentially saying to God that i wasn’t ready to let her go yet, but that I felt like she wasn’t going to make it much longer. At that point we could NOT get nutrition into her and NO ONE was taking her seriously. And then the last time I was scared we were losing her was this past Saturday afternoon and evening. I think I had put on my Facebook status that I was “very worried for her”. Even that was hard to put out there, let people know that i was anything but optimistic that this was a simple fix. But my true emotions were so far beyond worry…

I don’t want to rehash everything. Not yet. maybe not ever. Suffice it to say that when the resident had to ask ME what I thought “we should do” for her, I thought i was going to hit someone. Or faint. Or hit someone as I fainted. i wanted to scream at his N*Sync-littlepunkself – “YOU’RE the one with the initials after your name Dr. Dimwit – YOU figure that out!!! And do it QUICKLY!” So i suggested a course of Iv treatment which he reluctantly agreed to. Five hours later, when her sugars were going HIGHER rather than stabilizing, he smugly comes back and announces that my treatment wasn’t working. he is lucky he can still walk, stand and talk and that he has a fighting chance of possibly being able to pro-create someday. I could tell he was in over his head when he told us that he couldn’t figure out why her liver wasn’t stepping up to help the situation (ummm, could it be because I already told you we KNOW something is wrong with her liver function???), and that if her kidney’s didn’t “wake up” soon they were going to have to do something more drastic. I think I got that info in to my updated blog post that nite. I was still in shock a little at that point. Looking back now I should have asked for them to discharge her and driven straight up to milwaukee. But at the time, it felt too surreal. her nurses were all very good, and could see that we were clearly dealing with more than just “dehydration pursuant to gastroenteritis” (which was her official discharge diagnosis), but no one could argue with that punk resident.

Until Dr. A came in. As usual. I love how that man can kick booty and take names, with his calm quiet demeanor and almost unintelligible accent. He is unequivocal in his advocacy for Kendall in the face of “lab values being normal for expected”. He gives not one rip what one set of labs say when there’s three other highly abnormal ones and a clinical presentation slapping him in the face that SHE NEEDED HELP and he was going to see that it was provided for her. Between he and Dr. K (her surgeon, who was eventually able to get her tube in on Monday), a lot of forward progress was made while we were in.

so anyways, there it is in black and white. We almost lost her on Saturday. Not because doctor’s were having to employ heroic lifesaving efforts – but because they could not see how much distress she was in. They were looking at papers and lab values, not a limp lifeless baby who had vomited over 75 times by the time it was all said and done (in 12 hours). They were too busy patting themselves on the back with book knowledge, instead of looking back at their Hippocratic oath. She was saved by the mighty power of prayer, and i don’t care how much you do or don’t believe in that – I KNOW IT. There is no way she would have pulled out without it. Her gut had shut down to the point that bile was backing up from her intestines out of her stoma at quite an alarming rate (and color!). Her liver was at a very low percentage of function. Her kidneys were, for all intents and purposes, on the Injured Reserve list, not even suiting up. I am about 75% sure that at one point she was having seizures due to the lack of appropriate sugar maintenance and the possible buildup of acidic substances in her blood. (I videotaped one episode to show to her neuro for further evaluation. i could be wrong, but I know it definitely wasn’t right or normal what she was doing. Something neurological was starting to breakdown due to the other processes at work.) Four major organs headed toward massive calamity. About the ONLY thing that seemed to be working was her pulmonary function, and even that was pretty shaky at times. Her heart was working very very hard that first nite to keep her stable. I will say this – I was not even this worried about her when she was in PICU post-surgery in June. I knew she was in pain, sure. But I was never once worried that we were losing her.

And i guess that’s about all I have to say about that.

i don’t want to re-visit it again. I want to put it back in it’s nice little black box and padlock it up and shove it to the back of the {cluttery} closet that is my brain and leave it there. I am ready to get back to sunshine and rainbows and cute little woodland creature scenes now thanks.

so this isn’t exactly pollyanna-ish in and of itself, but it’s at least not death and doom. I tried to call milwaukee to schedule Kendall with the endocrinologist as we had discussed at our last visit with genetics (a few weeks ago). They said they were going to start the referral process,a nd clearly, after a blood sugar induced hospitalization, i was pretty gungho about getting that appointment. Except it turns out that you DO have to have a referral, especially if you’d like to be seen before 2018. So I call our genetic counselor and he actually answers! Tells me I was on his list to call today because they have actually been discussing Kendall’s case further and have decided that they DO need to send her muscle biopsy for the ETC testing and they DO need to see her quicker than November and they DO also need some skin biopsies. i was, quite honestly, taken back by this news. This is pretty much 180 degrees different than their attitude had been a few weeks ago. Back then, they were pretty sure we were looking at more of a chromosomal abnormality than a mitochondrial process. Mito was not even on Dr. D’s radar he so claimed. Leave the sample alone, we might need it in a few years if new tests come out. She’s too healthy for that. Blah blah blah. We usually don’t see patients for followup for 5-6 months, but why don’t you come back in three and see where we’re at?

I don’t know if they had conversations with colleagues that changed their minds, if they received something diagnostic in the tests that were run ($4600 worth of blood tests – ACTUAL insurance amount, they were charged closer to $9000!!!!! They better d**n well have gotten sOME kind of indication for that much!!!), or if they just really felt strongly that this needed to be ruled out. What made this news all the more fantastic was that Dr. A and Dr. K had come to the SAME CONCLUSION on monday. We have to do something with that frozen sample, and we HAVE to have a look at her ETC.

• {skip this part if you don’t need Terra’s intro to nursing 101 version of the ETC test – *beep!* Turn the page…
• ETC testing stands for Electron Transport Chain and is the process (simplistically) by which food is broken down into energy that is transported into each and every cell in each and every organ in your body, thus enabling you to live, operate, eat, digest, function, think, see, hear, pee, walk, talk, spit – you get the picture. It is how your body uses fuel for energy. If something is broken in this “chain” of getting energy into your mitochondria (what runs each cell, part of your DNA) – then you have a mitochondrial dysfunction. I’ll get more into what that means later, if any of this actually has bearing on Kendall’s situation. But that’s a short version of what we’re trying to figure out. don’t ask me why you need a muscle to test this. It’s just what happens. fools fall in love, the sun rises, ETC testing is done on muscle. Period the end. Thank you for reading. *beep*}

Anyhoo. the fact that FOUR out of her 12 doctors are on the same page floored me. Because they ALSO happen to be on the same page that *I* am on for once! wonders never cease. They will be sending her samples to Cleveland and Baylor – two very good labs from what I understand, to run this highly specialized testing. It will still take weeks to possibly months to hear results, but at least it is starting. The labs have already started the process of proper specimen handling and transport, so that is a good sign.

We also have a phone conference with Immunology tomorrow (Friday) to discuss the results that they have gotten back. I am so hopeful that they will have figured out what she can and can’t eat. That we have SOME direction to start taking her in. she is so ready to eat like a real baby. She’s nowhere READY to eat like a real baby yet, but her little brain wants to be there! What i mean by that is that she definitely knows what food is, and gets that WE are eating, but (prior to being taken off food), if you give her anything more solid than stage 1 baby foods, right down to the wrong pipe it goes. Or she swallows it whole (or attempts to, and the ensuing chaos of doing the “baby heimlich” is just too much excitement for one fry). Anyways – she still has a long way to go before she CAN eat like a baby, but she sure does want to!

So that’s what I know right now about her medical status update.  Oh – we DID get an appointment for endocrinology – this Tuesday at 2:45. Since Ben is going to be in the great state of Pennsylvania – i may be calling on those of you have volunteered to be my “backups”. The sheer logistics of bus stops, driving, kaylen, traffic and the million other things that tend to go wrong on these days is making my brain hurt. And then we get to do it all over again the next week too! And i am relatively positive that genetics will want us up the week AFTER that just to be cruel.

Thank you so much for all the continued prayers and support. The little cards of encouragement and the little treats are so often JUST the pick me up that I need. you are an amazing group of friends and family.

I hope you all have a GREAT weekend. It’s  supposed to be pretty nice weather around here – I hope to get at least one more good cookout in before the snow hits! (ok by that i mean that i hope Ben will grill out steaks and mushrooms for me one more time because I have been forbidden from touching anything as flammable as the gas tanks on the grill with a match in one hand ready to light the burners.)

TGIF!!!

 

terra