That was Dr. A’s assessment yesterday at Kendall’s routine 3 month checkup with him.
“she’s doing and looking great – and I don’t say that to minimize all that she has been through or that she still deals with every day. I say that because I know how hard you have had to work to hear those words – and so I tell you them today – she is doing great! NOW – let’s discuss how ve going to keep her this way…”
He is so amazingly special to us. He knows exactly how to word things just like I need to hear them. And he just knows his stuff. Anyways – we reviewed the huge packet from pittsburgh with all their specialists notes and tests and results and whatnot. Basically he told me that he knows why we had to go there, why we had to get confirmation from a “mito center” that mito is what we are dealing with, and to get everyone on the same page with regard to her myriad issues under one common “mito umbrella” – but he said that really, nothing has changed from how we have been or will be treating her. (Which is true – as long as HE is her attending – but when we are in milwaukee or the ER there at LG, different story!) So we discussed that – if we absolutely HAVE to come in through the ER, use the protocol letter, but he has put into her file already that no matter what her presenting issue is, he is to be called as the attending. The reason this is a good thing is that it means we may not have to trek up to milwaukee this winter for every little illness. Whereas last year we were still kind of being bounced around by everyone not knowing what the real deal was (Dr. N her ped wants her admitted to whatever hospital she is on service at or LG, but LG would not take her if it was a GI issue, and who wants to fight a snowstorm to drive 4-5 hours up to milwaukee only to be told to give her more fluids, etc etc etc) – this year we know that for most issues requiring “stabilization” and/or a short course of IV abx, we can probably still come to LG. As long as her gut isn’t completely shutting down, we won’t try to have the
idiots GI team put in their two cents (which is still overpriced in my opinion).
Between the bronchoscopy and the sedated echo – which was a lot of mumbo jumbo even to my medical journal reading self – Dr. A was able to see that her left airway floppiness is probably a large component of why her heart has to work so hard, which is probably a large component of why she needs oxygen so randomly. He broke it down really well and I wish I would have recorded it or something – because it was kind of like “Mito + Kendall 101”. Her body being in a near-constant state of “brownout” energy wise from the mitochondria not quite functioning correctly means that she has hypotonia. Not that she is always a floppy noodle, but that her muscles inherently have the strength of a stretched out rubber band. Since we have a pretty good idea that this is systemic/body wide, we know that her hypotonia contributes to her weak lower GI tract (necessitating the GJ tube to get nutrition moving to the proper place), it contributes to her bad reflux (which is why she has the fundo wrap), it contributes to her less –than-ideal swallow (which is why we now are thickening her oral feeds), it contributes to her weak accessory muscles (which is why she has crappy lung health, cause she can’t ever really inflate her lungs/move her ribcage like she should to get full deep breaths), and all of this is what we deal with when things are balanced and HEALTHY. This is her looking “great”. Add in a little virus, hot weather that throws her autonomic stability down the toilet, cold weather that does the same thing, being too tired from too much therapy or a fun weekend out with the family – and bam. Domino effect in action. Her body starts robbing Peter to pay Paul for this energy debt it creates trying to throw energy at whatever body system needs it the most – and that is why we end up on oxygen on a monday when she seemed fine on a Saturday.
So – what all of this means to us is that we now have proof of what has long been suspected. Her lungs and her heart are working pretty darn hard. And praise God they are keeping up with the demand thus far. Her little brain is SUPER smart and knows to suck energy from other parts in order to keep these two main things up to par. So – it makes sense then when some days she doesn’t seem to see real sharply in focus, or her hearing is about as good as her Poppa’s (Ronn, not Bob!!!), or she is doing the drunken sailor sloshy walk – those are days when her body has decided to temporarily suspend shipping energy packets to those systems so that her main systems can have the limited supply that is on hand. If we can get her to continue taking good restful naps, give her a little boost with some extra o2 at nite, cut back her risk of infection as much as possible, and keep her gut hydrated – we give her body the leg up it needs to continue making enough energy for all systems to have what they need. And sometimes we just won’t. And that’s when its time and when its "ok” to call in reinforcements for her – more neb treatments to keep her lungs clear, more prednisone to keep swollen airways, swollen guts, swollen bladders from becoming more inflamed, more fluids through the j-tube, more daytime o2 – and if all that is still not enough, its time to head in.
Again – nothing that is surprising. nothing really that entirely different from what we have always done. But now we KNOW the process by which things are seemingly coming out of the clear blue sky, so we can better analyze when to make the call to get her the help and support she needs. this, for me, is huge. I have always hated playing the ER guessing game – and now I have a little bit better grasp on when to go in and when and how to ride it out at home.
The only big changes he made for now are that we need to be a little bit more aggressive with her maintenance nebs during this coming up cold/flu season, and when we see her having her “blue spells”, immediately start a rescue neb and put her on o2. Leave her on the o2 for a few hours and if she is still bronchospasming, take her in. (can you hear me groaning and rolling my eyes at this?ugh. I am so bad at nebbing myself even when i can hardly breathe! I feel like this is a recipe for a one way pass to inpatientsville from december through april!!!) But – you know, maybe she won’t need any rescue meds at all and we’ll be a-ok! The other item that came up during this discussion was that Dr A was able to extrapolate from the pittsburgh reports that because her heart is doing so much work to compensate for her left airway collapsing so much, she is likely having essentially “asthma attacks” that aren’t really looking or sounding like asthma attacks, and that the reason we see such improvement on the o2 even at times when her vitals seem ok is because we are allowing her system to “relax” a little by giving it the oxygen it is working so hard to pull out/exchange in her one good lung during those times. Ummmm…..yeah….good point…..(me feeling like a sheepish idiot). I mean, I’ve only had asthma myself for some 20 years geez! It’s not like i actually have a good understanding of WHAT it all means –but you would think that MAYBE i could have recognized what was going on in my own kid??? Anyways – i felt dumb when he had to spell that out for me. But it makes so much sense….
And, in our never ending battle on this issue, he seems to have gained an upper hand with the trump card of the “swallow study”. @#(%)*@# At least we have a good compromise. She can have her oral feeds (bottles mostly), as long as we thicken EVERYTHING to nectar consistency. And she can still have her hard crunchies (cereal, toasted bagels, firm noodles) – but we should try to limit her drinking as much as possible, and at the first sign of respiratory illness (be it a cold or something worse), she is supposed to be basically NPO (no food by mouth). the ONLY good part of this is that I think even Kendall is starting to understand that food is sometimes what is hurting her, and has begun to be much more self-limiting in how much/when she wants to eat. of course her “bubba’s” are a whole other issue, and she hates when we thicken them, but we’ll cross that bridge when we come to it.
So there you have it. The latest and greatest on miss KQ. She is actually having a GREAT week. Her GI called in some steroid cream last week for her tube site (which was turning into a literally hot bloody mess), and that is seemingly calming down nicely, to the point where i am not not as scared that changing out her tube will cause massive skin tearing/breakdown. And now i need to schedule that fun trip up to milwaukee, unless I can somehow con her GI into calling in orders to children’s memorial and seeing if we can do it there. It’s at least a little bit closer to home! So – we’ll see. either way – it has to be done soon! i have her balloon so full of water that you can see the outline bulging up through her tummy skin! But it was the only way to stabilize it better so her site could heal! Never a dull moment around here. Less freak-outty ones than others…but never dull!
Thanks for checking on us!