We checked in to CHW Milwaukee just after midnite. That confuses me on how to say it. this morning? last nite? whichever. I signed discharge papers on the 29th and signed admit papers on the 30th, and in between we had one heckuva fast ride from Park Ridge to Milwaukee. Kendall GREATLY enjoyed sitting up in that big bed all by herself surrounded by all her pumps and machines and new stuffed puppies and new pretty blankets and all the gushing of the entire transport team from CHW. On the trip up she got to watch Veggie Tales in large screen high def – I had no idea they had such a rockin’ system in ambulances these days! I think it mighta made T-pain jealous of the screens in the dash watchin Saved By The Bell….(nevermind if you don’t get that part – its from a rap song…)
anyways. It was a long two hour process to go back through history with a new team, get her all switched over from the ambulance machines to the ones here, order the right fluids, etc etc etc.
I passed out finally somewhere around 3 pm and didn’t hear a thing till Genetics woke me up for morning rounds at 7.
And that was enlightening. essentially, he told me that he made the call to transfer her here because he was definitely under the impression that the doctor he was talking with (imagine this – it was a resident) was very much incapable of handling Kendall’s complex care needs. i told him he had a good instinct that he should continue to trust because that is exactly what was happening. We spoke for just over 2 hours about many different aspects of Kendall’s care and I don’t even know how to sum it all up, except that he is of the firm belief that Kendall is far more “mitochondrial” than “methylmalonic” – meaning that she presents like and should be treated like the mitochondrial disease is the primary factor, not the methylmalonic acidemia as the primary issue.
To that end, he definitely understands that the end goal here is not just to re-start feeds and send us on our merry way. The goal of this transfer is to actually MANAGE her metabolic care. And we don’t quite know what all that involves – be that assessing why she dehydrates even on feeds, or what functionality her kidneys have that may be affecting this situation, or just doing further testing on her GI tract itself. But we are at least on the same page that the level of care she has been receiving has been rather like a band-aid on a surgical wound, which is why we sought help in Pittsburgh to begin with. This doctor, who is new to the Genetics team here in milwaukee, definitely has a good sense of both the academic approach, as well as understanding that a lot of clinical medical papers that say “x, y or z should happen”, don’t actually help most kids/parents on a day in and day out basis.
He did a thorough job of reviewing different systems with me, discussing a few possible options for help (stool cultures, starting more of the “mito cocktail” to help support her mitochondria better, coordinating more consistent fluid support locally, etc.) – and then continued to check in with me as we switched her primary team over from genetics to GI. Luckily, they are on the same floor here so we didn’t have to physically move rooms, but – the timing of all of this meant that we missed morning rounds with the GI team, so we are still waiting to hear anything new that they might have to throw in to the mix as far as thoughts on how we go about testing what kendall’s hydration/nutrition status actually is.
We also had a lengthy talk with speech path – which is to help us assess kendall’s need to go “NPO” as I talked about in another post. Anne has walked us through more than a few of kendall’s swallowing issues, and was very open and honest with me about the fact that we just can’t really say one way or another whether kendall is safe to continue eating/drinking by mouth. We are left with this balance of “quality of life” vs. “risk of aspiration”. And kendall’s issues are very closely aligned. Her quality of life (being able to eat/drink orally) has a very high risk of aspiration. But – there is also the risk that she is just refluxing it up from below, up through the fundo. We discussed allowing her to feed while draining, so she is getting the comfort of eating, but not dealing with anything actually being in her stomach for very long. But – bottom line here is that she is being referred to the feeding team for their take on it. hopefully we have her all better by Jan 18th so they can assess her accurately and help us make a better plan of care with this regard.
Our complex care team was also by, where they got a mini-breakdown treat from me when i started KIND OF crying about how i just knew something else is going wrong here, but I don’t know what and I dont know how to fix it and I am afraid that we are really going to do some major damage by continuing to force her to have to compensate in ways that we aren’t even really understanding, all because everyone just wants to focus on how cute she looks and how funny it is that she likes to do “knuckles” (fist bumps), and how great that she wants to try to sit up and play – and meanwhile we aren’t really seeing WHAT is bothering her/going wrong at a cellular level. So then we all get to be shocked when she is having issues a mere 3, 4, 8 weeks later. Oh my gosh! i thought she was gonna be all better now!!! Yes. We all thought that. But she isn’t. so let’s try to focus. I think Complex Care got the picture that I was DONE trying to continue managing her issue without any kind of real info and very little as far as true support when it comes to understanding what is going on with her and how we can assess things better at home. They are going to probably have Dr. Gordon
ride roughshod talk with GI to let them know that this is NOT about starting feeds up right now, this is about figuring out the underlying issue that is preventing her from utilizing and absorbing what is IN those feeds.
To the degree that this may not be a GI issue inasmuch as it may be her kidneys not doing their job appropriately, complex care is also going to make sure that Nephrology is on standby to come consult as necessary and give us their two cents. And just to keep life interesting, we may have Endocrinology come join the party too because her sugars are doing some crazy things even while on the sugar water IV. Nothing dangerous yet – but enough that we may need to take a harder look at what part of this situation is causing issues, and what part is just an innocent bystander being dragged in by association.
Even with all of that – we still have hope to be home sometime this weekend. The bottom line is that we just may not get any more solid answers than “mito progression”. Dr. B from genetics was very open with me about that fact. Mito = progression. We don’t know where, or when, or how much. But as those cells die off, they just aren’t able to keep up with the demand to create new ones that are able to do the appropriate job. Trust me – if you don’t understand all of this mito talk – its’ ok. Not many people do. Not even some of the doctors themselves. Certainly not me.
But we are here, she is in great hands, and we are on the right path to possibly, hopefully, maybe getting her some real help and support. We are definitely turning around this acute issue of acidosis. Right now the working theory is that it was just a “mito crash” – she just pretty much ran out of energy because she got so dehydrated. Or she got so dehydrated because she ran out of energy. We’re still trying to herd all the kittens on this one and sort out what’s what.
Thank you from the bottom of my heart to all of you who have reached out or are reaching out to us in this time. i was overwhelmed at the amount of love and support pouring into my inbox from all of you last nite in response to our “need help with” list. I will try to update that later tonite or tomorrow possibly if we have a better idea of whats going on by then.
I don’t even know how to thank you all. And to those of you who say you can “only offer prayers” – trust me, that is enough. To my dear Coke/card fairy from this morning – you filled me with such a sense of awe and strength by your humble spirit in bringing something SO very needed, i can’t even begin to express it. And no, you are not crazy for coming here. I pray that God blesses you and your family for what you did for us today.
To the other angels who have been dropping stuff off at home or in the mail – thank you doesn’t even begin to cover it. If you all don’t stop soon my eyes will be permanently stuck in “ugly cry mode”. And you don’t wanna see that.
If you have been trying to call – my cell phone VM box has been emptied – so keep on trying. I don’t get the best cell coverage in the room here, but when i get to go out for a break I love hearing the messages.
We feel so very loved and supported by all of you tonite. Thank you.
all of the Atkinsons.