This is the medical update. For those who are here only for my fantastically witty posts about fluffy stuff….
there’s some laundry over in the corner you can help fold instead of reading this post!
I was going to do two separate posts about yesterdays’ immunology appointment, and tonite’s UMDF family meeting with Dr. Cohen (a mito doc from Cleveland Clinic) – but they kind of are competing for space in my crowded brain, so they’ll probably more easily form into one post full of medical stuffs.
First – immuno appt yesterday. At 8:30 in the morning. That I remembered at exactly 7:08 in the morning. It was crazy but we made it. Dr. Chung was amazing. One of those docs who walks in with questions for you, cause he’s already read every single piece of info in the computer about your kid (which, since the dreyer and advocate systems are connected, turns out to be an actually quite a lot!)
It was a two hour appointment, including the impromptu skin/scratch testing we did on Kendall. She was not a fan.
BUT!!!! – she is not allergic to kitties!!! Of all that I have obviously done wrong in growing/carrying/birthing/nourishing her, this is one less thing I have to feel guilty about! So anyways – this just went more towards one of the major “takeaways” from this appointment –
1.) Kendall does not have “typical” asthma, or even typical allergies. Dr. Chung believes that her asthma is of a chronic inflammation nature. Meaning that in spite of a surgery to stop the reflux from below, and the draining of her stomach contents so that there isn’t anything (in theory) to reflux, and the feeding of her nutrients even farther down her GI tract into the J-tube, and the thickening of feeds to limit her aspiration risk from above – in spite of allllllll of that – she is still somehow getting food into her lungs. Now, without hooking her up to an x-ray 24/7, this is just a theory, but the way he went through her labs/x-rays/swallow studies/clinical presentation to me, I gotta tell you, it makes a ton of sense.
So, ok. Her lungs are always (or almost always) dealing with trying to clear food out of them, and are therefore nearly always inflamed. BECAUSE they are always inflamed, any little virus that affects her Upper Repiratory tract, will immediately aggravate her lungs. Because energy is then diverted away from her muscles when she is fighting an illness of any kind, she’s at even more risk of aspiration than normal, so she is probably aspirating or at least micro-aspirating even more. And while her bronchoscopy in august showed no major damage as of yet due to all this inflammation, we know that they are headed there because of the degree of inflammation they did see on that bronch, and the fact that they would bleed from the slightest touch of the camera. And that was after about 3 “healthy” months that they were looking at her lungs. Blah blah blah – it’s a matter of time.
2.) Arguing over which meds are better – the ones pittsburgh switched her to, or the ones Dr. A has had her on for a year now? (which, Dr. Chung actually knows and respects dr. A – so that helps a lot – and they are both typically on the same page). And really, we’re talking about a po-tay-to/po-tah-to difference here (Qvar/Flovent), but in my mind, it’s a devil I know type of scenario. I saw her vastly improve on the Qvar. I saw her get RSV on the Flovent. I know the Qvar isn’t a magical pill. But I wanted him to tell me that the flovent was a miracle drug, or i was gonna switch her back. We really went back and forth over this for an incessant period of time, considering my children were throwing gloves all over the room and running poor Nurse Cindy ragged on the floor…Anyways – I think we agreed to keep her on the Flovent through her next respiratory illness, then switch her back to Qvar until the summer. Just to switch it up and keep covering both her larger and smaller airways.
3.) IVIG – the age old debate. Typically IVIG is used in kids who have what is called a “Primary Immunodeficiency” – meaning their immune system doesn’t produce enough of its own immunoglobulins, so they receive IVIG – intra-venous immunoglobulin, a blood product that offers a “fake” immune system to its recipients (and i know that fake is not the word i am trying to say there, but its late and i am losing vocab words!) Anyways – kendall isn’t a typical case for IVIG (I know, try not to be shocked by that.) And its not like you can just inject this IVIG in and call it a day. It comes with a large set of risks of its own, not the least of which is infection/reaction to the IVIG, and that’s even IF insurance will cover this super costly treatment. (to which my response is always – ok but they would rather pay for inpatient stays every couple months??? idiots…) But i digress. Anyways – Kendall is still pretty young to be able to say with any kind of certainty that her immune dysfunction is a true dysfunction. Her infections thus far have all been “normal” for the most part (she had the weird uro-sepsis when she was a baby, and its not normal to react to RSV and pneumonias like she does, but ok, we’ll call it normal based on what I know some of our friends have to deal with!). so because of all of that, we agreed to further table the IVIG as a treatment option, until or unless her infections prove to become overwhelming again (like the back to back hospitalizations last winter/spring). My personal thoughts/goals would be that I would, of course, like to NEVER have to even consider IVIG, but if we do, I’d love for her to make it another 2 years before we have to go to this. I don’t know how realistic that is. I am sure it will mean another two years of rather consistent infections – but hopefully we can find a way to start fighting more of them at home. In order to do that, we’d probably need some kind of IV access to run fluids/abx at home, which then opens her up to even more infections…..
Meet Hard place.
Such is the nature of miss KQ!
Which brought us back to
4.) If her lungs continue to be the source of most of her respiratory infections (and included in that would be ear/sinus infections, viral colds, pneumonias, etc), and we have a high suspicion of reflux/aspiration, we will need to consider, ARE STRONGLY URGED TO CONSIDER, taking her “npo” (nil per oral, nothing by mouth to eat), at least on a trial basis, and doing strict J-feeds. Her NPO status would be mainly liquids, even thickened would be mostly off limits,at least severely restricted and monitored – but we would be allowed to find two or three “real” foods that she could still have.
It would suck. no two ways around it. I cannot even begin to imagine how we will accomplish such a thing, considering that she LOVES to get “nnnnaaakkkk” (her milwaukee-accented nasally way of saying “snack”) – but i know that if this is the right way to go for her, she will walk through it with the same grace and strength and happiness that she walks through the rest of life with. Jesus will be with her as He always has been and always is. Somehow, she will receive the strength she needs to give up food, and gain health again. it will break a little piece of my heart off to take food away from her. it goes against every single fiber of my mommy being. But the fact that this is about the 89th doctor to tell us that we need to do this for kendall, and the way he laid it out, gave me a sense of peace.
My compromise was that in the next two weeks we are waiting for her blood counts to come back up (she’s still anemic from her illness of a couple weeks ago), Dr. Chung is going to speak with her immuno team in milwaukee, as well as dr. Natalie and dr. A, possibly the pittsburgh team, and a few other colleagues of his to formulate his plan of attack. This is, at the very least, going to require another full immuno panel, which requires about 8 vials of blood on its own, and probably a few other complementary tests. There is little doubt that she is not ready to handle vaccines again, so our only option is to build her a bubble and turn her into a human hamster. Or spend every 8th weekend in Hotel LG. That could be my frustration talking though…
The rest of the compromise is that while Dr. Chung is thinking, and while kendall is rebuilding her hemoglobin, we will let her enjoy any and everything she wants. Perhaps even a little bit of protein (read: Chick Fil A nuggets!). Cake! Pie! Christmas Cookies!!! Chocolate from Poppa (who then gets frustrated when she smears it all over him!) Live it up – Eat, Drink and Be Merry~ for come New Years Day she will go NPO. And we will trial NPO for three months, through the end of March, and hopefully the end of the bad “sick season”. And see if we can’t help her lungs heal a little bit, and hopefully not be further injured by any other URI’s/pneumonias.
that isn’t quite set in stone yet, but if I talk about it now, it may not be as shocking when it comes to fruition.
Dr. Chung seemed very pleased with the fact that I could at least say it out loud, because, in his words, his threshhold for “forcing NPO is low. very very very low”. Meaning essentially that if she had so much as coughed in his office, I think he would have said “NPO. now.” So – this way I feel still semi-in-control of the situation. It is something that has been brought up HUNDREDS of times before. I know we are probably way too cavalier with Kendall and her PO feeds based on her history of aspiration and crappy swallow study results. this is just a part of her journey, and I know there is a reason for it.
If we take her NPO, and she STILL continues to get hospital-level infections, we will know that her body just lacks the intrinsic energy to mount proper defenses. even if she has all the right “numbers” on the bloodwork, they don’t mean a whole lot if they aren’t being properly utilized. After looking through a few more of her labs and tests from pittsburgh, dr chung made this happy observation: “ well, geeze, she’s actually pretty much a heme/onc patient in the way you just have to dig through her bloodwork to find the real source of the problem!” (Heme/Onc, for the uninitiated, is hematology/oncology, or cancer.) He wasn’t saying she has cancer – he was just saying that we might need yet another specialist to come on board to help us better filter through kendall’s mystery labs, or at least have a better framework for understanding why certain labs of hers will act a certain way. For instance, instead of elevating, which would be what 99.8% of the human race would do in “x” situation, kendall’s values will plummet. Or vice versa. Pick any lab value – WBC’s, ammonia, creatinine – hers will almost always be diametrically opposite of what you would expect it to be given the circumstance. This makes it very hard for ANY doctor to really know what to do to treat her, or IF anything should be done to treat her – let alone Doogie Howser, ER resident who is barely of legal voting age.
And this, dear people, is why I must use dye from a bottle to cover the gray hairs.
Because I will quite often have a child who *I* know is sick, and who looks to me like she is seconds away from needing to have called 911 for transport –but to an ER doc who doesn’t know her, she seems “mildly ill”. this is also part of why our nurses in home are so important. They are starting to pick up on Kendall’s “baselines”, and the little variations she tends to make from that baseline that indicate that “something’s up”.
So if her body lacks the functionality, then we would know that IVIG truly is the best course of treatment/therapy for Kendall right now, at least for a temporary trial (6-9 months, receiving an infusion monthly). At least we would know then that we truly had exercised every other option/resort before going to the IVIG.
So anyways – Dr. Chung is officially “on the case”. We are going to enjoy the rest of this awesomely awesome magical month. We are going to pray without ceasing that she is protected from illness, and do our best to keep her in a bubble. We are going to use her oxygen and hook her up to the backpack as often as she needs in order to stay stable through Christmas.
And if the new year brings a change to all of that, we will deal with it then.
And apparently i AM going to have to do this in two updates!
so stay tuned!