Round and Round.

Right now I’m waiting for rounds. Not very patiently. I need coffee badly. And a reese’s cookie for good measure. But I don’t want to miss the team rounding because they will give the update on what is going on as a cohesive report, vs the piecemeal info I get from random nurses and doctors who come in.

Anyways – things are still going well. Kendall continues to look really good – not at all “bacteremic”. Very very good news! So far her cultures from yesterday, both peripheral and off her line, are negative. We are almost at the 24 hour mark so that is also very very good news!

It is looking like the original bug growing is enterococcus, a gram positive bug that CAN be a hospital acquired bug. They are pretty sure it’s not a contaminant as the original hope was, but are still stumped as to how she managed to pick it up. She also has two gram-negative bugs growing in her urine that are still pending identification. Given the NASTY laundry we were dealing with on Sunday and Monday (and the lingering horrid smell in her bedroom still) – I am not shocked by this. Her urine was basically so full of mucus that it looked like she was peeing skim milk.

All of this is making her doctors think that what we did was catch an infection EXTREMELY early,IMG_4658 mostly only by luck (although I tend to think it was probably more God-directed than anything). And that what could be happening is that Kendall is translocating stuff out of her bladder AND gut – but that because she routinely has these kind of “off days” and autonomic temps, *I* am (claiming sole responsibility here) not getting her checked/treated in time, and it’s going systemic. Now of course her doctors aren’t blaming me like this, they say “how could we know? she’s had just as many times where nothing has been wrong as has been times where something is wrong” – and that is true. But still – the conversations have been more about what will our protocol be for getting her “wonky days” checked out a little better. If we can just put a stop to this current cycle of infections, everyone thinks we will be good to go. we had her infection free for over a year – we can get her there again!

(clearly I got a little more sleep and food since my post last nite – I am able to think much more clearly and be more positive about where we are at!) so anyways – that’s what I know right now, early afternoon.

The doctor we have been waiting to have consult on Kendall’s case just came in. And luckily he lived up to expectations! (this may or may not be because he told me that moms like me are to be listened to very very closely, because we will usually teach doctors a thing or two!) Overall, he was just a breath of fresh air. He had read the history of kendall’s latest infection cycle, and just had a creepy understanding of how and why little organisms work in the human body. He will be changing a few of her meds around, which will mean a rather crazy schedule at home (where we don’t have the luxury of nurses around the clock to do all the work!) He wanted to know if I was going to be ok with that. i told him that if i had to administer IV antibiotics every 2 hours I would do it in order to keep this line clean. He laughed at me a little and said “john (kendall’s special needs doctor) told me you’d probably say that, but I wanted to hear it for myself!”

So we will do the crazy med schedule and new line protecting regimen and hopefully get on top of this cycle once and for all. But our conversation wasn’t entirely focused on this acute situation. In fact, we talked for longer about WHY this is currently happening, and what can we do to STOP it. Most CVL moms know/dread the term “cycle line infections”. It is when your body does what Kendall’s is doing – continuing to spew out infection after infection, as soon as the antibiotics are stopped, a new course is needed. Some kids get into cycles very often – other kids rarely have a cycle, and still others never cycle, but get random infections. No one really knows how or why this difference is. But at the end of the day – Kendall is in a cycle.

He found it very interesting that while Kendall has had cycles before, we also had a year+ of having NO infections. The main difference for that? Kendall’s overgrowth meds. I think I’ve talked about these meds before – they are a bunch of super strong IV meds that we get compounded down into liquid gooey nastiness that we can push through her tubes in order to kill the bad/overgrown gut bacteria that likes to leak into her bloodstream. Due to the nature of the meds and the fact that we need them compounded at a special pharmacy – they are NOT cheap. Insurance covers a nominal amount (roughly $30), and Kendall’s secondary Medicaid won’t even touch them because they are technically “black boxed” meds. Basically this summer, we couldn’t afford her meds. And now we have her back in a cycle. Our special needs nurse sent orders to a local pharmacy up here to see if they can get insurance to see the fallacy of their payment reasoning. $250,000 for a monthly hospital visit, or $250 for a monthly supply of drugs. Pretty sure even a politician could figure out that math. We have two doctors ready to write letters of medical necessity to support her needing these meds and hopefully between all of this, we can get our out-of-pocket costs back down to an affordable amount.

IMG_4591 But those meds are just one part of the equation. We will also be starting the ethanol locking of Kendall’s line to ensure that the broviac itself has kind of “bad bug repellent” on it. And when i asked Dr. Havens how to stop the cycle of infections, he looked at me and said “ Stop the antibiotics”.

Alrighty then!

Basically what he means is this (I’m borrowing his analogy – cause i think its so great! And easy to understand!) – Pretend like your gut is a church. And the walls of your gut are the pews people sit in. You want there to be GOOD people in those pew – nice, pearl-wearing, praise-singing little old ladies who love Jesus. (ok i get that theologically this is not sound, but go with it). Good people in the pews of your church – got it?

well, antibiotics come in and make the good people of church leave. Just kills them, makes them get up and stomp out, take their friends with them – gone. And THENNNNn – that leaves room for all the BAD PEOPLE to come sit in your church pews. Tattooed, bike-riding, swearing, drug-dealing bad people are now sparsely sitting in the pews of your church, and letting all sorts of chaos go on in your church (gut). They run rampant, knock holes in the walls of the church, and now you just have a bunch of tattooed Hell’s Angels roaming around your abdominal cavity and taking cruises in your bloodstream. Kendall’s church does not have good people in it!!! So the way to STOP this activity, is to stop antibiotics. Drink Activia, repopulate your church with good people sitting in nice strong pews, and non-holey walls in your church, eat yogurt and leafy greens. Poop regularly – so you can flush the bad guys down the toilet. Literally.

Clearly, that is a great theory for guts that actually function well. Kendall’s does not. So our goal is to keep the bad guys moving, and reduce Kendall’s antibiotic use as much as possible. This will mean that we treat even less of her UTI’s than we already do. This will mean that she will have more pain again – but there are ways we can hopefully lessen the pain, lessen the risk of re-infection, and keep things moving forward. AND GET OUTTA THIS CYCLE!!

So what I came away with is that Kendall does have an immune deficiency – but it is one of functionality vs. one of a “traditional” sense where her blood cells don’t function well. Her immune SYSTEM (the T-cells and IgG and all that good stuff) that is actually functioning pretty well, given what it has to fight on a week to week basis. It is part of why she DOES bounce back from her infections so well once we get the right meds in place to kick the bad guys out of her bloodstream. That is a very good thing! Finally something her body does RIGHT!! But a major component of a HEALTHY functional immune system, is a healthy functional gut and bladder. That is how germs leave your body – through your waste. Kendall’s body doesn’t have an effective waste management system, and THAT is why she is in this current cycle. Microbiology – I do not understand at all. Gram negative, gram positive, rods, chains, bacilli, fungal pseudohyphae – gibberish. Poop? That i can do.  Keep her pooping and thereby we will HOPEFULLY keep her ridding herself of the bad guys. This is so much easier said than done that i can’t even tell you. BUT – it is a plan that I can understand. There aren’t a whole lot of warm fuzzy answers out there right now – but if this is as good as we can get – I’ll definitely take it.

We still have to have a few more conversations with her team as far as when and how do we react to Kendall’s “off days”. How do we know when to treat and when to sit back? Lots of questions still –but i feel like we at least have a compass heading now. SO much better than yesterday. Again I am blown away with the good care she is receiving here this visit. She somehow convinced her student nurse to take her to the playroom today.IMG_4674 Honestly i am not sure how he managed to convince his instructor that he could only work with Kendall today, and that playing tea party with her was part of the care he ‘HAD’ to give her…But up and down the hallway I can hear people walking by the playroom – oh look! Kendall’s in there! Hi Kendall! How are you Kendall! I am SO relieved that she feels good enough to go play in the playroom, as that rarely happens when we are here! She is definitely getting ready for her Halloween character – Cinderella – by being in RARE princess form and ordering around basically everyone who comes into our room! If it wasn’t so brat-tastic it might actually be cute. Our main doctor (her special needs doc) actually sent a text to her team yesterday when she arrived in the ER:”Kendall is in the ED for the + cultures. She is just TOO CUTE! Wanted graham crackers. I got her some. Admitting and ordering abx.” (One of the nurses showed it to me!) And this is why she thinks she can get away with anything! Being cute is actually part of her charted record!

Anyways – obviously a much better update today than yesterday. That is mostly due to your prayers. So thank you for that! Even I feel much better, lighter-hearted even, than yesterday. It is by the grace of God that our doctor ordered those cultures. If he had not, we would have gone on our merry way with me continuing to chalk Kendall’s bad days up to a brewing UTI. The bugs growing in her urine are QUITE nasty (but not entirely unexpected). If we had let her body continue to get weaker from a presumed UTI, when in fact it was already fighting a bloodstream bug, and those UTI bugs leaked into her bloodstream….oy. I shudder at the thought. It would not have been a pretty picture. I am so beyond grateful to God for His constant protection over Kendall, even as I felt like He was leaving her hanging, leaving all of us on our own to fight another infection – it turns out that He had even MORE protection over her than I thought. you just never know sometimes – how even the darkest road you are on may be the very path you need to take to places beyond your imagination.

I hope and pray that all of you are having beautiful Thursdays.

We’ve been asked what do we need by so many of you wonderful friends and family – and honestly, I’m sure I could rattle off a list if I had enough time. But really, I am happy and blessed and content right at this very moment. I have a sleeping baby (FINALLYYYYYY!!!!!!), who is receiving her needed medications as she sleeps. I have a reese’s peanut butter cup cookie from the bakery downstairs. But most of all, I have HOPE again that she will get out of this cycle. Be her happy, pain-free, silly, strong beautiful self again. HOPE that we will be together again as a family by some point this weekend. It’s a good feeling, this hope. It covers an awful lot of wants/needs/desires. It does not mean that everything is fixed, or that we have solved all of our issues. But the warmth of the hope in my chest at this very moment – it is enough. Dayenu. (<—that links to a blog post i wrote almost exactly a year ago. It still holds true today. Maybe even more so. Go check it out again if you don’t remember it.)

I’m going to try to catch a few winks of sleep myself while Kendall is down for the count. She will want to go IV pole skateboarding later tonite (fun sport, you should try it sometime!), and I may try to go hunt down some non-moldy food in the cafeteria too. Thank you all for everything – the words of encouragement, the notes on facebook, the texts, the phone calls. I know I am so behind on responding to everyone and everything – but know that I read them all, and am encouraged and touched by every single one of them.

If things change tonite, i’ll try to do another update. As of right now, I have not heard of any growth past this mornings negative result, and I am hopeful that it will remain so. We will continue to work on tweaking the meds and the schedule tomorrow, and hopefully head back home sometime on Saturday or Sunday. Blessed be the name of the Lord.

Love you party people!

 

T-crest.

(and yes, the pictures are all random ones I’ve taken over the past couple weeks – nothing from in the hospital! my phone is currently full. I need to dump pics off of it in order to take more. anyone want to donate a 64gig iPhone to me?????? ;)  )

#11 of the Daily Decembers.

Why do I do this to myself? Wait for like five days and then try to catch up all at once?

Because that’s who I am, and that’s what i do.

Procrastination – one of the many services I offer. Or might offer. when I get around to it.

So – Saturday.

Started off (not-so-bright) but early with an awesomely fun shoot with my sissy for 2Sisters Photography of newborn twins. We had done the maternity shoot for mom back in September, and we have been anxiously awaiting the arrival of her precious baby girls! it was a good time. until we had to lug out all our stuff in the freezing rain after it was over!

Kendall woke up that morning soaking from leaking from her tube site. No idea what was causing it other than I think she is pretty backed up, in spite of having overgrowth treatment all week. We have slowed down her feeding pump rate from “mildly dehydrating” to “almost guaranteed to cause dehydration if she decides to stop drinking orally”. But at least the leaking lessened. It is just pouring back out into her farrell bag (a bag that attaches to her G-tube to allow her tummy juices to drain all nite, as well as anything coming back up that she might feel like trying to puke up). Either way – something’s up with her tummy cause she is super-ticked whenever you try to get near it, which is highly unusual for her (meaning – do NOT touch me, it hurts like a mo-fo). As is our typical M.O. with stuff like this, unless or until she needs obvious help in the form of IV meds/fluids or other testing to find the source of the pain, we will let her work it out, give her ibuprofen as she needs it, and hope she doesn’t tip over into autonomic mode (where her body’s autonomic system in trying to stabilize the pain or infection or whatever is going on in there swings too far to one side and things start going haywire.)

Like I said in another post – Whack-A-Mole. Fix one problem, and another one or two pop up. but she keeps life interesting, and she is who she is, and we love her for all her quirky craziness. i just hope she is actually feeling better soon.

So I left a crazy leaky Kendall with Ben so he could drive the big girls to dance, Zesty and I went to the shoot, I came home and got Kaylen, and we went to pick up the girls from dance.

gil3 I am working on a “yearbook” type project for our studio, so I went a little early so I could get some shots of the different classes/groups that were practicing at that time. I am so blown away by the amazing talent of some of these kids! We all had a good time at the studio…

and then we came home to get Daddy and Kendall and we all went to Dollar Tree!

I know. It sounds awesome. But the point was that the girls could each pick out something for everyone in the family. The concept was lost on everyone except for Kealey. Kaylen gil4 kept picking out things for herself and trying to convince me that her sisters wanted her to have it. Karissa was just worried that SHE wouldn’t get anything. And ben once again wiped out the freezer of the packs of Icee’s. It was a good thought. And i think they kind of had fun, and kind of got it a little bit. Obviously I have lots more work to do in the “better to give than receive” department!

We image then went to rent “A Christmas Carol” and got some chinese chicken to share amongst ourselves for a good fun family nite in, with the crappy freezing rainy weather that was trying to turn to snow. The movie kind of freaked Karissa out a little (but then again, so does her shadow on most days…), but Kealey was REALLY into it. I remember LOVING the story as a girl about her age. I love pretty much everything by Dickens though. And this Disney version that is animated really captures the story visually I feel like. (ok forgive the lack of grammatically correct/stunning reviews…I should have quit typing three hours ago.) But really, the movie was awesome. I’d like to own it someday for them, watch it every year, see if it ever does start sticking for the middle two! it could be a fun part of future family traditions for our December dailies….

And then we made brownies and hot cocoa and I put the babies’ hair up in curlers as is our typical Saturday nite routine. Kealey spiked a 103 dgree temp, and it is starting to really frustrate me because that is the third time she has done that this week – but she will inevitably respond to tylenol and be fine by the time its school or dance or whatever. She claims to have a super sore throat, has an annoying hacky cough – but that is really it. It’s gotta be some kind of lingering virus – and I am hoping that its not somehow connected to Kendall’s tummy stuff (i know that makes no sense, but kendall usually fights viruses by diverting energy away from her gut first,and THEN acting sick). Anyways – it is very unlike Kealey. She is definitely my high fever kid – but she’ll usually spike to 105-106, be knocked flat on her back and sleep for three straight days with only a few waking periods to take meds for a sore throat, and then she wakes up on day four really weak, but is good as new by day 6. Without fail. She gets this same virus every year, typically in January, early February.

So to have this one being so weird, and so early, is frustrating. And i hope her immune system will just kick it already!

But that was our Saturday.

It was definitely a lot of family fun, family memory and tradition building.

Medicalities.

This is the medical update. For those who are here only for my fantastically witty posts about fluffy stuff….

ummm.

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…..

rock.

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.

I know.

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!

 

terra.

She’s got a big head.

Why, yes, yes she does. The “she” here being referred to being, of course, Kendall.

I am thinking something like “tact 101” wasn’t part of Dr. B’s ESL/med school training based on this blurted out assessment at our neurology appointment yesterday. Luckily, I have grown thick D31_7934 enough skin to not be bothered by comments like this anymore, other than to recognize that yes, my child does indeed have a large noggin. Larger than the rest of us who have my genetic penchant for big heads. But anyways – that wasn’t the point of the visit yesterday. It just struck me as funny when the little teeny asian woman who is the neuro resident walked in, took one look at kendall, and made that proclamation like she had just figured out the key to world peace.

“She’s got a big head”.

Among the other things that we discussed were the fact that Kendall has had MULTIPLE clear EEG tests, 2 “ok” MRI’s (minimal findings that only supported what we see in her delays and presentation, nothing shocking), and most of all – I DO NOT THINK SHE IS HAVING SEIZURES. Most neuro’s are only good for one thing – seizure control. It’s all they see, it’s all they know, it’s all they (some of them) think their job is – to deal with kids who are having seizures, and make those seizures stop. This is frustrating when dealing with neurologists because your child has a “neuromuscular disorder” – because MOST of them aren’t sure what to tell us if she isn’t having seizures. Unfortunately, there is a LOT of stuff that the brain happens to control, most of which is what tends to go haywire in Kendall’s little body, and THAT is what we need help with! NOT another assessment about whether she is having seizures!

So thus far – most of our interactions with neurology have been “less than helpful”. Rather an exercise in futility, except that we usually receive more assurance that she is not having seizures. Excellent. Now, I will say, our first neuro up at LG, is the one who identified mitochondrial disease as possibly being the cause of Kendall’s issues, and he is the one who helped get us started on that path way back in the early days. Dr. G in pittsburgh is a neuro, and obviously you know we love her. She “gets” the whole connection of mito from the brain to the muscles and systemically, so she was obviously a huge help, IS still a huge help in getting the whole body approach to helping Kendall. But up until yesterday, we just didn’t have that connection at Milwaukee. our former neuro was helpful at explaining that IF mito was kendall’s diagnosis, her “seizure like activities” or vaccine reactions were more likely “brownouts”, just times where the energy was temporarily diverted elsewhere, so the brain has to go into kind of a “hibernate” mode to get other systems up to par. But beyond that, we got no real help/direction. Kendall would lose all sensation or movement in her legs for a few days – “oh….yeah that’s weird. Can’t help you. Hopefully she’ll be ok in a few days.” Kendall gets noticeably weaker on her right side when she is fighting a virus – “hmmm…..yeah, no idea what to tell you. most kids don’t show a hand preference till they’re 5.”

Luckily – he transferred somewhere else.

So our appointment yesterday was to meet the new resident and attending who will be taking his place as Kendall’s neuro.  And except for the big head comment – K4birthday 018 we absolutely loved them both. They both had as good of a grasp on “mito” as a systemic disease that ALSO involves the brain as anyone I have ever encountered, including the resident!  The overall takeaways from this appointment were essentially:

1.) Fluids – and that being specifically D10 – are SUPREME to preventing further mito damage/crashes when Kendall is otherwise compromised. Compromised can be anything from not tolerating full calorie feeds to definitely fighting a bug or a virus. If she’s off her baseline and headed towards “sick” in any way, she needs more fluids more faster. Period end of story.  And since we have a hard time convincing ER docs to stick essentially maple syrup into an otherwise mostly healthy seeming child, our complex care nurse is going to get all of our labs, analyze the patterns, and help us come up with an addition to our TWO protocol letters (that still get ignored) that state what her labs SHOULD look like, and if they aren’t THAT spic and span perfect, then she gets D10. There was a possibility raised of “scheduling” a fluid top-off on a monthly or bi-monthly basis, but then that got into the whole discussion of whether the IVIG will be started, in which case we would be discussing a port, and if we had a port we could just run fluids at home, etc etc etc. It got too overwhelming to think about all of that.

2.)  IF we end up avoiding the IVIG treatments, and Kendall manages to stay on a VERY healthy upswing in her health status, when she turns 3 we may be able to start a very slow vaccination process with her to offer her some desperately needed protection, and this would likely involve admitting her, starting fluids, and then administering the vaccine. I actually started laughing when the doc suggested this, then realized she wasn’t joking. It sounds like an insane plan. Surely there is a happy medium between not vaccinating an immune deficient kid at all, and hospitalizing them to protect them from the “side effects” of these supposedly innocuous vaccines. Kinda makes you scratch your head a little at the logic….I am definitely working on finding my OWN comfort level with this for her. Since we have a year to think about this one – I’m content to backburner it for now. But it is good to just have further support for our “no vaccinations for kendall” thought process right now.

3.)  We aren’t scheduling followup with neurology. She assured me that with mito kids, you just can’tk4hospital predict how often they will need to be seen. Kendall could end up inpatient in three weeks and do her weird loss of limb control thing, and they would be consulted then – OR – she could be absolutely peachy for 8 months and not need a thing from them. She assured me to just CALL her if we were having questions or concerns or anything. Come back in a year if we wanted to, or just wait till there was an issue. I know this seems like an odd thing to think is great – but when we have SO many other specialists/appts/therapies/meetings to deal with – being able to be in charge of our own scheduling for one is SO appreciated. On top of just hearing out of her mouth that we would not be thought of as crazy if we wanted to get something checked out if things changed in one or two months. Just that she GETS that mito is unpredictable, and it can cause some WEIRD symptoms, and you just can’t always fit mito kids into a nice predictable “see you in three months” box with things like neuro symptoms.

So that’s why I was so excited.

It’s not that we got any great answers or even any huge help. It was just that i felt heard, and understood, and I feel like Kendall will be understood and helped – in the way that she needs most.

And now I have to go start gathering up all the recent labs (because those have yet to be organized in any semblance in Kendall’s 5 inch thick notebook of medical stuffs), and get them up to the complex care team, and order all the imaging from LG and PGH to be sent to milwaukee also, so we can start making a cohesive plan of “what lab values actually signal impending doom for kendall, contrary to what you may have just learned in med school”. I can’t wait to whip that puppy out for a few residents…Actually, I am more just looking forward to being able to make sense of some of her wacked out labs for myself.

So that’s the big neuro update.

and now I have to go create a whole separate blog for “december daily”. Because I am crazy like that. And i had too much walmart sushi for lunch.

everybody wang-chung tonite!

T-money.

Two Mice Fell into a bucket of cream…

The first one quickly gave up and drowned.

The second one wouldn’t give up. He kicked and he kicked and he kicked so hard that he churned that cream into butter and out he crawled out.IMG_7679

Ladies and Gentlemen – Kendall IS that second (minnie) mouse!

{disclaimer": I just ate an inordinate amount of ice cream and am tired so you MIGHT call this a bit of “slap happiness/sugar high”…}

For those who aren’t lucky enough to be regaled with my random movie quotes on a daily basis, the above is from the 2002 movie “Catch Me If you Can”, based on the real life story of Frank Abagnale, Jr. as portrayed by Leonardo DiCaprio. (you do not even want to get me started on Leonardo Dicaprio…”gilberttttt, where’s mom????”….sigh. i’ll save it for another boring day.)

Anyways.

today was kendall’s IFSP meeting – which stands for something like individual family service plan – but I am tired and probably have that messed up – but the bottom line is – it’s how we assess what therapy is doing to help Kendall and how much she has caught up/is still delayed. The therapists each use an assessment tool that is based on percentages of delay, in order to quantify each child’s need for services based on their delays. Blah blah blah – basically it’s just a number. And in my head i know that, but it’s always still so hard for my heart to process that. Because I see her working SO hard, week in and week out, hour after hour. I see her completely conked out after therapy DSCN0025sessions because she has just utilized every last ounce of energy on putting shapes into containers and pointing at pictures in books. And while I know that the therapists are all only trying to help us get the services that kendall needs, it’s still a bit of a shock to the system to see “55% delay in gross motor, 67% delay fine motor, 88% delay expressive language”, and to realize that means kendall is still operating on the level of an average 12 month old. On a GOOD day.

I am not complaining – really I am not. I know that so many of my friends deal with SO much more and on such a more severe scale. we are so lucky and blessed to be able to receive such great services through our state’s EI (Early Intervention/Birth – Three) program. And kendall has made HUGE HUGE HUGE strides this past 6 months (we assess every 6 months). I know that my above paragraph sounds like it was a bad meeting today, when in reality it was anything but.

It was really and truly a celebration of how very far she HAS come, and how hard she HAS and IS working. And it was less about what her delays were, and more about, how can we keep her and help her keep moving forward? Especially when it’s so easy to see the drastic difference in her skills when she starts to get sick/too tired to function – how do we keep her at a good baseline, allowing her to retain MOST of what she works so hard to master?

So – we will continue with the insane therapy schedule. We will go back to the PMR doctor to get her feet re-casted for braces, but hopefully for lower foot braces than her knee high ones she got last year. We will begin the process to get her an augmentative communication device (I’ll have to do more research on this one and let you know what it means! I think it’s an electronic, or semi-electronic, little notepad type deal that allows kendall to use pictures/buttons to communicate her needs/desires/pain level/etc. with us during those times when she just doesn’t have the energy available to process language, either expressively or receptively.) I know that there’s a few of you out there who know EXACTLY what I am talking about and can probably explain it way better than what I am doing now! This will require another evaluation at a different clinic.

kendall1 And, we will continue to pray for many many more good days to be added to the good run of them we have had the past few weeks. We will continue to keep our fingers crossed every time one of the other girls comes home with a stuffy and runny nose. We will continue to know that the other shoe hangs precariously in the air, but we will try to ignore that nagging feeling that is ever-present that it might drop at any time.

(pic is kendall at 15 months)

Her 2-year “well child” checkup yesterday went, overall, pretty well. it’s never a normal visit. Of course she isn’t doing most of the questions that they ask at the beginning. And of course i have questions and concerns for the doctor. But most of them involve a crystal ball and a magical cure-all, so let’s just skip that part too, k? They put her on the scale once and she appeared to have lost  2 lbs, and then 1 lb, neither of which was giving me warm fuzzies (we have since weighed her on her regular scale and it seems like she has NOT in fact lost any weight from her week of overgrowth meds). And the dreaded discussion about placing a port (permanent IV access) again. We aren’t jumping in to it, but if she has to go inpatient for another infection this sick season, the surgical team will be consulted immediately. At least according to Dr. N. Which was a somewhat shocking, no, startling is a better word. Startling declaration to hear from her, and yet, she is right. We continue to dink around with these kinds of decisions, and it’s time to just start committing to something. This doesn’t mean she will definitely GET the port – it’s just that we will have a discussion regarding the best decisions for her based on the circumstances and projected future decisions (starting the IVIG therapy, needing IV antibiotics, needing more IV fluids, etc.). Trust me that there is a LOT of discussion that has already ensued to get to this point, and that it’s not something we would ever agree to lightly. But the fact that we are all tired of decisions being punted around while Kendall suffers brings us to have to make a hard and fast decision like this – which is simply that we will have a more in-depth discussion with an actual decision process IF she has to be inpatient again anytime soon. I have everything crossed that can be crossed that it is a moot point. We are also supposed to follow up with a local allergist/immunologist to discuss the whole vaccine reaction/possible new food reactions/IVIG options thing again. Yay. (obviously, i am uber excited at the prospect of going through kendall’s history/issues yet again only to be told that she makes no sense and they have no idea what else to do for her, but good luck and hopefully she just magically grows out of all this craziness! God bless modern medicine. I mean, at least back in the old days they’d stick a fricking LEECH on your torso or something if they were stumped. Geez.)

BUT –

overall, we are overjoyed at the progress Kendall has made, and is making, day by day. She is starting to show more and more glimpses of the impish little personality we have always known was in there. We are starting to hear her giggle far more than the whimpersDSC_0017 {banshee yells} of pain that have been her main form of communication for her first two years. It is hysterical (and just the teeniest bit sad, as you realize the implications) to hear her “imitating” her therapists with certain things. Singing something that resembles the “clean up song” is one, but my personal fave is when she “counts”. it is a sound that is very common in our house, as we have therapy going on more often than not, and in many aspects of each session, there comes a time when things are being counted. While you or I would say “onnnneee, twoooooo, threeeeeeee – GOOOOOO!!!!!”, what comes out of kendall’s mouth in her tiny little voice is more like this :”aaaaaa-ooooooooooonnnnn, aaaa-tooooooooooooooooo, aaaa-toooooooooooo” – oh i am not doing it justice. I’ll have to video tape it. It’s precious beyond words and cracks me up every time she does it, which makes her do it again.

(pic is kendall at 11 months)

but it’s just so danged awesome to see/hear her finally doing “regular” kid stuff.

So, for that, I am beyond grateful.

I have so many thank-you’s tonite, but I’ll try to sum them all up with her birthday post later this week. As we think of another mito angel who earned her wings last nite after a good long 13 year battle, i mostly am just thankful that she is here, with us, when things are super good, and when they start to take a dip to the not-so-good.

love and hugs~

T.

plans

They are good to have, but sometimes hard to make.

Today at Kendall’s one year well child check up with Dr. Natalie, that was how it was. I love her. She continues to be one of the best people we could ever have hoped to have on Kendall’s team of doctors/care specialists. She looked at me and said, “clearly this isn’t going to be like most other one year check ups where we get to talk about all the fun new things they are doing and eating, so why don’t you just catch me up on where we are with miss kendall.” I was so relieved. i hate going through the questionnaires with the nurses at the beginning of the appointments.

No she can’t say two or three words.

No she isn’t standing indepently.

No she isn’t cruising.

No she isn’t really crawling yet.

No she can’t drink from a sippy cup.

how about you back up to the questions you asked me at her 6 month check up??? <Maybe I can answer some of those with YES! SHE CAN DO THAT!!!! *sigh* I know they’re just doing their job. And I know that Kendall is still making amazing progress, even if she hasnt’ met the arbitrary milestones that most doc’s offices use. And I know she will catch up in her own time. But it still gets you down, just a little bit, having to answer no to all their questions. All the time. Every visit.

So i had printed off a bunch of info from the internet to go over with Dr. Natalie, specifically with regard to some of the weird things Kendall can sometimes do that would prompt a call from me to their office for guidance and help.

We talked about the MMA and what ramifications it could have, what ER we should go to since our local one told us they can’t handle her anymore, what the plan would be for different situations related to a metabolic issue. Dr. N has had one other patient with a similar metabolism error, so I do feel confident that she gets what I am saying when I say – “she’s just starting to be off a little”.  We went over some of the test results that have been sent to her office and what they were for and whether some of them would be repeated to look for patterns, etc.

And then we came to the second part of her diagnosis, the mito component. This is still an unknown area for Kendall, as we don’t have the testing back to tell us how and to what extent her mitochondria are affected. I feel like if I keep telling myself that it’s only secondary, that i don’t have to deal with the ramifications of what could happen if it is primary, or more affected than we are thinking right now. It’s like maybe some part of me knows that mito will play a bigger role here than other parts of me are willing to accept right now. But I am all about denial so we’ll muddle through.

Unfortunately (or maybe fortunately?) I stumbled upon an awesome resource for primary care providers (typically the pediatrician) when dealing with a mitochondrial patient. It goes through an overview of how “off days” can present themselves, the fact that most parents DO recognize early signs that may not be clinically present in bloodwork or other testing, and that things can go from bad to worse in a heartbeat. It also went through system by system the things that can be affected by mito dysfunction, and how that would look during good times, and how it would look during stress/illness/off days. Reading through it was like reading through a manual on Kendall! So I printed that out, highlighted the parts that we have already dealt with or see consistently with Kendall, and brought that. I didn’t expect Dr. Natalie to be SO open and receptive to it all. I’ve brought info before to doctor’s appts and they were just not going to read it. Anything I have off the internet clearly could never hold a candle to their vast learned knowledge. Which is true, I don’t dispute that. But I need to have a link between med school and my every day life fighting as an advocate for Kendall. And the internet does that for me.

But not Dr. Natalie – no she DOVE into the papers, going through a few of the more important pieces with me (things like the fact that when I call and say Kendall has a fever, it’s not about the number, its about the fact that this is different than her usual 3-4 degree daily swing, or when I say I think she is looking dehydrated and she isn’t tolerating volume through her tube, that we need to get an ER plan put in motion, things like that). She said that she was going to personally read it, and then have each of her nurses read it and check that they read it. A notes synopsis would be entered into Kendall’s file and anytime Kendall’s chart gets pulled up, it will flag the nurse (or doctor on call) to read these notes that summarize all this info. She called the nurses into the exam room and they all were shocked that it was the same Kendall (we have interacted with most of them at one time or another!) – and Dr. natalie let me tell them all about her diagnosis and how I needed their help.

I told Pam and Faye (our two main nurses) – “thank you for talking me down before with different situations, you’ve both been so great at hearing me out. Here with this new info we have on Kendall, there will be times when I am calling and I don’t so much need actual advice, I just need a nudge in the right direction. I need to know, yes that needs further examination or no, we can probably ride this one out. I may call and just need you to call ahead to the ER because I’ve already determined that it is time for IV fluids, or I may call and want your opinion on groupings of random symptoms. It’s just hard to tell with Kendall some days, especially when she is fighting something viral that isn’t real clear in it’s symptomology.” They are such nice ladies. they reassured me I was doing a great job and that they would be there to help with anything anytime.

I mean how can you NOT love a ped’s office like this?

and then, the last part of our conversation was the hardest. We talked about immunizations and what was going on from an immunological standpoint. I told her that we were waiting on official word from the neuro once we get her EEG scheduled and get results from that as to whether or not she has “seizure-like activity” that would predispose her to further electrical activity when her body has the stress of fighting off the vaccines. On top of the fact that she continues to prove to us with her other physical reactions to vaccines (fevers, irritability that is very uncharacteristic of kendall, lethargy, not eating, diarrhea and/or just not pooping at all – all of which typically leads to a hospitalization within a few weeks. WITHOUT FAIL. No, the hospitalization events are not always directly attributable to the vaccine, but they ARE invariably tied to the stress her body starts to undergo every time we have tried to vaccinate her this year. AND FOR WHAT???? Her body isn’t even making any response to the vaccines!

when you vaccinate your child or yourself, you are injecting antibodies against certain diseases into your system. Sometimes a micro component of those antibodies are dead strains of the very thing you are hoping to protect your child against. And what happens in persons with regular immunological function, is that your body will have these antibodies enter their bloodstream and the “soldiers” of your blood and immuno system will “read” the antibodies and create little copies of it, realizing that it is a form of ammo against certain invaders.  These little copies are called “titers” (and I am skipping through years of high level medical knowledge here to try to simplify this into terms that I can grasp, so it may not be the most scientifically accurate here and should definitely not be searchable by any google bots so that no one else has to be subjected to my pseudo-medical knowledge!). Anyways – the titers are what is the hopeful end goal of any vaccination. Your body now has a mini-army to protect you against those specific invaders. And as you grow older, your titers continue to copy themselves, you stop licking the snot off your friends sippy cups (hopefully), your whole system matures a bit, gets stronger, and there you have it. Mostly healthy adults who wear themselves down and then wonder why they get sick. But that’s a whole other post.

anyways – so Kendall’s body has these little mini-invaders from the vaccines, but has no idea what to do with them. So it tries to mount a defense, but it can’t borrow too much energy from other places to do this, so her immune system just starts sending red flag memo’s all over the body – “You! Stomach – divert energy away from digesting food to come help us fight these invaders! BRAIN! – shut her down for a few hours so we can store up some energy to fight this next battle! ALL SYSTEMS MUST CONTRIBUTE WATER! Alert the fever Alert the fever!!!” But instead of reading the antibodies and turning them into covert spies and making good copies of them, her body basically throws the equivalent of nerf darts at them and lets them just meander their way through her body until they all die of lack of supply. And no copies of those invaders are ever made to help boost her own army of disease fighting cells. 

This is what I do not understand is so hard for her docs to comprehend. I am not anti-vaccine. I think they serve a GREAT purpose! I wish there was less money involved with some of the drug companies because I think we’d get more straightforward answers and products, but really, it’s not about the politics of it in this case. Vaccines work for 99% of the population and that is a great thing. YES kids like Kendall need all the protection they can get, which would mean we SHOULD continue to try to vaccinate her.

BUT THEY AREn’T GIVING HER PROTECTION!

We are shooting her up with these little toxins with the hopes that her body will kick in and do its part – but its not. So we’re just shooting her up with a very hard to process, illness-inducing placebo. It’s not that I am against the vaccines – I am against the fact that THIS plan isn’t working for Kendall, so why are we continuing to beat our heads against this wall? Either tell me she’ll be fine without vaccines, or come up with Plan B. Well, the problem is, they have a plan B, but it’s IVIG. And really, I am becoming more accepting of Plan B, because we’ve discussed that it would be only for about a year, through next cold and flu season. It would get us over that 2 year hump when most systems are mature enough to deal with the stress of vaccines without flipping out. It would cover so many more illnesses than just the vaccines, and would eliminate having to fight insurance for separate Synagis shots (against RSV).

but again, we are waiting for neurology to tell us that she may or may not have more seizures if we try to vaccinate her again before we make this decision. Her metabolic/genetic docs feel that vaccines are always the appropriate course of treatment, so they recommend that she continue with the vaccines (although realistically, I don’t think they have the whole picture of what is going on to make that decision. I think I just got the “party line” from them on their recommendations when I asked.) Immunology is willing to start the IVIG as soon as someone ELSE makes the call. And then there’s Dr. Natalie.

“That is the most ridiculous thing I’ve ever heard!!! WHY do they want to try to continue to vaccinate her even if its NOT seizures?!?!?! Clearly her body can’t handle it! START IVIG NOW!!!!” I was laughing pretty hard at her because she is just so “no non-sense” when it comes to certain things.

So we had a talk about the fact that if it were Dr. Natalie’s call, she would refer us to get a “port" (easily accessible IV that is permanently placed in Kendall’s chest) within the next week or so, so that we could just start the IVIG treatments sooner than later, as well as have that more easy, permanent access for any IV fluid boosts that Kendall might need. I was stunned to say the least. And relieved. And resistant. And a little bit…I can’t think of the right word. Ready? To do whatever we have to do to keep Kendall healthy – or at least MUCH healthier than last winter! So we talked about waiting to hear from Neuro (we have to get the stupid EEG test SCHEDULED first before they can even make a recommendation), but that Dr. Natalie would write up her recommendations and send them up to our care team in Milwaukee for further consideration on the matter.

It was a relief because while a part of me thinks that we will probably end up on this plan, it isn’t one that I would hope for for kendall. And yet, it would have probably fallen to me to be the one pushing for something to help ease the IVIG treatments since Kendall has a penchant for losing peripheral access (IV’s) at inopportune times, after going through the trauma of trying to get a line started in the first place. At LG on the peds floor (where every nurse is used to starting lines on small babies), we still have to have NICU or PICU nurses come start them (who are used to starting REALLY difficult lines in one lb babies), and even then they usually blow a few spots before getting one to stick. The problem is that by the time Kendall needs an IV started, she’s usually so dehydrated that her veins are starting to show instability. It’s a catch 22 for her. But anyways. The burden of advocating for Kendall as her only voice has again been lifted a little bit by Dr. Natalie’s plan and absolute committment to PROACTIVELY caring for Kendall.

Another plus to this plan (if indeed any of these can be counted as pluses when talking about putting a line into place in your baby), is that requiring nursing care would more than likely allow us to qualify for state services to help with easing that gap between what insurance covers and what we have to get out of pocket. It would allow us to have a nurse come to US with IV fluids if that’s what we needed (vs waiting in a germ filled ER where they may or may not be able to help us anyways!), especially as sometimes it really is just a matter of getting fluids throughout her body in a quicker manner than her crazy gut determining how much fluid to share with the rest of the body.

anyways – it’s not a perfect plan by any means. It isn’t one that will be put into place for a least a month if we do need to go to it at all. But it is at least a PLAN. It is something solid we can hold on to as hope that we can help Kendall better than just RE-acting to her crashes when they come.

Part of me does still hope we can just forget all about any of this kind of talk. Cause right now, she looks great. She has a slight yeast overgrowth around her tube stoma. Yeast can be a nasty little bug to get rid of, so we’re hoping that a nice tube of jock itch creme can help nip it in the bud. Next step if the cream doesn’t help is to give her one shot of yeast-killing antibiotic. If THAT doesn’t help, we’ll pull out a few big hitters and put her on a course of oral antibiotics. There is still some question that the yeast on the outside COULD indicate that she is dealing with a yeast overgrowth in her gut somewhere also (which would be why she is currently dealing with some nasty diarrhea and stomach cramping) – but right now, we’re all ok with hoping its just an external problem that is treatable without antibiotics. Kendall does not handle antibiotics very well (because her gut is already so sensitive to changes in its flora/bacteria), as well as we know that she rarely responds to oral antibiotics and ends up needing IV antibiotics and could start building resistance to some of the few that she responds to. So we have to weigh all of that against kendall’s health/discomfort.

But for right now, she’s good. She’s great actually. That, of course, can change with the blowing of the wind, but we’ll take it for this few minutes. BEcause at least there’s a plan, or a few of them, for what to do when that does change. And THAT makes all the difference in the world for my peace of mind, and ergo the peace of mind for the whole family!

So that’s our ped visit update.

terra

Today will be a good day

I posted this as my morning facebook/tweet, and have gotten so many comments/emails/encouraging words from it that i have decided to make it a daily mantra. Attitude IS so much of what happens in our day – CHOOSING to have a good attitude goes so far to making any day good.

so in spite of the fact that I currently feel like…well…not so healthy I guess, I am trying to choose to have a good day.

I spent the majority of yesterday on the phone with the genetics assistant, home health care company, 5 or 6 pharmacies – all trying to get this new b12 therapy in place for Kendall. The home health nursing agency recommended we just go to our ped’s office daily for these shots – “that will be so much easier”.

Really? It’s 45 minutes away – ONE WAY. I have three other kids who have to be at school, on the bus, off the bus, at dance class – not to mention a LIFE. and really, how advisable is it, do ya think, to take all these children, one of whom has a known immunodeficiency, into an office where sick kids are laying around like so many zombies, EVERY DAY for the next month? Are you REALLY telling me that will be easier????

Needless to say, we are setting up home nursing training.

cyanokit1Still can’t find the right meds though – and by meds i mean vitamin b12, but prepared in a slightly different way than is commercially available. Found one pharmacy that CAN make it the way Kendall needs it, but they don’t take our insurance. The ballpark number I have been given is $800. I am praying they were doing math off the top of their head and carried an extra zero or something.

So today is going to be  more of the same. 2 therapies today – speech and occupational. Which is kind of funny when you think about it because she neither talks nor works. But, you know, we’re working on it.

In some GREAT news – I just got off the phone with our Immuno doctor and we DO NOT need to start IVIG immediately as they were discussing/thinking! It’s not totally off the table and Ben and I need to discuss still what we think is the next best thing for her (attempting to give her monthly vaccines of the most likely to harm her infections, maintenance antibiotics, etc.). We will still meet on the 25th with the immunodeficiency team to see what they recommend based on the plan we come up with, and IVIG may still be in the future, just given the fact that with her metabolic issues, sickness is clearly going to complicate things even more for kendall. But HALLELUJAH we don’t need to get it started yesterday like they were first thinking.

i may or may not have just tripped this over to italicized printing. if i did, sorry. my editor is kind of wonky with showing me what it’s actually doing.

So. like i was saying. Attitude reflects leadership, cap’n. Oh wait, wrong movie…

Attitude changes things. Prayer changes things. today is going to be a good day. we’ll just keep adding up good days one by one.

I feel like I had more to tell you all. but my efforts to ignore the fact that I think I am slowly being hit over the head with some kind of sickness aren’t staving off the fact that indeed my throat is now swelling shut and my ears feel like they’re going to explode off my head.

perhaps I’ll be back later with more.

IF YOU’RE LUCKYYYYYY!!!!!!

Fun contest of the day – if you notice that you have the number 26078 in the counter over to the right ———————-> LEAVE A COMMENT! There will be a prize for you!

 

Did you guys think of your favorite childhood 80’s movies yet for my big post? Tell me what you remember watching over and over and over again!

 

hope you are all having a GREAT day!

 

t-shizz.

Cautiously optimistic?

Not sure if its too early to get excited yet – but Kendall seems to be handling her vaccine from Wednesday pretty darn well!

Typically after vaccines her gut shuts down, and she starts first dumping a lot of bile, then not tolerating as fast of a rate/flow, then not tolerating full strength formula, then not tolerating formula at all and just crumpling into a hot mess who doesn’t even want pedialyte going into her tube.

So far – none of that.

the ONLY weird things were that she lost head control for a few hours that evening after the vax on our way home, and her temp was 94-95 rectally for almost 2 days after that. The head control thing scared me, i gotta admit. You hear all these horror stories about babies who get one random vax and then two months later have full blown autism – or worse. And when I went to pick her up out of her carseat after we got to my mom’s from milwaukee, and her head was just floppy like a newborn again, and she couldn’t lift it up or control it real well at all till much later that nite, i kind of panicked. BUT – she did eventually get it back, even though it took right around 24 hours for her to be back to her usual baseline (still floppy and loosey-goosey – but at least having some control over her head and other body movements!)

I am very hopeful that she has made it through one vaccine! Not sure what this means for our big plan for getting her through a winter illness/infection-free, but at least for the short-term, we aren’t dealing with a miserable baby, so I thank God for that! she really and truly seems to be doing SO WELL since her last hospitalization! I personally chalk it up a.) to the massive amounts of prayers poured out for and over her at that time, and b.) to the fluid overload she got for those three days. Her gut got total rest, and she got pumped full of good fluids. While she has lost most of that “water weight”, i think the help it gave her little system is not being given proper consideration. I do plan to bring it up with GI/immuno/genetics as soon as we see them next, just to get a feel for what their professional insights on it are, and see if that helps direct them towards any more issues that we may be missing.

Her stamina and energy have continued to improve since that time, which was a huge concern of mine just prior to that. It seemed like she would never start putting pieces together on her own (from a developmental standpoint), because just making it through an hour of therapy was the MAX she could do, and even that was inconsistent. There was just no energy leftover for learning to DO anything on her own! She doesn’t have a TON of extra energy now still, but I do think she is getting onto more of a normal baby routine with her sleep. She still gets in two naps a day, sometimes three, for a total of about 6-7 hours of sleep in the day, and then 12 hours at nite. Some days a little more, rarely less than that though.

She’s working on it!

She is bound and determined to learn how to stand up soon! She wants to be up up UP! and looking around, seeing the chaos going on around her, keeping an eye on Kaylen for any wayward flying body parts or toys that may be coming her way, watching kealey and karissa dance/fight/whine at each other…it is so awesome to see!

I cannot believe she will be 11 months this week. Where has the time gone???

oh yeah. in doctors office, waiting rooms and hospitals.

But oh what a ride its been.

I need to do a post about her birthday celebration party!

Have a great day!

 

 

terra

More Info.

Probably more than you will WANT to know, as you’ll get my own personal opinions of vaccines, but since i can think a little more clearly today, here goes.

PERSONAL view of vaccinations

First – let me say that I don’t intend this to turn into a debate about them. It’s a personal choice and one that I hope people make after doing their own research. I did only a little bit of research before having Kealey, but we were luckily on the West Coast still at that point, where, in our experience, our doctors were ENTIRELY more laid back about things than here in the midwest. SO – whatever your choice, great. This is just how our family has done it.

For the most part, I have no problem with most vaccines. They are a part of childhood in the US, and i think that most of what we get vaccinated against is not prevalent anymore BECAUSE of the vaccines. So for kids who are healthy and strong and can handle them, I think they’re a GREAT thing. They provide herd immunization not only for kids, but also for the adults that interact with them on a daily basis. It works. My first three were all vaccinated just about on time. Like I said, our WA and CA doctors were very laid back  – “ oh you want to delay this one today?> great call us when you’re ready, or we’ll just catch her up on her next visit.” So if I was still reading up on one or two of the recommended vax, we’d just wait. if i felt like kealey was just slightly under the weather, we’d wait. we delayed the MMR till she was 2. Karissa – due to her issues, was also on a slightly delayed schedule, but, being here in the midwest, I had to really do some convincing of the docs that this was the appropriate route. Once she seemed healthier and more stable, we caught her up and all was well. Not a big deal. Same thing with Kaylen. She was constantly with the ear infections her first year so she had more than a few catch up shot days, which did cause minor reactions, but nothing that i coudln’t deal with.

We have gotten chiropractic care for our entire family since we moved here to IL, before karissa was even born. Our chiro, God bless him, is VERY anti-vax. They’re all poisons, made with fetal tissue and battery acid, completely unnecessary and more harm than good, etc etc etc. Now while I am pretty sure they’re not the HEALTHIEST thing, i think I fall somewhere in the middle. I think some of what gets put out is just lots of drug company propaganda/money making, as evidenced by docs who are all too willing to push certain vaccines without much backup. (Again, this is JUST MY PERSONAL EXPERIENCE.) But – whatever the case, we always take our kids to get adjustments right after they get any vaccines, so that their bodies can be in optimal condition to be able to absorb the titers of whatever vaccine they just received, fight if off appropriately, and let their bodies do their jobs to store those antibodies till they’re needed. For the older three – this has been a good way of doing it. Sure they still get the occasional sicknesses, and they all have some slight reactions to the vaccines (crankiness, tiredness, fevers) in SPITE of the propaganda  paperwork the nurses give you that swear that NEVER happens. Whatever. It is what it is and we just deal with it for a few days.

So in general – I am not Anti-Vaccination (Vax), nor am I necessarily gung-ho PRO-vaccination. I believe in the concept of vaccinations, just not always the execution of that concept the way that our country has accepted it. There are more than a few vax that I personally feel are complete bunk, but i’ll keep those opinions to myself since that’s all they are – MY opinions. anyways – this has been a good course for our family for the past 7 years.

THEN along comes Kendall. With more issues than months in her life, and throwing ALL my parenting wisdom out the door. She was in the blog2 NICU still for her first set of vaccinations, and I can honestly not say whether she had reactions or not. Things weren’t normal for her, that’s for sure, but its hard to know which was the horse and which was the cart there. At her 2 month check up was when she turned into exorcist child when they gave her the vaccinations, and that’s when her bad downward spiral began. So at 4 months, we did just one shot (I BELIEVE it was just the Hib at that time as well, single vax, not combo) and sure enough, a week later she was back in the hospital with pneumonia. 6 months, we held off on vaccinations because she was again dealing with pneumonia (but just not inpatient for that one! YAY!). At her 9 month check she got the Prevnar (for pneumonia and lots of sepsis infections, which she dealt with this past spring) and the DTaP, combo’d because that’s the most effective one against Pertussis (whooping cough), which IS actually coming back around in IL this season, and we felt like it would be good if she had SOME protection against it. And please believe, nurses don’t just walk in the room and administer shots without a LOT of discussion from me. I ask lots of questions specifically about the pro’s and con’s of vaccinating vs. NOt vaccinating, in KENDALL’s situation. We discuss things about her history and overall status before doing anything. It’s not something that is done lightly by any means.  I was a little hesitant to give her two shots at her 9 month check (which was at the end of August), but she HAD been in a pretty good run of health/weight gain/stamina, so i thought it was ok. And sure enough within three weeks of that, she was inpatient. I get that it MAY be all circumstantial, highly coincidental, all of that, with regard to going inpatient after every round of vax. However, given what we are also seeing on the bloodwork, and the fact that she does appear to be constantly fighting SOMETHING, this is what is raising the red flags to her immunologist.

blog1 The discussion yesterday about giving her the one vax again was not short. It was a good discussion though, in that i felt like Dr. R was REALLY hearing me out, and that we were having a two-sided conversation. We went back and forth about the benefits of vaxing her right then and there. But in the end, the plan he laid out was one I could not argue with. She just IS stuck between a rock and a hard place. She has a good chance of growing out of this issue, as a lot of kids do. However, in the absence of any real protection against MANY viruses and infections, it could be a scary winter. We need to balance projected growth out of this problem with the fact that she needs to make it TO that point alive in order for that to be helpful. I think if we had dealt with ear infections, colds, croup, that kind of thing last winter, Dr. R might not be so keyed up to fix the problem. What we dealt with though were very bad, life-threatening infections. Her body bypasses the ability to keep the infection at bay in just her ears, or just her sinuses, and lets it go right to its favorite warm incubation spot – her lungs. She more than likely always had secondary infections along with her pneumonias – but clearly we were more worried about the pneumonia/breathing issues than the ear infection. And the horse antibiotics she got put on killed everything. And then the fact that she would always improve SO WELL on antibiotics, and start crashing again the day she finished each course also played into the entire history Dr. R was looking at when outlining his plan of attack.

so that’s why I consented to the vax yesterday.

Not because I just blindly go into each appt accepting whatever the doc says will be best. not because I am a huge fan of either vaccinations OR inpatient stays. And certainly not because I like to torture Kendall. We try to make very informed and researched decisions when it comes to all the girls and their medical issues, and no decision is ever just lightly jumped into.

So – now that that’s all off my chest!

ummm what else did i have to blog about today…

Oh – Dr. Terra’s personal (completely unmedical) version of IvIg. This is JUST what I understand from reading the helpful medical documentation on google and from a few other online sources. Basically, it would involve going to the hospital monthly, getting an IV started, and staying hooked up for a few hours for the globulin product to be infused. I guess it can cause quite a few reactions, so sometimes there’s some pre-treatment (Iv benadryl or other anti-histamines), and then a period of observation afterwards for the first few especially. It is a blood product, so in some ways it is similar to a blood transfusion. But it’s not whole blood, nor is it medication. It is somehow a conglommeration of thousands of antibodies taken from different donors and combined into one product. That could be WAY off, but in essence, that’s what is happening.  {UPDATE! I found a better link – read this!} Since Kendall’s body can’t make antibodies, she would be getting them from other sources. Which is why its a monthly therapy. And it would last for a couple years, until, hopefully, her own body can kick in and start creating its own antibodies, a.k.a. – using what she makes!

Today definitely feels like fall. So glad I stopped by Yankee Candle for my new fall tarts! they definitely make it seem/smell/feel more autumny. *today my house smells like Macintosh spice! the girls were all excited that I had baked cookies….uhhhhh….no, sorry – just a tart burning!*

Ben came home early today and we were able to go have lunch – which was all fun and enjoyable till Kendall started choking on…who knows what – her own saliva? Reflux? something anyways, and then Kaylen peed her pants and clearly her diaper was at capacity because it created a nice little puddle on the floor. So we had to cut our fun little date short and speed home. C’est la vie.

And now we are deep into the throes of afterschool homework and snacks before getting ready for Awana’s tonite. And I am freezing in spite of the fact that we succumbed and turned our heat on today. I think Kendall’s waking temp of 94.5 kind of pushed us in that direction. She is now back up to her normal of 97! So the hawklike watching continues. Thus far her stomach seems to be holding up , which is usually the first sign of something going wrong. She is refluxing a lot more today, and then choking on it, but that could be just tiredness, not a harbinger of worse things to come. I hope. Thanks for the prayers and thoughts you are sending on Kendall’s behalf! I think getting through the next three or so days without massive reactions will be HUGE!

Ok the guilt over not having baked goods ready and warm from the oven for my girls is getting to me so I am off to try to retroactively have some!

Have a happy Thursday!!!

 

T-Crest

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