Our mornings are getting back into a rhythm and routine with the return to school. My children are all still up at the crack o’ dawn – in spite of the fact that their bus does not come until 8:40 am. It was weird yesterday to have such an obvious line of demarcation between my “olders” – both going to school all day, and my “youngers” – staying home with me all day. K3 and K4 seemed to enjoy being able to play with ANYTHING they wanted without having it ripped out of their hands by their older sisters who “were going to play with that in a few months” or “wanted that to be special!!!” (referring to a mcdonald’s happy meal toy or some such trinket).

In spite of the fact that she was clearly pretty weak from her recent hospitalizations, Kendall tried bravely to keep up with Kaylen, until she finally passed out for a four hour nap. Luckily, Kaylen also had a nap during this time, and if THAT happens every day – I will be one productive super trooper indeed! unfortunately yesterday I felt like I should have taken a nap too. Monday night was the 5th nite in three weeks that was spent in my own bed – it was a welcome collapse into bed after getting home from the hospital at almost 11 pm!

As to how Kendall Quinn is doing…well, that remains to be seen. I have been, let’s just call it “distracted” thinking about what Plan B is from her GI doctor. They let me bring her home from the hospital on “feeds” of 5cc/hr. Most preemies can handle more than this into their tummies. It is one dose of medicine, one medicine dropper-ful, every hour, very slowly. It is nowhere near enough to even keep her hydrated let alone fed let alone catch her up on calories which she really desperately needs. She absorbed almost 2 lbs of fluid over the weekend – meaning either that she was WAY more super dehydrated than her labwork even showed, or that it is somehow leaking into her tissues instead of being turned into urine by her kidneys, or – she’s got the world’s hugest bladder right about now! It is probably a combo of all three of those – lumped together under “autonomic dysfunction”. On her best days Kendall’s autonomic system does some crazy stuff, but it usually stabilizes itself, or has adjusted to the craziness, so we don’t mess with it too much (this is things like her body temp, her sugar reaction to certain foods or to “fasting”, peeing too much or not peeing enough). However, after a huge stressor like this weekend was to her (metabolic acidosis), her autonomic system is pretty much haywire.

So they let her bring me home because a.) their only plan was to continue to dump her full of fluids even though they weren’t really coming out as well as they should have, and b.) her pulmo HATES playing GI/metabolic/geneticist to her! He does it out of his love for her and his concern that she get the best level of care as close to home as possible – but he hates it. So – I called our GI, got plan B, and convinced them it was ok for me to bring her home that I could work on feeds just as good at home as I could there. She was interested in some oral eating again, could sit up without puking, and had managed to keep her sugar up for about 4 hours after stopping the IV sugar water. All good signs to me.

Unfortunately – not quite enough for what Kendall’s body NEEDS right now though. I was relatively pleased with her feeding progress yesterday, till I added up her 24 hour intake from stopping the fluids. Between what I was able to pump into her intestines at half her normal rate, and what she wanted to take in orally – she had 16 total ounces. About half of that was pedialyte, meaning she had 8 ounces of “nutrition/calories”. Subtract the 10 ounces that she dumped back out of her g-tube, and we aren’t making a whole lot of progress. Yesterday afternoon she was seeming to perk up a bit more, and we got a few more ounces of full strength formula in (4 to be exact), plus some more pedialyte-diluted formula, and i was able to bump her nite feed up a little higher. If she has a super-excellent day – she might be at half of the calories she needs for TODAY.

Her urine output is back down, the pressure in her intestines is popping open her med-ports (they are little openings on the tubes so you can push meds in without having to unhook the feeding pump – helpful if you have a kid whose motility is good, not so helpful when things are like this!), making her lose even more formula and bile, and she has not had a BM in 5 days. All things making us think that while she has gotten herself out of the metabolic acidosis danger zone – she is still not up to par for what her body needs. Now in a regular kid who had maybe a post-op ileus or even just a touch of gastroenteritis – you can have a little leeway. they will lose weight, sure, because their body will dig into its stores for calorie sources to keep itself going.

With Kendall, its not quite that easy. 1 – she just doesn’t have a whole lot of stores. She has more NOW than she ever has in the past, which is excellent, but her body burned through those real quick with all the vomiting. 2 – her body doesn’t effectively know how to make energy from calories (food) and oxygen on a good day, let alone when its “stressed. That much we do know about whatever metabolic dysfunction she has.

So – we are left with Plan B. I am not quite ready to commit Plan B to “paper” yet – those of you who have been through gastrointestinal slowdown/shutdown know what it is. For those who don’t know – let’s just say we may have to head up to milwaukee in the next 48 or so hours to be put on the OR schedule, and come home with a drastically increased medical load. Right now – it’s all up to God and the doctors to decide. I know she needs more than what she is getting right at this moment. I am sure that Plan B would give her a huge boost even beyond just this immediate deficiency. Plan B would enable us to meet with one of the leading motility experts in the country as he happens to be the GI on service this week. Plan B is not a bad plan….it’s just a hard to swallow one. (HA! get it! hard to swallow…cause….kendall isn’t swallowing well…..ok nevermind. too soon…)

All joking aside – it is weighing pretty heavily on my shoulders right now. I am not sure that I will be “happy” either way – if we don’t decide on plan B, i’ll just keep worrying about her and am afraid that plan B will become inevitable at some point in the very near future, perhaps after permanent damage to her minimal gut function has occurred. If we DO enact it – well, it’s just a lot to wrap my mind around. But I think going FOR plan b is probably the best thing to do right now – to ensure that it is the shortest amount of time possible. It will make this WEEK crazy – but hey, i am pretty sure we’re firmly into crazyville already.

So please pray – for our doctor as he makes the decisions and necessary arrangements. For the other girls as they will once again be in upheaval if we have to be admitted in milwaukee for a couple days. For kendall to endure another procedure if that’s what is decided, or to have the strength to endure through another couple weeks of minimal nutrition as we ramp her back up slowly from home. And for me – to retain the shred of sanity I have left. My mind is consumed with this plan and the decisions contained therein. And I would REALLY like to get off this topic already. Or even this merrygoround.

Thank you to all the good friends who have listened to me vent and process and whine through this past week of chaos. Thank you to all of you who pray and lift us up even when I haven’t asked for it. Thank you to our wonderful family who have rearranged their lives this past couple weeks to make sure that we don’t all fall apart. Words are not enough to thank everyone.

Off to see if some dunkin’ donuts’ X-tra X-tra can’t help sort things out a little better…and MAYBE a chocolate frosted. maybe.

t-rock.

(that’s gangsta for pittsburgh by the way. I have no idea why. I just made it so.)

So this post will be just the details of the appointment – or as best as I can recount the conversation without too much editorial comment. That will come later. Either later on in this post or in another post.

So our appointment was scheduled for 1 pm, but this morning the nurse called to change our appointment to 3 pm so that the doctor would have more time/all the time we wanted with her. I will refrain from the editorial comments here about what a mess this was, but let’s just say that when you are calling to leave a message for someone that has a somewhat urgent component to it, like say an out of state doctor appointment occurring within the next few hours, LEAVE YOUR NAME AND PHONE NUMBER. That is all.

It ended up working out well because a.) I would have hogged up that much time anyways, and b.) Kendall actually took a nap ( I know! in someplace NOT her room! Unheard of!!!).

In nothing short of a miracle, we found the hospital. This crazy city of one way streets and construction every other block – oh wait, I am reserving editorial comments for later. Anyways, we found it. Thank you Jesus for your guidance.

We were called back for our appointment right away after we registered (probably because it took me forever to park and get up to finding the clinic), and we didn’t even fill out the paperwork because Dr. G came right in (her name is Dr Goldstein, but i am just used to referring to her as Dr. G, so, there you go. ok that was the last editorial comment).

Dr. G came in with a stack of papers and file folders, welcomed us and said she felt like she already knew Kendall and was SO glad to meet her in person. I gotta tell ya, NOTHING warms the heart like hearing that from a doc! She had clearly done her homework on the paperwork I had sent over back in March. She had made notes on our lab tests, and had a few other pages of her own notes. She said she had a lot of questions for us, and I said that worked for me to start with hers and then go on to mine.

Each question she would ask would inevitably lead to one of my “issues” i had wanted to discuss, so we’d go back and forth in this manner going body system by body system. It was amazing to hear MY THOUGHTS echoed out of this doctor’s mouth. For instance, she said ” I noted in the paperwork that there is a lot of talk about IVIG, and that her immune testing is all relatively {not good} (I’ll get in trouble from my mom if i use the word she really said here), but WHY is she not on IVIG yet?”  I was dumbfounded. I have wondered this same thing myself many times! Not that we WANT Kendall to need IVIG infusions, but in the face of what we are up against with the active immunizations not working, and the fact that we deal with an average of a bacterial infection of some kind every 8 weeks or so, and that those TYPICALLY end up with kendall needing inpatient support, why isn’t more being done to support her?

There were about five or six instances where this kind of thing happened – we talked about the MRI results report not making anyone feel warm and fuzzy (my thoughts exactly), her possibly needing a cough assist device (again, discussed many times, never actually ordered), her possibly needing a port/access of some kind in the future (second verse, same as the first), and the fact that there is little testing to support our theories/treatment of Kendall’s gut function, because we all ASSUME/have good confidence that it’s just {not good}. Just about everything i have ever questioned/researched/been frustrated with in regards to Kendall and her issues, Dr G brought up and her thinking was just so right on with what I have always felt.

After about a half hour of this, she just kind of stopped and said “Clearly there is a LOT going on here, and I am just going to tell you, it is going to take a while to get to the bottom of all these assorted pieces with the different people involved in her care. Is there any way you can come back next Monday to be admitted and just let me do it all here inpatient?”

My mouth still hurts from where my jaw dropped open and hit the floor.

Her options were actually “Next Monday, or the week of Christmas, and I just don’t think she’s going to make it to Christmas without some good solid direction from the testing we need to have run.”

so we picked next week. * the reason those were the only two options are because those are the weeks that Dr. G is the attending neurologist on the floor. Any other week would be some different doctor, not familiar with Kendall, and with drastically different goals for outcome.*

Now if you have ever had the privilege of having a medically complex kid, you know EXACTLY what i mean by “getting things done inpatient”. As long as its not a holiday weekend, you will get done in about three days what would take about six months if you did it all “outpatient”. Getting to run testing inpatient means you get answers/results/help on a much accelerated timeline. (I know – not always, but in general, you get way more done, right???) so for HER to suggest this to me,  I knew she wasn’t fooling around with it. I told her I needed to run it by Ben, but that given what we have had to go through this past almost two years, we were pretty much ready to do whatever was needed to get Kendall the help/answers to live life to the fullest.

I called Ben and told him what was up, and he said “well, I guess it works out then that I am supposed to be in pittsburgh next week”. I could not believe it. now I don’t believe that everything is going to magically fall into place just because THIS happens to be one major coincidence. But – to my heart and mind – it was just one way of feeling reassured that we were doing the right thing.

Dr G started the paperwork for the admit right there and told me that while she had many more questions for us, we would definitely be going through all of them next week. She gave me her email address and a list of things I should try to get from the hospitals/her docs before we come and told me to send her any questions I still had so that we could finish getting a solid game plan in place.

And that was the end of the appointment.

It was just….

A breath of fresh air. A huge infusion of hope. Exactly what I NEEDED to hear.

sure I went in hoping we would get THE ANSWER – whatever that is. But that isn’t what I NEEDED. What I needed was to feel like I was being heard, that everything I have noticed or tracked or brought up to docs for the last 21 months wasn’t for nothing.

That i wasn’t crazy.

And i mean, the jury is still out on that last one in MANY ways – but today, I got confirmation that at least with regard to my baby, I am not crazy.

She is beautiful and she has bright blue eyes and she gets most of her nutrition through a tube in her intestines and she likes to make her heartrate do funny things at random times that scare the bejeezus out of me – and she has a metabolic condition that is causing every cell of her body to misfire.

SHE IS DOING GREAT - and we hope to keep her that way.

We do not know what to call her metabolic condition yet. Dr G had printed off page after page, chart after chart of the “metabolic cycle”, urea cycle disorders, Krebs cycle disorders, fumarate something or others – my head started spinning at the sheer volume of info she had compiled. The bottom line is this – what we DO KNOW right now is that the cycle that each cell has to go through in order to turn food and oxygen into energy is not happening correctly for Kendall. EVERYTHING in her testing points to this as a definite concrete thing.

If you picture the metabolic cycle as a figure 8, the bottom half of the cycle is what we’ll call “metabolic” and the top half of the 8 is “mitochondrial”. The bottom half is supposed to complete its little circle and then hand off its by-product to the top half, which creates “energy”. Energy to digest food, fight infection, receive messages from your ears and eyes, swallow food correctly, keep your brain functioning, keep your heart pumping, etc etc etc. Everything needs energy. Still with me?

For those of you who know kendall, you will see where we hit the crossroads. Many of those things don’t happen, or at least don’t happen properly, because there’s not enough energy.

so that right there is enough to say – yes, kendall has a mitochondrial metabolic disorder. Or a metabolic mitochondrial disorder.

The problem is – that’s not enough to actually HELP Kendall. If we can pinpoint WHERE the breakdown is occurring in that figure 8, we may be able to provide what her body is lacking, plug in a cog or a sprocket in the form of a supplement or a medicine that will help the process keep moving.

where we are running into problems is that we can’t pinpoint it just yet. Last fall when we thought she had the genetic defect for the MMA – methymalonic acidemia – we thought THAT was the missing cog. And sure enough – the b12 shots helped IMMENSELY.  But it turns out, kendall doesn’t “genetically” have that defect. That was just one part of the assembly line that had a kink. As we worked that kink out – it became apparent that there were more kinks.

As Dr G explained it to me today (and those of you who are way more knowledgeable in this than me, please correct me if i am mis-speaking here) – we may not ever be able to distinguish for Kendall if her “kink” is in the top half or the bottom half of that figure 8 cycle. They are both affecting the other. She may have a primary mito defect that is causing a lack of energy to the bottom half of the cycle, thereby not allowing the top half to ever function properly because, well, it doesn’t function properly. She may have a metabolic defect that prevents energy from getting to that top half so it doesn’t have enough energy to force the bottom half to do its job right. There are literally dozens of parts to the bottom half cycle alone, and Kendall’s testing points in at least one dozen of those directions!

So – that’s what we are going to attempt to narrow down next week.

We will also be having the sub-specialty parade in our room:

Pulmonology, Cardiology, Genetics, Endocrinology, Immunology, Opthalmology (i can’t spell that word tonite), Intestinal Care, GI, and of course Neurology (Dr. G) will all be coming in to give us “second opinions”, or at least opinions that are cohesive under the metabolic mitochondrial disorder genre. Right now, we have most of those people on our “team” already – but none of them are really working TOGETHER, with one big picture in mind. Dr. G has certain docs in each of these specialities who she knows KNOW her metabolic kiddos. They know that what might be normal, or abnormal, for a kid with any other issues might not be what works for a metabolic kid. They know that there are different threshholds for metabolic kids.

And they know how to make all of that work together for what is best for each child individually.

Now this doesn’t mean we are done with our docs back home. It just means that NOW we will have some direction for them all! I know that Kendall scares the whoopie out of the majority of them because, well, metabolic stuff is a scary thing to mess with, and because they just don’t know what to do with her. That is part of what is so amazing about Dr G – when I would describe part of what I see happening with Kendall, she could FINISH THE SENTENCE FOR ME. She got it. She got that if “x” was starting to happen, “y” would be the final result. I am still in shock and awe, sorry.

What we KNOW will be happening next week: Kendall will need good veinous access all week for certain tests that Dr G wants to run. I laughed out loud when she said that and told her that i hoped they could get it all in one shot because they would have an average of ten hours from the first IV placement til they needed a new one. And again – she said that didn’t surprise her given kendall’s history. So – we MAY end up going for a PICC placement first thing upon admitting. She will more than likely be on a Holter monitor for her heartrate stuff for a couple of days, and will more than likely end up with a video EEG – which is so many levels of fun to imagine that it will take its own post. You know kendall? my child with sensory issues who hates anyone touching her head or face? They will have to hook her up to about 20 leads all over her head, with sticky tape/glue stuff, and LEAVE THEM THERE for three days straight. I think I might rather be mauled by small woodland creatures….And then at the end of the week, once everyone else has evaluated her in her “regular” state, she will be put under GA for a sedated MRI and spinal tap, and possibly a few other procedures as may be recommended by the other team members. This may or may not include her PDA closure, a chest CT/bronchoscopy, GI testing, etc.

our insurance is going to hate us.

And there are about eightyfourthousand loose ends we still need to tie up.

For instance, do we bring the girls with us? It’s the week before school starts. I have countless other appointments next week that will need to be rescheduled. I have to WORK at some point. I need to bedazzle the girls’ backpacks. Dance tryouts are coming up. We have no idea if we can stay in the Ronald Mcdonald House yet, and no way can we afford a hotel for a week. Ben has to be at his work monday through wednesday nites which means that I have to leave Kendall alone in the hospital at nite so i can stay with the other girls at the RMH – IF we get in there.

Blah.

I can’t think of that – its going to make my head explode.

I have probably already made all of you all’s heads explode with all this info.

So that’s that. you got a little more than “just the facts”. I am still sorting out “how I feel” about it all. Mostly peace. It’s a super good feeling to feel like you are being HEARD. And that you aren’t crazy. And that maybe, just maybe, there’s hope on the horizon. I mean, there’s ALWAYS hope, but it is harder to see at some times than others.

Remember my favorite movie? Anyone, anyone?

Here’s part of why I love it – one of my favorite quotes:

“Hope is a good thing, Red, maybe the best of things. And no good thing ever dies.”

Full of hope tonite,

terra

This is kind of how I feel tonite.
Like I have the jitters a little bit about this upcoming week.
It’s not so much the fact that we will be flying in essentially a tin cigar with an engine on it, although I think that plays into it.
And it’s not even that we’ll be in a strange city for 2 and a half days by ourselves.
I think its just kind of the whole thing of it.
What this trip represents. Going searching for answers again. Or still. Depending on how you look at it. It was exactly one year ago yesterday that we were in Milwaukee hoping to find ANSWERS for Kendall. And one year later, here we are going to ANOTHER state, Another hospital, another doctor with another opinion. And really, I think we are doing the right thing. I think that Dr. G will have some very good direction for us at the very least.
But it’s hard.
It’s oh so hard to think about it all.
What if she DOES have the answer we have sought, but it’s not the one we want to hear? Will I have the strength to hear that all by myself without my rock of a husband and best friend to be sitting next to me? Will I have the strength to NOT get any answers and be back to wondering why/for how long my baby will need to have a mostly elemental formula diet pumped into her intestines instead of eating normal toddler things in normal toddler ways?
Oh there’s so much more but I don’t even know how to get it out of my heart and head and into words here. Plus they feel a little too vulnerable typed out here.
But I know that some of you who are blessed enough to have to deal with me on a day to day basis want to know why i am in such a funk. So this is my attempt to explain to you how my heart hurts. How every cell of my being is on edge for the weight of the journey we are about to embark on. How my head feels so clogged up with questions that I am shocked that it even remembers how to do its usual involuntary actions like breathing and digesting.

So please don’t take it personally dear friends and wonderful family. By this time next week, I will be in a much different, hopefully better, headspace. I will have a direction to set the navigational beacons in, and I will gather up the chicks and lead them towards that direction.
Thank you for sticking by me through it.

And now I need to go figure out how I am going to get kendall, all her medical equipment, me and at least one laptop on the plane and come in under our 40 lb cargo weight limit. Because the laptop and my makeup bag alone are about 10 lbs…
this should be fun!

t

(PS – I know some of you have so many questions about where we are going and why pittsburgh and why the small plane and what are we hoping to learn in this appointment – and I promise that I will get to that. Just not tonite! But feel free to leave any questions in the comments and I will do my best to answer them!)

I had such good intentions for blogging every day! Too bad all these children running around underfoot gets in the way of my best laid plans and intentions…

Anyways – in typical Ben fashion we are sitting here flipping back and forth between a ridiculously hilarious movie, and a tear-jerker. I hardly know what to do at any given moment, be sobbing my eyes out or laughing hysterically. So instead i’ll blog! Since I have so many days to go back and catch up on, I’ll try to keep this one short and somewhat sweet.

Today our fun event (besides actually making it home for long enough to finally go to the bathroom – trust me – it was no small feat!) was getting a little cooler full of fun vials and needles. It was my mail-order “start your own meth lab” business in a box!  HA – i am only kidding. Kind of.

It was full of a bunch of scary sounding IV medications for Kendall – except she doesn’t have an IV. So in keeping with my theory that her doctors are using her as their own science experiment, we are going to be mixing up these IV meds with some water, and putting it through her tubes directly into her stomach in the hopes that we can eradicate the overgrowth of “bad bugs” that are causing issues in her GI tract, re-planting some “good bugs”, and continuing to do this until…well, i guess until we don’t see any signs that too many bad bugs are coming back too quickly.

At the very least, I am looking at a couple months of learning to mix/compound these IV meds into something we can get into her tummy, and hoping I don’t screw up the math too badly. Considering I had to repeat 3rd grade math three times, and even then didn’t get much farther past that, this may not be as easy as it sounds! I spent an hour on the phone with the pharmacist yesterday (while i was simultaneously trying to explain to the apple genius that I just needed a new battery and that i knew it was more than likely under warranty still and i had already researched that there was a known batch of bad batteries that matched my battery’s serial number so don’t try to talk me into anything else) – where he was trying to tell me how simple it really was to mix all these things up. Notice the stop sign and the big bold letters “FOR INTRAVENOUS INFUSION ONLY” on the box. Yikes.

And really, I am sure it will be that simple, if I manage to get more than ten uninterrupted minutes to sit and think and read directions in any day! Here’s to hoping that can happen soon! we have the girls’ (K1, K2, AND K3!) dance recital tomorrow afternoon, our Kidstown Appreciation picnic on Sunday after church (that i still have to finish up the lesson for), and then it’s time to start packing up for our trip to Rhode Island! Which i am sure will include finding a way to keep these meds stable all week that need refrigeration. Thank God we aren’t flying. These things seriously look like I am trying to cook up some street meds. I can JUST imagine the scene at the airport…

Annnnnnd right on cue here is the stupid crying part of this movie (for those who care , it’s “The War”, and we’re both a sobbing mess).

Ok – laundry and dishes are still mocking me so I have to go shut them up.

peace out.

t-crest

That is the kind of day Kendall had yesterday. It was really and truly awesome.

She was awake for almost 5 straight hours without crashing or melting down too bad. I really don’t think that has ever happened in her whole life! It gave me hope that maybe we CAN get out of the house more this summer without having to rush home for naps! She didn’t need any oxygen (or rather, she didn’t turn colors or do anything else to indicate that she needed it, so I didn’t lug a tank around with us!) I got to see her face all day with no grippies/tape/tubes on it – I didn’t realize that it had been a few weeks since I had!

She was trying to stabilize herself more when cruising along furniture and even took on quick faltering half-step to get from one piece of furniture to another! She learned “duck” in speech therapy, went up to a harder level of beads to string in OT – i mean – really, it was a SPECTACULAR day!

I hope we get to string a few more like that together, and maybe even a couple weeks like that!

I know so many of you are waiting for the update on the cardiology appointment from Tuesday, so, here goes. First – a link to what the main issue is: Pulmonary Hypertension.

A few weeks ago Dr. A recommended that we get in with her cardiologist quicker than our scheduled follow up (in October) in order to more effectively rule out whether we were dealing with PH or not. Given the fact that Kendall has dealt with respiratory symptoms, the open PDA (heart defect), and feeding/growth problems from day one, reading about the symptoms and effects of PH was, understandably, a little scary in that it all made sense. And is a scary thing in itself to think that Kendall’s heart could be working much harder than any of us could even imagine – and what the long term ramifications of all that might mean.

So – I was apprehensive for tuesday’s appointment. Have been for a few weeks, but just couldn’t bring myself to breathe life into those words here. Or even out loud really. I was worried that there WAS a reason she was struggling so much without oxygen, and I was worried that there WASN’T a reason she was struggling so much. And I guess – as much as you can find a happy middle of the road with something like heart issues – we found out that there WAS and WASN’T.

Dr. T (heart doc) reviewed the ECG (getting hooked up to a bunch of electrodes placed all over the body) and the EKG (heart ultrasound) from this visit and compared them to last year’s tests. Her PDA (extra valve in the heart that all developing fetuses have that is supposed to close with a few hours after birth to allow proper blood flow) is still open. It was open last year at almost 6 months, and it has not changed in size at all in one year. In essence, she will likely never be able to close it on her own – although he did say that he HAS seen a few spontaneous closures over the years. Because of this open extra valve, she is having some blood flow backwards and get re-circulated in her system that has NOT gone to her lungs and gotten oxygenated, as all your blood should. This un-oxygenated blood is part of what we’re seeing when she has her “off-color” times/days.

So there is the reason there IS a problem.

BUT – here’s where there ISN’T a problem – she right now doesn’t appear to be in a bad state of hypertension (extra pressure in her heart/lungs). He feels that she probably suffers from “intermittent” pulmonary hypertension, probably during times of extra stress on her body. Which, given her body’s reactions to things, means basically anytime she is slightly off her baseline: any illness, too hot, too cold, retaining fluid, being slightly dehydrated – any of those times can put extra stress on her heart.

The good news is that if it is truly just intermittent, she will probably not even need medication. The super great news is that right now, we don’t even have to talk about surgery to close the hole. Those were my two big worries – more meds and surgery. HOWEVER, that doesn’t mean she is totally out of the woods. Part of his deciding factor in saying no surgery right now is that she has such a hard time with anesthesia (she reacted to one GA with something called “malignant hyperthermia”, meaning she needs lots of crazy precautions taken anytime she is under GA. blah blah blah its a big pain in the booty for everyone involved, mostly her, period the end.) Anyways – the bottom line is that since she is not really failing to thrive anymore, and the heart defect doesn’t appear to be THE MAIN issue that she deals with with all the other issues, its not worth putting her under GA for JUST that. If she has to go under for anything else, we will probably have a discussion about adding the PDA closure to that. It’s a relatively simple procedure as far as heart surgeries go, its really just a matter of her handling the anesthesia.

And even with the meds, it doesn’t mean that she won’t EVER need them. In fact we have a pretty short leash with trying to get her respiratory issues under control before he wants to start the meds. And i know that sounds like a tall order for miss wrench-in-the-plans, but what he means is that if she is still having some of her issues with dropping her sats, higher HR’s, blue colors, breathing too hard/fast over the next week, we will know that she isn’t just dealing with a random virus/illness right now, and that it is time for the meds. So in true Terra (all or nothing) fashion – I took her right off the O2 to ENSURE that we can hit that one week mark! No seriously – I went based on the fact that everything else seemed to be going so well, why not see how she did without the O2. And yesterday she did great.

Last nite when I got her up to get her ready for bed (hook her up to all her tubes and wires and give meds and such), she was crank-o-matic. She seemed to be in pretty bad pain, but she is just losing the ibuprofen or tylenol right back out her G-tube venting bag, so that wasn’t going to be super-helpful. I am assuming at this point that it is related to something with her gut function – since last Saturday when she vomited up some mostly undigested french fries from 12 hours earlier, she has not been real hungry acting at all, and in fact has screamed at me when i tried to give her food. VERY unlike miss K4, but she seems otherwise to be holding her own, so maybe it is just a “normal” picky toddler thing. Of course, in typical K4 fashion, there are other factors playing into it – namely her tummy is hugely distended and is hardly relieved by venting (typically when her tummy is huge its just a lot of air that once we get the air bubbles out, her tummy goes down), as well as what is coming out the other end is just indicative that things aren’t actually moving like they should. A fellow SN mom suggested to me that it might be the overgrowth in her tummy acting up again – and that wouldn’t surprise me really. It’s just that there’s so many theories and factors going into one little body – it’s hard to say. Bottom line – she is going on about the last day she can probably tolerate having less than half her normal intake (of calories – whether that is food she eats or formula she drinks/gets tubed in). And then she will find herself trying to walk around with a backpack strapped on and food running continuously.

Luckily, she has her 18 month “well-child” checkup with Dr. Natalie tomorrow, so I am sure that we can come up with any other thoughts on what may be going on. She is definitely not acting “UTI’-ish” like she was last weekend – so either the double dosing of abx all week helped keep the bugs under control (and probably resulted in the crazy tummy issues we see now), OR – it was never UTI’ish to begin with, and is instead just some other viral silliness throwing her precariously balanced system offline. Either way – I am sure she will tip one way or the other over the next couple of days and then we’ll have our answer.

But for now, we will take yesterday’s spectacularness. And hope that we continue on that path for today. And tomorrow. And maybe even the day after that…

Hope you are all having a spectacular day!

 

terra

I seriously cannot believe Kendall is inpatient for the third time this year already. We have been VERY lucky that they have all been quick stays (well, we hope this one is quick!), and that we have known what we are fighting each time we have been in.

I am tired and need to try to sleep while I finally have Kendall asleep, especially since 6 am lab draws come WAY too early around these parts, but a quick update for those of you still up and reading this –

 

Kendall has been showing signs of  her usual “NQR” status (not quite right) for about a week now – things like spiking a slight fever out of the blue, having high HR’s, breathing too fast (being tachypnic), having really weird (meaning super gross smelling) urine output, and in general just being “off”. By Monday i was starting to think we might be dealing with another UTI (thats what she was inpatient for last month in milwaukee), by Tuesday i knew we were more than likely headed that direction, and then this morning I finally made the call to call the ped’s office, knowing they would want to catheterize her for a culture.

Sure enough, we get there at 12:30 and they IMMEDIATELY take us back to the treatment room to get her labs drawn/cathed urine collected and hand delivered to the lab for a stat reading. I wasn’t really honestly TOO worried about kendall until dr. natalie tells me that she is pretty worried for her based on how bad she looks, and that we need a GOOD plan and we need it SOON. Knowing that we are fighting against the bacteria overgrowth in kendall’s gut, we needed to find out from Dr. Noe in milwaukee what his recommendation was for starting the ampho (antibiotic to kill bad gut bacteria), as well as anything else related to oral abx. We needed to figure out if we should have a PICC line placed for longer term antibiotic therapy, and if so, where? It was a long afternoon of waiting, running around, waiting some more, and then fighting traffic, but here we are.

Home away from home, 2Center at Lutheran General.

The HOPE is that she will get started on IV antibiotics here, they will run a few more kidney specific tests to figure out WHY she is either fighting the same UTI from last month, or has a whole new one, and hopefully she will have a PICC placed when they run any tests that need sedation. The PICC will allow her to be discharged to home quicker (vs staying inpatient for ten days for a full course of antibiotics), as well as be able to address whatever crazy thing is going on with her fluids, kidneys, bladder, etc. As of right now we are not on the surgery schedule for first thing in the morning (and i say this only because no one has come in to take kendall NPO, switch her to IV fluids only, or tell me she is having surgery in the morning!) – so i HOPE that someone gets their act together overnite and we are still scheduled for a quick placement at some point tomorrow so that we can have home nursing set up and waiting for us and POSSIBLY get out of here tomorrow nite, or barring that, friday morning sometime.

We just have way too much going on this weekend to wait around for bureaucratic orders to be followed and written!

So that is what is going on in a nutshell.

She is responding nicely to the first two doses of antibiotic she has gotten – that is encouraging. She is finally sleeping, although she keeps crying out in pain and having a subsequent rise in HR and drop in O2 sats with that. I just want a plan to be magically put into place overnite….

sorry this is so discombobulated – i’ll hopefully have a more coherent post in the morning!

 

terra

Holy cow. Poor little neglected blog! I need to figure out how to get my facebook posts to update here, because i feel like the little random snippets off of FB would help explain why it is so long between updates.

Tonite I am tired. And kinda sick. I get it every time the weather changes, but today’s was particularly hard as it was just a cycle of pain, vomiting from the pain, vomiting up any meds i tried to take to stop the vomiting or the pain, etc. Pain meaning a headache from the outer recesses of hellish places, and i swear to you it was pushing my eyeball out of the socket. At least this is what it felt like. but that may or may not have actually been happening.

Anyways – nice spring break. Our fun family stay-cation to chicago started with kendall waking up tachypnic, tachycardic, and slightly febrile. Not wanting to disrupt our nicely planned vacation, we decided to not pack any of her monitors or O2. I also thought it might be a nice touch to not pack ANYTHING that a medically complex kid might possibly need to survive a nite away from home (syringes, meds, gauze pads, etc). It’s fun times! Knowing it was only for one nite though, i realized you just gotta let go and do the best with what you have (or don’t have)! i can say though that kendall is NOT a light traveller! We have two family trips in June/July that are probably going to require a small u-haul JUST for kendall stuffs!

So after surviving the nite, we wanted to take the girls to the Museum of Science and Industry downtown, mostly to go on the coal mine ride there. Now i am going to tell you a story about the coal mine ride. From the time i was, oh, I think in Jr. High (12 years old) and we first went on that ride on a field trip – i have been IN LOVE with that ride. I don’t know why. I love taking the elevator down below the museum, below the parking structure, down into the depths of the IL coal mines, making the machines start up, watching the gas lamp go out and kill the bird, etc etc. All of this was a REAL reality to me until my darling Father In Law decides one day to tell me “Terra, you DO realize that the elevator is just going down about 15 feet into the basement? It’s all just an exhibit.”  I was dumbfounded. i was 22 years old at the time and No, I did NOT realize it was only going down into the basement. He may as well have told me that Santa isn’t real and the Tooth Fairy doesn’t exist. To this day i am not sure that i really believe him because that ride is SO REAL!!!! (and i still love you Poppa Bob, even if you do burst my make-believe bubbles!)

So i really wanted to take the girls to see this. But the line was at least two miles long, and the babies were crank-o-matic just getting from the parking lot to the entrance. So we had to make a tough call to try to come back on a NOT spring break week, and without two babies in strollers. Instead we went to have a fun family lunch, and while we were there, we get a call from some dear friends inviting us to a nite of fun at a resort west of here. I’ll refer to this place as “BFE”. which means “berry far e-way” to the uninitiated, or any of my mother’s friends who are reading this who may be offended by my potty mouth. (my mommy makes me say that). Anyways – so we rush home, I finish up some work stuffs, we re-pack, and me and the olders (K1 and K2) start the drive out to BFE. We arrive in BFE to find NO CELL PHONE SIGNAL, so we can’t find our friends. At this BFE resort, there is an indoor waterpark that we THINK everyone is at, so the girls change into their swimsuits, and we trek through the bathrooms to go hang out while we find them. Long story short – we found them the girls swam till they were sick from chlorine exposure, and we went back to the condo for a nite of fun, hyperness, and lots of Twister games with friends. But back to the fun-ness of BFE.

on the way to BFE i hear a commercial that advertises it’s location as being “on your way to the Big Super-Walmart in Ottawa”!!!!! (which is a slightly larger version of BFE 20 miles up the road.) We pull in to BFE to find that they have a “Get your picture taken with our baby bear cub/tiger/other animal that may or may not turn rogue and rip your child’s face off while we capture the moment with a polaroid” exhibit. And NO KIDDING, they really had a for real baby bear cub IN A PACK-N-PLAY, waiting for you to get your child’s picture taken HOLDING this wild animal. I just do not understand this phenomenon….If God wanted us all to have black bears as pets to hold and cuddle and love it like a naughty pet, we’d all live in Alaska and have leathery skin. That may be just my opinion though. Anyways. The final straw to being in BFE and knowing it was when we walked into the bathroom/locker room and they had a HUGE sign on the wall that said “Please do not swim if you currently do or have had diarrhea in the past 24 hours. This especially applies to babies in diapers.”

I don’t even know what else to say about that. I mean really? People do this??? WHY would you even WANT to go swimming if you are having to run out of the pool every ten minutes to hit the pot? And if you are changing diapers like it’s your job cause your baby has the hershey squirts – perhaps you should consider keeping the baby in the hotel room and watching some cartoons. But i just kind of thought it was a given that you don’t go swimming when you have diarrhea. Apparently it’s not.

Anyways – we had a very fun nite with friends, and I was so happy that I got to be doing something fun with JUST the bigger girls for their spring break. All of that was shattered though at breakfast Tuesday morning when i open my phone to see a series of missed calls from Ben, right as he calls my friends cell phone and asks for me. I hear kendall screaming in the background and ben is asking where the old NG tubes are. NOT a good combo. Sure enough, she had pulled her GJ tube out. She used to pull her G-tube/button out with some regularity, and while it did involve a couple of ER trips to have an x-ray confirm placement, it was not really ever a HUGE emergency (except for the time in September that she ended up crashing really bad because of dehydration/blood sugar issues). But usually you just pin her down, make sure the stoma is open still, and jam the button back in (after you’ve checked the balloon to make sure it’s not leaking). Blow the balloon back up with a shot of water and there ya go. Good as new.

With this new tube – the part that you can see dangling out of her about ten inches, has a similar looking tube on the INSIDE that goes down into her intestines about a foot. So she had pulled a 3 cc balloon PLUS 12 inches of tubing out of her stoma. This happened because her balloon SHOULD have had closer to 5-7 ccs of water in it, holding it more firmly against her inner tummy wall. So clearly the greenish black stuff floating around in the balloon (once we could inspect it after she had pulled it out) must have somehow eaten a small hole through the plastic, caused it to have a slow leak, and out it came. Since i was still in BFE, Ben called a neighbor to come help hold a THERMOMETER in her stoma (because the main goal is to keep that stoma open SOMEHOW, with SOME kind of firm tube in place, so we can eventually replace the tube.) He couldn’t get a G-button to go back in, couldn’t find another kind of tubing up here in our medical cabinet/countertop area, was searching the basement for something, and nothing was really going in. So I told him it was time to take her to the ER (because, God love him, but if you’ve ever tried to un-do a lid that Ben has put on, or take something out of a container it is not supposed to fit into but Ben thought it should fit so he made it – you will know that if even HE can’t get a tube to go in the stoma, we must have issues). I flew as fast as I dared back up from BFE (an hour away) and met Ben in the ER, where they were as equally unsuccessful as Ben had been (but will still probably charge us a couple thousand dollars for “surgical procedures and supplies”, because they tried to place a BLADDER catheter into her stoma, and our poor nurse had to run up three flights of stairs to personally look for a more appropriate tube). So we thanked them, packed her up, went home so i could re-re-pack, pack kendall back up, and drive up to milwaukee for an appointment in IR (interventional radiology – basically it’s a “live view” X-ray. And yes Kendall does officially now glow in the dark from all the radiation she has received in her life.) Anyways, after finding the freeway i take to the hospital is GONE (they are trying to re-do it???), I manage to figure out the crazy detour and get her in to IR with fifteen minutes to spare before close! YESSS! I basically have to present a thesis statement to the nurse about why I think Kendall needs the sedation meds (are you kidding me? she has had random objects poked into a HOLE in her STOMACH for the last 7 hours, and now you have to manually DILATE her stoma and THEN place a whole new tube in it and you want to do all that while she is totally awake and aware???? Good luck! I’ll be down the street banging my head on the cement sidewalk to block out the sounds of her screams – call me when you’re done!) And luckily, the doctor agreed with my assessment of the situation.

now, when they set the legal drinking age at 21 years old, I am going to guess that they lined a bunch of kids up at random ages, gave them some sedation meds, and the age that it stopped being HILARIOUS to watch them at was probably 21. I am not encouraging sedating children (not all the time at least!). But I will say that seeing those meds hit Kendall’s system is one of the funniest things I have seen in a long time. She thought EVERYTHING was hysterical, and just had this totally goofy laugh going non-stop. She couldn’t figure out what to do with her binky and just kept holding it up to me and then laughing, and then laughing harder when i would laugh at her. It was all fun and games till they strapped her down to the table and moved the big machine over her head. She said “hi kitty!” to the x-ray, they yanked the tube we had gotten in, and the screams then ensued. They only had to use two sized dilators (vs. the five they had to use last time this happened), then they put the tube in, it got “stuck” going around the curve of her colon so they had to start over again, and this time they got it. We shot her full of some barium for good measure, watched it flow out into her intestines, and called it good. She was literally smiling and saying hi to random (non-existent) kitties within about forty-five seconds of getting out of the restraints and into her stroller, so I think the sedation was a GREAT call. I don’t know if she will need sedation before every tube change from here on out (GJ’s get changed out every 3 months as scheduled changes in IR. Unless you are Kendall. Then you set your own schedule which is more like every 4-5 weeks), but i can say that at least until we can “reason” with her a little more about how much quicker the procedure goes if you AREn’T squeezing your stomach so tight they can’t push the tube through, i think we may be looking at the sedation.

Anyways – we stayed the nite with my mom so i didn’t have to drive all the way home in rush hour traffic, only to turn around and drive back up to milwaukee the next morning for our regularly scheduled GI appt/followup. I didn’t really have any huge expectations for this appt, as I felt like things were mostly under control. BUT – in a huge ironical twist, because she had just pulled out her tube and i was able to show it to the nurse and say – what is this gross stuff growing on the inside of her tube? it was starting to clog up the tube and is IMPOSSIBLE to wash off – and she said she would have the doc look at it. He comes in and immediately wants to see the tube and says “uhhh, yeah that looks like yeast!” I encouraged him to smell inside the ziploc baggie and see if the beer factory smell emanating from it supported that theory and he said it certainly did! So what does this mean?

IT MEANS I WAS RIGHT!!!!!!! (not that i am gloating over this or anything).

I have been telling her docs for MONTHS now that I just don’t feel like oral abx are working on her. I question what is happening with other oral meds, but oral abx typically give us a couple days of good stomach functioning, then nothing. No diarrhea like you would expect when most babies are on heavy duty antibiotics, no real relief of symptoms (be they respiratory, ear infection, or UTI related), it is as if i am putting water through her tube. And these little yeasty dots confirm that!

She is believed to have what is called “Small Intestinal Bacterial Overgrowth” (SIBO). This is not a main diagnosis – rather, you can just add this onto the pile of other random diagnoses she has. This is a large piece of the puzzle though, in that it confirms that her motility (movement of food through the intestines) is really as crappy as we thought (sorry grandma and mom’s friends). Having yeast (and more than likely other fun bugs that should never see daylight but ARE supposed to help turn your food into poop lower in the intestines) coming out of her jejunal and stomach tubes, and indeed leaking out around her stoma and irritating the skin all over her tummy most of the time – means that when her lower intestines are shutting down, or moving VERRRY slowly, everything is kind of backing up, including that not good stuff. It is getting up to where there’s air, and more food, and other kinds of normal gut flora and it is just having a PARTY up in there, multiplying and wreaking all kinds of havoc on her stomach (most likely why we had to go to the J-tube in the first place). But most of all, when oral antibiotics are going into her stomach, they are encountering these “bad” bacteria, thinking THAT is what they were sent there to destroy, maybe controlling them for one or two days, and thereafter every dose is just consumed by the bad bacteria. Never reaching her bloodstream and attacking the REAL infection she is on them for in the first place.

i think that my initital reaction to this insight from Dr. N was to jump up and go “BOOO-YAHHHHHH!” I KNEW IT! I have had no way to quantify this concern to the docs, so they have blown me off for the most part, BUT NOT ANYMORE!!!!! I realize I am ridiculously giddy over what is still a not good diagnosis. But I’ll think about what it really means later. for now, there is a REASON, and for now, we think/hope that we can get it back under control. The plan as it stands right now is to let her system see what it can do on its own with this new tube. It may take three days, three weeks, or three months to see the yeast overgrowing the tube again. But when we see it, Kendall will have this tube pulled out, a new one put back in (hopefully the button this time since I convinced IR that 20 and a half lbs is actually VERY close to their random 22 lb mark for getting the buttons!), and she will start on a heavy heavy duty antibiotic into her gut and another one that will just sit in her tubes to keep them from letting the bacteria and yeast attach to them again. She will cycle through this antibiotic (Ampho-B – i am not sure how to spell the full name) for one week on, three weeks off, for at least a few months until we seem to have the bacteria under control and growing only in the place they should be growing. We will discuss whether a probiotic would be appropriate or helpful at that time. (Since I get asked that question a lot!) MOST probiotics work by putting MORE bacteria into the stomach/upper gut that get eradicated by antibiotics in otherwise healthy individuals. Kendall’s gut flora is already a little out of balance, and adding in MORE bacteria to a bacteria party is not something we want to just do willy-nilly. Trust me that I am the FIRST ONE to go for a more naturopathic approach to things with all my kids, and if a probiotic will be a better alternative than this big gun antibiotic, we will do it.

Anyways – it’s been a very very long week.

I am hopeful that we will get to still do our easter egg coloring, easter egg hunt, easter basket finding this weekend in spite of all the craziness. This will involve me getting a quick healing from this headache/pain so that i can fight the Good friday shopping crowds, and just a lot of other logistical issues to be worked out.

There is a lot more I wanted to /need to blog about. hopefully that will come tomorrow. Basically the jist of it is this – they believe they have narrowed down Kendall’s issues to a “probable” mitochondrial defect, but they do not know exactly WHAT defect she has (they have only identified about 50 out of what they presume to be thousands). They don’t feel like she has one of the more known to be very life-shortening ones (that is just their words, i am not sure what they mean in the greater scheme of mito defects), and do hold out hope that she will continue to grow and progress along a good curve. That is about as much hard and fast info as I could get out of the geneticist on the Monday we saw him inpatient.

Her paperwork/records at the hospital have her official diagnosis listed as “mitochondrial disease”. This is a change from the previous “MMA” (metabolic error). She still has a metabolic issue – her MMA levels are typically all over the map with no rhyme or reason. But since she doesn’t have any of the known genetic defects that cause MMA as a disease, they are attributing her MMA levels/issues to a bigger picture “mitochondrial defect/disease”.

This is still kind of sobering, and i readily admit that I do not have all the answers about this. We do know that we are in the process of gathering up all of her records to send to a mitochondrial disease specialist in Pittsburgh for review, waiting to hear if they think she should be seen by their mito team, and if so, making travel arrangements and hoping our insurance (which is based in Pittsburgh oddly enough), will cover the visit out of state. Many questions still in the air, but at least a few more arrows pointing us in the right direction hopefully.

 

With that, i am off to try to sleep off the rest of this horrible headache.

 

Sorry its been so long – I’ll try to be better over the next couple of weeks!

 

terra

Ok so it wasn’t bloody so much as it was…..sucky.

Sunday morning Kendall had to get labs drawn early, but blessedly they found a good vein rather quickly and she was soon back asleep. She ended up sleeping till almost noon when her sissies and daddy came to visit. She instantly tried to laugh when she saw them, but it was so pitiful how weak/tired she obviously still felt. After about an hour of seeing them, trying to sit up and play with the toys they kept bringing her from the playroom here on the floor, and just enjoying the chaos that only they can bring to the room, she was ready for a nap again. We slowly bumped up her J-tube feed of pedialyte another ml per hour, got a little more fussiness, but she took a pretty good nap while I enjoyed just being with the other babies playing in the playroom, doing crafts, etc.

The rest of sunday was just – gross. I was done. Kendall was done. There were too many people with differing opinions gathered in one small place, too many plans and we were all just too far away from home to deal well. I ended up going to my grandmas house to take a shower and a nap while Poppa stayed with Kendall and got another taste of hospital life (thanks Dad!) – the nonstop flow of nurses and CNA’s and residents asking question after question that you may or may not know the answer to. He was sick of it after four hours! I was also able to get some happy blue medicine which is a surefire way to fall asleep even while inpatient. Or if a tornado happens to be hitting your home. Either way. You’ll probably sleep right through it.

So I came back, fought a little bit with the nurse about the idiocy of the fact that THEY stopped her calories (her IV fluids per her protocol have some calories in them, mixed up special in the pharmacy), and put her on regular maintenance fluids (which is a bag of potassium – which i thought was ridiculous for a kid who is, at baseline, on the very high end of hyperkalemic) and then wanted ME to get some calories into her. I told her she could figure it out, I was going to bed. But if you make my baby scream in pain cause you put too much food in her at once, there will be some words exchanged.

So they managed to get 2 ounces of half strength formula/half pedialyte into her before she had to be “NPO” for the nite because of her test the next day. She cramped and whined through the whole nite because of it, had a few sat drops and coughs indicating that it was being refluxed up from her jejunem into her stomach, and that then her stomach was trying to get rid of it.But overall, she survived the nite ok with it. And then we were on to Monday morning. I love writing these posts 2 days after the fact – it always makes for such an awkward wrap up/transition/segue….

terra

Better known as “breathing”. Taking a breath. Having oxygen exchanged for carbon dioxide in your lungs.

you take for granted that all of this AMAZING stuff happens when you inhale and then exhale – indeed, how often do you stop and even have to focus on inhaling or exhaling? (Assuming that you aren’t currently suffering from a cold/sinus/allergy/asthma type illness, that is). I mean really – do you KNOW how awesome it is that things work RIGHT most of the time??? I didn’t – and still really don’t get all of it. But I have learned enough to know that when one small part of this process is interrupted, it can definitely start causing a lot of big wreckage further down the line. This is kind of where we are at with Miss KQ.

My thoughts are a little scattered on this all tonite – probably because there is just SO much to sort through and try to process and turn into understandable info. Basically – she is doing “ok” – as in she isn’t in any kind of downward spiral at the moment. She is playing and laughing and learning new things every day. For all intents and purposes, she seems like a normal healthy baby from the outside. Unfortunately, you can’t always judge a book by its cover though – and you can’t always judge a Kendall by her few good daytime hours. Starting last week – almost exactly one week off of antibiotics, 10 days after being in the hospital for her last respiratory illness, she started up with MORE cold symptoms. And while I was about 97% sure it was (is) just your basic average common cold – it REALLY started taking a toll on her little body. What does that mean exactly? Well – it started with something so small that probably no one else would ever have noticed it – but it just seemed so ODD to me – her right eye was purple-y and swollen. It was really noticeable in pictures that day, and I could not think of any reason why she would have a black eye – she hadn’t banged it, there wasn’t really a bruise there – it was just the shadow of the puffiness. The next day it was the other eye that looked the same way, and that nite her pulse ox alarm kept going off because her HR was in the 190’s (while she was in supposedly deep sleep). She was satting well (keeping her oxygen saturation number up), so I just raised the parameter on the alarms and tried to get some sleep myself. The next day she started in with the respiratory symptoms and that’s how i knew everything else was just signs of her body trying to fight something.

when she is getting sick, she does pretty much what you would expect: her nose starts running and doesn’t seem to stop, she gets cranky, she sleeps a lot more, she coughs, she doesn’t want to eat. AND she does things that you wouldn’t expect: her runny nose looks eerily like it MIGHT be her formula regurgitating up and out her nose, she goes completely rain-man cranky over the craziest little things (changing her diaper, giving her too many pieces of cereal on her tray,  putting shoes on/taking shoes off, take your pick), she goes from sleeping 17-19 hours a day to 20-22 hours a day, her cough often makes her gag/retch/vomit, and not eating means that the little food she is taking in orally causes major choking episodes, the food that goes into her tube either comes back up or causes tummy pain, so it has to be diluted with pedialyte or changed over to straight pedialyte. All of this combines then to make more problems in that she then will either be peeing out too much fluid (and thereby looking more puny/bony), or she is retaining water and not peeing for the whole day (making her look nice and chubby, except it’s all just water puffiness!). We were supposed to follow up with nephrology last year after her Feb/March admissions – but got so sidetracked by all her GI issues that kidney issues took a back seat. We know that there is SOME dysfunction going on in there (as evidenced by every set of labs we draw on this kid), but aren’t sure how it all ties in with the metabolic stuff. I am hopeful that our marathon of appts next week can help us get some direction in this area.

So anyways – that is kind of what is going on for the last week – nothing that by itself is horrible, life-threatening, or ER worthy – but it is an overall picture of many processes not quite working the way they are intended to, and me feeling like I don’t know which hole in the dam to plug first, or really, how to even go about plugging dam holes in the first place!  By Thursday she was vomiting bile, having a lot of bile and pressure come spewing out her g and j tubes, having the high heart rates but without the good sats, and was just a sick baby. i spent Wednesday nite and Thursday nite on “ER watch”, where I am making plans B-G about how to get her to the ER, which ER, what to do with the girls, etc. – and so by Friday I knew that if i didn’t call and talk to SOMEONE, that I would be regretting it by 4 am Saturday morning when i was once again on ER watch! So I called Margie, Dr. A’s dear long-suffering nurse and left a message something to the effect of  ” Hi Margie! i am only calling cause it’s Friday and we all know how kendall likes to get sick on the weekends! So if i call, then she’ll be fine, and if i don’t call then she’ll be sick so I am calling! here’s what she’s been doing – I know Dr. A isn’t even in the office today, so there, I called, you know, we will see you on Monday for our regular appt!”   She called me back about 3 hours later and told me that oxygen would be delivered that afternoon.  She had paged Dr. A, told him what i told her, and he said enough is enough – she needs the support.

Sure enough – that evening, Ben and I were officially “oxygen therapy” trained on how to use an oxygen concentrator and portable oxygen  pumps for Kendall. I guess i was thinking that Dr. A meant “at nite, if she is having a hard time with sats and/or her heart rate, then turn it on super low, only going up if she isn’t responding”. I knew it would help keep us out of the ER for the weekend, but really thought we might get in to his office on Monday for our appointment (which was just our post-hospital follow up) and that he would say that she didn’t really NEED it, and we could send it back. I think Ben thought even less than that. Kendall HATES the nasal cannula (well she hates anything on her face or hands, all the time, any time) – we found it around her neck after an hour of trying to get her to sleep with it – so we took it off and just prayed that she would be ok without it. Tried to not give it another thought.

Until today’s appointment.

Basically I came out of the office today with four main takeaways:

1.) The oxygen is needed. She needs it to support her overall energy levels, to boost her breathing, to help the metabolic processes that are being disrupted by either the MMA or just her current illness. Bottom line from the big man: “I ordered it for a reason – and that is not to just sit around your house. Use it on her, use it liberally, and HELP her get better and stronger.” Ok! Point taken!

2.) Her lung issues/recurrent colds are more than likely caused by aspiration. either from above (when she is attempting to swallow) or from below (when she is vomiting/retching as a reaction to who knows what – metabolic stress? reflux? pluto not being a planet anymore? take your pick!). WHERE it’s coming from isn’t really the issue. We’ve done what we can to stop it from coming UP (the fundo surgery and now j-tube feeds), so we need to look at eliminating it being aspirated (swallowed into the lungs) from above. which means taking her “NPO” (no more bottles, possibly no more food by mouth). He is willing to wait a week to enact “PLAN A” – which is that her video swallow/follow-thru test next week in milwaukee shows that she IS aspirating, and her GI will direct the orders to go NPO.

3.) Plan B would be that the test “appears” normal (meaning that she doesn’t directly aspirate or reflux the food during the 20 minutes of the test) – but we decide to do a 2-3 month trial of making her NPO on our own (with the blessing of all of her doctors), to see if we don’t see improvement in her lung issues. Dr. A is pretty confident that either way – Plan A or Plan B – that we are dealing with an aspiration issue here, but that if we take her NPO for a year or two, it will protect her lung tissue from any further damage (and is hopeful that because she is so young that any damage she DOES have right now can be undone because lung tissue is still regenerating at such a fast pace the first 3 years of  life), it will eliminate these seemingly non-stop respiratory illnesses she is dealing with, and will allow her to build her strength and coordination up to be able to re-learn how to properly eat/swallow/chew without needing the Heimlich once a day (at least!). It sounds like an awesome theory. But I know it would be devastating to her (and us) to have to take her NPO. she LOVES eating. loves it. loves the taste, loves being with us at the table, loves the social interactiong of it – everything. It would be very hard to deal with. BUT – like Dr. A pointed out – at least we have SOMETHING we can try. Many kids do’nt even have a Plan A OR a Plan B. So I know I should be thankful that we can at least TRY this.

4.) the last part of our conversation was about the fact that, while he feels the chances are slim, there IS still a chance that this could just be her baseline – that the hypotonia (muscle weakness) in her trunk/accessory muscles (used for breathing), in the smooth muscle of her diaphragm and lungs – that its just TOO much for her body to ever be able to really breathe/cough/function right. Her heart might be more of a culprit here (again – hypotonia affects the muscles – heart being mostly muscle). There are a few other darker paths that we may need to go down if we get to Plan C – but for right now – we are very hopeful that one of the first two plans will help get Kendall on a good path. One without a lot of food on it – but a good path nonetheless!

So that’s what I am working through tonite. I am sorry if it doesn’t make much sense. It got a LOT later while I was trying to finish this up…

and I still have a good 30 minutes of tube attaching, cannula taping, food preparing and machine priming ahead of me…..

Hoping that tonite you are all inhaling and exhaling without much ado. Thank God for that awesome ability the next time you think about it.

Peace out.

t

I tell you – it is always somethin’ around here.

I will try to do a real update tomorrow, or very soon.

Between last week’s ER visit for the hives (me), Kendall’s random day of violent retching/body trying to digest her tube on Saturday, and another day in the ER for stupid abdominal pains of unknown etiology (me again), it has just been a week around here.

Besides realizing that I have a serious love of the narcotic medicine Dilaudid, nothing spectacular has come of any of our random medical issues. Luckily – everything else has been smooth sailing.

So after I try to sleep tonite (because the half a glass of hot chocolate i tried to consume two hours ago is still causing me enough pain to swear off hot chocolate for at least a year) – i will attempt to do a real update.

Thank you so much for all of you who are praying continually for us.

ONE of these days – i will have not updated simply because our life is so boring it would bore me to tears to write about it.

 

keep on keepin’ on.

 

terra