Doing Great!`

That was Dr. A’s assessment yesterday at Kendall’s routine 3 month checkup with him.

“she’s doing and looking great – and I don’t say that to minimize all that she has been through or that she still deals with every day. I say that because I know how hard you have had to work to hear those words – and so I tell you them today – she is doing great! NOW – let’s discuss how ve going to keep her this way…”

He is so amazingly special to us. He knows exactly how to word things just like I need to hear them. And he just knows his stuff.  Anyways – we reviewed the huge packet from pittsburgh with all their specialists notes and tests and results and whatnot. Basically he told me that he knows why we had to go there, why we had to get confirmation from a “mito center” that mito is what we are dealing with, and to get everyone on the same page with regard to her myriad issues under one common “mito umbrella” – but he said that really, nothing has changed from how we have been or will be treating her. (Which is true – as long as HE is her attending – but when we are in milwaukee or the ER there at LG, different story!) So we discussed that – if we absolutely HAVE to come in through the ER, use the protocol letter, but he has put into her file already that no matter what her presenting issue is, he is to be called as the attending. The reason this is a good thing is that it means we may not have to trek up to milwaukee this winter for every little illness. Whereas last year we were still kind of being bounced around by everyone not knowing what the real deal was (Dr. N her ped wants her admitted to whatever hospital she is on service at or LG, but LG would not take her if it was a GI issue, and who wants to fight a snowstorm to drive 4-5 hours up to milwaukee only to be told to give her more fluids, etc etc etc) – this year we know that for most issues requiring “stabilization” and/or a short course of IV abx, we can probably still come to LG. As long as her gut isn’t completely shutting down, we won’t try to have the idiots GI team put in their two cents (which is still overpriced in my opinion).

Between the bronchoscopy and the sedated echo – which was a lot of mumbo jumbo even to my medical journal reading self – Dr. A was able to see that her left airway floppiness is probably a large component of why her heart has to work so hard, which is probably a large component of why she needs oxygen so randomly. He broke it down really well and I wish I would have recorded it or something – because it was kind of like “Mito + Kendall 101”. Her body being in a near-constant state of “brownout” energy wise from the mitochondria not quite functioning correctly means that she has hypotonia. Not that she is always a floppy noodle, but that her muscles inherently have the strength of a stretched out rubber band. Since we have a pretty good idea that this is systemic/body wide, we know that her hypotonia contributes to her weak lower GI tract (necessitating the GJ tube to get nutrition moving to the proper place), it contributes to her bad reflux (which is why she has the fundo wrap), it contributes to her less –than-ideal swallow (which is why we now are thickening her oral feeds), it contributes to her weak accessory muscles (which is why she has crappy lung health, cause she can’t ever really inflate her lungs/move her ribcage like she should to get full deep breaths), and all of this is what we deal with when things are balanced and HEALTHY. This is her looking “great”. Add in a little virus, hot weather that throws her autonomic stability down the toilet, cold weather that does the same thing, being too tired from too much therapy or a fun weekend out with the family – and bam. Domino effect in action. Her body starts robbing Peter to pay Paul for this energy debt it creates trying to throw energy at whatever body system needs it the most – and that is why we end up on oxygen on a monday when she seemed fine on a Saturday.

So – what all of this means to us is that we now have proof of what has long been suspected. Her lungs and her heart are working pretty darn hard. And praise God they are keeping up with the demand thus far. Her little brain is SUPER smart and knows to suck energy from other parts in order to keep these two main things up to par. So – it makes sense then when some days she doesn’t seem to see real sharply in focus, or her hearing is about as good as her Poppa’s (Ronn, not Bob!!!), or she is doing the drunken sailor sloshy walk – those are days when her body has decided to temporarily suspend shipping energy packets to those systems so that her main systems can have the limited supply that is on hand. If we can get her to continue taking good restful naps, give her a little boost with some extra o2 at nite, cut back her risk of infection as much as possible, and keep her gut hydrated – we give her body the leg up it needs to continue making enough energy for all systems to have what they need. And sometimes we just won’t. And that’s when its time and when its "ok” to call in reinforcements for her – more neb treatments to keep her lungs clear, more prednisone to keep swollen airways, swollen guts, swollen bladders from becoming more inflamed, more fluids through the j-tube, more daytime o2 – and if all that is still not enough, its time to head in.

Again – nothing that is surprising. nothing really that entirely different from what we have always done. But now we KNOW the process by which things are seemingly coming out of the clear blue sky, so we can better analyze when to make the call to get her the help and support she needs. this, for me, is huge. I have always hated playing the ER guessing game – and now I have a little bit better grasp on when to go in and when and how to ride it out at home.

The only big changes he made for now are that we need to be a little bit more aggressive with her maintenance nebs during this coming up cold/flu season, and when we see her having her “blue spells”, immediately start a rescue neb and put her on o2. Leave her on the o2 for a few hours and if she is still bronchospasming, take her in. (can you hear me groaning and rolling my eyes at this?ugh. I am so bad at nebbing myself even when i can hardly breathe! I feel like this is a recipe for a one way pass to inpatientsville from december through april!!!) But – you know, maybe she won’t need any rescue meds at all and we’ll be a-ok! The other item that came up during this discussion was that Dr A was able to extrapolate from the pittsburgh reports that because her heart is doing so much work to compensate for her left airway collapsing so much, she is likely having essentially “asthma attacks” that aren’t really looking or sounding like asthma attacks, and that the reason we see such improvement on the o2 even at times when her vitals seem ok is because we are allowing her system to “relax” a little by giving it the oxygen it is working so hard to pull out/exchange in her one good lung during those times.  Ummmm…..yeah….good point…..(me feeling like a sheepish idiot). I mean, I’ve only had asthma myself for some 20 years geez! It’s not like i actually have a good understanding of WHAT it all means –but you would think that MAYBE i could have recognized what was going on in my own kid??? Anyways – i felt dumb when he had to spell that out for me. But it makes so much sense….

And, in our never ending battle on this issue, he seems to have gained an upper hand with the trump card of the “swallow study”. @#(%)*@#  At least we have a good compromise. She can have her oral feeds (bottles mostly), as long as we thicken EVERYTHING to nectar consistency. And she can still have her hard crunchies (cereal, toasted bagels, firm noodles) – but we should try to limit her drinking as much as possible, and at the first sign of respiratory illness (be it a cold or something worse), she is supposed to be basically NPO (no food by mouth). the ONLY good part of this is that I think even Kendall is starting to understand that food is sometimes what is hurting her, and has begun to be much more self-limiting in how much/when she wants to eat. of course her “bubba’s” are a whole other issue, and she hates when we thicken them, but we’ll cross that bridge when we come to it.

So there you have it. The latest and greatest on miss KQ.  She is actually having a GREAT week. Her GI called in some steroid cream last week for her tube site (which was turning into a literally hot bloody mess), and that is seemingly calming down nicely, to the point where i am not not as scared that changing out her tube will cause massive skin tearing/breakdown. And now i need to schedule that fun trip up to milwaukee, unless I can somehow con her GI into calling in orders to children’s memorial and seeing if we can do it there. It’s at least a little bit closer to home! So – we’ll see. either way – it has to be done soon! i have her balloon so full of water that you can see the outline bulging up through her tummy skin! But it was the only way to stabilize it better so her site could heal! Never a dull moment around here. Less freak-outty ones than others…but never dull!

Thanks for checking on us!

terra

Keep on Fightin’

So I never blogged how I felt or how hard i was hit by one of our mito friends’ journey Home last week. Or how it all fit together in a week that was tough and surreal to begin with. Or how I managed to stay upright without curling into a hot sobbing ball of emotions on the floor.

First of all – please lift up the Knight family in your prayers. Today are the services to celebrate the altogether too short and yet still amazing life of their little boy, Samuel, who received his healing from Mitochondrial Disease last Thursday. Even though on earth he was never able to run around free of tubes and wires, or enjoy pizza and brownies and ice cream, I know he is enjoying all of those things and so much more up in Heaven right now.

Samuel’s death rocked our little mito community. Being a relatively rare/new/often misunderstood and underdiagnosed disease, we have formed our own little band of friendships, support and strength. We are scattered across the country, indeed across the continent – and we touch each other’s lives through the words that come out of our fingertips, in emails, and blog posts, and facebook chats and message boards. We love each other’s kids like they were our own because in some way, they ARE ours. The struggles and the victories – we all share them. The times of tears and the times of rejoicing – we all know them. The fears that are too hard to put into words to our “real life” friends, we know without having to spell out loud. When news of Samuel’s passing was still being processed, we had only to ask someone else – “did you hear?” – no other details necessary. And although we know that our grieving and sadness are nothing compared to those who actually HAD him in their lives – the sadness still weighs heavily.

Any time a parent survives a child, it is sad.

Any time a mom is forced to pick out a headstone instead of a new school backpack, it rocks your world.

It is unfathomable.

And yet is is reality for so many people that I have come to know over the past two years.

Samuel had just turned 4 years old. He had a beautiful infectious smile, and the way his mom described his giggle I can only imagine how contagious it really was. His journey started out much like Kendall’s. His gut function was more severely affected earlier on, which led to an earlier reliance on a PICC line, which led to a horrific systemic infection that nearly took his life two years ago. He has always been a fighter. His family’s motto was “Expect a Miracle”. And boy was he one!

But the part that rocked us all so hard was that this was so unexpected. This was the crux of what keeps so many of us up hovering over our babies at nite – worried that this small fever or that nasty sounding cough could signal THE TIME that we will lose some function forever. When the mitochondria in each cell can no longer make or process any energy, that cell dies. When enough cells die in any organ, you lose function. when they all die, you lose that organ. When enough organs die….well….i don’t have to spell it out for you.

Samuel had been through a couple nasty hospitalizations this year. His body was probably fighting harder than anyone really thought. But he was playing around at his sister’s ballet class on a Saturday and was dancing in heaven 5 days later. How does that happen? And yes, that is, sadly a reality for every human being on earth. An accident could take the lives of anyone unexpectedly. but you tend to not live your life expecting an accident. with mito – every day could be THE ACCIDENT. And nothing reminds you of that like the loss of a beautiful little boy named Samuel.

So this has spurred us, my mito family as well as my family family, to fight harder. To be more vigilant. to enjoy every hour of every day with our beautiful little fighters.

But I know we aren’t in this alone.

So many of you are fighting this good fight with us. some of you may not understand all of it. But you know enough to know when to say “i’m praying”, or “i’m sorry”, or “I don’t even know what to say but I can hug you”. Some of you have made an AMAZING difference in our ability to keep fighting by sending $20 in a card, or by donating your wedding favor money to Kendall and her care instead. We have been SO very touched by so many of you and your true actions of love and support…

THAT is how we are able to keep going, keep doing it, keep fighting. That is probably one of the most asked questions I get – “how do you do it?” My friend/sherpa Allison wrote a blog post on this a couple weeks back. and she summed it up so well. I do it because I just do. I do it for my little goofballs. I do it because I know I am loved and supported from all of you. I do it because I have to – and because I wouldn’t have it any other way.

Life with Kendall is hard. There are no two ways around it. Yes, some of that pressure is now relieved with the addition of two wonderful nurses who come into our home every week to help lighten the load of Kendall’s care. And yet – she now requires 4 trained adults to ensure that she is getting everything she needs.  Our lives have had to re-adjust to having an extra person here all day with us, in our schedule (crazy as it sometimes is!), seeing every facet of our lives. It is knowing that right now – something is going on with her little body that I can’t quite put my finger on, but that it means I may be sleeping on a hospital bench at some point in the next week or so. It is having to find a balance between having her with us, out and about, running errands, enjoying the craziness of our lives – and her overall health and safety. It is a constant running algorithm in my head of intake vs. output, coloring, temp, energy levels and movements. And yet as hard as that all is – I know so many others who would gladly trade places with us. our lives are their version of “easy”.

So I pull up my big girl underroos and march onward. Because that is the only way to go.

we march on because we have to – because to stop and give up is unthinkable. To stop and whine is ridiculous and unnecessary. to look back at what we have lost or given up serves no purpose. The good fight lies ahead of us, and together we will all go forward towards it.

Every day is a gift.

What are you going to do with yours?

I know what I am going to do – I am going to go color with Kaylen Hope while Kendall sleeps. I am going to try to calm the voices in my head that are worried about Kendall not eating and having way too much “puking” through her tubies the last few days, and the fact that she wanted the oxygen on at naptime. I am going to go work on tying up some of the loose ends that come when you are suddenly fired from your job. I am going to think about what I can make for dinner after I come home from dropping Kealey off for dance class. I am going to watch a little DVR’d Survivor/Intervention/House. And I am going to think about how very lucky we are to just be alive.

And then I am going to think about how to not have such a depressing blog post tomorrow!

But for now, we keep on fighting – for Kendall, for Riley, for Samuel S., for Eithene, for Cayleigh, for Quinn, for Nate, for Brigid…..for so many others who are fighting every day of their lives.

Keep on fightin’ babies –

 

terra

Goodbye Summer!

Holy hot day today. i was actually shocked to see the car temp gauge thingamajigger on the rearview mirror (you know – that thing) reading 95!!!!!! On Sept 21! Which I guess, is technically the last day of summer. It just seems a little late in the game to be THIS hot! but I will take it! i have LOVED how hot and humid and SUMMERY it was this summer. I hate winter. Well, I should say, I hate winters here in the cornfields where its FUH-REEEEZINNNGGG, and no snow to accompany it. Cold is pointless. anyways…

It was gorgeous weather and I wanted to bottle it up so I could open it on some gross freezing January day when I am sucking down steroids to keep my lungs open and shoveling tylenol into many small children to keep their own crappy colds at bay. (Isn’t that such an amazingly happy thought??? I know. Call me Pollyanna Sunshine.)

I feel like I have (as usual) 8934 things to do before this weekend, and all I want to do is go to sleep. But I am waiting up to give the final dose of meds to Kendall for tonite, and killing the time by blogging. And since I don’t have anything astounding to say other than – dang, I have a crapload of stuff to do and no energy to do it right now…I’ll ramble a bit. consider this your forewarning to hit the little red X up in the right upper corner of your screen! I promise my feelings won’t be hurt!

Anyways – I have wanted to do a post like this for a while  – so why not now in honor of mito awareness week? Now, to be honest, I am not sure how much true blog2 “mito education” this will be, because a lot of it I am still trying to figure out for myself. But here you go – a glimpse into what life is like for Kendall in the OTHER 22 hours of her day from what most of you see her in! Here is her bedtime routine: First, prepare all your supplies – mix formula, open new sterile bags, draw up meds (this pic is ALL of her daily meds, including her PICC flushes when she was on that). And that little group to the far right? The crappy IV meds we have to reconstitute – I say crappy only because of the insane amount of time it takes me (and now her nurse!!!) to prepare! They are actually good – all of them are – in that they really do make a huge difference in her functioning, every one of them.

Next, take a freshly bathed baby and wrestle her floppy little self into pj’s. blog9 These pics were taken a few months ago before we realized that she wasn’t actually absorbing any meds through her gut. She used to take a couple of her meds orally (sucking on the syringe by herself), but now that we NEED most of these to actually be absorbed/metabolized, they go through her J. So she gets just a couple ml’s of tylenol or gatorade in a syringe to keep her entertained while I do everything else that has to be done.

This all actually takes so much longer in real life that I have more pictures than words here! So – I’ll just do my usual rambly nonsense to fill in the gaps…

anyways – so the other pictures entail her modeling her long tubey (PEG-J), which is obviously now the much easier to handle G-J button (GJ = gastrojejunal, in blog8 through the stomach, down to the intestines). Her food/formula bag gets plugged into her J-port so her intestines get a “continuous slow drip” of formula all nite long. Did you know that your intestines do not expand like your stomach does? This is why you can’t “bolus”, or let food go in fast all at once, into a J-tube. Fun fact for the day. A “Farrell bag” (so named for it’s inventor I have heard) is hooked up to her G-port, which allows air/pressure/backflowed food (ie, reflux or vomit) to exit into the bag and hopefully not up through her fundo and into her lungs (since she is laying down at nite). Some nites we set the blog5 farrell to “drain” her stomach contents, meaning if she is having a bad tummy day (which can be anything from lots of bloating, to hiccuping/retching most of the day, to just not having had a bowel movement in a few days), we will help her tummy be able to relieve the pressure and hopefully get a little rest by allowing the vacuum action of the bag to pull anything out of her stomach that gets into it. Other days when she is doing ok, we set the farrell to “vent”, which means stuff will only come back into it if there is a LOT of backpressure or air being trapped. This all happens because her gut motility (movement/contracting action of the intestines) is VERY affected by the amount of energy she has in any given day.

She is also then hooked up to her pulse ox probe (we call it “pretty toe” because it lights up red, and because this is honestly the only way I have been able to come up with to get her to tolerate blog6 having the probe placed on her foot for hospital stays. she gets INSANELY sensory overloaded sometimes in the hospital, and since they usually want the probe on her to see what crazy heart things she is doing, she has to get used to having this heavy sticker on her foot.) Now normally, since we have learned to “read” her autonomic signs a little bit better, she doesn’t have to be hooked up to the monitor continuously unless she is getting sick. Back when i took these pics though, she was hooked up to the monitor almost 24/7 because this was when we were fighting a pneumonia from home. Notice the amount of tape I have to use to keep the stupid thing in place because she likes to try to pick it off and there is NOTHING like a low sat monitor to wake you up out of a beautiful sleep…(even if it IS only cause the probe has come off!)

and again, she was on the oxygen continuously when I took the pics, and now only uses it intermittently or continuous with illness. Today was one of those days she probably needed it, and now that I look at ALL that she was doing over the course of the day, blog4 I should have just TOLD the nurse to hook her up (she kept trying to tell me her sats/numbers were beautiful but they were not “kendall” beautiful, as evidenced by the fact that she was turning colors AND not sleeping all day – two big signs that she is going into autonomic dysfunction mode and needs a “reset” with the o2. So , ok, add that to the list of things I have to do when I get done blogging and go hook her up to stuff.

where was i…

blog3 Oh yeah. So she gets hooked up to all of that which leaves a hot trendy mess of wires. If you have ever freaked out over having a bumper pad or stuffed animal in your child’s crib for fear of choking/strangling – try putting your baby to bed with a cord actually PRE-WRAPPED around her head!!!! Yeah – good times! So – we have had to get creative and borrow ideas from many other great moms before us who deal with all of this and more at bedtime.  We gather all the cords up and pull them through the snaps of her pajama leg and voila – a much reduced strangle hazard. She still manages to get SO tangled up overnite…blog2

anyways. So that’s bedtime for Kendall. Do not mistake the cute little buddha belly in the picture for “chubbiness” – i mean, yes, she is definitely finally getting some good meat on her bones, but more often than not her “chub” is painful gas from food that isn’t travelling anywhere in her slowed down system. So she cries for more food because food = comfort, except she doesn’t get that food = pain. Maybe she is now, a little bit. She didn’t want to eat much of anything after this last gastroparesis (the august hospitalizations), and when she did start eating, it was just mac and cheese (with very little “cheese” powder actually on the noodles). That has pretty much been the staple of her diet for almost 2 weeks now. She did have some “dipydipydipydipydip” (translation: guacamole) on sunday, so, you know, she’s getting SOME vegetable in…

blog1

Holy cow this post has gone in a whole other direction than what i started with…

oh well.

Anyways – i have other pics – of my OTHER beautiful babies. And a lot of existential talk about “worry” vs. “concern” for the future, and what that might look like for our family. But I might not get to that one this week. It’s probably only good to rip off so many scabs all at once. I don’t know how much emotional bleeding I can handle in one week. For right now, things are good. I will focus on that and hold on to that for right now, because really, that’s all we’re guaranteed. That’s all anyone is guaranteed.

and I’ll keep on keepin’ on. Cause that’s just how I roll.

gosh I should not end blog posts when i am this tired. i am sure this makes no sense!

 

t

Chicken, tiredness & awareness.

Yep. We’re gonna cover all three real quick here so buckle up and hold on.

{disclaimer – I am beyond tired at the moment so this post may be full of typo’s and/or swear words. Because sometimes my typos ARE swear words. accidentally though. Just – be warned. Grandma, mom, people who think I am perfect….forgive me. }

Gosh it’s been so long since i last updated…I don’t even remember where I left off. Oh – an after  PGH update. Yeah i am not even sure how to catch back up after that. So we’ll hop into something nice and warm and fuzzy first, and then you’ll get all the leftover crap at the end, which you can then decide whether or not you want to pilfer through, kk??? good.

THEY OPENED A CHICK-FIL-A RESTAURANT BY MY HOUSE!!!!!!!! Yes, Jesus loves me.

I know that some of you have like 87 cfa’s within driving distance and you don’t get it.

I know that some of you have no idea what the big deal is about our new cfa.

I know also that MOST of you who know me and my insane addiction to cfa as of late that you are calling Jeff Van Vonderen and his A&E crew and preparing your “Your addiction to cfa is affecting me negatively in the following ways…” speeches.

AND I DON’T CARE!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

I have yummy nuggety goodness just a few minutes down the street now – whenever I please!!! And REAL sweet tea, not the fake stuff from mcD’s. And some of the best chocolate shakes in the world! And just because they already know me by name when I pull up to the drive thru (no i am not kidding), and because I am on a first name basis with the awesome Operators, Doug and Becky, with their adorable son Ethan – this, I promise you, does NOT mean I have an addiction!

I will tell you what it DOES mean though – I recognize quality and goodness when I see it and THAT i can get behind. it does not help matters that i sat at the fricking wendy’s by my house tonite for 20 minutes while ONE person halfheartedly sorted frosty sprinkle packets and ONE person was….kind of making food, I guess, and THREE people sat on the counter talking!!!! OH my gosh my blood pressure is still elevated from waiting there while they would not give me my money back and did not know enough english to explain to me what the fricking problem was!!!! Ok yes, i should maybe consider cooking at home more often – but ….. that’s besides the point. The point is – that would never have happened at CFA. Ok, now I am starting to sound a little bit like a junkie….

Second topic: tiredness. This is a large part of my absence for a couple weeks. Work has been beyond crazy and just not a whole lot of something I can get into, but its been a huge heavy weight on my shoulders since we were in pittsburgh, and while there is still no resolution to the problem, I am at least learning to move forward through the chaos. But I am still very tired from it. All of the stress and sleeping on hospital window bed for almost 3 weeks/the month of august, and eating not the greatest led to an inevitable flare-up of the fibromyalgia I was diagnosed with a few years ago. It’s not something I like to think about or even really acknowledge that much (apparently this is a theme with all of my medical issues – hello asthma mgmt meds? what? Nahhh I’d rather just get pumped full of steroids when things flare up real bad). But it took a MAJOR toll on me the past couple of weeks, leading up to a pretty bad week this past week.  And by bad – i mean that fibro is painful, and a nearly debilitating fatigue, and it makes your brain so foggy you can hardly form words or say your own name. I really don’t know HOW I made it through this week except that I know so many of you are praying for us and I know that God is amazing and awesome and gives us the strength we need when we need it the most. but that’s all I really want to talk about that.

I am feeling a LOT better after getting a lot of good rest in over the weekend. blog1So – onward and upward. And on to more chicken!!!

Third topic: Awareness. Yes I know its a broad topic. As in, wouldn’t the world be a better place if we were all more AWARE of things around us – people’s feelings, other cars, life-threatening diseases, etc.

See, today, Kendall and I went to the Chicago Energy for Life Walk for Mitochondrial Disease Awareness. This is Mitochondrial Disease Awareness week. This is me finally realizing and acknowledging that mitochondrial disease is a part of our life. Part of what I haven’t been able to process from our pittsburgh trip was the notes from the geneticist who consulted on our case while there, who has previously reviewed our records. It’s a lot of technical mumbo jumbo which makes most people’s eyes glaze over when i start talking about it – but basically, he felt that if her mitochondria/muscles were already showing signs of disease at 7 months old (when her biopsy was taken), that it was more than likely progressing to a “diagnosable” deficiency by this point in time. I know that doesn’t make sense  if you have no idea what mito or metabolic disorders entail. So as best as I can explain it – reading that note was like a punch  in the gut. It was the moment I realized that this might all be true – all this existential talk of mito as a diagnosis for Kendall. That all the months and long nites of research and reading medical journals and studies on lab results and looking up words that I didn’t know the meaning of and putting the pieces of the puzzle that is her together all of a sudden were REALITY instead of just thoughts/fears/a catch in the back of my throat.

i mean, don’t get me wrong. It’s not like this comes out of the blue. This is part of why we sought the help of a “mito expert” in pittsburgh, knowing that mito was our likely diagnosis. I don’t know. i can’t explain it good right now (see aforementioned fibro brain fog…)  But basically – it was like a tiny corner of my heart still wanted to hold on to the hope that there is something ELSE we will find. Some other disease – one that has a cure, a treatment, an easy to pronounce name – something everyone can understand. Because mito is none of those things.

Mito – mitochondrial disease – has no cure. You can “treat” symptoms with a cocktail of expensively compounded liquid vitamins (similar to the b12 shots we sometimes have to give kendall), you can “treat” with lots of therapy (at least we have a leg up there!), and of course you can “treat” the damage done to organs by failing mitochondria (like a GJ tube to bypass a failing stomach, oxygen to boost failing lungs, antibiotics to boost a failing immune system, etc.). But there is no magical THING we can do to fix Kendall’s genetic material. It is inherently defective. the best we can hope for is SLOW progression of the disease, and GOOD progression of her own strength and skills. It is hard for people to understand how she can “look so good”, and yet be so sick. It is hard for me to be her constant advocate, knowing that if I let up for a second, we might miss that one little sign that is my clue to getting her the help she needs BEFORE it gets bad. She has been hospitalized 6 times this year. Granted, a few of them were kind of back-to-backs that SHOULD have been one long hospitalization if we had a continuum of care in place! But the point is – she has still been in that many times in SPITE of me doing my best to continue treating things at home. I avoid taking her to the ped, or even calling her, for as long as I think I can possibly do so, because I usually KNOW that the end result is an admission, or at least a long ER trip. And our new ER protocol letters instructs the docs to admit kendall until full feeds can be resumed. Oy. I hope I am wrong about this – but I think it’s going to be a very long winter.

Anyways – I am coming to terms with it a little more. Not that it will ever be an “easy” thing to accept – and I don’t know that I will ever fully ACCEPT mito. But we have now named the monster – we know what we are fighting against. So as my own “awareness” of mito is coming out more this week, I want to share that with you all.

to start, here is a good, condensed, not-too-medical version of some mito FAQ that might help explain things a little more.

I will have more info as we go through mito awareness week. But to those of you who have already taken up the torch for Kendall and for mito – by posting on your FB wall, or by voting in the Pepsi Refresh project, or by just asking me how I am doing lately, and then being the support to hold me up however I need at that moment – thank you. I cannot put my true gratefulness into words. Some of you have gone above and beyond this week – and you may not have even known it.

Here’s to a great week this week. And lots and lots of chick-fil-a. 😉

t-crest

Monday morning. Take 2.

how are we still “in the hospital” one week later? I feel like my life has revolved around the revolving door of a hospital for the past three weeks.

sorry – I am going to try to not be overly whiny in this post.

I know so many of you are faithfully praying for our sweet girl and want some updates!

As best as I can recap –

Friday morning when we were due for discharge, Kendall stopped walking, would not bear weight on her legs, and in fact would just crumple into a screaming mess on the floor if we tried to put her down. She was ok if she was laying flat in her bed, but would not make much of an effort to move at all. We dosed her up with tylenol, which was not holding the screaming pain fits off, so we went to IV Tordol (basically it’s the IV version of ibuprofen), which knocked her out for four hours. She woke up happier than she had been all day. we put her down and she walked around so we told the nurse we were ready for discharge. The floor resident said if we were ready to go, we could go. No one came in to examine her or to go over discharge stuff with us. We packed up and were pulling out of the hospital parking lot when kendall started retching (trying to puke, but it hits her fundo and can’t get up). I jokingly said – “no ma’am! Let’s not puke the whole way home!” Oh the propheticness…

Twenty minutes later Kendall violently vomited all over herself and everything in her seat. And let me tell you – if you thought Oreo’s were messy going IN – well, wait till you see them come back out. She had had about one fourth of an oreo on our way out of the hospital and that small bite came back to haunt me for the next five hours! We stopped about an hour away, changed her, hooked her up to drainage bags and pedialyte and oxygen and thought we were good. We thought maybe it was a reaction to the Tordol, or just maybe being overly tired from a long hard week – we thought it was the end of it. But it wasn’t. She continued to violently vomit the whole 8 hour drive home. We stopped counting the times and stopped even trying to get her cleaned up. We just changed out towels and blankets as best as we could. She was definitely hurting.

We knew we were going to pass two children’s hospitals on the way home, but every time we would get near them, it seemed like she was improving. you have to understand how very tired we all were, how we just wanted to get home, how much we missed the other children – we weren’t TRYING to be stupid about Kendall, we just really had no idea which was the lesser of two evils. And plus you’re thinking  – ok surely there’s nothing left in there now, THIS will be the last time she pukes.

We made it home, she laid right down in her crib and went to bed. I slept on the floor next to her crib because by this time she was puking so hard that she would choke/not clear her airway, and I was so afraid to leave her alone. She slept for about four hours, woke up excited to see her sisters, and started puking again. Called Dr. Natalie who gave me a virtual beat-down through the nurse “ YOU KNOW BETTER!!! YOU SHOULD HAVE TURNED RIGHT AROUND!!! WHAT WERE YOU THINKING?!?!?! YOU GET TO THE ER RIGHT NOW!”

So off we went. Good thing I hadn’t unpacked a THING.

i took kaylen with me, Ben had the older girls at the softball tournament, my parents met us at the ER. We were met outside the doors by an angel of a nurse who must have seen how floppy kendall was, or maybe i just looked like as crappy as I felt – but he took kendall and ran us past the line of 30 people waiting to be triaged and into a room. They took one look at the protocol letter i had in my hand and got to work. It was darn near impossible to get a new line started – kendall’s arms and legs are all bruised up like she’s been in a prize fight from losing spots and blowing veins all week. But they got a line in, got fluids started, and I turned off her pump of pedialyte I had been running into her J-tube. And instantly she started to calm down.

She was lethargic and floppy and didn’t want lights on and didn’t want us to touch her or talk to her that whole afternoon. That nite the doctor comes in to tell me that Kendall’s labs were going a little wonky (heading towards metabolic acidosis), and that she had put her on PICU consult – meaning that the PICU doctor would make the calls about Kendall being able to stay on the floor or having to transfer to the PICU for higher level care. Kendall was on an IV fluid containing 5% dextrose, and her sugars were still dropping. She had not made any pee in over 18 hours – she was just in a bad way. At one point I was really super scared for her – she was pale as a ghost, even her lips, felt cold and clammy, and was floppy when i tried to pick up her arms or legs. Her body temp was 95 degrees.

Somewhere in the early morning she started to seem to stabilize, so we stayed out of the PICU, but they were still worried about what she was doing. Dr Aljadeff came in to check on her, took one look at the black bile draining out of her stomach and said in his wonderful accent “ clearly, what we are dealing with here is much bigger than a possible and rare CSF leak”.  wonderful. So we talked about “paralytic ileus”, where the gut THINKS its obstructed, so it stops moving. And yes, in essence, this is what has happened to kendall. We took a KUB (tummy x-ray) – and there is no obvious obstruction, so we can’t just give her an enema and get things moving. We can’t go in surgically and “fix” anything and wake it up. Her body just has to wake it up on its own.

and we have no idea how long that might take.

I tried valiantly to start the pedialyte back up yesterday afternoon – and kendall immediately started in with her pain signs – kicking the bed, writhing around, moaning, crying, grabbing at her head and her chest. So I turned it off. Understand that the rate I am dripping into her intestines is about equivalent to a teaspoon over the course of an hour. We are talking droplets of moisture hitting her tummy – and causing that much of a reaction. She instantly starts making more bubbles in her farrell bag when we do this too – so clearly her whole gut is reacting to the feed attempts.

That is all i know right now. Thank you to my dad and mom who went to the hospital yesterday so I could come get the big girls ready for school to start tomorrow. They are being inducted into the injustices of hospital life – including lab techs who think nothing of flipping on every light on the unit at o’dark thirty to get their lab draw, living off of vending machine food, and the kind of crazy things you start doing to entertain babies when the mind-numbing boredom sets in! so if you see them and they are jabbering non-sense, take it easy on them!

I hear reports from Poppa that Kendall is anxious to get something/anything to EAT right now – so we are trying a sucker to see how that goes! The problem is that she doesn’t understand yet that eating is what is making her feel so bad, and its VERY hard to say no to a kid who hasn’t really eaten anything in a few days, but if we try to put too much into her semi-functional gut, we could make the problem worse, and set her back another day or two! it’s a tough balance.

Hopefully her gut really has just decided to wake up on its own. No one at LG has any clue what else to do for her except pumping her full of sugar water. I am afraid to take her out of the hospital prematurely again, because I just don’t know if we will get another IV started if we have to go back in two days or a week. Ok I know I am the one jabbering non-sense right now. that is the udpate as I know it.

I will go back and cover all that we learned last week. It was full of answers, some good, some helpful, some…..harder to swallow. literally.

thank you for the prayers – for the gifts (colleen – I about died at the cuteness of the minnie mouse overload!!!) – for thinking of us – for the emails and facebook posts – for everything.

Keep thinking good tummy thoughts for Kendall! Your prayers are always so appreciated!

 

T-crest

Wednesday.

This is going to be a quickie – well, as quick as it gets around here.

Seriously – this will be bare bones stuff that I will come back and fill in the gaps on tomorrow.

Today started out by me coming over from RMH (the ronald mcdonald house where i come to sleep for a few hours every nite) to our nurse telling me she had orders to take Kendall NPO cause of her MRI in a few hours. This was further compounded by pulmo coming in to talk to me about the bronchoscopy and having me sign consents – it all made me think we were indeed doing the “big testing” today.

turns out that apparently “MBSS” (modified barium swallow study) and “MRI” look a little too much alike for a night nurse at the end of her shift!

So once we got that all figured out, Kendall was able to eat a quick breakfast (three trix balls, a bite of english muffin, and two bites of pancakes), and then got taken NPO again (this also means tube feeds – it means no food at all. Well, ok NPO means nil per oral …. i digress.

what was on the agenda for today was that she had a one hour EEG (leads hooked to her head to watch for seizures or seizure activity in her brain waves – tons of fun for a very sensory kid), the MBSS to see if she is still aspirating or an aspiration risk, and the EKG on her heart (not to be confused with the ECG or “echo” that was a dismal failure yesterday).

I will get into details tomorrow – I promise – but the long and short of today is that she does not appear to be having any seizure like activity on the EEG – GOOD NEWS!

She can safely swallow purees (which we previously did not think she was swallowing safely) – GOOD NEWS.

we were able to get a calm quiet 20 second picture printed out of her heart from the EKG – GOOD NEWS.

We finally had immuno lay out a plan for us with the IVIG – this is GREAT news!!!! One of the biggest questions we had in coming here.

And – she got essentially a D+ on her thin liquid portion of the swallow study – BAD NEWS. I mean, don’t get me wrong – its not the worst news. It’s just – saddening. If you are lucky enough to know Kendall “in real life”, you have probably seen her happily sucking away on her “bubba” (bottle). (Dear Megan the SLP – I know you hate that she still has the bubba!) It is as much a part of her as her crazy curly hair or her infectious giggle. If she was not able to take almost half of her calories in by “bubba” every day, she would be hurting in a bad way not only for calories, but for hydration. Even hooked up to the pump 24/7, she would still make just over half her daily goal. Plus her ability to walk around uninhibited would be greatly reduced. But – in more than a few conversations over the past couple of days, it is becoming clear that not all is as well in the swallowing/keeping food out of her lungs/keeping food in her stomach arena as we have lulled ourselves into thinking these past few months.

Don’t worry – she can STILL HAVE her bubbas! (I know all of you who aren’t grandparents of KQA were just waiting on the edge of your seat for that announcement).

but – we have to start thickening any liquids she takes by mouth with a super thick clear goop called – watch out this is creative genius right here – “Simply Thick”. I know!!! BRILLIANT!!!

Because I just needed one more thing to do in my day. Now I get to make my daughters bottles the equivalent of trying to drink a chocolate shake through a coffee stirrer. We gave her her first thickened feed tonite and she took one tug on it, pulled it back and looked at me like – “you have GOT to be kidding me, right???? oh well, I’m tired – BOTTOMS UP!!!” The effort gave her a wicked case of the hiccups for no joke an HOUR afterwards, but hopefully she managed to figure out which was her esophagus and which was her trachea while she was sucking like a madwoman on that bottle.

In the long run – I know this is a good thing. It’s just hard to hear it today – because it seems like just one more area she should be “growing out of” her problems, and instead they are just getting worse. But – like I said – it will be a good thing in the long run to have this info because it will help us protect her lungs better, which means hopefully protecting her immune system better, which means hopefully not as many hospitalizations.

We (or at least I – Ben has been at work all week and hasn’t talked to her for more than three seconds) continue to be amazed at Dr. G’s innate sense of being in tune with what Kendall is doing, what things she is looking for, what all these consult reports from different docs coming in hourly every day mean for the bigger picture. She sat with me today for a LONG while and explained through a lot of things, gave me some good perspective. I need to process through it a little more –b ut I will put it all on here soon enough.

for now, I need to get in bed. I am going to need my rest for tomorrow, when the REAL MRI is scheduled for. Tonite we are stressing kendall’s system out (on purpose) in order to get good baseline metabolic labs. It scares the whoopie out of me because this is what we kill ourselves to AVOID on a daily basis at home. But – i know we need this labwork, and I know she is in very capable hands here at the hospital if she DOES crash. She will be getting fluids only – no dextrose, no feeds. But at the first sign of stress, test over, sugar in. So – since no one knows her random little signs better than me, I need to be there. I need to be there in case she starts getting stressed out in her sleep and can’t give us any good signs. I need to be there when they come in to draw labs off her already failing IV without the aid of a tourniquet to get good blood flow. I need to be there when she wakes up to a team of people “doing stuff” to her and she scans the crowd for that one familiar face, mine. so I can sing twinkle twinkle to her even though she is screaming “NO” at me and biting through her binkies in frustration and pain.

so I need my sleep.

more details will come tomorrow. And in case I haven’t put it somewhere else – She is first on the OR list for the morning – 7 am Eastern time she will have an MRI/MRS, spinal tap, bronchoscopy, and then sedated echo. Anesthesia will keep her under while cardio determines route (PDA closure or not), and then that will either be performed right then and there or she will be taken up to recovery.  I have been told that anesthesiologists know all about her prior reactions to certain kinds of anesthetic and that she WILL need a PICU bed if they decide to hang a LR (a solution they like to use in the OR for ????? no one knows why they like it when it causes so many side effects for kiddo’s mostly, and especially for kiddo’s like kendall). This is what happened after her last GA (general anesthesia) – and it was brutal to watch. I know that with some mito/metabolic kids they say no LR as a precaution – it is a proven FACT for kendall that it will cause issues. I am prepared to go in with my boxing gloves on – but I have a feeling that they already know exactly what to do for kendall quinn (from her awesome doctor!)

So – that’s that for tonite.

Thanks so much for all the awesome e=cards you all have sent – they nearly cover one whole wall across from kendall’s bed! SO very heartwarming! and the prayers are definitely being felt! So thank you all!

have a good nite!

 

terra

Elephants.

I really do like elephants. They are probably my favorite thing to go see at the zoo, if I had to pick a wild animal to ride, I’d definitely go for an elephant, and I absolutely adore watching them do tricks at the circus.

And all of that is a good thing since we apparently have an elephant living in the middle of our family room. You know the one, the elephant in the room that no one addresses.

Our elephant’s name is “Complex Special Needs Child”. She is citrus-colored, and wears a pink tu-tu with purple polka dots. And for the most part she stays very much to herself, not really intruding upon my life, or the life of anyone else in the family. We all do a really super good job of ignoring her on a day to day basis. But yesterday at our GI appt in milwaukee, she was acknowledged, and that gave her the freedom to kind of go trampling through my thoughts and the room.

I knew going in to this appointment, that it was probably going to lead to some hard discussions and choices about what else to do for Kendall health-wise. We have danced around the issue of placing a “GJ tube” for Kendall’s feeding for a few months now. When it was first brought up, I was SO relieved to have avoided it. I saw it as a major step backwards when we were just starting to FINALLY have some forward momentum. It has continued to be part of what I personally consider to be “background noise” – blah blah blah GJ tube, blah blah. Last week when we were up in milwaukee for the EEG (brainwave testing), our complex care nurse stopped in to check on Kendall because of all the other issues she has been dealing with for the past few weeks. When she brought it up again, and made a note that she was going to send a note to our GI, I knew then that we were going to be barreling towards a switch sooner than later. And when Kendall started a pretty hefty amount of vomiting this past Saturday night for no real apparent reason, I knew it was not a matter of “if”, but “when”.

So at the GI appointment yesterday, when Dr. Noe came in relatively concerned about the increased vomiting, and started asking some very pointed questions (when do we notice it, is it worse at nite, what is the consistency, etc) – I could tell it was coming. He did a good job trying to ease me into it, i’ll give him style points for that much. He offered to do an endoscopy, offered taking her completely NPO (nil per oral = no more bottles or cheerios or food – only slow feeds all day long through her G-tube), and then said, “orrrrr….we could talk about the GJ”. I kind of smirked at him that since this was the third time he had brought it up that maybe I should start considering it! He told me “right – cause I like to ease most parents into it, but hey, we’re already past that point. I think it’s time”

So – end of discussion.

And here’s what the rest of the conversation basically contained:

I went in to this appointment knowing that i was frustrated. I knew it was time to start asking the harder questions about what is going on with Kendall, and what do we expect from Kendall, and how do I fix Kendall, and the bottom line is…no one really knows. Dr. N looked me in the eye after i got finished ranting about my frustrations over the fact that it’s not like I am dealing with “well, hmm, do i give her tylenol or not? it MIGHT be an ear infection or it MIGHT be her molars…” – no, it’s, well ok, here’s a semi-lethargic baby whose appetite has noticeably decreased, been sick for almost 2 straight months, is breathing real rapidly and shallowly, and her core body temp is 93 degrees rectally…..is this a medical emergency? or do we ride this one out with a few pairs of pajamas and some warm water flushed through the tube? – and he told me basically

Look, I know we don’t have the muscle biopsy back yet, but from everything I can see in her chart, we don’t have anything else working at this point BUT the mitochondrial myopathy. i think we have to all work towards that, in order to protect her better, keep her better comforted, until we DO get more solid answers. i’m going to be straight up honest with you – I do not expect Kendall to EVER be off tube feeds. She plain and simple does not have the function in her gut and intestines to support herself nutritionally by what she can handle by mouth in any given day. Especially in the case of a myopathy, we don’t ever expect to see that function increase. So our goal with Kendall isn’t to “help her be normal” – our goal with her is to keep her functioning as well as we can, for as long as we can.”

And therein lies the birth of our elephant in the room that no one wants to discuss.

The fact that Kendall is not only more complex in her special needs than I think either Ben or I have allowed ourselves to believe over this past year, coupled with the slap-in-the-face reality that Kendall may actually have mitochondrial disease. These are two things I have hardly allowed myself to even really consider over the past year. They are my little pets that I like to just tuck back into my brain somewhere and not really acknowledge them, even while I have to live with their presence. And really, the good news is that probably within the next couple weeks, I will be able to shove them back into their little hiding places, even if I was forced to take them out and look at them yesterday.

But that’s just a coping mechanism. Cause really, i get it. I know that Kendall is different and special and may have….life-shortening problems. i know that by going to the GJ, we are hoping to preserve her stomach function. But that eventually, someday, that part of the muscle will probably wear out. And someday after that, her intestines will probably start wearing out. And then, maybe some far far far away day, she will end up on TPN (nutrition through her veins). And while I hope with every ounce of my being that this ISN’T what happens for her, i know that sometimes we have to be willing to take it out and look at it, as a possibility. I think we have to come to terms with the fact that Kendall is indeed a more complex kid than we tend to treat her like. And that needs a balance. No, we don’t want to  treat her differently, or be constantly consumed with her “needs” – and yet, it will not behoove her at all for us to blithely ignore all that she does require in the name of “treating her like a regular kid”. I think it’s going to be a mindset readjustment for our family. Learning to figure out what our end goals are – protecting her energy levels as much as possible, preventing, as much as possible, any major sources of stress which would suck even more energy out of her, and trying to allow her to use her energy on an even keel so we stop seeing some of these random swings in her health.

Ok so I am kind of rambling…processing out loud. There will be plenty more of THAT in the days to come I am sure!

In as quick of a nutshell as I can – I’ll try to explain this newblog4 switch to a G-J tube. i’ll try to do a post with links tomorrow for those of you who read too much google like I do. But basically, what Kendall has now is a “G-tube” – gastrostomy tube, which drips the food directly into her stomach. It gets held in place by a balloon that we inflate with water through one of two openings on the outside. One opening is in the middle, we plug an extension tube up to that hole, and the food goes in there into her tummy. The other opening is on the side, and that’s how we fill and check the water level in the balloon to keep it in place.

blog5 The reason we need to advance to a GJ tube is to bypass her stomach  with the majority of her food. For reasons not known to us, the muscles of Kendall’s body have never quite worked right. When she was born, it was her lung muscles not allowing her to breathe right. She is in various therapies because her arm and leg and neck and trunk muscles don’t quite have the strength in them to do what they should do. And we have always known that her Gi tract (which is one big long smooth muscle from top to bottom!), doesn’t quite function like it should. And right now, her stomach part of that muscle just doesn’t seem to want to play nice with the rest of her. it just doesn’t have the energy it needs to hold in and process foods in quite the normal way. Maybe two months of sickness is just finally taking its toll – or maybe this would have happened without all of that stress. But either way, we need to find a way to ensure that MOST of her food can be properly absorbed, so she continues to gain weight, and grow, and get as much energy from her food and her meds as possible.

So to do that, we have to switch out the G-tube, for a G-J tube – gastro-jejunal tube. Meaning the food will now  go through an extension tube and drip slowly into her intestines, or jejunum – hence the name! isn’t that clever???

Anyways  – hoping that kind of explains it a little more.

We will be making a trek back up to Milwaukee next Friday, February 5th for an Upper GI study to rule out the .001% chance that Kendall’s tube just may sit too low for her be absorbing her food. Her GI doesn’t really believe that this is the issue because we would have seen the increased vomiting almost immediately after her surgery, and it would be with pretty much every feeding. Which is not the case. So once we have ruled that out, we will head over to Interventional Radiology, where they will hopefully NOT have to sedate Kendall, nor dilate her stoma too much (yes i promise that is as painful as it actually sounds), and they will insert the G-J button.

And then we can all go back to ignoring the large citrus colored elephant in the room again. For a good long while hopefully.

 

It has taken me about four hours to get this post written – and i am sure it doesn’t make a whole ton of sense – but there you have it.

 

There is a lot more that i need to get out, process, spew/vent/whine and then Praise the Lord about. But it will have to wait for another day. Preferably one after which I have had a great nite’s sleep.

Thanks for reading. And really, I know its tough to know what to say. I don’t expect you to say…anything, really, I guess. I am the queen of never knowing what to say to some people when they drop news on you that is….less than sunshiney? So I promise you – there is really no right or wrong thing to say. Something inappropriately funny always works though!

hope you all have wonderful Fridays!

 

terra

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

a gift of hope

set1mito

For the many many families affected by Mitochondrial Disease, HOPE is all they have to hold on to.

A dear friend of mine  named Allison who I met on this path to a diagnosis for Kendall has a daughter affected by a strain of mito that was originally thought to be MELAS (which meant she had encephalomyopathy, lactic adidosis, and stroke like symptoms), along with a mitochondrial depletion syndrome. Yeah. The bad one. The one Kendall was thought to have. We avoided it by a tweak of an A,T,C or G in the right place on a double helix strand of DNA. Miss B did not.  

She is a beautiful little girl with an infectious smile. imageShe fights a battle every day to maintain enough energy in all her critical systems to fight infections and function as normally as her body can. She is just one face of mitochondrial disease. As we have walked down this road with Kendall this past year, I have had opportunity to “meet” many other amazing children and their families who are also dealing with this heartbreaking diagnosis. As Allison puts it

“A week and a half ago, I feared that Brigid was nearing the end of her journey with us – she had a supposedly unsurvivable fever, her little heart was beating 4 times a second, she was bleeding from her nose, mouth and stomach. Her blood pressure barely registered. No parent should ever see anything like this, and it will stay with me until my last breath. We know Brigid will not outlive us, but we are not ready to let her go. We asked you to pray, send happy thoughts, whatever you could, and you stepped up. Thank you – thank you for thinking of Brigid, and of us. Thank you friends, and friends of friends, and so on – all who thought and prayed for Brigid. Thank you for your prayers, for your energy, for your support. Thank you for helping us get the new chair for Brigid – it is on its way. Above all thank you for reminding us we are not alone on this journey. Brigid is the embodiment of why we should never give up hope, and at the end of the day, hope is bigger than all of us.When you have a condition with no treatment and no cure, if you don`t have hope, you have nothing. We are selling the jewellery to help raise funds for MitoCanada and UMDF, the organizations that help give us hope, when we think we have lost it all.”

 Allison and her friend have created some beautiful hand-crafted jewelry to help raise funds for UMDF/MitoCanada. I am personally honored to be able to purchase one of these sets because not only is it gorgeous jewelry – but the meaning behind it makes it worth so very much more than the amazingly low pricetag attached to it. 

I remember when I first heard about mitochondrial disorders – back in February when the neurologist assessing Kendall in the hospital brought it up as a remote possibility based on how very sick Kendall was at that time. As soon as he left the room I of course was googling everything I could find on the topic. The UMDF website was an invaluable resource at that time, and is a great organization that is furthering research and education efforts about this disease and the havoc it wreaks on little ones and their families. What I learned about mito at that time was more than I think I wanted to know, as it is not a “curable” disease. And as we have continued to go down this path of looking for answers for Kendall, mito has continued to pop up over and over and over again. Knowing now that she has some form of disease affecting her mitochondria and their energy production or use in appropriate ways of course makes this cause very near and dear to my heart. I haven’t talked about it here a whole lot simply because it’s one of those concepts that when it is just a thought in your head, you can reshape it and make it what you want. You can carry something that is light as air around for a while. But the second you make those thoughts words, and speak them out loud, or commit them to paper that other people can read – they become an object. Something with weight and strength behind them. They are more real. And I don’t like real. I much prefer pretend. (guess karissa and i have a lot in common….)

So I don’t talk about it much because there isn’t much solid info to go on at this point. Right now we are hoping that Kendall’s mitochondrial dysfunction is a secondary process to the metabolic disorder she deals with. Getting yesterday’s news that she does not have a mitochondrial depletion was great and amazing – but it does not change that there is still some functional disease process here which falls under the “mito” umbrella. And I think I am just now learning to say that, accept it, be able to carry it around as a real object. It is something I will have to learn to adjust to still, probably for many years to come.

And the first step of that is for me to wear my mito hope necklace with pride.

And love.

And of course, with lots of HOPE.

Because that’s what we have always clung to, and will continue to cling to.

If you are looking for an awesome gift this holiday season – for yourself, a friend or maybe your mom or sister, please consider this beautiful jewelry for that gift! Wear it because it’s gorgeous, wear it because it supports a great organization, wear it because you want a reminder to pray for Kendall and Brigid and the thousands of other beautiful children affected by mito.

There are a few beautiful options for color choices.

mitoset2

I love this blue color. It is probably my favorite color ever.

mitogreen

and yet I am drawn to the “mito green” as well, just because it’s “our” color, our little magnetic flag on the back of the car, it represents the camaraderie of being united against something.

brown black

Apparently I like the black the most because it decided to be a bigger picture! But these are the brown and black options which I also love because they would just go with so many things. There are also gorgeous yellow, red, red and black, pink and a few other combo’s available.

If you would like to consider purchase a full set (necklace, bracelet and earrings) for just $35, please email me and I can get you the correct info. This money is not going to just our family, or Brigid’s family, or any other family in particular. All of the proceeds will be donated directly to the UMDF organization to allow them to continue providing supportive and research services to all families affected by mito.

 

I am sure I will have more great gift ideas coming up in future posts too, so stay tuned!

Have a great Thursday! I just found out that Ben is coming home today instead of tomorrow like i’ve thought all week – so that is a great surprise! But I better get movin’ on cleaning up the chaos of Hurricane Kaylen having hit hard this morning!

APPLES!!!!!

      terra

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