September the First.

I don’t know why i’m always so shocked to see how long it’s been since I last posted.

But still…. June?

September will be better,  I promise. I already have lots of fun mildly eye-roll-inducing SUPER INTERESTING posts lined up.

And even a few vlogs! (speaking of, I have a burning question regarding vlog vs blog on a specific topic on the Terra Talking Facebook Page – go over there, like it if you already don’t, and help decide the fate of my awesome story!!)

So since most of you come here to read about Kendall, and how she’s doing, I’ll start the month off with that.

Overall, she’s doing good. Other than the crazy bug bite from hell that turned into an abcess/cellulitis that required hospitalization and ten days of IV antibiotics and a week of IV stress dose steroids, she’s been doing a good job holding her own.2SS_6375

But oh…how I wish you could see what I mean when i say she is exhausted. How much my heart breaks for this little girl who shoulders up her backpack, full of two pumps and two bags of fluids the last couple weeks, and wears it on the hot sticky days like a champ, never complaining, never whining about it, just maneuvering around with it because this is her life and this is what she knows how to do: LIVE.

She loves going to school. Absolutely ADORES it! It wears her out so badly (and yes, I get that all first graders are tired and adjusting to a new schedule – I was prepared for some tiredness but this is a whole other level of exhaustion for Kendall!), but she puts on that crazy smile of hers and she keeps her chin up and she does her absolute best to pay attention to the teacher and to keep up with the class and she’s trying so hard. i’m so proud of her. She’s amazing.

This infection she had – well, it hit me almost as hard as it hit her. She had JUST gotten IVIG, the magical unicorn tears I have held up for the past two years as being the reason she has stayed so healthy.  And as a quick primer on IVIG for those who are newer around here – it stands for IntraVenous ImmunoGlobulin.  Your body’s “immune system” is complex and multi-faceted but for space and time constraints i’ll boil it down to this: your blood has three main components of immunoglobulin which are technically your “fighters” against bacteria, viruses, fungi, etc. You have IgA, IgM and IgG. IgG is the main one you need in order to have an immune system as most people consider it.  Contrary to popular belief, “picking up every cold under the sun” is not actually a sign of a weak immune system. It’s a sign of a good one – your fighters kick in! You get the fever (WHICH IS A GOOD THING!!! It means your body is fighting invaders!), you get a runny nose, etc etc – and you fight off the infection thanks to your IgG.

Then we have Kendall. She technically HAS IgG in her blood, and sometimes she even has a really good amount of it – but it’s like having an army of soldiers who are all narcoleptic and/or otherwise incapacitated. Her own native immune system doesn’t recognize invaders until it’s WAY too late. Even with the IgG that we infuse into her bloodstream every few weeks, her immune system is extremely lazy. So what happened a few weeks ago is that when she got a bug bite on her little booty cheek, her body didn’t send the soldiers to help control the spread of the bite reaction as is typical. Hers went from “regular mosquito bite” to “holy crap what IS THAT THING!?!?!?!!?” in about 72 hours. And we were running IV benadryl to try to stop it from getting worse and it was just getting out of our control almost before our eyes.

And i was glad that we are at a place with her team that when I send an email (with a picture) and say  “ummmmmm…..help me?”, they jumped into action. I didn’t have to spell my fears out for her doc, he knew what I was thinking. This is the kind of stuff that takes Kendall down hard. Maybe not right then, but if we had not nipped that in the bud, it would have easily been systemic within the week. Septic, most likely. A blood stream infection that would wreak havoc on her organs and little body – all from a bug bite.

Now of course we have no way of knowing for sure – and no one really wants to try to find out. But the fact of the matter remains that Kendall looks amazing on the outside, and yet her body fights a raging war on the inside, every single day.  I am SO thankful for our new nurses who are very on top of learning all of Kendall’s “Kendallisms” and helping me manage the delicate balance of meds/fluids/vitals that keep her able to go to school with a smile on her face and sit in that little desk like the other kids in her class and  live her life the best way she knows how.

A bug bite. A little regular probably mosquito bite. And it was as if her body didn’t even recognize that it was fighting what was quickly becoming a massive infection. I’d be lying if i told you it doesn’t scare me a little bit for this winter.  I have NEVER wanted to live my life, or hers, keeping her away from living her life. I’ve never tried to keep her quarantined from things (although I like to think i make decisions in her best interest as far as when to go and when to stay home). I don’t make it a huge deal that my kid has basically no functional immune system. It’s just not how I’ve ever wanted to treat her or live. But my gosh…to see what just happened, it makes me stop and think. And overthink probably….

So that’s where she’s at. With currently a very minimally functioning immune system, but off the antibiotics and steroids for now (YAY!!!! Those drugs are brutal on her and I hate when she needs them, even though I am SO very grateful for them.)
We are still going up to Milwaukee once a week for iron infusions – I think we only have three more left though! After that course is done, we will re-run the labs and see if we have done enough boosting to let her coast for a while. My impression is that we have definitely helped her feel TONS better, and I don’t see the massive crash after a few days that we were at the beginning of the infusions. But I do wonder how long that will “stick” once we stop the infusions.

School is going…..well…..it’s going! It is becoming more obvious and apparent that there are some MAJOR lags in Kendall’s ability to keep up with the class – and while that is in part due to her needing to miss one day each week, it is in larger part due to the fact that she has ALWAYS had struggles with actually learning and retaining the information taught in class. That isn’t to say that she isn’t smart as a whip – cause she is! She can throw around medical terminology correctly and tell you about feeding pumps and IV pumps and discuss IV medications better than a lot of medical students probably. Yet – she can’t recall the sounds each letter makes and spell a word off the top of her head from her word wall list. She can’t tell you how to sort a group of like words. It is hard to put it into words exactly, what we see and observe, all I know is – she is struggling and I refuse to let her continue to struggle without some major help.
So next Tuesday we will be shlepping BACK up to milwaukee for all day neuropsych testing to determine what, if any, learning disabilities Kendall has that we can identify and hopefully help address. It isn’t like this news comes as a shock. Her doctor actually put in the referral for this testing LAST YEAR, but insurance has finally approved it and the clinic finally has an opening. While i know this will be yet another “thing” to add to the diagnosis pile, I am hopeful that at least this one we can attack. This time we can maybe help solve a problem instead of just minimizing damage.
This post is all over the place and I feel like there’s so much more I could say about where Kendall is at….or my feelings on the matter. But I’m sure they will come out in bits and pieces in other posts over the next month.

Thanks for sticking around if you’re still here. I hope to have lots more to talk about over the next few weeks and it’s so nice to know you’re listening!

Have a beautiful evening, party people. See you on the flip side…

Terra.

Kendall Update.

Just because it’s probably been a while since I’ve done a good update on Kendall. If you aren’t on her facebook page – Hope For Kendall – (you can easily click on the “like” button over there —>), you should be. I try to do some updates there on a weekly basis, and if anything huge comes up, that’s where I post it.

Overall, I’m so pleased to report that she is doing amazingly well. 6 months ago, I did not know if we would be here. I hoped – I hoped even against what I was being told by her doctors – and I prayed, oh how i prayed. And I WILLED her to get better, be better, push through, fight through, prove the odds wrong once again.

And she has.

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I know God has big plans for this little miss. He is the reason that she is here, thriving, doing so well. Your prayers to our merciful God are why she continues to do what she does on a daily basis.  It has not been an easy road by any means though.

You will not always hear a whole lot about the true ins and outs of all Kendall deals with on a daily or weekly basis. Part of that is because there are a few people involved peripherally in Kendall’s care who like to misuse the information they read on the internet about her, part of it is just that I don’t feel the need to post about every single time something goes wrong or is hard to deal with. A large part of it is because I never want to be one of “those moms” – the ones who seek attention for themselves via plastering their child’s medical issues everywhere. Sure, I share a lot of Kendall’s struggles. Maybe sometimes I have been guilty of sharing too much, but everything I share, I do because I feel at the time that it is the right thing to do. There are times when I think people need to hear what she is up against, because sometimes we all need to be reminded to stop complaining about the small stuff, and sometimes we need to stop and realize what a miracle it is that our bodies function as well as they do every day. And sometimes I just want people to understand how very amazing my beautiful warrior princess is.

Whatever my reasoning on that day – there are many times when I do not update simply because there wasn’t a whole lot of “good” or positive to share that day. It was just another day we all survived. It was full of nausea, tummy pains, headaches, meltdowns, behavior challenges, lots of therapy, lots of sleeping, lots of medicines, lots of my brain working overtime trying to plan for 12 different contingency plans based on the next hour. And really – these things don’t always need an update. Because at the end of the day, or the week, Kendall is still doing GOOD.

My definition and boundary of “doing good” has expanded so far these past few months I can’t even describe it20131222_003921910_iOS to you. It’s hard because the other girls get caught in that same super wide web of “oh, well as long as you aren’t requiring a ventilator to breathe right now, you’re probably healthy enough to go to school/dance/the store.” Surviving what we all survived those scary nights in July has changed all of us a little bit. Hopefully for the better. Sometimes for the not so good. When we were in the ER a few weeks ago with Kendall she knocked one of her leads off her chest and THAT alarm started going off and I felt my chest constrict and the room start spinning a little bit – it was just the NOISE that set me right back in that moment of panic and it took me almost an hour to calm back down.  I hate even looking at ambulances sometimes, thinking of her ride up to the hospital that day. I sometimes envy the “problems” of regular childhood illnesses that I see my friends dealing with. Sometimes I hold someones baby and I instinctively adjust for GJ tubes and pulse ox probes that aren’t there, and then I panic when I feel their smooth bellies like maybe I’ve pulled their tubey out. (Pardon my little foray into my own personal therapy session here….)

So maybe it isnt just the last few months that has changed us but the whole of Kendall’s life experience.

Whatever the definition of time we use – I know that it has changed me. It has changed how I perceive HER, and her issues. It has stretched us both, mother and daughter, in our indefinable intertwined relationship, to learn to understand the other better, to trust the other more, to become both more dependent and independent of each other.

And now when I try to update about how she’s doing – it has come to this. She is doing good. She is doing better than anyone expected. We don’t know for how long we have this awesomeness, which makes it all the more awesome because we try so very hard to not take the good days for granted.  She has adjusted to her IVIG infusions like a champ as we have learned to tweak the protocol under which she receives it. And to say “oh they are going great, she handles them fine” – even saying that is to neglect to convey to you what her body endures on that long day. She is awoken at 4:30 am to be placed into a cold car for a 2.5 hour drive. She is nauseated by all the movement in what should be the middle of a REM cycle. She is nauseated by the movement of the car for the first 30-45 minutes until her brain catches up with her a little bit. She retches and gags quietly to herself in the backseat while she tries to fall back asleep in spite of how bad she feels. We get to the hospital and walk down to the infusion center where she is swooped upon by 2-3 nurses hooking her up to vitals, taking blood, bringing medicines in and hooking her up to the machine she will be tethered to for 6+ hours. In order to help the IVIG be absorbed better into her bloodstream, she receives a high amount of IV 5% dextrose in addition to her TPN that also runs concurrent. The dextrose makes her jittery, but she can’t do much to burn off the energy because she is tired from the high doses of IV benadryl she must also receive in order to keep her body from attacking the IVIG. She receives almost 2 liters of fluid into her little body during her infusion. The demand on her heart to keep up with pumping this much fluid is enormous. She must also have her foley cath in her “Cinderella” during this time because it would be impossible to keep up with the amount of fluids retained in her bladder otherwise. And then when it is all done, and her other appointments have been completed for the day, we pack up in the car again for the long drive home, often getting home just in time for bedtime, so she can wake up early the next day and go to school.

20131211_215320000_iOS She does all of this with so very little complaining, it nearly breaks my heart when I think about it. She doesn’t question “why me?” – she just takes it for the part of her life that it is. She doesn’t try to take any of her “attachments” off, or get frustrated when she is tangled up in them like so much spaghetti tied in knots. She doesn’t whine that her head hurts or her tummy hurts or her heart hurts or her bladder hurts – she whispers these hurts to me when the door is shut and it is just her and I, knowing that there is not much I can do to make it better, just hold her and stroke her hair and tell her she will feel better soon.  This is just who she is. The strongest kid I know.

But this is just life in the trenches. We keep on keepin on. Really, it’s not so bad. I know so many of my friends would love to “only” have Kendall’s issues to deal with. It is what our every day lives are like – this give and take of good days and bad days.

We will soon be starting what we hope is “intestinal rehabilitation”. Her team has been working very hard to come up with a modular formula that will have the correct amount of nutrients and the least amount of “bad” stuff (sugars, fillers, random chemicals found in most commercial formulas). It is a complicated process that would require us to basically have a lab in our kitchen to properly prepare it – so as such it has to be prepared at a pharmacy and delivered to us every few days. As you might imagine, the cost of this will be redonkulous. We are awaiting and hopeful that insurance will approve this process. Originally we were supposed to admit Kendall to the ICU in order to start this process, but one of our awesome doctors pointed out that we could not define success or failure based on a few days, and that our overall goal for Kendall has always been to care for her at home. We have strict orders to head in to the ICU if she does appear to be not handling the starting of formula into her gut (even though the list of “signs of intolerance” her GI gave me she already does on a near daily basis with just pedialyte going into her gut). ..I guess it’s just going to be a lot of trial and error. It is scary to be in this place – truly stuck between a rock and a hard place. She is stable right now, her gut is as happy as I’ve ever seen it be. She is growing and thriving with her ridiculous amount of parenteral calories (her IV nutrition). Every once in a while she decides she wants to snack on something, and she can if that’s what she feels like, and if she doesn’t want to eat for days on end, no worries either. But the risks of continuing to rely on that IV are horrible to think about.  Even though it comes with it’s own set of risks, we HAVE to give this a try. We could push her gut right into a translocation (infections that make her sick like this summer), which is why we need to have an almost immediate response time to any issues so she can be admitted and started on antibiotics quickly, or we could lose all the ground we have already gained with being able to have her getting some enteral pedialyte. Both of these risks are very much worth it compared to the huge risk that it is to continue relying on TPN for all of her nutritional needs.

No one knows what to expect – even though we all hope for a miracle. The miracle would be that she could get back on full enteral (into her J tube) feeds.  But at this point, if we can get her consistently tolerating ANY amount of formula, and can taper her TPN calories and even maybe some of her TPN time, it is that much more HOPE that we have that we can stay one step ahead of her whacked out little system. I know we can make some big strides. I know she is as strong as she has ever been and is ready for this challenge, even 20131229_023725000_iOS though part of me wishes it was not going to be SUCH a challenge for her. Part of me would love to keep her this happy and functional and not ever have to put her through the hard stuff. But the hard stuff is what keeps her doing so well. the long hours of therapy and the discomfort of making her learn to pee on her own without the foley in all the time, and all of the other things we push her to do – this is what is saving her life.   I know this. So we will push her through this too, and know that in the end she will come out stronger than ever.

I don’t know when all of this will start – I know only that it has been in the works since the week after Thanksgiving.  Whenever it happens, it will be the best time.

So that is where she’s at right now. We are still working hard to get all the pieces in place for her Make a Wish trip – i cannot believe the logistics we have been working through just to try to find a week that will work – and now to get a nurse approved, then i think we can start our official “Yay Get Excited It’s a Real Countdown”. IF the dates manage to get locked in! I am sure the MAW people are going to hate us by the time all of this is said and done…it’s just craziness trying to find the right time between dance/seasons/enough time to plan for all the other issues we need….It’s making my head spin just thinking about it. So i will stop for now until I have more solid details to share with you!

Anyways – thats our update as of this moment.

Thanks for checking in on us!

Love and hugs~

Terra.

Snowflakes that stay on my nose

And eyelashes. And brown paper packages and kitten’s whiskers and all that happy horsecaca. My favorite things – but the best of all – IT FINALLY SNOWED!!!!!!!
It’s starting to really feel more Christmasy around here and I’m loving it!

What a crazy weekend – lots of fun – just pretty busy! Got to see the girls’ grandparents from Florida and had so much fun hanging out with them, did a little Christmas shopping, Kendall went to her first ballet class with her BFF, and today got to go to a fun family christmas party with the Starlight Foundation! And now I need a weekend to recover from the weekend!

20131207_150401000_iOS Tomorrow will be spent trying to get everything set for our monthly Milwaukee trip – logistics for the girls to get to/from school and dance, hot lunch money scrounged for,  all of Kendall’s necessities packed up. This trip up will actually be two days worth of stuff – so I have to schlep all of her meds/pumps/fluids/chargers/supplies with us. We are also going to be changing out her GJ tube. Normally this is just something that happens – no thought from me needed, no complaining or making a huge deal out of it. It’s just a part of life for Kendall – sucky as it may feel to be pinned down and have a long tube threaded through her intestines.
But this time it’s different. For me at least.

See, last time she got her tube changed, she was fighting for her life three days later. While there is no hard evidence that the two are actually connected, there’s a high percentage that something happened during that tube change that allowed the bacteria an easy translocation into her bloodstream. Whether her gut was actually punctured by the guidewire or whether the act of removing and replacing the tube just stirred up too much bacteria for her body to handle – it’s not super far outside the realm of “risks” associated with a GJ tube change. She has needed her tube changed for two and a half months now. But i have been unable to bring myself to do it. I am scared. I daresay I have a touch of PTSD about it. I know God is in control of this situation, but I am still nervous.

However, it is time. Her tube is likely the reason she has been having some increased GI bleeding this past week. Plastic tubes that are soaked in bile for a long time get stiff. Stiff tubes of plastic in ones intestines lead to scrapes/pain/nausea/bleeding – all of which we are seeing on an increasing level with Kendall. Thank God for her doctor who understands that Kendall likes to deal in the unexpected and is helping us come up with a plan that will hopefully lessen the impact this tube change could have on her body. I am hopeful that the increased antibiotics she will be receiving this week via her tube and IV, coupled with Tuesday’s IVIG infusion, will help her body fight off any increased bacteria.  And hopefully then this tube change will help decrease the nausea/vomiting and bloody stools and just overall pain and yuckiness Kendall’s been dealing with the past few weeks. I know I don’t always talk a lot about the details of all she endures, and that I put up pictures of her smiling and having a good time, and i try really super hard to act like we are all just cruisin right along week to week. Because really, that’s what I hope for. I cannot imagine how hard it would be to deal with life if I were to focus on all the little (and big) issues we deal with every day.

Take today for instance. We are almost ready to get out the door to this christmas party – and Karissa notices that Kendall is dripping and leaking out of her backpack. Sure enough, her TPN bag has a huge hole in it. While it might seem that this is only a minor nuisance, a laundry issue – it is so much more serious than that. If Kendall has been infusing from a bag that was open to a cut all night, bacteria could have made their way into what should be a closed and sterile environment, and could have been infusing into her bloodstream all night.  Like she needs any help in that department. On the other hand – it could have just busted open about ten minutes before when she dropped her backpack so she could put her coat on. We had no real way of knowing. So what do we do? Do we panic, take her right to the ER where they could have run cultures/sat there and waited for labs/ruined the day for everyone else who was looking forward to this event? Do we just pray for God’s protection and go about our day trying to ignore the fact that kendall is now a ticking time bomb of possible septic infection? We tried to find a happy medium. I’m glad Ben was here – we were able to tag team unhooking the bad bag, rehooking up a new bag, doing our own set of baseline vitals, getting a contingency plan in place should one be needed, and going about our day with smiles plastered to our faces for the big girls. (for the record – her doctor is aware and happy with our current plan of treatment and if anything should change, they are ready and waiting for us. For now she appears to be safe from infection, and we are running extra fluids and keeping a close eye on her vitals.)
Nearly every day there is some kind of medical mayhem or issues that I don’t address in full detail – simply because I cannot live my life focused on all that COULD go wrong. Each day is precious. if i spent it focusing only on the bad stuff – it would get pretty darn depressing around here real fast. So instead I try to just keep moving forward – taking care of the blood coming out of Kendall’s bag and stoma, doing the incessant laundry created by the mass amounts of diarrhea, ordering the supplies that get forgotten, dealing with the insurance companies for the things they deny that we need, and praying that somehow, in the end, it all works out for God’s glory the way it should.  Kendall’s daytime oxygen needs have increased – our ability to give her that oxygen has made things like getting out of the house a little more tricky. would it be easier if our insurance would pay for a portable concentrator or if i beat someone over the head to carry teeny little tanks that would fit on kendall’s wheelchair easier? Sure it would. But that isn’t my life goal.  At the end of the day, we have the tanks we need to provide what kendall needs.  And that frees up my time to deal with trying to get the plethora other meds and supplies we need to keep flowing through here on a constant basis to keep her home and functional.
And if what you see at the end of all that effort is a happy, smiling, full of life little girl – then my job is complete. If it comes off as seamless and like “things are going fabulously” – all the better.  Because really – she is doing as well as can be expected. I do wish I knew exactly what was happening inside her little gut and I wish the meds we had could provide enough relief for her to make it all the way through each day without bad periods of puking and pain and bleeding.  I am hopeful her team can help us come up with a better plan this coming week as we are able to sit and discuss what is going on. I hope the tube change helps makes a major difference for the better, and that her body is able to fight off the inevitable stirring up of bad gut bacteria.  I hope her body is able to assimilate the IVIG with no reactions in the midst of all that is being asked of it this week.
I hope I don’t forget to pack anything important and I hope my other girls are angels at their home away from home again.  I REALLY hope we can find time to have someone help us get the stitches out of kendall’s broviac too! (It is stitched into place to allow the internal cuff to grow in to anchor the broviac firmly in the tissue of the chest – usually the stitches dissolve/fall out on their own. Unless you’re kendall. then they just sit there and irritate your broviac site and drive your momma crazy trying to keep them from filling up with gross stuff every dressing change.)

Anyways – it’s late now and I have to figure out how I’m going to fit four bajillion things into tomorrow and still get some sleep to make our trek north at 4 am Tuesday morning.

I hope your weekends were all magical and filled with your favorite things as well.

love and hugs~

T-crest.

success!

Maybe not success by very many people’s standards – but on a day like today, where I have been awake for far too long and dealt with far too many medical issues – success is measured by being safely home in our own beds.
We succesfully got kendall’s ivig infusion for this month. i’ll call the infusion a complete success if we stay out of the hospital the rest of this week (I don’t think we will have to go there, i’m just hedging my bets!)

It’s been a super long day, draining not only physically but emotionally too.

I’m about to crash but gosh darn it – i said i would blog every day so i’m going to do it if it durn near kills me. (For the record – ive also done the planking every day, even though that crap is getting HARD. And i most definitely did NOT want to do it tonight but i did it anyways.)

So here it is – in all it’s super short glory – today’s blog.

we surived. we made it home. we had success.

 

the end.

(i’ll write more tomorrow cause i really do have a lot more to say but i cannot possibly prop my eyelids open any longer.)

 

t

Celebrate good times.

Come on!!!

(If you don’t already have a Kool and the Gang song in your mind then……you need more fun in your life.)

So now that we’re all jammin out to some good 80’s tunage, here’s my actual blog for today.

As usual, it’s a motley mix of emotions and different things on my mind.

My heart still aches for my friends who lost their little boys recently , and for all my friends who have lost their children to this cruel disease. Every day, at least once a day, I am reminded of the fragility of life. My facebook news feed is filled with medical crises and issues that i never would have thought I would be able to read with a calm sense (and understand them even!). Daily I am playing a balancing game of running Kendall’s outward actions against what I perceive to be her internal issues. I am constantly counting down the days until our next IVIG infusion, and counting UP the days  we are removed from her last infection, or from broviac placement, or from any number of different medical milestones. It is always present. Even when it might seem like are “getting a break”, there really is no such thing.

She has had a GREAT past couple of weeks. I have been able to breathe a little bit easier. I haven’t had to post a whole lot of crazy medicalese. But this does not mean that we are just cruising along. I may make it look like things are smooth sailing – but underneath the surface I am treading water with all I have. Kendall’s care never stops. We have good nursing these days, and that IS helpful, don’t get me wrong. I am able to get a couple hours here and there where I can just be “me” – go read a book in a quiet spot, peruse the aisles at target leisurely for an hour, spend a little more time with the big girls apart from having to constantly be giving meds or assessing kendall. But at night, pumps still alarm. Pumps still malfunction and I have to try to calculate missed fluids x hours remaining co-sign tangent (y) to reprogram the pump so she doesn’t have a crash in the morning. i have a funky little device that tracks my daily activity and my sleeping habits (because i need more of both!) – and seeing my sleep patterns every morning actually explains SO MUCH about why i feel exhausted every day of my life.

So yes, Kendall IS DOING GREAT – but it still requires Herculean effort to keep her there. Effort from me, and prayers from all of you. I know it is not a solo effort on my part. yes i am the most hands-on, but it is not by my power that Kendall is doing well. It literally takes a village to keep our family running. I do not for one second take all of that credit. It is the village of our family pitching in to help with the other kids, close friends who arrange and bring meals because cooking is just one more thing that i am too exhausted to do by the end of the day, friends and acquaintances who can’t be hands-on help but can send a gas card or $10 for something fun for the girls.

And this weekend, it will literally be a VILLAGE, the village of our neighborhood, gathering together to celebrate this sassy little red-head who has touched a whole lotta lives with her spunk and feisty will to live. I am honored and humbled at all the effort of our friends and neighbors to put this amazing event together. I cannot fathom how many hours have been poured into making an event of this size come together – and I know there are people who we have never even met pulling strings for this to be an EPIC amazing day for Kendall! I try not to let some of the ins and outs of a kid with needs like Kendall’s creep in to the blog or her page too much. But it is often overwhelming. We have been so very blessed, and we will constantly seek ways to pay forward all that we have been blessed with. Beyond the fundraising aspect of this event, I hope that it is successful in getting more awareness of mitochondrial disease, and the battles that some littles fight every single day. I hope that the people who have poured heart and soul into this event have an inkling of how amazing their efforts are and how  Kendall has had……a year.

In many ways it has been a good year, and in so many others it has been one of the scariest years of my life. here is one of those other countdowns that i rarely breathe out loud. The countdown to 5. 5 years old. I know WAY too many children who never made it to that milestone age. And there were far too many days this year when i didn’t know if Kendall would make it there either. But here we are 30 days out. thirty more days. One month. And yes, we will be celebrating a tidge early by having her birthday celebration be this weekend –but the point is – we have SO MUCH to celebrate. So we will be doing it up big. If you are anywhere in the area – we’d love to see you! Just come by and say hi – see all the fun activities on the flyer i’m attaching. If you can make it in the morning for the 5K – awesome! If you can’t make it till the afternoon – that’s great too!

 KendallKrewFlyerFinal

We have some amazing friends and family scattered around the country who are doing their own versions of the Princess 5K – and there’s a link for that too if you wanted to do something to celebrate in your own way!
one of our friends even challenged her workplace that if they raised a certain amount she would wear a princess costume to work all day!!! I hope someone is taking pictures of that!

It is this Saturday, October 19th. We have a huge countdown between now and then – the countdown against IVIG reactions! For those keeping score at home, last time, Kendall started reacting at 36 hours, was in the ER by 48 hours, and was in the ICU 6 hours after that initial bad crash. And stayed through the weekend. PLEASE PLEASE PLEASE pray against a reaction of that kind with us.  The countdown is on!

so let’s celebrate the GOOD times – the every day battles won – like waking up and breathing in and breathing out and grabbing life by the horns and LIVING it. I hope and pray for you that you have something GOOD to celebrate about the everyday.

Pumps are alarming again – that must mean IVIG is done infusing and it’s time to flush and pack up this little cubicle that is destroyed with dried playdoh and tongue depressors and sample cups turned playdoh tools….

Keep on Keeping On.

 

terra.

Home away from home.

For those who don’t know – Kendall was re-admitted to her home away from home here on PICU 5 up in Milwaukee on Thursday night. At this point we are still unsure if it is an infection trying to brew that we caught super early, or if it is a delayed and “abnormal” reaction to the IVIG treatment she received on tuesday.

She started acting “off” on Wednesday afternoon, was definitely off on Thursday morning, and was a certifiable “hot mess” by Thursday afternoon. She spiked up to 102.8 fever, very high heart rates and blood pressures, and was becoming inconsolable from pain – so I ran around like a chicken with my head cut off for an hour waiting for babies to get off buses and hoping Ben could cancel his work trip in order to fight rush hour traffic and get home. My mind immediately began playing back the track of her doctor telling me “she will not survive another infection back to back” – and i was struggling to keep my panic in check while trying to stay calm enough to get the big girls and their logistics settled down.
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Thank God for good friends, for gas tanks that manage to run on fumes, and for a husband who can drive like a bat outta hell when one of his baby girls needs him. At the end of the day, the girls went to a friends house, Ben made it to the hospital with me and kendall, and as smooth as things can go in a situation like this, they went smoothly. It was So hard to run out the door, the girls had just come in off the bus all ready to tell me stories of their day, and instead i was waiting at the door so they could kiss their febrile, cranky sister goodbye and i could tell them to sit inside quietly with the door locked until daddy came home. They all had such stiff upper lips, but kaylen’s little lower lip quivered just a little bit as she was saying goodbye. i hate that they all have to know this kind of “fear” – are you coming home with sissy tonight? how long will it be until daddy is here? when will we see you again? – and yet have learned coping mechanisms to shove the fear down, sit at the table and get right to homework. My dear friend sharla who has stepped in and become a second mommy to my girls over this past crazy year was able to run and rescue them so that I could have Ben at the hospital with me. At that time i had no idea if Kendall was headed towards another bad crash or if it was just something “simple”. I had no idea if the hospital was going to follow Kendall’s protocol or if i was going to have to pull the momma bear routine again. Sometimes you just need poppa bear to come in and set some people right.

Thank the Lord we did not either of us have to get to that point. Edwards was already on top of everything by the time we got into a room thanks to a proactive phone call from our Milwaukee team explaining the severity of Kendall’s last few infections and that we just cannot tell ahead of time what is or isn’t Kendall “doing ok”. They started her protocol procedures immediately, gave Kendall extra comfort meds and Ben and I were able to focus on just praying over Kendall and getting logistics worked out. I was able to drive myself back home, get gas in the VERY empty car, and pack calmly for what is hopefully only a couple day stay. I went with the theory that if i packed for three weeks, we would only be here for three days. I got a small lecture about eating a real meal from “someone”, so I stopped to eat, and by the time I got back to Edwards, the milwaukee transport team had an ETA within 30 minutes. once I saw them get her all shifted over to their machines, I took off so I could be at the hospital ahead of them. I figured Kendall would sleep the whole trip up, but would be asking for me once she woke up when they stopped, and that is exactly what happened. We got about 30 minutes away from CHW and the skies just opened up into a torrential downpour! I was trying again to be calm about the fact that I was in one vehicle with zero visibility while my baby laid in the back of ambulance somewhere behind me, barreling towards the ICU, and I had no idea if she was still stable or if she was pulling crazy stuff. Again, the peace that passes understanding washed over me and I just knew she would be ok. Somehow, some way – she was going to be ok.

I pulled in to the garage, parked and unloaded our stuff, and began the long slow walk up to our unit. I knew we were in a room far away from where we were last month, but it was still a little hard to not start breathing a little hyperventilatery when i had to be buzzed in. I turned the corner around the welcome desk and down the hallway came one of our beautiful amazing nurses, one i am blessed to call my friend, running and jumping to come give me a hug. Its the kind of place you never want to be “excited” about being, and yet if you have to be here, its so good to be among friends and family. right then, I knew we would be ok. I knew i would get “some” sleep that night because once I answered all the questions I had to for check in, i knew our nurses would be able to handle anything Kendall threw their way.

And then the next day we had our angel nurse rachel back on the case – and when a new to us attending asked if we could transfer kendall up to the floor for observation, her firm and emphatic “no we can NOT” that went along with the horrified shakes of their heads “no” from the other rounding team members who know kendall again gave me such a peace that no matter what – we would all be ok. We were in the right place. It is hard and expensive and drives me CRAZY sometimes to make this drive – and yet, there is no other place. For better or for worse, milwaukee is where kendall belongs when she cannot be at home anymore.  I cannot put it into words for those who have never walked this path of medicalities before. And for those who have – you know exactly what I mean.

Anyways – Kendall is hangin tough. As part of her “emergency protocol” we started stress dose steroids in the ER and are dosing her every few hours. So far, Kendall has not had the cliff dive of craziness that we would normally see if she were septic (had something growing in her blood cultures). SO SO SO grateful for that.  But it leaves us all scratching our heads a little bit because her labs otherwise “look” like she could be fighting something, but she’s not “acting” that sick. So is that because the steroids are doing their job and stopping the catastrophic shock her body has been going in to? Or is it because this isn’t really sepsis, but rather a reaction to the IVIG treatment that is mimicking sepsis because her body is still recognizing those immunities as antibodies, something to be fought and rejected vs helpers to be assimilated.
Tough questions, and no one has any magical 8 ball to shake for good answers.
All we have is time, antibiotics, and a whole group of really smart doctors and nurses putting their minds to the grind and hashing through what they know of complex kids, and what they know of Kendall and her “-isms”. The hope right now, Saturday night, is that we get a final “all clear” report from the cultures drawn on thursday night, and that the labs we draw at 4 am show that her blood counts are going UP instead of the downward trend they’ve been on. If those two things happen, we will likely make plans for discharge tomorrow, call it a bad reaction, and have a conference at some point in the next couple weeks about how we will make a better plan for NEXT infusion time to support kendall’s body a little better to make it not cause this kind of reaction.
Mostly – I will be overjoyed to call this a reaction.

If it were another infection, it would be disheartening. we had just started gaining some good momentum with getting her back to baseline. It would feel a little….hopeless? I guess that’s the word i’m looking for….to think that her body was already spitting out bad bacteria. In spite of doing “all the right stuff” for her care. I do’nt really think it is an infection. i really feel like its just her crazy body showing us that her baseline is still razor thin. she has no reserves to deal with any little thing that pushes her out of that teeny tiny zone of “ok”. that in and of itself is a hard realization.
“but she looks so GOOD!” we hear this so often. It is a phrase that is the bane of every complex kiddo’s mom’s existence.We rejoice in the good days! They are awesome! We are glad that you get to see our kiddo’s who fight day in and day out just to have a semblance of normalcy “looking good”. I pour myself into making sure Kendall “looks good”. I do take it as a compliment when I hear that – but it is really sometimes hard to hear it. Because what it doesn’t say is “I can’t imagine how much it takes to keep her looking good”, or “how is she doing on the inside to be looking so good on the outside?” Not that i expect most of you to say that! If we are out and about in any kind of way where you are seeing us in public, she probably really IS doing as good as she looks. But for me – it is hard to look at her sometimes – wanting to chase her sisters and run to Target with mommy and just wanting to LIVE her life – and have to wonder what is really going on inside? 
but this is our life right now.

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we live by faith. Faith and hope that what we see is what we get – that good equals GOOD.
I am tired and feel like i’m rambling now. i didn’t get to many of my “mito awareness” plans for posts this week – but hopefully Kendall’s real life adventures are good mito awareness for you all. More so than any facts I could spout out – I hope that Kendall’s story brings awareness to the fact that none of us should ever take anything for granted. Grab hold of each minute, each boring hour at work, each tedious trip to the grocery store, each back-breaking loading of the dishwasher – relish that each of them has a good side. That’s what my mito awareness is. As I think of friends we’ve met on this journey who are watching their children near the end of their journeys on earth, I consider myself lucky. I will think of them, and think of our own story and the stories of so many of our friends with mito – and I will BE AWARE, that life is a gift.  All of the bad times and all of the boring times and all of the amazing yippee-skippee times – they are all worthy of being cherished. Because they all make up LIFE.

I hope you are all grabbing hold of life and living it to the fullest as best as you know how.

sorry for my crazy scattered thoughts tonight! if you have questions I didn’t answer – let me know!

 

much love to you all –

keep on keepin on~

 

terra

Medicalities.

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

ummm.

there’s some laundry over in the corner you can help fold instead of reading this post!

I was going to do two separate posts about yesterdays’ immunology appointment, and tonite’s UMDF family meeting with Dr. Cohen (a mito doc from Cleveland Clinic) – but they kind of are competing for space in my crowded brain, so they’ll probably more easily form into one post full of medical stuffs.

First – immuno appt yesterday. At 8:30 in the morning. That I remembered at exactly 7:08 in the morning. It was crazy but we made it. Dr. Chung was amazing. One of those docs who walks in with questions for you, cause he’s already read every single piece of info in the computer about your kid (which, since the dreyer and advocate systems are connected, turns out to be an actually quite a lot!)

It was a two hour appointment, including the impromptu skin/scratch testing we did on Kendall. She was not a fan.

BUT!!!!  – she is not allergic to kitties!!! Of all that I have obviously done wrong in growing/carrying/birthing/nourishing her, this is one less thing I have to feel guilty about! So anyways – this just went more towards one of the major “takeaways” from this appointment –

1.) Kendall does not have “typical” asthma, or even typical allergies. Dr. Chung believes that her asthma is of a chronic inflammation nature. Meaning that in spite of a surgery to stop the reflux from below, and the draining of her stomach contents so that there isn’t anything (in theory) to reflux, and the feeding of her nutrients even farther down her GI tract into the J-tube, and the thickening of feeds to limit her aspiration risk from above – in spite of allllllll of that – she is still somehow getting food into her lungs. Now, without hooking her up to an x-ray 24/7, this is just a theory, but the way he went through her labs/x-rays/swallow studies/clinical presentation to me, I gotta tell you, it makes a ton of sense.

So, ok. Her lungs are always (or almost always) dealing with trying to clear food out of them, and are therefore nearly always inflamed. BECAUSE they are always inflamed, any little virus that affects her Upper Repiratory tract, will immediately aggravate her lungs. Because energy is then diverted away from her muscles when she is fighting an illness of any kind, she’s at even more risk of aspiration than normal, so she is probably aspirating or at least micro-aspirating even more. And while her bronchoscopy in august showed no major damage as of yet due to all this inflammation, we know that they are headed there because of the degree of inflammation they did see on that bronch, and the fact that they would bleed from the slightest touch of the camera. And that was after about 3 “healthy” months that they were looking at her lungs. Blah blah blah – it’s a matter of time.

2.) Arguing over which meds are better – the ones pittsburgh switched her to, or the ones Dr. A has had her on for a year now? (which, Dr. Chung actually knows and respects dr. A – so that helps a lot – and they are both typically on the same page). And really, we’re talking about a po-tay-to/po-tah-to difference here (Qvar/Flovent), but in my mind, it’s a devil I know type of scenario. I saw her vastly improve on the Qvar. I saw her get RSV on the Flovent. I know the Qvar isn’t a magical pill. But I wanted him to tell me that the flovent was a miracle drug, or i was gonna switch her back. We really went back and forth over this for an incessant period of time, considering my children were throwing gloves all over the room and running poor Nurse Cindy ragged on the floor…Anyways – I think we agreed to keep her on the Flovent through her next respiratory illness, then switch her back to Qvar until the summer. Just to switch it up and keep covering both her larger and smaller airways.

3.) IVIG – the age old debate. Typically IVIG is used in kids who have what is called a “Primary Immunodeficiency” – meaning their immune system doesn’t produce enough of its own immunoglobulins, so they receive IVIG – intra-venous immunoglobulin, a blood product that offers a “fake” immune system to its recipients (and i know that fake is not the word i am trying to say there, but its late and i am losing vocab words!) Anyways – kendall isn’t a typical case for IVIG (I know, try not to be shocked by that.) And its not like you can just inject this IVIG in and call it a day. It comes with a large set of risks of its own, not the least of which is infection/reaction to the IVIG, and that’s even IF insurance will cover this super costly treatment. (to which my response is always – ok but they would rather pay for inpatient stays every couple months??? idiots…) But i digress. Anyways – Kendall is still pretty young to be able to say with any kind of certainty that her immune dysfunction is a true dysfunction. Her infections thus far have all been “normal” for the most part (she had the weird uro-sepsis when she was a baby, and its not normal to react to RSV and pneumonias like she does, but ok, we’ll call it normal based on what I know some of our friends have to deal with!). so because of all of that, we agreed to further table the IVIG as a treatment option, until or unless her infections prove to become overwhelming again (like the back to back hospitalizations last winter/spring). My personal thoughts/goals would be that I would, of course, like to NEVER have to even consider IVIG, but if we do, I’d love for her to make it another 2 years before we have to go to this. I don’t know how realistic that is. I am sure it will mean another two years of rather consistent infections – but hopefully we can find a way to start fighting more of them at home. In order to do that, we’d probably need some kind of IV access to run fluids/abx at home, which then opens her up to even more infections…..

rock.

Meet Hard place.

Such is the nature of miss KQ!

Which brought us back to

4.) If her lungs continue to be the source of most of her respiratory infections (and included in that would be ear/sinus infections, viral colds, pneumonias, etc), and we have a high suspicion of reflux/aspiration, we will need to consider, ARE STRONGLY URGED TO CONSIDER, taking her “npo” (nil per oral, nothing by  mouth to eat), at least on a trial basis, and doing strict J-feeds. Her NPO status would be mainly liquids, even thickened would be mostly off limits,at least severely restricted and monitored – but we would be allowed to find two or three “real” foods that she could still have.

I know.

It would suck. no two ways around it. I cannot even begin to imagine how we will accomplish such a thing, considering that she LOVES to get “nnnnaaakkkk” (her milwaukee-accented nasally way of saying “snack”) – but i know that if this is the right way to go for her, she will walk through it with the same grace and strength and happiness that she walks through the rest of life with. Jesus will be with her as He always has been and always is. Somehow, she will receive the strength she needs to give up food, and gain health again. it will break a little piece of my heart off to take food away from her. it goes against every single fiber of my mommy being. But the fact that this is about the 89th doctor to tell us that we need to do this for kendall, and the way he laid it out, gave me a sense of peace.

My compromise was that in the next two weeks we are waiting for her blood counts to come back up (she’s still anemic from her illness of a couple weeks ago), Dr. Chung is going to speak with her immuno team in milwaukee, as well as dr. Natalie and dr. A, possibly the pittsburgh team, and a few other colleagues of his to formulate his plan of attack. This is, at the very least, going to require another full immuno panel, which requires about 8 vials of blood on its own, and probably a few other complementary tests. There is little doubt that she is not ready to handle vaccines again, so our only option is to build her a bubble and turn her into a human hamster. Or spend every 8th weekend in Hotel LG. That could be my frustration talking though…

The rest of the compromise is that while Dr. Chung is thinking, and while kendall is rebuilding her hemoglobin, we will let her enjoy any and everything she wants. Perhaps even a little bit of protein (read: Chick Fil A nuggets!). Cake! Pie! Christmas Cookies!!! Chocolate from Poppa (who then gets frustrated when she smears it all over him!) Live it up – Eat, Drink and Be Merry~ for come New Years Day she will go NPO. And we will trial NPO for three months, through the end of March, and hopefully the end of the bad “sick season”. And see if we can’t help her lungs heal a little bit, and hopefully not be further injured by any other URI’s/pneumonias.

that isn’t quite set in stone yet, but if I talk about it now, it may not be as shocking when it comes to fruition.

Dr. Chung seemed very pleased with the fact that I could at least say it out loud, because, in his words, his threshhold for “forcing NPO is low. very very very low”. Meaning essentially that if she had so much as coughed in his office, I think he would have said “NPO. now.” So – this way I feel still semi-in-control of the situation. It is something that has been brought up HUNDREDS of times before. I know we are probably way too cavalier with Kendall and her PO feeds based on her history of aspiration and crappy swallow study results. this is just a part of her journey, and I know there is a reason for it.

If we take her NPO, and she STILL continues to get hospital-level infections, we will know that her body just lacks the intrinsic energy to mount proper defenses. even if she has all the right “numbers” on the bloodwork, they don’t mean a whole lot if they aren’t being properly utilized. After looking through a few more of her labs and tests from pittsburgh, dr chung made this happy observation: “ well, geeze, she’s actually pretty much a heme/onc patient in the way you just have to dig through her bloodwork to find the real source of the problem!” (Heme/Onc, for the uninitiated, is hematology/oncology, or cancer.) He wasn’t saying she has cancer – he was just saying that we might need yet another specialist to come on board to help us better filter through kendall’s mystery labs, or at least have a better framework for understanding why certain labs of hers will act a certain way. For instance, instead of elevating, which would be what 99.8% of the human race would do in “x” situation, kendall’s values will plummet. Or vice versa. Pick any lab value – WBC’s, ammonia, creatinine – hers will almost always be diametrically opposite of what you would expect it to be given the circumstance. This makes it very hard for ANY doctor to really know what to do to treat her, or IF anything should be done to treat her – let alone Doogie Howser, ER resident who is barely of legal voting age.

And this, dear people, is why I must use dye from a bottle to cover the gray hairs.

Because I will quite often have a child who *I* know is sick, and who looks to me like she is seconds away from needing to have called 911 for transport –but to an ER doc who doesn’t know her, she seems “mildly ill”. this is also part of why our nurses in home are so important. They are starting to pick up on Kendall’s “baselines”, and the little variations she tends to make from that baseline that indicate that “something’s up”.

So if her body lacks the functionality, then we would know that IVIG truly is the best course of treatment/therapy for Kendall right now, at least for a temporary trial (6-9 months, receiving an infusion monthly). At least we would know then that we truly had exercised every other option/resort before going to the IVIG.

So anyways – Dr. Chung is officially “on the case”. We are going to enjoy the rest of this awesomely awesome magical month. We are going to pray without ceasing that she is protected from illness, and do our best to keep her in a bubble. We are going to use her oxygen and hook her up to the backpack as often as she needs in order to stay stable through Christmas.

And if the new year brings a change to all of that, we will deal with it then.

And apparently i AM going to have to do this in two updates!

so stay tuned!

 

terra.

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