The condiment queen.

If you’ve ever met Kendall in real life, chances are, you’ve seen her live up to her nickname “the Condiment Queen”. And what that means is that rather than actually eating real food, Kendall is mildly obsessed with eating the “condiments” – butter, jelly, syrup, whipped cream (yes she’s basically on the Elf diet).
And what this has to do with anything is that the condiment queen is about to reign supreme.

I hinted last week on Kendall’s page that we had had a VERY encouraging GI appointment in Milwaukee but that I needed to blog the news in order to do it justice.

The back story to why this is such good news:

*sigh as I try to figure out where to back up to in order to capture all of the feelings associated herewith*…..

Ok the back story is too long to fully explain. Basically – three years ago this month, Kendall was miserable. She was constantly sick, needing a ton of oxygen on a daily basis, was making no progress in the daily therapy she was undergoing, and we were at  a complete loss as to how to keep her healthy, get her to grow, stop the intense pain she was in (pain/nausea due to feeds into her jejunem/intestines). At that time her doctors decided to start her on TPN and we began “gut rest”.  The rest is kind of history. Ups, downs, good times, bad times…..but through it all, we have tried to keep a sense of “normalcy” with regard to food. It has never seemed right or normal to me to not have her sit at the table with us for family meals. Not that we are the Cleavers who sit down every night for a fancy meal – but when we are together and are eating, we put her at the table with us and make her a plate of appropriate foods and if she wants to taste/lick/eat a bite or two – great! And if not, no pressure.
Food is such a social thing. So many events in life involve food or are centered around food – and I never wanted her “issues” to be anything that made her stand out or hold her back. Of course we have learned what does or doesn’t work for her (meaning basically we’ve learned that some things cause more pain than they are worth and some things are MUCH easier to suck back out of her gastric tube than others!) And it’s not like Kendall eats a large amount of food by any stretch of the imagination.

But she WILL eat some things. She grazes. A bite or two here, go play, a bite or two an hour later, maybe a lick of butter as a snack, then a couple more bites of applesauce later.  I’m really really proud of how well we have done at keeping her “oral” in spite of all the gut issues she endures. And even though it doesn’t always go all the way through her system (most of it ends up sitting in her very weak stomach until it drains out hours later) – she eats.

How this relates to our current news:
Her GI doctor has come to the realization after the last few years of trialing multiple formulas, that maybe Kendall’s body is just not ever going to accept a commercially prepared formula as a means of nutrition. This really isn’t surprising considering the ingredients of most of them (corn syrup, etc.) – but it’s a confusing and hard to understand thing.  i don’t know WHY her body doesn’t accept the formula – and really, even that is just a theory at this point – but clearly, j-feeds have only led to a vicious cycle of bad septic episodes.  The last few months we have given her a break from j-feeds, and she is doing fabulously. Coincidence? It’s hard to say. But it’s not really a theory anyone wants to put to the test again.

so for right now – we are saying “see ya” to j-feeds of formula, and we are saying “come on, condiment queen!” to the rest of her gut! We are going to let Kendall prove to us what she can do for herself nutritionally. we are going to be SLOWLY SLOWLY SLOWLY weaning the calories down out of her TPN, and seeing if she can continue to maintain her nutritional status based on what she can take in orally.
i’ve gone over her diet with a fine tooth comb with our TPN dietician, and she is aware that we are talking miniscule amounts of food right now. But the hope is that if we can keep Kendall’s lower GI tract “cleaned out”, and maybe force her into “hunger” by reducing her calories, that she will inherently take in more calories on her own. Again – it’s only a theory at this point – but it is at least a PLAN and it at least FEELS like we are making forward progress!

right now Kendall’s TPN calories are three times what a “normal” 6 year old needs based on metabolic testing that we’ve done. Weaning her calories will be a painstakingly slow progress before we can say we’ve done anything major or noteworthy – but the point is – we ARE making progress. We are moving forward – and this is HUGE!!!!!
I feel like i’ve not done a fabulous job of fully explaining why this is all so awesome or why certain things work while others don’t.  I guess on some level – I don’t have all the answers. I just plain don’t know why my kid is the way she is. I just know she’s awesome, and she’s forging her own path. And i don’t know where this path will take us. But I’m glad to be on it.

So that’s our news!

I have lots more other blog posts floating around in my head – but my computer is still being slow and stupid and I’m feeling not very coherent right now so i’ll try to post more tomorrow.

Peace out party people~

T-crest.

Hope In a Can.

So much hope in one little can. And a big bag of pure sugar.

With this crazy mixture, we hope we can rehabilitate Kendall’s gut. We hope her body will accept this blend of nutrition, absorb it, move it, grow on it, thrive on it.

Hope.

It’s the special ingredient not listed on that can.

If I were to pour out my heart here, all my feelings about this experiment – it would leave me feeling too vulnerable, raw, open. So i’ll try to sum up the ones that are closest to the surface.

To fully understand why I am emotional over this, you’d probably have to have more than a working knowledge of exactly why Kendall is on TPN in the first place. And then you’d have to understand that while TPN has been a lifeline, it has also been part of what has tried to kill her. It is the devil you learn to love and live with. And the truth of the matter is, the reason Kendall’s body has grown and thrived only on the super boosted TPN is somewhat of a medical mystery. Her issue lies somewhere in the nerves and how they communicate with her brain to communicate with her muscles. The messages get screwed up a lot. This leads to what is known as “pseudo-obstructions” – her body believes there is an actual blockage in her gut and it just shuts down operations in an attempt to protect itself. Except there really isn’t a blockage, there’s just an area of her gut that isn’t moving because the nerves are not working or the muscles aren’t getting the message or ??????

There is no real rhyme or reason to why these things happen. She could have a few good weeks with some minimal movement – these are the weeks that she is mostly happy, pain-free, not retching from nausea, wants to sit at the table and attempt to “eat” dinner with the family. And then she’ll have a “PO episode” – and her body will try to fight, and she gets her low-grade fevers, and has bad crampy diarrhea all day long, and gets nauseated at the drop of a hat, and lives from zofran dose to zofran dose.  those are the hard weeks. The weeks that I am quiet because the words I want to say sometimes aren’t “acceptable” language, and it mostly would just come out as a whine, a complaint about something over which I have no control. As she has had to fight off the regular colds and illnesses that a cold hard winter brings, the bad weeks have been more frequent lately. But then she’ll have a few good days and we try to hold on to those.

Overall – we keep moving forward. We focus on the good, the positive, the happy days. We choose to see that the overall big picture is one of awesomeness, and improvement, and yes – much hope. I am too superstitious to actually say out loud what next Saturday’s milestone will be – but if you have been around since the beginning, you’ll realize there’s a certain pattern regarding this current month.  And while I have no reason to think we won’t make it until next Saturday – I have a child who likes to pull crazy stunts. So i’m not gonna say it out loud yet.

So it is time to attempt to use Kendall’s gut for nutrition again.

For those of you who have seen her snacking before, you may be confused. I’ll try to explain.

Kendall CAN eat. On the good days when she isn’t doubled over with pain and nausea – she actually asks for a lot of food. It is confusing to understand if you aren’t around someone whose brain is routinely getting bombarded with the wrong messages from their messed up gut nerves.  Think about how you feel sometimes after having a tummy bug. You’ve puked up everything there could possibly be to puke up- and you’ve sworn you will never eat again so you don’t have to puke anymore. But your body needs nutrition. So you get messages to eat. And eat a lot. Eat whatever feels good, even if its telling you to run to Outback Steakhouse and get the biggest steak they have and load it up with mashed potatoes and grilled onions. The LOGICAL part of your brain tells you that no, that is not a good idea. But still, the message keeps coming – EAT EAT EAT!!! you may even have hunger pangs. Eventually you get brave enough to maybe nibble a saltine even though you know the risks are that you set off pukemageddon all over again.

Kendall’s problem is that she has never been able to logically connect that putting food into her mouth leads to the bad tummy pains. We spent the entire first year of her life hoping/believing the doctors who told us that she just needed to “wake up and eat” and all would be solved. She was in so much feeding therapy, speech therapy, we even took her to have her throat shocked twice a week trying to wake up the nerves so she would just EAT. After all that work – she finally learned to eat. She loved sitting with the family, she loved the social experience of “eating”. So trying later to undo all that work – it was hard. In her life, there’s not much she has control over. Some days, her wanting to snack on a few goldfish or have a butter sandwich is just not a battle I want to fight with her. We try to control WHAT she is eating – make it something that can eventually drain out her g-tube port if it decides to not move through very well. But if the girl wants a snack – well, she gets a snack.  It does not mean she is getting any nutritional value from it. Most of the time it sits in her stomach until she is hooked up to her drainage bag at night and then it makes its way back out her tummy. sometimes it slowly moves through, still mostly undigested, and shows up on the “other end” a few days later. sometimes it causes a massive bout of immediate diarrhea and it literally travels right through her. We try to help her connect the dots so she can start making smarter choices for herself – and sometimes that works and sometimes it doesn’t. 

But the bottom line is – food is her choice. the amount that she actually ingests is roughly equivalent to 1-2 snacks the average toddler would consume. That’s on a good day. The actual nutritional intake from that is very close to nothing. so even though you may see her “eating” – it is not a sign that she is “fixed”. The kid has literally been puking/retching into a bucket and asking me for a snack in between heaves. She just doesn’t get the connection. She fights through so much pain and nausea I cannot even fathom. We have tried to cut down on the major swings by just keeping her on a scheduled regimen of zofran (vs only using it when she is in obvious discomfort). We have adjusted her meds that have to go into her gut as those make her nauseated also. We have given her body 6 months of “rest” from having to do any of the work of absorbing nutrition, while still keeping it “moving” in a way by running pedialyte every night into her jejunal feeding port (into her small intestine).

And now, after 6 months of increasing stability and an overall positive direction towards “good health” – it is time to put her gut to the test again. Since we have previously tried every commercial formula out there considered “elemental” (already broken down so digestion is easier on kiddo’s with tummy issues), our dietician and tpn team got real creative with their plan. Due to some of what we know about her body does or doesn’t break down foods, we are going with a formula that is completely devoid of carbohydrates (since the assumption is that this is one of the harder parts her body has with malabsorption and why she requires such a high amount of carbs in her tpn in order to maintain growth). So the formula is carb free, but nutritionally it doesn’t work very well to have a 5 year old on a strict Atkins diet, so we have to add in a simple carb like dextrose. So we get a huge box full of sterile water, the carb free formula, and huge bags of D20 delivered to us, and we mix this up into a super unpalatable blend of nutrition.

We plug her feeding extension in to her jejunal port, turn on the pump, and we wait….

Last night was the first night of running this formula.

I have strict instructions that she is to be brought to the hospital (Milwaukee) immediately upon any sign of intolerance. Even if I think I can push her through it – she has to be admitted. Her GI is not a huge fan of doing this outpatient, but our team as a whole knows that our goal always has been and always will be “happy days at home”. So here we go.

she will be running this formula at a very low rate 24/7. If you know Kendall, you know she already wears her backpack with her heavy TPN bag and pump in it 24/7. So now we add in another bag, another pump, another tube she is constantly hooked up to. We will slowly be working our way up in her rate over the next couple of months. At some point around June, assuming she tolerates the rate increases, we will begin weaning down her TPN support. This will be HUGE. ANY amount of time we can get off the TPN is that much more boost to her liver, that much more of a shot at “surviving” tpn.

I am oh so very hopeful. And yet a teeny bit afraid to get my hopes up too high. I know God knows. I know God is in control. This is only so helpful on a day to day basis when it’s my baby, and the stakes for this succeeding or failing are so high. I have to hold on to that Hope though.

so here we go.

Mixing up a can of hope.

Cheers.

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terra.

Atkinson General Hospital.

 

Part 1.

That’s what we’ve decided to rename it here at our abode.

I don’t even know how to go back and begin trying to start at the beginning of the medical saga that is our lives – so we’ll just kind of jump right in!

I think I had posted a while back about Karissa’s issues. Add to that now Kaylen and as of last nite, ME TOOOOO!!!!!

YAYHOO.

So  – a quick recap/update/intro on what’s going on here so you can keep up when I am a completely nonsensical mess in January. K?

Karissa

I think in my last post referencing K2, I discussed her having had a spina bifida defect at birth and a few of the complications that we have seen over the years from that. And I also talked about her general issues/decline over the better part of the past year – things like cognitive issues in school, a noticeable decrease in her k2aTT energy levels, and just a basic blah-ness to her. If you’ve seen her recently you’ve also noticed the addition of some new hardware to her – she has had a pretty shocking and quick decline in her vision since last spring. Of course this is a rather “common” occurrence for many kids – to need glasses at around this age. Even the doctor was rather concerned though about how quickly it went SO far downhill – and I was shocked that Karissa had never once verbalized that things were “fuzzy” or in any way hard to see! Getting the glasses though was a huge help to a lot of the issues we had become very concerned about (cognitive issues at school, behavioral outbursts/super-emotional, headaches, etc). Unfortunately, the glasses didn’t fix everything – as she was still dealing with a lot of urinary and bowel issues – all of which she had very badly right before Kendall was born also, but were just pushed to the backburner when it seemed like everything else was crashing in.

So when we finally got to talk with the pediatrician about all of our concerns, she agreed that it was time to talk to a neurologist who deals with spina bifida, and to order an MRI to look for a condition called “Tethered Cord Syndrome”. Basically, in a healthy spine, the spinal cord is able to move freely within the vertebral space, and this is part of what allows us all our flexibility and ability to deal with a variety of injuries that seem like they should otherwise be debilitating. In kids with a spina bifida defect like Karissa’s, where surgeons often DON’T go in and correct any abnormalities like happens with the more severe and open forms of spina bifida, the spinal cord unknowingly can connect or “tether” to the backbone via a variety of mechanisms (bony or fatty growths usually), and the spinal cord is no longer able to float freely in that space. As the child with tethered cord grows taller and becomes more active, more problems from having a tethered spinal cord start to arise. Understandably, the more stress and strain that is put on a cord not designed to stretch like a rubber band, the more problems will start to pop up and get worse. And that’s where we are at with Karissa.

She may not have a tethered cord. She could just be having a really really rough year and having it show up in a lot of different areas in a lot of different ways. I mean, admittedly, our lives are far from “normal”, and it has only been in the past couple months that we have seen ANY kind of “stability” for the first time in years! I go back and forth a lot with thinking she definitely has a tethered cord one minute, and then the next thinking its all just ridiculous and over-reactive. But watching her change and withdraw more over the past few months – I know its the right thing to do, to at least get it checked out.

When the nurse called to do the pre-procedure questionnaire for the originally scheduled MRIphoto (which we had scheduled at an outpatient center), she said that due to Karissa’s snoring (she sounds like a train most nites!) and the fact that Karissa is needing near daily ibuprofen to help control her pain, that we would need to reschedule the procedure at the main childrens hospital (the one actually here in chicago! yay!), and she would need to be put under GA (general anesthesia) and be monitored for a while afterwards. So now the MRI is scheduled for January 9th, with followup with the neuro on January 12th.

From what we understand right now (based on conversations with the pediatrician and dr. google only) is that if  there IS evidence of tethered cord, a decision would probably be made by neurology/neurosurgery to “de-tether” the cord in order to protect some of her organs from further or worsening nerve damage. I really have no idea how soon they would recommend surgery or IF they will recommend surgery. We know from X-rays at her chiropractor’s office that there has been a definite change in the lower curve of her spine – where it should bump towards her stomach like an S – hers has become much more C like, and bumps out towards her back. It’s hard to put into words – but I can say that all of the above, and more that I can’t even describe, have left me feeling very sad for Karissa. Wishing I could take away the pain, make her happier again, see her bubbly cloud gathering again for more than a few minutes at a time on random days. It’s still there, don’t get me wrong – but it’s changed. and I can’t wait to see it come back!

 

 

Kendall

IMG_3061 As a quick Kendall update – she is hangin’ in there! She has been in a LOT of pain the past few weeks – seemingly stemming from her GI tract. It is causing oral and enteral (by mouth and through her tube) feeds to not be super successful – in that she seems like she is STARVING, but then can’t handle more than a nibble or two. She tells us multiple times every day how she is in pain, doesn’t feel good, has a bad tummy, wants her bag on (her bag is how we relieve pressure that builds up in her stomach – we typically only vent/drain her at nite time so her formula doesn’t build up and try to get puked out/into her lungs, but when she is feeling really badly, we hook the bag up during the day when she is too sick to move off the couch much anyways). Anyways – we have been able to give her an anti-nausea medication orally up to this point, but since we know her gut does not absorb most medications very well, and since we would need to be using it nearly non stop in order to stay on top of the nauseating pain, it has really not been much of a weapon in the fight against “kendall’s crappy gut pain” as of late. After going back and forth with the doctor for FAR too long, we were finally able to get this medication ordered as an IV med, and wouldn’t you know – halfway through the first dose being infused, Kendall sat up in my arms, announced that her tummy didn’t hurt, and her temp instantly dropped two degrees (she had been running a slight fever, which she does as a pain response quite often). I’m so glad for the relief, and yet frustrated that she had to go through so many weeks with ZERO relief to get here. We see GI and complex care again the first week of January – and I am REALLY hoping they have some better ideas to help get this gut moving again. TPN has been an amazing boost for her – and i love watching her learn new things in school, which i know she could never tolerate if she was getting her energy zapped by having to use her gut for all her nutrition/hydration – but it would be really nice to get some native functioning back here SOON! Overall – she is doing well, and for that we are absolutely BEYOND grateful!

 

Since Kaylen and my updates will be so long, I’ll end Part 1 here and hopefully get to Part 2 tomorrow!

hope all is well with all of you~ thanks for still checking in on us!

Have a wonderful Monday!

 

terra.

The Kendall Update.

Oh my gosh. I have seriously got to be better about updating this thing!

So – since we got out of the hospital, Kendall has been steadily moving onward and upward. Thank God! We had a pretty “iffy” first week at home, basically because her body had been without major nutrition for over a week at that point, and that just doesn’t work real well for kids of ANY level of health, but especially for kiddo’s like Kendall! Her lab values all reflected a very depleted little girl, and that is definitely what we had on our hands for those first few days at home. I admit that at one point, I was just this side of “scared” for her. Scared that any and all absorption had definitely tanked (we know she doesn’t have GREAT absorption of nutrients through her gut, but she still has SOME), that we had caused even greater damage by letting her go without protein/fats for too long (she was getting mainly carbs her entire hospital stay, in the form of mass amounts of sugar water in her IV), that something BAD was happening. I work really hard usually to keep my mind out of that bad place, and instead focus on everything GOOD she is doing. But one day last week when everyone had commented on how pale/weak/bad she looked, it was hard to shove those feelings inside.

IMG_2379 Eventually she DID start making that turn back to good though, and I am happy to report that this week has been much better overall! We were able to make HUGE progress while in the hospital with regard to a better plan for getting Kendall’s gut working again, and that in itself is a relief. The hope now is that she is OFF of TPN by her birthday (about three months), and onto a better formula for her. The problem is that she is still on an infant formula that is elemental (all the way broken down so her gut doesn’t have to do too much work), but obviously, the makeup of an infant formula isn’t enough to sustain a growing child. Infant formulas are calculated to be roughly 20kcal/ounce. Toddler (and up) formulas are calculated to be 30kCal/oz.  It seems like a simple enough equation, and yet somewhere in the middle of that kCal crap, Kendall’s stomach revolts. We have never, in spite of our best efforts at ignoring symptoms and pushing her through crampy doubled-over retchfests, been able to get her to truly tolerate more than a 22kCal/oz concentration. So we have been stuck with an infant formula that has thus far mostly met her nutritional needs. Especially when you throw in the random lick of donut frosting, handful of Goldfish crackers, and couple of Saltines. She’s been ok. (Negating all the goodness of the TPN at actually keeping her ALIVE – the nutrition into her gut has been GREAT at protecting her liver and keeping things mostly moving “forward”. Kind of.)

So our days are spent trying to weigh out if her symptoms/pain level that day are manageable enough that we might be able to bump her rate forward by 1cc and hour that week, and continue to challenge the concentration of calories so that we can eventually switch her to the more appropriate toddler formula. Mostly, she’s been doing great. If you are willing to believe that the chubby face is from nutrition (good) and NOT from third spacing her fluids (bad), and if you only see her for the 1 hour of frantic activity she has when we are out and about anywhere, you might even think she is  doing SPECTACULAR. And indeed, she really is. I’m not even going to be superstitious about it, I’ll say it out loud. She is doing great right now. We are making SOME forward progress, however painstakingly slow it feels like it is going. We are actually getting more words out of her and even got a spontaneous 3-word sentence out of her yesterday. Wanna know what it was?

“Chill OUT, Kaylen”. Except it sounds more like “shee-oo ottt La-La”. But there it was, yesterday when we went to get her out of bed and Kaylen (aka La-La) was running around like a banshee. Apparently she picked up this little gem from me, seeing as I probably say it a good 39 times a day. Oh my kids. They drive me absolutely INSANE some days, but God love ‘em…they keep me laughing.

She still gives us scares now and then. Last Friday for instance, when her nurse and I are trying to keep each other calm by not freaking out over the attempts at puking and nearly catatonic state Kendall seemed to be in while we ran out for a few groceries and to Ikea (we had taken the babies with us so Ben could rest some more). Checking temps and HR and draining a tummy are somewhat challenging in the stroller in the middle of the Bjellkat and Dorsdung bedroom displays. I just about needed a paper bag (Fjellsta, aisle 8) to hyperventilate into because I could just SEE this turning into another @#(%*) line infection for her. But we hung up her bag of extra IV fluids, gave her some more meds, put her to bed for a few hours, and breathed a sigh of relief when she seemed much better after that nap. She is back to taking 2 naps a day more days than not, even though the 2nd one is often only around an hour.  But in between those times, she is looking great. So we will take it!

She had her evaluation for the special needs preschool (where kids who are in Early Intervention go once they age out of the system on their 3rd birthday). Of course in some areas she was “within normal limits”, which tends to go up and down depending on the kind of day she is having, and in others she was “referred”, meaning they need to evaluate her further in order to make educational recommendations. It was the usual mess of emotions that come from hearing your child has delays. But one bright moment, our evaluator told me “Kendall actually seems ADVANCED cognitively, its like she just can’t get that message out to her arms and legs to do what her brain wants her to do!” Puff my heart up with pride and hand me a brick. Pride that i think for the first time EVER Kendall was “advanced” in a good way, brick so I can bang my head against it cause I have told her doctors this for YEARS. Actually 2 years and 9 months to be exact. Anyways, it was just a mommy moment.

We went to pick up her new wheels this morning. Our awesome wheelchair/seating tech called it “her highness’s throne”, and I think that’s gonna stick! I’ll have to get pics up of her in it when she wakes up from her second nap!

So there’s the Kendall update. now I gotta go type one up on the Gimpster and how he is doing post-surgery.

 

Thanks for checkin’ on the K-Quinnster!

 

terra

Manometry.

In case you don’t know what manometry means – let me tell you what a few synonyms for it are:

Torture.

Hellish Pain.

Cruelty.

And I know its not that bad for everyone. But it was my worst fear going into this test – that it would be so overwhelmingly painful for Kendall, and indeed, it was. I am not going to go into a whole lot of details about that day simply because it was truly one of the most horrific things I have ever watched her have to deal with. I did not even take pictures of it because I never want her to remember the horrifying details. Basically they removed her GJ-tube/button that is always in her tummy, and replaced it with a tube containing 8 smaller leads/wires. They also attempted to place a foley catheter in the same stoma, and none of the nurses could do it, so the doctor said “let me try to jam it in.” Which indeed he was successful at. These wires threaded down her stomach into her intestines similar to the path her GJ tube takes, except it extended just the slightest bit past where her tube goes. Horrifically, a similar tube was also placed up her rectum and threaded to the junction where her large and small intestine meet. This sounds like no big deal until you watch almost 4 feet of thick tubing get pulled back OUT at the end of the test. I just about threw up. She had more tubing in her than she is tall.

On top of all this funness, she was grumpy because it took enough anesthetic to knock her out to put an adult to sleep. Typically she is able to get morphine coming out of anesthesia to help combat the grumpiness, but for this test, she could have no meds that might interefere with her motility. No morphine, no tylenol, no ibuprofen. No Zofran for nausea, no venting of her tummy for pain relief, not even numbing cream around her stretched out stoma. She couldn’t even keep her eyes focused in front of her at the beginning due to the pain and nausea. She could only give me glares every few minutes in between arched back screaming fits. We had to try to convince her to lay down/lay still during the entire test (supposed to be about 4-5), and she was not having it. She was just grunting/screaming in sheer pain. At one point I just finally laid down on top of her, we both had our heads buried in pillows crying – she crying AT me, I crying FOR her. After an hour, she had finally calmed enough to stay laying down or at least reclined enough for one of the nurses to step in and distract her, while the other one pulled me away and sent me down the hall to regain my composure.

At one point almost five hours into the test, we had still not seen any kind of good pattern, contraction or rhythm on the test. It was very tense in our room. We had given the first medicine meant to induce stomach contractions and it was not working. The second had just been administered, Kendall was finally just about to pass out from the pain, and it happened. One semi-weak, but rhythmic contraction. The nurses cheered, paged the doctor who came right in and looked at it and had a half-grin on his face. We NEEDED that contraction he told me. Three hours later, the test was complete. Three more hours of horrific screaming that had people on our floor shutting their doors and avoiding our room like the plague. They gave her a small bolus of her formula into her stomach and her jejunum, and then gave an enema from the top of her colon down and we waited again…..and FINALLY saw/heard/witnessed the five contractions we needed from her lower colon (meaning that she pooped! luckily it was just water, since she had a $50,000 tube blocking the exit…gag!)

The initial results we received in the one hour meeting we had with the doctor leave us with much hope. She CAN have normal function. She does not right now, and the normal function we did see obviously came at a very high price (her pain and nausea levels are not sustainable right now for the payoff of still very weak and too few contractions). But we were able to eliminate a lot of bad stuff from the table right now (transplant, organ failure, surgical anatomical changes to her GI tract, ostomies, among others). In my heart, I always knew Kendall still had SOME function. I have never once given up on her gut. It has been horrid and crappy from the start of her little life, but i have always known that it was still working on some level. Even on the TPN, we have retained some very good function. She is able to get about 1/4th of her nutritional and fluid needs met with the J-feeds we are able to run (well, at least half of the time…). So the results are not shocking to me that there is the capability of normal function. She does not have  an inherent motility disease – meaning that the nerves inside the GI tract and the muscles of the GI tract CAN contract in an appropriate fashion and play nice together – IF the right environment for such is created for them.

She does, however, have an outside or secondary neuropathy. The nerves from her GI muscles to her brain are not communicating in an appropriate fashion. They are misfiring at times (leading to the increased pain or the reverse motility patterns we saw more often than not), or not firing at all at other times (leading to the times when everything that we are able to get into her gut comes pouring right back out any exit hole it can find). This is believed to be due to her autonomic dysfunction, which is a part of the larger (quite poorly understood) connection to the mitochondrial disease. Our motility doctor was not comfortable giving us an overall picture of what to expect from her gut simply because he is not a mito doctor or even a genetics doctor – he is a motility doctor. and right now, we CAN get some motility. What I find to be ironic is that these are the exact nerves that we get corrected by our family chiropractor every week. And while I know some people’s thoughts on chiropractic range from very positive to thinking they are just this side of quacks, for us, it works. I have seen it help countless times. I know that the limited function we ARE able to see is because Kendall’s nerve pathways are constantly being reset back to as good as we can get them. I shudder to think of how bad her testing or her function in general would be without the benefit of direct therapy to those nerves each and every week of her life. Because while we got SOME good contractions – it was essentially less than five minutes worth. Out of almost 8 hours of testing and stimulating and recording and watching….five minutes. But we will cling to the hope that those five minutes offer, and pray that we can turn it into ten. And then fifteen. And then an hour.

Essentially – the capability for normal function is there, but the pain it causes to reach that function are extremely ABnormal.

It will not come easily.

But if, in the end, it gets Kendall off the TPN and helps relieve her pain and lets her LIVE her life again, it will be worth it. A team of Kendall’s doctors from Milwaukee conferenced me in on a call yesterday where we came up with Phase 1 of our plan. We will be trialing a few different medications in an effort to help slowly stimulate those same contraction patterns we did see. We are doing the trials in a very methodical and controlled manner, which I appreciate. I hate nothing more than when doctors just throw meds around at symptoms and you never know the real story about what is helping what or what isn’t working about a particular plan. Especially with the ramifications of what happens if we CAN’T find a good medication option, I want to know that we have given each and every one a good hard look at what is working and what isn’t. Once we have ramped up the first medication and kept her steady on feeds, we will add in a second one slowly. If those go ok, we will begin advancing her rate of feedings very slowly and taking nutrients out of her TPN very slowly. The impatient side of me (ok that’s pretty much the only side there is to me) wants to just stop TPN next week and turn up feeds and beg and plead with her body to just please please please work.

The way it was explained to me though is that what we are trying to do is work around a bad nerve pathway that has been created. If we change things too quickly, we risk really pissing those nerves off even more, and losing any ground we have been able to gain or maintain. Right now, she is on a good enough rate of enteral feeds that we have been able to really protect her liver from having to do ALL of the work. (Liver failure is one of the biggest and most real risks of TPN as a long-term nutrition solution.) If we lose any of that ground, it would be devastating to her system. So everyone else (besides me) wants to move very slowly on this. I think the biggest shock to me about how serious they are about going slowly is that one of her doctors on the conference call who has been very “upbeat” about kendall’s issues (brushing off some of my concerns, telling me she’s way too healthy for this that or the other thing, etc.) – was one of the biggest voices in the room on how slowly we will go with this, how we aren’t looking at getting rid of the line for many many months, if indeed she ever is able to get rid of one. So while the situation overall is very much full of hope and ambition, there is still a very long road to “success”. And it will be a bumpy road. And it will probably have setbacks. And at the end of the day, it still might not totally work.

But we HOPE it will. And our HOPE lies in the Author of all HOPE. And as has always been our view on this journey with  our strong leader in the valley,

“Hope is a good thing – maybe the best of things. And no good thing ever dies. I hope…I hope…I hope.”

So in spite of the absolute horrific trauma that the testing was, we are so very grateful for the awesome team of doctors we have, who can interpret this information into a real and hopeful plan for rehabilitating Kendall’s GI tract, and allowing her a chance to get off the TPN, if even for a little while. As Dr. S (the motility doctor) put it to me – without that one good contraction we saw, you and I would be having a VERY different conversation right now, one that I hope we never have to have.  So we rejoice that her system showed us that indeed it is Kendall’s. It may not feel like doing a whole lot, but it’s got the fight in it that is so totally her!  I really really really do not ever feel like even talking about that day again. I know some of my special needs momma friends have had to witness so much worse, and on a near constant basis. But I still feel afraid to close my eyes or have it be too quiet. I can hear her screaming if it is…(does that sound like Hannibal Lecter to Clarice? “can you hear them clarice…the lambs, do they still scream??? run run run…..run run run……”)

anyways – now that i’ve completely lost it (and proven it on the interwebs once and for all!), I’ll wrap it up.

Thank you to everyone who was praying for us on that day. As you may have noticed, I was SO dreading it I couldn’t even really talk about it in advance. Every time I would think about it I would be in tears. So to the few who knew ahead of time, thank you. To those who prayed even without knowing, thank you too. And to everyone of you who is hoping and praying for us that this new information and new plan is the KEY to getting things working again – THANK YOU!

I have so much more on my heart and mind to share –

and I will hopefully get the chance to soon, but please keep the Hilliard family in your thoughts and prayers. Eithene is a little girl who has battled against mitochondrial disease her entire four and a half years on earth, and now she is weary.  Her family is preparing to allow her earthly body to earn its rest in this coming week. Eithene’s mom has been an amazing friend to me, helping me navigate some of the more crazy aspects of this disease and the weird things it can do to kids’ bodies, sharing of her spirit and her amazing love for God in the midst of her own storms raging around her. She, her husband, and Eithene’s little brother will need to be lifted up in ways I cannot even begin to imagine over the coming days, weeks, and months.

So this just seemed like a fitting video for today. I don’t know why. Cause it usually makes me cry pretty good. Anyways.

That’s about all I have to say about that.

 

T.

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