8 hour cycles.

This is what our life has become – 8 hour cycles.

I know I should have updated way before this – but now I am time crunched and can’t get into all the details like i’d like to – so here’s a few quick bullet points to hopefully help answer some of the questions you all have about what’s going on with miss Kendall Quinn.

Our lives are in 8 hour cycles because that is Kendall’s IV antibiotic dosing routine right now. I am starting to see why doctors try to avoid sending kids home on IV antibiotics because what is easy for nurses in the hospital on a 24 hour care routine is MUCH harder in real life actuality. (and to all my friends who do this on a very routine basis – please know I am not complaining – just “processing”! )

So kind of a chronological timeline/stream of consciousness spewing from Friday to today:

  • IMG_1822 Friday morning we got confirmation from the Milwaukee lab that Kendall’s cultures from there were growing the same strange bug as the lab in Naperville – Pantoea Agglomerans. You can google it if you want – you won’t find a whole lot! It’s a gram negative bug that is plant based, and seems to only affect severely immunocompromised people in hospital settings (although if you happened to have a nasty run in with a thorny bush or say a tree limb impaled through a body part, and then you later developed an infection in your joint or in the area of impalement, it would very likely be a P. Agglomerans infection). In short – they have no idea how Kendall got this bug, but bets are on a TPN contaminant that may or may not have made other kids sick, but in her “still recovering from a crappy spring full of blood infections and sepsis’” immunocompromised state, she got sick from it.
  • The good news about this bug, is that it tends to play nice with the right antibiotic. We had luckily added in one of the “right ones” the day after she was admitted, and that’s why we saw such rapid improvement. The problem though was that she was on three different antibiotics with a crazy dosing schedule, and the team all knew we could not go home and sustain that level of care in our home setting.
  • I am not good at math or explaining things like this but here’s part of the problem: Kendall has what is called a “double lumen” PICC line in.  The point of this being that since her TPN (food/fluids) runs for 16 hours of the day, instead of stopping and starting that all the time, we should in theory be able to use the other “lumen” or end to hook up to her antibiotics. Except that for Kendall, her other lumen (the white one) has never worked very well. It’s a good bit of a wrist workout to push a flush (saline that we use to clear the line out), and you NEVER get blood return on it. You may recall that we had this same exact problem with her LAST PICC line in April, that was placed in essentially the same spot as this one. We had to “TPA” it – which is a long name of a medicine used to break up blood clots. We don’t know if Kendall is clotting off this line (or the last one), or if its “going positional” – meaning that the ending of the plastic tubing that threads up her arm and ends near her heart is getting “sucked into” or embedded in the wall of the vein somehow, giving us the illusion of trouble/pressure/etc. It is supremely frustrating to have issues with this lumen, but given the fact that Kendall has already had a few issues finding access for central lines, and we don’t know how likely she is going to need those access spots, it’s not the kind of thing we want to go ripping out a line for.
  • SO – we were trying to juggle getting SOME antibiotic coverage through that lazy lumen, with the other lumen having a “tri-fuse” on it so we could run her TPN/lipids/antibiotics in the hospital. We had a separate pump for each lumen dedicated solely to the antibiotics, and our nurses had a handy little chart by which they knew exactly which med was going into which lumen at which time on which pump. This worked great with round the clock care. There are obvious problems when trying to translate this into the home setting. Namely, I am not that organized, we don’t have that many pumps, and we don’t have round the clock help!
  • So to consolidate things and get us home in time for the holiday weekend, our docs consulted with ID again, they decided to order her this fancy-dancy super supreme antibiotic that they don’t like to use because its a “big gun” and you want to save the big guns for the big battles. BUT – since the lab had never even had to identify the bug that Kendall is growing before, and there isn’t a whole lot of literature out there to say even what “normal” kids would do with this bug (let alone Kendall who writes her own medical journals), they felt it was our best bet for getting home.
  • They sent us home based on how well Kendall looked clinically. We were all IMG_1825 waiting for and even expecting the “fallout crash” to occur – and it never did. At least not there. It was very hard to keep a kid there who really looked GREAT, especially given what she was dealing with. No one could figure it out. I admit I was a little wary of a few smallish things she was doing, but i was able to convince myself I was just on edge and they probably meant nothing (things like her sleeping HR jumped from its normal of 90’s up to the 120’s, she was having a few more apneas than she normally did, her coloring all of a sudden went from pale to ghostly, she got very irritable, etc. – all pretty much nothing on their own, but they had my antenna up definitely.)
  • our AMAZINGLY AWESOME home pharmacy ThriveRx got the orders from our docs for the new meds around 1 pm on Friday afternoon, and we got word at 3 that they would have it all ready for us that nite and we could safely be discharged to home. So we packed up, said goodbye to our nurses for a couple weeks, and off we went!
  • Kendall CONKED in the car, slept almost the whole way home, and was ready to PLAY by the time we got her all unloaded/unhooked/etc. She rode around in the Barbie Jeep with her sister driving and Ben and I just sat on the porch with tears in our eyes thanking God that she got through another one, that we were home, together as a family.
  • our delivery of meds was driven from Ohio (where our pharmacy is) through the nite by an old man who arrived at our house at 11":45 pm. This kind of dedication to our family, and keeping our family HOME and TOGETHER is exactly why our pharmacy is in Ohio. Because that is where they are based. They are the best!!! Ben hooked up Kendall’s antibiotic at midnite, it ran until 1 am, he unhooked her and crashed for a couple hours. I, on the other hand, had crashed at about 9, and did not hear a THING until the next morning. Ben had checked on Kendall at around 4 and she was sleeping peacefully. Because she gets hooked up to so much in the hospital, we usually try to give her a break when she first comes home if we think all is well. Which we did. Which is why we didn’t have any warning of what was coming the next morning.
  • Saturday morning I was jolted awake by the unmistakable scream of a Kendall in a ton of pain. I ran in there to see her puking/gagging/choking (she can’t really vomit because her stomach is wrapped around her esophagus, a surgery she had when she was 7 months old as a way to try to keep food in her stomach and out of her lungs) – so watching her body fight with itself to try to puke past this knot in her stomach is not for the faint of heart. I turned her on her side and started trying to relieve the pressure by opening up her stomach ports, checking for anything obvious to signal why she was puking, assess what else was going on – and then I felt her head. I instantly knew something was very wrong. Ben was in there by this point and he started helping sort out all the wires and such from around Kendall’s body, hooking her back up to the pulse ox monitor, catching more puke, which was actually nothing more than bloody bile – looking back on it now it seems like the opening scene to an episode of ER. It does amaze me how Ben and I have learned how to do the “Kendall dance” together, both taking a part of her care, both moving around each other to work on the different parts of her little body, handing a flush here, tape there, syringes over there.
  • The picture was that Kendall had a fever of 103.5, probably prompting the puking, was pouring the same bloody bile she was trying to puke up out into her G-tube drainage bag, was rather limp, very tachypnic (breathing about 65 times a minute), was having a hard time maintaining her oxygen saturations above 88 percent, heartrates in the 190’s just laying in her bed – a very bad and sudden crash. I was racing through my mind what it could all mean and trying to formulate a plan  – ANY plan – that didn’t involve packing her back up and driving to milwaukee less than 12 hours after we had pulled in the driveway. Not feeling so super hot myself (thanks to the less than stellar sleep that one gets in the hospital, and not having been able to sneak away for real food at all this trip), I decided to shove her full of tylenol suppositories (because with what was coming out of her g-tube, it was quite obvious that we weren’t going to get a WHOLE lot of absorption through her gut even if I could have figured out a way to push a bunch of tylenol in past all the pressure pushing stuff back out). Because in my mind – if her fever responded to tylenol, it was a virus, and if it didn’t, she was either septic or fungemic again.
  • An hour after all of that chaos, her temp was up to 105.2, and i knew we had to do something else. I had already called milwaukee and spoken with our awesome resident (I know you’re shocked to hear those two words together come out of my mouth in a non-sarcastic way) – we pored back over her lab results from the day before, double checked on all her cultures to see if anything new had grown, and basically ended up shrugging our shoulders and hoping the attending had a better plan. Luckily she did.
  • We were sent to Edwards hospital (the local peds ER), where she had already called ahead, and they were going to re-draw all cultures, check her urine for a UTI, do a chest X-ray, and re-start two IV meds that we had discontinued earlier in the week to get broader coverage over ANYTHING that might be growing.
  • After a couple hours in the ER getting IV fluids bolused into her (run at a fast rate), and the Vanco and Micafungin doses done – she had definitely improved with much less work of breathing (we had had her on 2L of oxygen when we took her in, which is the max we can run at home, and even that wasn’t helping her out a whole lot respiratory wise), her fever finally responded to the horse doses of tylenol and ibuprofen that we were pushing in to her J-tube, and overall we all felt much better about her clinical status. The decision was made with Milwaukee to let us go home, with STRICT orders to come right back if she started being off again at all.
  • A few hours after we got home, Edwards called back and said that since our pharmacy would not be able to get meds to us until much later in the nite (3 am), that we needed to come back to the ER in order to have her next dose of antibiotics run, but that we could just zip in and zip out with that as our only mission. Done and done. Unfortunately, “zippy” and “ER” are just not two words that EVER go together, but all in all, sitting in the ER for another 2.5 hours SURE beat having to stay there overnite, or a couple overnites, or a helicopter ride up to Wisconsin.
  • We are very very very blessed to have  a really good team of docs who “get” Kendall, and who can communicate to a whole new IMG_1836 set of docs who have no idea what to do with Kendall! our Pharmacy company REALLY stepped up to the plate also to make her new meds and get them sent out with a driver from OH to our house ASAP – this also played greatly into the doctors’ decision to let us stay at home. There was really nothing else that they would do for her on the floor at Edwards that we couldn’t do at home, so unless she needed PICU level care, we could comfortably manage her care at home.
  • Unfortunately – all of this led to what we are now dealing with – 8 hour cycles. I have a spreadsheet on the fridge detailing what medicine has to be run at what time on which lumen for how long. Added in to this is her TPN schedule, and the timing of when to take the right med out of the fridge so it warms up in time to be run. Again – not complaining – because I will GLADLY give us sleep and any other coherent thoughts except “red lumen/white lumen/vanco/mica/mero” in order to all be home as a family this week!
  • Overall – Kendall is hanging in there. We have not gotten any word if the new cultures from yesterday are growing anything, but given Kendall’s drastically improved response to SOMETHING we changed yesterday, I’d say that we managed to cover whatever was still growing to make her so sick yesterday morning. My bet is on the yeast again, just cause it seems to be the soup du jour for little miss. BUT – she did grow out a gram positive bug last week, and we had only stopped the vanco right before we left the hospital on friday. So really – it could be either. or both. Leave it to her to have both!
  • She is not currently septic, and hopefully with the right meds on board she will avoid becoming so. If she does go septic (basically another crash like yesterday), we will be heading up to milwaukee as fast as we can, or possibly going to Edwards to stabilize and then be transported depending on her needed level of care.  Her labs looked about as crappy as they can without actually being considered “septic” (it’s a semi-subjective thing, based on how the doc interprets certain clinical impressions plus the lab results) – so I personally don’t have a whole lot of warm fuzzies about her ability to handle another crash like yesterday without some big issues. BUT – I have faith that she is more stable on the treatments we started yesterday, so I’m shoving all of that to the back of my mind and hopefully ignoring it.

And that’s about all I have to say about that right now. She looks very very tired as you would expect of someone who has fought with all they had against a crazy bug (or bugS) all week. She is weak and nauseated in a way that her usual meds are hardly touching (or, as I suspect, her gut just isn’t absorbing them properly right now, or in enough time to get her relief during the worst times). That is probably the hardest thing to see – watching her want to play with sissies, but not being able to climb the stairs after them, or keep up with them running around the house, or want so badly to enjoy the ice cream we are all having, but pushing it away in anger/frustration/pain after just a  small lick. Hoping her docs have some other ideas about ways to relieve that while she has to be on this insane amount of medication. If you’ve ever been on even one antibiotic and had the upset stomach that goes along with that – times that by about 50 and you’ll have an idea of the amount of nausea and pain Kendall is in, with a gut that is already pretty much non-functional from a mechanical standpoint, add in the slowdown that comes when you’re sick, and then the chaos that ensues when you have a bunch of antibiotics coursing through your system – bleck. It makes me nauseous just to think about it!

Now we just wait to see if anything else grows from yesterday’s cultures. IMG_1839 The plan right now is to keep her covered with everything through this coming week, stop everything on Saturday, and hope hope hope that she is fine/better/healthy when all is said and done. I think if we can get the tummy pain under control, she’ll have a much better week and be able to get the real rest she needs in order to get back her strength to let her bone marrow do its job and grow new cells to be able to fight off infections again. Circle of life and all that jazz. She’s in a bad cycle right now, but we are SO grateful that she is doing so well with it. She is an amazing little fighter. I know that if our doctors didn’t have so much faith in our ability to handle all of her issues at home and know the signs of her taking a turn downward, she would NOT be at home right now.

As always – thank you all SO VERY MUCH for all the amazing support you are to our family – the prayers and meals and thoughtful cards, emails, facebook message – they all truly mean so much and I am so sorry that I am not capable of writing a thank you note to every one of you. I want you to all know that we ARE so very thankful, and could not survive without you, our support system.

I’ll try to update a little more regularly this week. There’s more coming up in the next couple weeks that all deserves its own post! Does anyone know where the stop button is for this crazy carousel of life? I’d like to slow it down for just a few days please!!!

Hope you all have a very awesome holiday weekend (in the states at least  – to our international friends – carry on with life as usual – but i hope its good too!!)

love,

IMG_1841

Terra, Ben, and the K-crew

One More Night.

It is hard to believe that last Thursday nite we checked into Hotel Milwaukee, with a whole different kiddo on our hands. I think I knew she was sick, but I don’t think I knew HOW sick she really was or would get. And honestly, I don’t think the gravity of this week will hit me for a while. I kind of hope it doesn’t because what you will encounter if you see me in real life is probably a hot sobbing mess of me.

I guess tonite I should kind of recount a little more of what happened up until last thursday to kind of get us here. Or at least hit the highlight reel.

In essence, we knew from the first time we accessed Kendall’s port that we would need different needles. To access a medi-port, you use a special kind of needle that is called a “Huber needle” (I have no idea why), that is bent at a 90 degree angle, that pokes right down into the port well, and then you secure the whole thing. Ideally, the needle is just about flush to the skin through the port, leaving you enough room to slide a teeny foam skin protector patch right underneath it. Also just as ideally, you do’nt have to leave a port “accessed” (having the needle stuck into it) for more than a couple days at a time. That is the beauty of a port. It’s there when you need it, and very unobtrusive when you don’t. Unless you’re Kendall. Dealing with our HHC (Home Healthy company – the place that delivers all your home medical supplies. For those times when you want your kitchen to resemble a hospital pharmacy.) Now, God love them, they have been great for two years. But with this port, they have been less than helpful. We told them after the first delivery that we would need smaller needles. They do make smaller needles. apparently our HHC needs to get them forged from iron milled in the Maldives or something because they STILL do not have smaller needles. A month later. The reason we need smaller (shorter) needles is because kendall has zero body fat on her chest, and what happens is that the needle sticks up off of her chest a good half inch still. The needles are made of rather thin metal, and when Kendall lays on her belly, it crushes the needle.

what happened last week (if you saw on facebook) is that Kendall’s needle had “fish-hooked”, and made kind of a J, coming back up through her skin. This meant that we had to de-access Kendall and that her chest was sore, and the skin over the port was a little angry from all that trauma. Because it was also pouring TPN out onto her skin that was poked through twice, it just added insult to injury. We got her cleaned up and gave her a bath (something she can’t have while she’s accessed, you don’t want a central line getting water over it cause it could grow bacteria and fun stuff that is always in your water unless you bathe in sterilized water), and then we went to re-access her. It took two more pokes to get a needle to stay in at that point. Kendall then got hooked back up to her TPN, went to therapy, took a nap, and woke up to watch Hot Dog. As I was putting her in her chair, i noticed her lines looked a little funny.

What caught my eye was actually her G-tube bag, I thought I saw blood draining in it. But then I realized her tpn line was clear. It should be milky white. I yelled for her nurse to help me turn off the pump and get kendall laid down so we could rip into the tpn bag and see what was going on. I frantically searched all the way up the line and realized there was NO TPN in the line at all, and that blood was in fact coming down out of her line into the tpn tubing. There was air in the tpn bag (it should deflate/suck in on itself as it empties), and all the pump had been pulling was air, no liquid. If you’ve ever seen a James Bond movie, you know that you can kill someone by injecting air into their bloodstream. Well it usually requires a lot more than they use in the movies, but the amount of air that was SECONDS away from being pumped into Kendall would have killed her instantly. There is an air filter in both the pump and on the tubing, and somehow, neither of them were working. I do not know how or why I was able to see it and stop it. I just know that at 2 pm on Monday February 14th, someone was praying for Kendall. And that someone, or someoneS, saved her life.

I was SO shaken up by this, it felt like I had stopped a car accident with my bare hands. It was brutal and insane and i wanted to rip every single tube out of her body right then and just WILL IT to work ok. And I know that some of you may be thinking – well why didn’t you? And I guess, until you have sat vigil at your child’s bedside, and seen them walk in the valley of the shadow of death, or until you have heard things come out of a doctor’s mouth that no mother should ever have to hear, or until you have seen your child bounce back to health and happiness BECAUSE of those tubes supplying life-giving nutrients – then I don’t think you can understand the love-hate relationship that develops with the tubes. It was something i hope to never see again – that empty clear line, the mental visualization of what it could have meant, any of it – and yet, this is life with Kendall.

Where she is at with the battle against the disease that affects every cell of her body has put us in the position of having to make some choices that are either the rock or the hard place. And while we hope and pray and seek the prayers and hope of all of you that she can rise above where she’s at right now, this is our CURRENT reality. Stopping the tpn would mean we could de-access her port a lot more, and therefore reduce or even mostly eliminate the risk of any further life-threatening complications (like air in the line) or infections (like the bacteremia/fungemia she has fought this past week). But stopping the tpn would also mean that right now, she would be back in a place of near starvation. I had avoided talking a whole lot about the downward spiral Kendall has been on since December simply because it is not a happy realization. It is so much easier and nicer to try to stay on your little cloud of denial. But the fact of the matter is – Kendall’s gut was never really working well after we were discharged on New Year’s Day. And right now, we don’t know why.

By the time we ran the first set of labs at Dr. Natalie’s office, it was clear, according to her that they reflected “ the labs of someone who has not eaten in many many many days”. And yet, we were in fact feeding her. Every day. But it was a losing battle. Getting worse by the day. With a goal rate of 40 oz of her formula per day, we had been making slow downward trends to 35, then only 29, then 20, and by the last few days before starting the tpn, kendalls net intake was less than 4 oz a day. She was puking/dumping/pooping/spewing/leaking/draining everything out faster than we could get it into her. And the faster/harder we tried to pump it in or convince her to drink it (which she really was NOT into doing because by then she was in so much pain), the worse we made the situation. The day we decided to just let her belly drain from both ports and she dumped out almost two days worth of food in a matter of hours, she had THE MOST energy, and the most smiles we have seen in WEEKS. Kealey even said to me “I’m so glad Kendall felt so good today – it was so nice to see her laughing again and trying to play and dance with us!” It nearly broke my heart, because what I saw was a little girl picking up and carrying two VERY heavy drainage bags, with hardly a drop of food left in her, yet happy as could be. A happy little girl we had not seen in a long long time.

So that is how we got here.  To the point where we are staring down the face of a road to recovery from hell and back. There were many points over the past few days where Kendall could have taken a very very very bad turn. Those of our friends who deal with line infections as a part of life know exactly how bad this infection was. I don’t even think that I myself grasp how bad it could have been. I just know that yeast is bad. very very bad. And to look at kendall, even though she is smiling, you can see that she has been in a battle this week. She has bruises and petechiae all up and down her little body. Where the fluid retention has finally started to go down (because her protein levels have just been decimated by the lack of nutrition in the face of all that has been needed), you can see bones through paper thin skin. Her eyes are deeply sunken into her head under some heavy purple bags. Her hair, besides the Bob Marley dreads she has going on again in spite of my best efforts with a bottle of conditioner and a fine tooth comb, is brittle and wiry again (a sign of malabsorption/malnutrition). It is not a place I hope we are ever at again.

I do not know what the future holds for Kendall. I know that God can heal her, and He WILL heal her as he sees fit. We hope and pray that that healing comes this side of heaven. But I also know this – God gave us Kendall just the way she is. He knit her together and He whispered into her ear the plans HE has for her. I look at my blog stats and I think – my baby is doing things that I could never have hoped she could do – she is touching the lives of so many people. She is teaching me, and I have heard from many of you, what it means to be grateful for every day. How to stop and enjoy the simple small victories of life. How to fight when it seems impossible to fight anymore, and how to battle against the odds nearly every day of your life.

And so that is what we do. We tell her to keep on fighting and to keep on proving people wrong and to keep on teaching residents at teaching hospitals to NEVER underestimate what a complex kiddo can and will do. And she does.

Tonight she will probably spike another random fever. I will get shaken awake by our nurse to tell me they are pulling more cultures from Kendall’s lines. They will probably not grow anything, but we have to do it anyways. She may or may not spike so high so fast that she gets the “rigors” – painful shaking that is uncontrollable from either the pain or the temp change (hard to say). She wil eventually calm down. And we will still have no answers. This is Kendall’s new norm, for a few weeks at least. We are going to be packing up tomorrow to head home. I am so glad we are, and I am SO GRATEFUL that it is happening in just over a week. Kendall is truly an amazing little trooper. She will have a long road of recovery. Sitting up for more than a few minutes at a time is still hard for her, and she still cannot stand up. That will take a few more days probably, and then we will have to work with our PT (physical therapist) to see how long it will be before Kendall is steady enough to walk on her own again. Her muscles are very weak right now. She will still for right now be on full time tpn (IV food). They have doubled her calories in an attempt to get some of her lab values up, not to mention her weight that is continuing to drop every day in an effort to keep on fighting back from the bugs that invaded her body last week.

But through it all, I see her true colors shining through. I see that impish grin behind her green “nee-nee”, and I see, past the purply tired eyes, that sparkle that says – watch out – the kid is back. I know that we will get her tummy working again this time because failure is not an option. We refuse to let it be. We know that so many of you – near and far, strangers and family, are praying with us and believing and hoping for Kendall to keep proving doctors wrong. She has definitely made believers out of more than a few of her docs this visit. I do believe that we will have a VERY hard time convincing anyone to ever attempt to place a PIV in her again…but other than that, I think she’s done an amazing job.

So we will go home and try to find a way to fit all of her new medicine and tpn and vitamins into the one sterile drawer in our fridge. We will rearrange a few drawers and shelves so that we have more room for more medical supplies, more pumps, more bags. We will be together again as a family and that will be enough, that will be more than enough. We are surrounded by an amazing group of friends and family, who have already been banding together in their efforts to help us ease into the transition of home living again. Again, I know that there are so many amazing people who are part of this journey with us that I can’t even begin to thank them all.

I have been interrupted countless times trying to get this update finished and posted, so if I trailed off a thought or two, or you don’t quite understand anything I’ve put here, please let me know.  The double shot latte I just drank an hour ago is STILL not combating the extreme amounts of tiredness I feel at this moment. I am so excited at the thought of sleeping in my own bed again, and not having to deal with vitals checks and alarming pumps every few hours…

just one more night.

 

terra.

The Triple Threat.

I am sure this actually happens a lot – but it was something that I really didn’t think was THAT possible….at least not in my child. But then again, this IS Kendall we are talking about.

And by this I mean: She has a bacterial, a viral AND a fungal infection, all at the same time. a true triple threat.

She is positive for influenza (viral), but on top of that, she also has bacteria (some unpronouncable gram-positive bad guy) and yeast (very very stubborn very bad bad guy) hanging out in her blood. At first we thought they were just hanging out in her port. But then they decided to procreate and set up a party in her whole body. she is just all sorts of a hot mess right about now.

Ben has been with her for the better part of the last two days. During those two days by all accounts, she has been doing serious battle against these invaders by burning at an average temp of 103 degrees. Considering that earlier this week her core temp was 95 degrees, you can see how hard her body is working to wage war on the bad guys. She spent the nite packed in cold towels and ice packs (which i hear she just LOVED! 😉 ), and then this afternoon getting double doses of tylenol shoved into, well…you know. the place where you put tylenol when its not being absorbed from above…Four little tylenol bullets, every two hours….brutal.

So tonite she was sent down to the OR (operating room) to have her port removed. Exactly one month after she had it placed. Nobody was very happy about having to take it out. Especially not the surgeon who was pretty livid when he heard that a large part of why we think she is having problems is because of all the idiocy surrounding her too big needles. He IMMEDIATELY started dictating a note to some poor soul essentially telling them that they should either start helping the children they are hired to help, or find a new job…seriously – I have no idea who he is writing a letter to, but I don’t think it’s going to be a happy pat on the back kind of letter. I believe we will also be receiving a pretty hefty supply of ANYTHING we need for better port access prior to going home!

we had an AMAZING team in the OR. Even the residents. Who i did not even want to kick once. (be proud!)  Truly felt like we were leaving our baby in the best hands possible, which is always a comforting feeling when they are sticking a tube down her throat and ripping one out of her chest. For all of the ups and downs and questions of the past few days, these folks made it really worth it. I felt heard and validated and like someone is finally “getting it”, that we don’t get to just bask in Kendall’s cuteness, we have to actually deal with all the medical complexities every.day.of.her.life.  I do feel kind of sorry for the amazing anesthesiologist who was so convinced that the meds she gave to kendall would buy us a few hours of sleep – only to find that kendall was waking up FAR too soon, and with a lot of vengeance too!

I should wrap this up since Ben and I are having to take turns standing at her bedside and calming her back down when she wakes up raging at the pain or the fever. It has been a very long few days, and i have a feeling we are in for quite a few more like it.

To everyone who came out to the Kendall Quinn Medical Fund Benefit last nite (or the KQMFB as our awesome emcee put it!), thank you. I hope you know that even though those words seem so small and inadequate, they hold the enormity of all that that entire nite meant to our family. I want to do its own special post for this awesome nite, so that is all I am going to say about that right now. To everyone who helped put on the KQMFB – thank you is FAR too trite. I would have to borrow a plane and write it in the sky to even begin to come close to capturing how very very thankful I am for each and every one of you absolutely amazing folks.

And to all of you who are reading this post and praying, thank you.

Kendall is in a pretty big fight right now, and it is a horrible feeling to stand by and watch her, and not be able in any way to take some of that pain away from her, to do some of the fighting for her, or better yet, ALL of the fighting for her. It is against every fiber of my mommy being to not be able to DO anything to help her. Except advocate for all the support I can for her. So that is what we do. We push for more tylenol and we tell them to start the oxygen and we ask them for more pain meds and zofran and we run our hands over her burning little forehead and we pray for her. We pray that dear Jesus who can heal a blind man will heal our baby girl of the sicknesses she is fighting tonight. We pray that He would scoop her in His arms, and cradle her close, and give her His rest. We stand and we pray and we beg and we plead because that is all that we can do right now. And we pray knowing that we believe in a God who hears us, and listens and is faithful.

“This is my anthem, this is my song

the theme of the stories I’ve heard for so long.

God has been faithful, He will be again –

His lovingkindness knows no end.

All i have need of – His hand will provide –

He’s always been faithful to me.”

-Sara Groves, He’s Always Been Faithful

 

k4web2 Sleep little baby Kendall. Sleep and restore your energy. Sleep so you can fight, little fighter. Mommy’s got your boxing gloves all ready – we just need you to be strong enough to start fighting. Everybody’s praying for you – cause we all love you. Just rest now. Jesus is with you.

love you~

mommy.

A long night.

It’s actually been just a long week, really.

I figured i should get this all out before life gets too awful crazy and I leave everyone hanging.

In a nutshell – Kendall is sick. She’s either really really sick (if it’s a line infection stemming from her port and all the crazy needle crap she’s been dealing with all week) – or she has this wicked flu virus that has hit everyone else in our house so hard, and is just SICK sick. (it’s a fine line! 😉 )

So after her spiking a fever last nite, we knew we had to come up to milwaukee today (because any fever in a kid with a line means you need to have blood cultures drawn to make sure it doesn’t go systemic). Even though the simplest explanation would be that she has the same flu we’ve all been fighting, it’s not a chance we can take. They were actually going to let us go from the ER this afternoon when our GI decided it would be better to have at least one nite of observation in case anything popped up on all the bloodwork the ER was running.

And here we sit up on the floor, about to start some IV antibiotics “just in case”, and waiting to see if her viral panel grows anything in the flu category so we can get some tamiflu started to try to help her body fight it.

Just like we sat on the floor of a little hospital two years ago on this nite…the nite that started this whole crazy journey. I have learned so much since then, seen so much, experienced so much – of all of the good and the bad that life can sometimes throw at you.

i wish i wasn’t so completely brain dead right now so i could type more. But its been a very very crazy week, and that is really putting it mildly.

Please keep Kendall in your prayers – and I know that so many of you already do so faithfully!

Her little body is working really hard tonite to fight this nasty fever (she is almost ten degrees hotter right now than she was on monday!). This in turn is causing her heartrate to skyrocket, which leads to more pain/less sleep. She has not been able to sleep well in a few nites because of the severe tummy pain she is in right now that we also can’t pinpoint the cause of exactly. She is very tired, and very sick. And very upset at mommy that i can’t just turn “hot-gog” on. (Mickey Mouse Clubhouse, her newest obsession. It unfortunately doesn’t play here 24/7 like it does at home!) Still got that Kendall spunk in her, in spite of being so absolutely wiped out. Right now they are still trying to push all her meds enterally (through her tubes into her intestines). This causes a LOT of pain for her right afterwards, and so much nausea that she gags and retches for about 15-20 minutes after each dose. Hopefully her gut absorbs some of these meds so that they can help control the fever, which will help her sleep better, which will help her body fight whatever it is fighting better. She’s in a tight spot right now – even though I am confident she’ll pull out of it soon, its tough seeing a little fighter go down this hard.

But we thank you all so much for your prayers and thoughts and help and concern and just everything. I do apologize for the incoherency of this post. I am beyond exhausted and trying to fight this crappy bug myself which is sucking every last brain cell i thought I had left.

Hopefully we have good news in the morning!

 

terra

The Saga Continues.

Sometimes I struggle with updating the blog simply because I feel like we are just “that drama” that everyone is tired of hearing of because it just piles up until its at an unbelievable point. This week has definitely been one of those weeks.

So remember how in my last post I talked about Kendall’s love of being inpatient in February? Yeah, she’s been trying REAL hard to end up there. Turns out though that her momma is actually even MORE bullheaded and stubborn than she is, and as much as she is trying to be in the hospital, i am trying even harder to keep her OUT of there. Sometimes my bullheadedness in this regard comes back to bite me in the butt.

Warning: the rest of this post will contain a lot of medicalities that will probably be over the heads of most of you. I do not mean that to be condescending – I am just saying – I know its a lot to take in or try to wrap your mind around. It’s a lot for ME to wrap my mind around. I do’nt blame you if you “don’t get it” or “have no idea what I am talking about”. I’ll try to summarize at the end so you can skip the detailed portion of this quiz!

As much as I had hoped it would be a long long long time before I ever had to write these words, it was a decision that was really out of my hands. Kendall was started on TPN last week, and made NPO, in an effort to give her gut enough rest to kind of “reset” itself and work back up to her baseline of gut function. Translation: Kendall cannot eat or drink anything by mouth, we are bypassing her GI tract completely by using her port to feed “iv food” directly into her bloodstream in an effort to divert energy away from her gut so it can rest and heal by not having to work so hard. And if this sounds to you like the kind of thing that happens every day, and you’re thinking – why didn’t they do this sooner? – let me assure you that this is not something we entered into lightly. The way it came about is a testament to the fact that having a medically complex kid with multiple doctors in multiple states is indeed as much of a nightmare as it sounds like. Thank God for Dr. Natalie who is definitely a no holds barred kind of person, and had no problem ordering whatever was necessary to help get Kendall better NOW, not waiting and seeing any longer, not playing pass the buck, not treating kendall like a file instead of a little girl who was in a lot of pain and distress.

So while I had hoped that it would be a quick “in and out” kind of thing with the TPN – give her a three day break, turn the feeding pump back on, go on with our merry lives, and only have to mention this episode in passing, it is becoming obvious that this may be a tad bit more involved than that. We still hope and pray for a VERY temporary need for this intervention. Our goal is still to have her off of full time tpn within this week-possibly 10 days. We are praying very very hard every day that Jesus would touch Kendall’s system and allow it to heal and rest and restart with no more pain and issues. Those of you who know exactly what TPN involves and means know all that is NOT being said in this post. And those of you who don’t, well, I pray that you never have to.

Besides the emotional issues that come as a mom having to realize that her baby’s organs are not functioning like they should, and that you are having to choose between the rock and the hard place, this has been one of the most physically challenging and stressful weeks I think we have ever had with Kendall. Without getting into too many medical details, there are very high risks involved with TPN infusions. That’s not to say that the benefits don’t sometimes outweigh those risks – but they are there nonetheless. Cliffnotes version of TPN: it is basically milky looking IV fluid that is made up of amino acids (protein), lipids (fat), and dextrose (carbs/sugar), with added electrolytes and vitamins as needed to give a person basically all the nutrition their bodies should be pulling out of their food/diets. While we do dextrose a lot for Kendall (sugar water as I often refer to it), we have never had to add in proteins or lipids. Both of these could cause a reaction in Kendall, the proteins because we have no idea why her body sometimes can’t break whole proteins down, and the lipids because it is very hard to find the right combo of fat that someone needs to grow/thrive, and it is very hard on the liver to process through this form of fat. TPN itself is very hard on the liver, and in a child whose body fights a disease that likes to not have enough energy to always go around, protecting the liver is paramount. It’s kind of a catch 22. She can’t produce energy without any fuel (food), but she can’t get enough fuel in her because she doesn’t have the energy to make her gut work hard enough to do that. Anyways – it’s not a simple thing to just say, here is a bag of tpn, hook it up to that needle hanging off of your child’s chest and call us in the morning.

We have been having to make some major rearrangements in our nursing shift coverage, schedules, etc in order to do lab draws every morning (over the first three days we started the tpn last week). I have lost major amounts of sleep staying up or getting up multiple times in the night in order to check on Kendall that her tpn/iv pump was running fine, that milk was still flowing, that she was still breathing/not third=spacing tons of fluid into her lungs/not breaking out in hives as a reaction to the lipids/not puking from dropping blood sugars too low or spiking them too high with the new formula of dextrose. We have had to watch Kendall essentially be confined to the couch or her bed because the pump and bag of food she requires 24/7 weighs at least half of what she does right now, and there is no way she can push/pull/drag/wear the bag in order to self-ambulate, even if she wasn’t super weak from lacking nutrition for a couple weeks now. My normally high stress levels have hit peak levels over the past week and I am snappy with just about anyone or anything that crosses my path. I feel like a rock has taken up residence in between my shoulder blades and my normally stoic self has been replaced with one of Sybil’s personalities who cries at the drop of a hat.

But through it all – that baby girl with the bright blue eyes is smiling. And why not? she pretty much has all of us wrapped around her fingers and being at her every beck/call/whine/yell! Want sissy’s DS to play with? here you go! Want Mickey Mouse (aka “hawt-gog” – for the hot dog dance at the end of the cartoon) on the TV every waking moment? OK!!! Want to use sissy’s special markers to color in the brand new coloring book? Sure thing! She’s a smart one that K4… And she is a trooper. When we tell her its time for “poke” (getting her port accessed with the needle), she climbs up onto the table (with a lot of help!), and lays right down to get sterile field started. She helps wipe her hub off with alcohol wipes. She shows us which toe or finger she wants us to use for her accuchecks (blood sugar monitor). She has adjusted so well to this new attachment, she is absolutely amazing. I know she will be anxious to start eating again soon, but I think she feels so much relief at not having to deal with all the pain right now from a non-functioning gut. She isn’t having major autonomic spells or bouts of horrible pain like she was just a couple weeks ago. We see more of HER coming through, and it is so very nice.

So the plan for right now is to just hold tight. We will restart j-feeds with her feeding pump really slowly tomorrow with pedialyte, and work our way up from there based on kendall’s symptoms. We want to walk a very fine line between pushing her gut to wake up, and not pushing so hard that we undo all the good we have just done with the gut rest by forcing it to work too hard too fast. But we desperately want her back off of tpn very soon.

There is ever so much more I want to get out about this whole part of the journey, but I don’t want this turning into a small novel.

I’ll wrap this one up with this observation I had the first nite we had Kendall NPO/on iv fluids only. I walked into her room to say goodnite like I do to all my babies every nite before I go to bed. And I had a mini moment of panic. Her food! I’d forgotten to make her food, mix it into the feeding pump bag, hook up her extensions….And then I realized, that for the first time in almost exactly 2 years to the day, I didn’t have to. what a part of our lives kendall’s pumps and bags and attachments have become. It made me tear up a little bit. It was like I felt I wasn’t doing my whole job as her mommy. But I quickly realized I was. I’m just doing it in a different way for a few weeks.

Keep on fightin’ baby. We all love you and are praying for you.

IMG_1174

 

xoxo,

T

Let’s Review.

I was going to go all the way back to Valentine’s Day of 2001 (ok not quite ON that day, but around that time), when we had an earthquake in Seattle that resulted in me breaking my shoulder blade, but I decided to spare you.  We’ll pick up the fun walk down memory lane in 2008.

February of 2008 – I was getting sick because of Miss Kendall, but did not know it yet. And man there is some FUNNY STUFF from back there…that hyperlink (click on the february of 2008 text) takes you to all of my posts from that month. Hilarious. At least in my own mind. As Ben likes to say, the Read family is the funniest people we know. Admitting it is the first step…kqNG

February of 2009 – Right AFTER Valentine’s Day, little missy decided to  get sick in a big way. It’s what started this whole fun fiasco that we are still waiting for her to “grow out of”. Failure to thrive, RSV, bilateral pneumonia, urosepsis, staring death in the face and yelling “you’ll never take my freedom”, PICC lines, feedings pumps – and a partridge in a pear tree. We barely made it home in time for Kealey’s birthday. Kendall got her first tubey – an NG tube that was inserted into her nose and down into her tummy. She also got technically another tubey – the PICC, but that wasn’t for nutrition.

February of 2010 – Second verse same as the first. Kendall decides to stop breathing one nite, and back in she goes. This started a fun merry-go-round of springtime hospital March 2010 064 stays. Again, we get out JUST in time for the Valentine’s Day parties at school (one of which i was in charge of), and go SQUEAKING in to the dance competition with about 30 minutes to spare. (Kendall also apparently likes to go into the hospitals around every dance comp the girls have. Just to, you know, keep things interesting.) Kendall also got her third tubey – we switched her G-tube button into a G-J long tube in order to give her overworked/hardly working tummy a break, and feed her straight into her intestines. 

(and yes I do realize this photo is labelled march – i didn’t have pics of her february tube change like i was looking for – and this shows her GJ tube coming out of her tummy along with a few other random wires from being inpatient at the time.)

February of 2011 – I am not one to breathe life into things, but I gotta tell you, this kid’s in a tight spot right now.  So far, no new tubes (yay! – unless you count the new “tube”/port a couple weeks ago), but her gut is definitely not in love with february at all. i’ll spare you the gory details, but let’s just say that anything we can get into her, is quickly coming right back out. Via many different orifices. Around any kind of plugging mechanism we try to stop it with (and i mean her tubes. I’m not even going to address it if you think i am trying to plug up other holes!) Even with the port for fluid replacing, it doesn’t help us replace calories/electrolytes, and THAT is where she starts to run into problems. I think the hardest part of seeing how bad it got yesterday (with the fluid output that we could measure), was how HAPPY she was, seemingly because she was not in pain, as she has been for a few weeks now. Let’s just say that part of what we were getting out yesterday afternoon was stuff that had been put in the day before. She isn’t seemingly “pseudo-obstructed” as she gets when she has another illness going on that leads to this kind of “gut shutdown” that she deals with on an occasional but temporary basis. she just seems like she would rather not have a lick of food/fluid in her GI tract! Last nite was a little worrisome only because her nurse had advised us to stop all feeds, which is a good thought, except metabolically she can’t handle an overnite fast. So we just ran sugary pedialyte at a very slow rate, and i set my alarm to get up a couple times to do accuchecks (poke her finger and check her blood sugars) to make sure that even at the slow rate she was a.) absorbing some of the sugar and b.) not dipping too low/otherwise reacting. Or c.) choking herself with one of the 6 tubes hanging off of different body parts.

So – that’s where she’s at now. Hinged precariously on a cliff edge of “check-in to Hotel LG” or “Casa de CHW”.  I am trying to not let the past history bring me down, but it’s definitely a tough game mentally to not go there.  I don’t know what else they would really do for her inpatient (except give her more appropriate fluids than what we have at home – D5/D10 vs. the normal saline we have here), and possibly monitor her labs a little bit more. Considering that her GI already wanted us to stop in the ER on Friday when we were there, I just think his leash is not real long as far as letting Kendall just go on like this willy nilly with not tolerating full feeds, or really any feeds to speak of. But – there’s always hope! Maybe she just needs a short break for her tummy (HA! try telling her that!), or maybe she has some strange little virus that is just hitting her this way…

I need to just be done with this post since i started it this morning.

 

I have lots more to say. Mostly about my special amazing beautiful wonderful hug from Jesus called a “Keurig”. But also a few other things. That may or may not hold your interest. Don’t say I didn’t warn you.

Much love to my peeps tonite.

The T.

Long time, no talk.

I am not even sure i remember how to publish a blog it’s been so long…

It has been a rather “challenging” couple weeks since Kendall’s surgery. Nothing that was horrifically bad, well, if you don’t count the whole watching Kendall disintegrate with pain for two straight weeks thing. But other than that, it’s just been kind of…buckle down and keep moving forward mode around here.  I am not even sure where to begin with “catch up” on all that’s been happening, and since most of it is just a lot of medical mumbo jumbo, i’ll probably just try to sum it all up and move on from here! And then you’ll just get a lot of the random terra drivel that keeps you coming back for more.

Sound good?

Ok so – since our last post, it has been a mind-numbing blur of trying to keep up withIMG_1186 a very ticked off Kendall, trying to stay on top of her pain (that is the cause of the pissed-offness), trying to not question our decision to keep her home vs. going in for an eval at the ER, trying to just survive as best as we know how, adjust to this new “normal”.  And yes, I realize that some of this sounds super melodramatic. I am really not trying to make it seem that way, because I get that its not really “THAT” bad.  But her pain has been at epic levels, and it did not appear to be solely from the surgery. In fact, after the first two days, I am pretty sure none of it was from the surgery. She was very bruised and swollen where they had to push pull and tug at her skin to get the port implanted under the skin, and thread the catheter (iv tube) through her jugular vein. But the pain she was having was definitely of a more intense nature. As best as we can piece together after the fact (we being ben and I, her nurses, and our GI in milwaukee), it seems like the GA just did a number on her whole gut. I’ll spare you all the grisly details, but it was not pretty. We went through two bottles each of childrens tylenol and ibuprofen. No kid should need that much pain reliever. But to go to anything stronger would have only led to even slower gut motility than what she had, and that would have been much worse. It was a definite rock and a hard place. And we kept thinking that any minute she would turn the corner; after this nap, or tomorrow morning, she’ll wake up and be herself again.

Today is exactly three weeks past surgery, and I would say that she JUST started turning that corner on Sunday morning. as in two days ago. It has just been very draining. That is the best way I knowIMG_1199 how to put it. Her needs on an emotional level have been so high – not understanding why she felt so bad, and not knowing how to communicate her pain, or how to allow herself to be comforted. I think it was one of the first times that I have ever realized how “complex” her care actually is – and not from a purely medical standpoint. Complex in that nothing is straightforward. She has so many different “parts” to what affects her, that to try to fix one causes another to crop up. You can’t solve this problem because that solution aggravates this other problem. She is a virtual “whack-a-mole” of issues, and while our issues aren’t super critical at the moment, they are still difficult to deal with because one always leads to another. And its just hard to hold it all together some days.

i want so badly to take her pain away. I want it to be me with all the tubes and wires, so that she can run and play like a regular little girl. But you know what – SHE is the amazing one. She is the one who can be pinned down and jabbed in the middle of her chest with a needle, and be crying only because she can’t eat her oreo while we have the “sterile field” set up. She is the one who has learned to pick up all the tubes behind her and stumble her way from room to room, falling here, and running into that wall, or falling off of that chair – and pop right back up with a smile. And i am so sad that that smile has been mostly M.I.A. for the last two weeks.

But I think it’s gonna make a comeback here real soon.

Cause that’s just how my girl rolls. She gets knocked down, but she gets up again (and without the aid of the whiskey drink and the lager drink as per Chumbawumba’s instructions).

editor’s note: if that sentence makes no sense to you whatsoever, it’s totally fine. It’s just a song reference. Carry on.

It has just been a kind of crappy couple of weeks. We lost another mito angel, a sweet little girl named Ellie. I did not know of her until after her death, but she had an adorable smile from the pictures I have seen, and just seemed to exude life. She was 4 years old, and had a very aggressive form of mito known as Alpers Disease. Another little boy is in the fight of his life (for about the 3rd or 4th time in his short 4 years here on Planet Earth). Our little friend Eithene continues to struggle with the effects of a disease ravaged body trying to fight superbug infections. My heart hurts to even pray for these families, and the so many others who are dealing with the pain of living with an incurable disease.

See – I’m a real Polly Sunshine lately. It’s part of why I’ve avoided blogging. IMG_1175 I still believe in our God who is bigger than all of these problems, and who loves us enough to hold us through them. He is faithful, and He is always in charge. I know this unswervingly. I know that as much as it pains me to see Kendall in pain, it pains Him even more.  I don’t know the answers to any of this. But they are not mine to know.

We will all be ok. Because of the amazing generosity of so many of you. The cards, the gift cards, the chick-fil-a coupons, the face lotion, the offers of help, and the help that didn’t wait to be asked…YOU are all an amazing part of this story. I need to still do a proper “thank you” post. I hope you all know how very very very appreciated you are, even if I haven’t been able to scrape the energy together to send real thank you cards.

It’s been a rough few weeks. Having to deal with some of the worst pain I have ever been in in my whole life last week from {unidentified issues that i am trying to pretend aren’t gallbladder related} did not help me get ANY kind of my groove back. I am so grateful for those of you who put up with my whining, my disappearing, my moodiness, my….just me…..thank you. You know who you are.  I am hopefully clawing my way back out of the murkiness of the past couple weeks, although I know there’s still so much processing to do. I was just talking with a friend about the fact that while Kendall herself is not doing any “worse” than she was before the surgery, my view of her has kind of been “expanded”. I have been able to happily deny many of the other issues she has had to deal with in her life. This one, not so much. I am not sure why. I just know it is what it is. And that’s about all I have to say about that…

We are hunkered down for the impending arrival of “Snowmageddon 11” – a storm predicted to dump up to 24 inches of snow on Chicago=land, IMG_1203 and then blow it all around with 45 mph winds. Sounds like a rockin’ time. A time just perfect for Kendall to pull a Kendall, and do some strange thing that will require hospitalization. (and i’m only mostly kidding…) The girls already have a snowday for tomorrow, and Ben is halfway done with his first bowl of queso dip. I just hope we have enough cokes.  And really, I’m kinda excited for the big snow! We hardly ever get good REAL snowstorms around here, and its pretty awesome! I have the camera set up to get a shot every half hour or so of the amount of snow being dumped – I’ll try to have some of those up tonite to show you all!

And I think that concludes this episode of randomness. I need to go get some of the queso dip before it’s all gone.

And if you have any questions about anything having to do with her surgery or anything – please feel free to email me. I feel like I have had so many individual conversations with people over the past couple weeks, that I don’t know who I’ve told what to, and what  I said I would just blog about so everyone could hear it…

terra@2sisphotos.com

I hope that link works.

Ok seriously. the queso is almost gone. I’m going.

peace!

 

t-crest

More tubes, more tests, less answers.

The long awaited GI appointment update at last.

I actually had MOST of this typed up a few days ago, and then my blog editor ate it. Talk about frustration.

Although sometimes that is maybe a good thing, cause it might help me ramble less….

on second thought, no, probably nothing can help that.

Anyways.

To attempt to clarify this appointment – it was not because we had been in the hospital. It just so happened to be scheduled for a few days after we left the hospital. We typically see the GI every 3 months for regular followups and to make sure that everything is hunky-dory with kendall’s gut (which it rarely is, but we have learned to have a relaxed definition of “hunky dory”, so it’s copasetic.) Back in the week before Christmas, when Kendall started doing more weird Kendall stuff, and we started upping our home interventions, and then called him for some more support, he moved her appointment up from the last week of January to the first week, and gave us the new med to “buy us the holidays at least. we hope.” Obviously, it bought us christmas, but not new year’s. Close though.

so – it wasn’t because of anything that did or didn’t happen in the hospital, but it WAS because he knew something was going more wrong than typical. Make sense?

So – at the appointment was K, the nurse who deals with my frantic GI calls, M, the nurse from complex care who helps coordinate all of kendall’s specialists, Dr. N, the GI, and me and kendall.  K the nurse takes all of our info first, relays it to Dr. N, who then usually comes in and says, ok, address this issue with this fix, this one with that, add this, try this, see you in three months. It sounds like a not good system, but it actually really works well for us. I am obviously condensing a lot of talking. So this time he comes in and he is obviously up to speed on the fact that Kendall is getting the port put in, has spent 5 days in the hospital the past week, and that I am fed up with not having any help or answers.

Somehow he gets all worked up over “this output situation”. I don’t even remember how it came up. gj-tubeBut it really was bugging him that Kendall is putting out so much more bile. Now if you have a kid with special needs whose gut is not working optimally, you know the vast discussions that can be had on “bile” vs. “stomach acid”, normal vs. not normal, yellow vs. green, etc. So he wanted to get into a discussion on what was coming out and I opened up kendall’s backpack and showed him, and he did his deep thinking face rubbing thing.

Then he said that right now, our best option is definitely putting the port in, but that we would most likely need to talk about doing a surgical J soon, and that it was definitely time to do the motility testing. Our complex care nurse is nodding along like she totally agrees, Kendall is eating baby soap out of her diaper bag, and i am like “homey come again???” How did we get from “I think she needs a different formula” (which was my big question for this appt) to “she needs more surgery and more painful testing to confirm that things have gotten much worse for her gut”???

i was seriously baffled. Dumbfounded even. I felt like I had missed a major part of the conversation. I mean, I know things haven’t been super-fine in the gut department as of late, but how do we know its not her wonky kidneys or another metabolic process out of whack or heck, even just autonomic dysfunction at play here? Obviously none of these things are great answers. None of them are even really just good answers. But something about his jump to more surgery, and the manometry testing just seemed very defeatist to me. His whole demeanor changed, and I was left wondering how things got this bad this quick.

Now- as to what all of this really MEANS on a practical level –

no, we are not doing another surgery anytime soon. I will need some REALLY strong evidence that a surgical J-tube is going to be the absolute best option for Kendall before Ben and I consent to something like this.  Here is a link that kind of describes the separate J-tube surgery (which I don’t really understand myself very well). And its not that I think the surgery itself is a bad thing. I know it has helped out more than a few of our mito friends whose tummies have needed the help. For instance, our awesome model Samuel here, sporting the newest look in chronic tummy issues – he is showing us his new port (its the little round bump near his left shoulder, just past midline), his G-tube on top and his separate J-tube underneath that. It’s just that, in my mind, right now, its most definitely a step backwards for Kendall. photo It is to admit defeat in a small way, more so than doing the G-J was in the first place.  (aNd just to clarify, this is a picture of how her tube looks NOW – not the separate J) I know that J-feeds have made a WORLD of difference to Kendall and were one of the best decisions we ever made for her. And who knows, i may feel that way about this surgery some day too. but it just seems so young, too early to be having talks like this. It was just all a little too REAL. And it sucked.

In Dr. N’s opinion, we are tabling the J-tube surgery discussion till we get the manometry (motility) testing results back; in my opinion we are tabling it until further notice from me, which might be never. The manometry testing is a controversial thing among kids with gut issues. As Dr. N put it to us “We might catch Kendall on a great day, and get no answers from this test about what her gut is doing. Or, we do get answers from this test, and they are not the kind of answers we want to hear. But at least they will help us know what other directions we can look to get her help.”

so let me get this straight.

you want to put kendall through a rather painful testing procedure in order to either disregard any “good” results we might get, because we assume that they aren’t a true reflection of her gut function, or, we will get devastating news from this painful test that might mean we have to make hard decisions about her future care? Yeah, sign us RIGHT up for that. Cause that sounds like a GREAT way to spend a few days and a couple G’s worth of money. Sounds like Disney on crack right there – every kid’s dream come true!!!

Unfortunately, I think he is going forward with the manometry testing scheduling at least – which takes a few months to get into at our hospital. There are apparently only a handful of places in the US that even do this kind of testing on kids, and luckily, CHW is one of them. i mean, luckily if you are excited about this kind of thing. Which I am not. I was not excited about ANY of that appointment. I am not excited about ANY of the news or issues we have received or are dealing with for Kendall lately.

When Dr. B asked me the morning after we were transferred up there to CHW if I felt like Kendall has gotten better, rollercoastered, or gotten worse in the past 6 months, it really made me stop and think. I wanted to say that she has made great strides over the past six months. i wanted to tell him that we were all done being sick and yank all the tubes out and take back the formula and the pumps and the machines and the wires and give me back my healthy baby now please. I wanted him to be as excited as I was about the fact that she can walk now, and she says a few more words than she did 6 months ago, and that I don’t worry that she will stop breathing any given nite anymore. And I wanted to point out that she has only had 1 pneumonia in the past 6 months, (except then we’d have to forget the scary RSV issues she also just had a few weeks ago). and I wanted him to see that of COURSE I want to say that my baby is getting better every day because in my eyes she is. She’s my baby. I will NEVER give up hope for her. i will NEVER stop rejoicing in the inchstones, or the major milestones, no matter how delayed they are. I will probably NEVER accept that she has a disease that could continue to zap her organs of precious life-giving energy. Because I am her mommy and I just can’t give voice to those words. My head can kind of form the words from a semantic standpoint, but my heart never will.

k4web But I looked up at Dr. B that cold morning with tears in my eyes and I told him that I just wanted her to be fixed. I just want her to be better. And I still want that. And I always will.

Because the bottom line is, right now, her body is doing it’s own weird little thing. I am not going to say things are getting worse because I just don’t have solid proof of that right now. I know that we are dealing with a lot more issues now than we were say, 3-4 months ago, especially with regard to her gut. But  that does not mean “getting worse” necessarily. It just means we need to learn to support her a little better, a little differently than we are doing now. Hopefully this port surgery on the 18th will be a large part of that. Hopefully being able to keep her all hydrated up in a way that her gut isn’t able to do right now will give her tummy the break it needs to start working on its own again. Hopefully we are able to find the things and ways of support that she needs right now.

I hope…

I never stop hoping.

I hold on to hope because I believe in and know the author of all Hope. And I know He is holding my baby girl in His hands, and that He has all the answers that our doctors are so frantically searching for. He knows whether Kendall will need the surgery and He knows what the results of her testing will be and He knows the plans He has made for her.k4web2

So – even though I am writing this a week after the fact, it still doesn’t quite take all the sting out of that appointment. GI appointments are rarely good around here. But that’s ok. Because today Kendall is laughing. yesterday was rough, but today is good. And I hold on to the good days, and try to forget the bad ones.

Because really, that’s all any of us do.

Thanks for checking in on us~

 

terra

A Long December.

But there’s reason to believe that maybe this year will be better than the last.

I can’t remember all the times I tried to tell myself

to hold on to these moments as they pass.

And lest you think I am just a super poet (on top of all my other great skills – ba dum dum)…here is the song where those lyrics come from. It’s the kind of semi-depressing angst you can only appreciate as a college freshman stuck in the frozen tundra of Minne-suck-ta. But, it still kind of fits here. If you are so inclined to listen. It really has nothing to do with the rest of this post, other than, it explains the title. and kind of how I am feeling about this week, this month, this year, kind of just life in general at the moment. It’s just been a long year. but we always have hope.  Things can ALWAYS look up. and I know they will! As soon as we all get some sleep. On a bed that is actually the same height the entire way around, Instead of six random squares that all form a “bed” from the pullout couch in the hospital.

Anyways.

Today was a long day. We anxiously waited for GI to round first thing this morning, and when they did, wow. It was the kind of bomb dropping you always are hoping for when inpatient, and rarely get. Dr. B, the attending on service GI came in and asked a few questions confirming why we were there, what her history was, and what was our biggest frustration/problem we needed resolved. And after we had answered those things, he said, “well, I’m gonna tell you, Kendall and her issues – the mito, the pseudo-obstructions, the dehydration – all of these things fit into a box we have to label ‘things we can’t fix’. We can’t FIX them, but we can support them a whole lot better, and one of the ways we do that is with a permanently placed IV called a port.” At this point my shoulders just fell – in relief, disbelief, shock, i do not know. But Ben was nodding his head and Dr B said, ok obviously not the first time this has been brought up. Ben explained that we have had a port brought up in the past, and that it was exactly the kind of thing we know she needs. We HAVE to find a way to get fluids into her faster in a way her body can absorb and utilize them.

I asked if he felt like this was really what she needed, where she was at, and his response was “ok, how many inpatient stays has she had this year? 8? Yeah, this is what she needs. If you want me to put a PICC in, I’ll do that, but all you’re doing is buying an expensive 3-month long bandaid. This is a chronic and ongoing problem. Now, the only question is, do you want to stay here this weekend and have the port placed first thing Monday morning and we can have you out of here Monday nite? Or do you want to have the surgery consult now, and schedule it outpatient when it works better in your schedules?” After some discussion (once the team had left to go finish rounds), we decided that the other girls needed us home, Kendall was probably ok in the short term, and that it would be better for us to kind of process through the decision on our own turf. you kind of get into a weird freaky twilight zone when you’re inpatient and under lots of stress. It’s not the best scenario for decision making sometimes. In fact, I sometimes stand in front of the vending machine for ten minutes just unable to decide whether i want something chocolatey or salty. You can imagine how paralyzed with indecision I get then when it comes to medical things.

So we sat and waited some more, for surgery to come consult this time. This would eliminate us having to add another outpatient appointment, drive back up here, then schedule surgery, then drive back up here for that. Surgery came by and was very thorough with regard to her ability to maintain an access point for the port, explained the process to us a little bit more, answered the few questions we  had, got kendall’s anesthesia history, and went to call the head surgeon to get “approval”. Head surgeon was apparently in the OR with anesthesia, and when our doctor started to list off kendall’s anesthesia protocol, the anesthesiologist (this is a super annoying word to keep typing by the way) said “uhhh, is she a mito kid? We do ALL the mito kids exactly that way here, we know exactly what you’re talking about!” And THAT, folks, is why its worth it to drive 3 hours away. When Ben and I heard that, we were both just super at ease.

So, we will hear on Monday what day they are scheduling her for.

It is shocking to hear, and yet, not suprising. We know she has been headed for this for a while now. And we also know that this won’t be a “cure-all”. It comes with its own set of risks, along with the benefits. It is the ultimate in “rock meeting hard place”. I will do a post on what “the port” is soon, going into details, etc. about it. If you have questions about it, feel free to ask me – either with a comment here or an email to me :  terra@2sisphotos.com    It is a sobering part of Kendall’s reality right now. In that right now all of her docs (complex care, genetics and GI) up there in milwaukee were of the mindset that this is just “mito progression”.  They aren’t sure why or where exactly (either her GI tract getting worse at doing its job, or possibly kidneys having more trouble regulating themselves), but they were all pretty much in agreement that this is not suprising for a “mito kid”.

So – lots of stuff to be pondering on this New Year’s Eve. LizCarl1210 288

2010 has brought a lot of good stuff, and its share of other news.

I need to wax poetic on this topic a little bit later cause I am so tired now I can hardly prop my eyelids open.

I hope 2011 brings much love and happiness and God’s many blessings upon all of you, friends and family near and far.

 

Terra.

on the 30th day of December.

We checked in to CHW Milwaukee just after midnite. That confuses me on how photo2 to say it. this morning? last nite? whichever. I signed discharge papers on the 29th and signed admit papers on the 30th, and in between we had one heckuva fast ride from Park Ridge to Milwaukee. Kendall GREATLY enjoyed sitting up in that big bed all by herself surrounded by all her pumps and machines and new stuffed puppies and new pretty blankets and all the gushing of the entire transport team from CHW. On the trip up she got to watch Veggie Tales in large screen high def – I had no idea they had such a rockin’ system in ambulances these days! I think it mighta made T-pain jealous of the screens in the dash watchin Saved By The Bell….(nevermind if you don’t get that part – its from a rap song…)

anyways. It was a long two hour process to go back through history with a new team, get her all switched over from the ambulance machines to the ones here, order the right fluids, etc etc etc.

I passed out finally somewhere around 3 pm and didn’t hear a thing till Genetics woke me up for morning rounds at 7.

And that was enlightening. essentially, he told me that he made the call to transfer her here because he was definitely under the impression that the doctor he was talking with (imagine this – it was a resident) was very much incapable of handling Kendall’s complex care needs. i told him he had a good instinct that he should continue to trust because that is exactly what was happening. We spoke for just over 2 hours about many different aspects of Kendall’s care and I don’t even know how to sum it all up, except that he is of the firm belief that Kendall is far more “mitochondrial” than “methylmalonic” – meaning that she presents like and should be treated like the mitochondrial disease is the primary factor, not the methylmalonic acidemia as the primary issue.

To that end, he definitely understands that the end goal here is not just to re-start feeds and send us on our merry way. The goal of this transfer is to actually MANAGE her metabolic care. And we don’t quite know what all that involves – be that assessing why she dehydrates even on feeds, or what functionality her kidneys have that may be affecting this situation, or just doing further testing on her GI tract itself. But we are at least on the same page that the level of care she has been receiving has been rather like a band-aid on a surgical wound, which is why we sought help in Pittsburgh to begin with. This doctor, who is new to the Genetics team here in milwaukee, definitely has a good sense of both the academic approach, as well as understanding that a lot of clinical medical papers that say “x, y or z should happen”, don’t actually help most kids/parents on a day in and day out basis.

He did a thorough job of reviewing different systems with me, discussing a few possible options for help (stool cultures, starting more of the “mito cocktail” to help support her mitochondria better, coordinating more consistent fluid support locally, etc.) – and then continued to check in with me as we switched her primary team over from genetics to GI. Luckily, they are on the same floor here so we didn’t have to physically move rooms, but – the timing of all of this meant that we missed morning rounds with the GI team, so we are still waiting to hear anything new that they might have to throw in to the mix as far as thoughts on how we go about testing what kendall’s hydration/nutrition status actually is.

We also had a lengthy talk with speech path – which is to help us assess kendall’s need to go “NPO” as I talked about in another post. Anne has walked us through more than a few of kendall’s swallowing issues, and was very open and honest with me about the fact that we just can’t really say one way or another whether kendall is safe to continue eating/drinking by mouth. We are left with this balance of  “quality of life” vs. “risk of aspiration”. And kendall’s issues are very closely aligned. Her quality of life (being able to eat/drink orally) has a very high risk of aspiration. But – there is also the risk that she is just refluxing it up from below, up through the fundo. We discussed allowing her to feed while draining, so she is getting the comfort of eating, but not dealing with anything actually being in her stomach for very long. But – bottom line here is that she is being referred to the feeding team for their take on it. hopefully we have her all better by Jan 18th so they can assess her accurately and help us make a better plan of care with this regard.

Our complex care team was also by, where they got a mini-breakdown treat from me when i started KIND OF crying about how i just knew something else is going wrong here, but I don’t know what and I dont know how to fix it and I am afraid that we are really going to do some major damage by continuing to force her to have to compensate in ways that we aren’t even really understanding, all because everyone just wants to focus on how cute she looks and how funny it is that she likes to do “knuckles” (fist bumps), and how great that she wants to try to sit up and play – and meanwhile we aren’t really seeing WHAT is bothering her/going wrong at a cellular level. So then we all get to be shocked when she is having issues a mere 3, 4, 8 weeks later. Oh my gosh! i thought she was gonna be all better now!!! Yes. We all thought that. But she isn’t. so let’s try to focus. I think Complex Care got the picture that I was DONE trying to continue managing her issue without any kind of real info and very little as far as true support when it comes to understanding what is going on with her and how we can assess things better at home. They are going to probably have Dr. Gordon ride roughshod talk with GI to let them know that this is NOT about starting feeds up right now, this is about figuring out the underlying issue that is preventing her from utilizing and absorbing what is IN those feeds.

Photo1 To the degree that this may not be a GI issue inasmuch as it may be her kidneys not doing their job appropriately, complex care is also going to make sure that Nephrology is on standby to come consult as necessary and give us their two cents.  And just to keep life interesting, we may have Endocrinology come join the party too because her sugars are doing some crazy things even while on the sugar water IV. Nothing dangerous yet – but enough that we may need to take a harder look at what part of this situation is causing issues, and what part is just an innocent bystander being dragged in by association.

Even with all of that – we still have hope to be home sometime this weekend. The bottom line is that we just may not get any more solid answers than “mito progression”. Dr. B from genetics was very open with me about that fact. Mito = progression. We don’t know where, or when, or how much. But as those cells die off, they just aren’t able to keep up with the demand to create new ones that are able to do the appropriate job. Trust me – if you don’t understand all of this mito talk – its’ ok. Not many people do. Not even some of the doctors themselves. Certainly not me.

But we are here, she is in great hands, and we are on the right path to possibly, hopefully, maybe getting her some real help and support. We are definitely turning around this acute issue of acidosis. Right now the working theory is that it was just a “mito crash” – she just pretty much ran out of energy because she got so dehydrated. Or she got so dehydrated because she ran out of energy. We’re still trying to herd all the kittens on this one and sort out what’s what.

Thank you from the bottom of my heart to all of you who have reached out or are reaching out to us in this time. i was overwhelmed at the amount of love and support pouring into my inbox from all of you last nite in response to our “need help with” list. I will try to update that later tonite or tomorrow possibly if we have a better idea of whats going on by then.

I don’t even know how to thank you all. And to those of you who say you can “only offer prayers” – trust me, that is enough. To my dear Coke/card fairy from this morning – you filled me with such a sense of awe and strength by your humble spirit in bringing something SO very needed, i can’t even begin to express it. And no, you are not crazy for coming here. I pray that God blesses you and your family for what you did for us today.

To the other angels who have been dropping stuff off at home or in the mail – thank you doesn’t even begin to cover it. If you all don’t stop soon my eyes will be permanently stuck in “ugly cry mode”. And you don’t wanna see that.

If you have been trying to call – my cell phone VM box has been emptied – so keep on trying. I don’t get the best cell coverage in the room here, but when i get to go out for a break I love hearing the messages.

We feel so very loved and supported by all of you tonite. Thank you.

Love –

all of the Atkinsons.

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