Gastroparesis, ketosis, and an abscess...oh my!

It has been a rough few days! We are currently fighting against a return trip (we were already in the ER on Saturday) to the hospital because KayTar still isn't doing well.

It all started Thursday afternoon when KayTar made me stop her feed and she promptly vomited it ALL up while I was rushing to get her venting equipment. We did clears that evening and slow formula on Friday morning. We managed to get her to school for part of the day and part of her party before going to get her already scheduled flu shot (maybe we should have skipped this!). Friday she got about 16 ounces of Elecare plus 8 ounces of Powerade over night for a total of 24 ounces, rather than her normal 32 ounces in a day. Normally a deficit like that isn't a big deal, but when we checked her urine on Saturday, she was already spilling a large amount of ketones. Not good! It has been years since she has decompensated like that and I was quite worried we would end up in the hospital on Christmas if we didn't get her buffed up, so I called the on call neuro and he agreed we should bring her in to get fluids.

This was our first time in the ER with our protocol letter and we were taken back quickly! The rest of it was sloooow once we were back and hooked up, though. KayTar was not thrilled with any of it, except for the Angry Birds toy she got for a reward for the IV and the teddy bear we got at registration. I was a teeny bit frustrated that they forgot to order half of her urgent labs until hours after she had been on D10 and fluids, so they were all normal by then...who knows how they were when we got there! We were discharged around midnight and headed home. It was a long afternoon/night. (And I was forced to watch Santa Paws. Good Lord.)

Sunday morning, I noticed that KayTar was wincing when I gave her meds and plugged in her feed, which is totally weird. I consulted the collective wisdom of my tubie parent friends and Heather suggested that it may be an abscess and to feel around for a lump or ridge. Bingo! There it was...right where it hurt worst. I took a photo at the pediatrician's request and she decided to treat her with oral and topical antibiotics. We started them midday yesterday. I don't know if the brewing abscess caused the earlier vomiting, feed intolerance, and decompensation, but we are hoping that treating it will help. Unfortunately, last night she was spilling ketones again!! And when I tried to vent her, I pulled out a lot of old formula. It is like nothing is moving through. We can run feeds for a time and it is fine until it hits maximum capacity and then we can't get anything more in. Ugh! We hooked her up to her pump last night to try and get 16 ounces of Powerade in over 12 hours, but she unplugged herself in the night (and now there is red Powerade everywhere!!) and I don't know how much she really got. Grrr.

This morning her glucose was low-ish (62) and she was spilling ketones still. We are trying to alternate 4 ounces of Powerade (dye-free!! I learned my lesson!) and 2 ounces of formula at a rate of 60ml/hr (2 ounces an hour for those of you that don't speak this language). At last check, she was still spilling ketones, but her glucose was at 80. She has diarrhea now, though. She is also getting congested. I am at a loss as to what exactly is going on and we are struggling to avoid a bounce-back to the ER. I don't think they will let her go unless she starts tolerating normal feeds if we have to go back. This is the first time we've dealt with both a gastroparesis flare AND metabolic problems and they are both working hard against us. So if you are the praying type, please pray that we can get this under control and avoid another hospital trip. KayTar would be heartbroken to miss Christmas at home!

Surprise! Surgery!

Wednesday night: I noticed that the closure nub (seriously, there has to be a better name for it, but I can't think of one!) on KayTar's g-button was breaking. Except, I noticed it in the middle of the night and by morning, I thought it was a dream!

Thursday: After school, I remembered the dream, so I checked her button to reassure myself...except that didn't go to plan! It was breaking and almost completely broken off by then. Oops. I called the surgery clinic and left a message that her button was breaking and it wasn't urgent yet, but maybe they could get us on the schedule for Monday?? It felt a little too hopeful to me, but I REALLY did not want her to miss another performance. I bandaged her button up, so it wouldn't get tugged on during costume changes and off we went to the musical! I was the backstage parent that night so Josh would watch the play with his family, but evidently it was a PACKED house and the applause went on FOREVER! We could hear it from where we were! So cool. 

A picture a cast mate drew for KayTar backstage. 

Thursday night: When it was time to hook KayTar up for her last feed, I called Josh upstairs to show him what condition the button was in, except when I opened the button the closure nub was GONE. It had broken off and was lodged inside. Ack. Josh used the venting tube to push the nub through, then we fed her, then we hooked an extension to her tube, clamped it off, and secured it to her belly to keep stomach contents from leaking out. Normally it doesn't matter if the nub is broken, the internal anti-reflux valve should keep stomach contents in, but her valve has been blown since she got this button which complicated things a bit. I secured the tubing to her with hypoallergenic paper tape, BIG MISTAKE! I think that was the worst part of this whole ordeal. She broke out in hives in the morning and her skin is still damaged from it. Josh had the brilliant idea to use an ACE bandage instead and it worked like a charm!

With her new surgery buddy...

Friday morning: I called surgery again and left a message that the button was totally broken and we would need a replacement sooner than later and was told the nurse would call me around 8am. I gave KayTar Gatorade and cornstarch to keep her glucose up, but also get her closer to NPO status in case she was going to have surgery. Around 9, nobody had called me, so I called and left a message and marked it as urgent, only because I needed to know if I should keep KayTar NPO or not, it is a dangerous game with a kid who deals with hypoglycemia. Nobody returned my call, so around 10:30, I called again and was in the process of leaving a message when the nurse beeped in. She was SUPER rude at first, "Mrs. 'Tar! That tab breaking is NOT urgent! Unless stomach contents are shooting out of her tube, it is NOT urgent!!" To which I replied, "The internal valve has been been blown since she had this button placed, so YES, stomach contents WOULD be shooting out if I hadn't attached and clamped off an extension tube and secured it to her belly, but that is not the urgent part...the urgent part is that I've only given clears to a child with ketotic hypoglycemia in case she has to be NPO for surgery today." Then she was sweet as pie to me and very helpful. People should not mistake me for an imbecile, honestly. She said to keep KayTar NPO and she would call me back within an hour with more information. She called back and said that KayTar's surgeon couldn't do it, but she had called the on-call surgeon and would call me back as soon as she heard from her.

Friday afternoon: A bit after noon, the nurse called me and said the surgery load was light and we could come down and go to day surgery right away. We signed in, went through all the pre-op paperwork and interviews, talked with Child Life (which triggered a very sad little breakdown from KayTar about the gas mask they use), and all of that jazz. During her history, I mentioned to the NP that KayTar had had a possible bad reaction to LR during her sedated MRI (this is important later) and it caused persistent hypoglycemia/labile blood sugars for 24-48 hours. I guess she did not document this in her chart. (hint: foreshadowing )I spoke with the resident and asked him if they could start an IV for her and give her fluids, because her glucose was sitting at 70 which is the bottom of okay for her. He said that they would give her saline and D5, which is exactly what she needs in that scenario, so I thought that was that. (hint: more foreshadowing) In the holding area, KayTar decided that she would rather have an IV that be put to sleep with the gas, so the nurse anesthetist gave her one, complete with a "rainbow fist of power" as KayTar had requested. I got a call shortly after from the vice principal of the kids' school asking if BubTar could stay for detention to finish a delinquent assignment. AGH! For the record, this is not how I had imagined my Friday. Once they took KayTar back, I went back to the waiting area and updated Facebook and watched The Office via Netflix while I waited. She was in and out quick, as usual.

Friday night: When I went to see her in recovery, one of the first things I noticed was the bag of fluids she was connected to....LR! Agh! I told the nurse that she had had a previous possible adverse reaction to LR and he shut it off right away and called the anesthesiologist. Turns out the NP hadn't made a note of it and the resident who was supposed to be in her surgery and told me that he would start her on D5 and saline was not in her surgery, because she was moved to a different OR. The anesthesiologist was very apologetic and from now on I will tell EVERYONE on her case that she shouldn't have LR, so this won't happen again. Thank goodness it isn't a super serious life threatening thing, but crazy blood sugars are no picnic! Her glucose was 71 when she came out of surgery. I gave her 2 ounces of Pedialyte. Her glucose was 69 at the next check. Then I gave her some Sprite. Her glucose was 62 at the next check. WRONG DIRECTION. I gave her 2 ounces of apple juice and it finally came up to 90. I wanted to wait for one more check before we left, and it was down to 80 just 15 minutes later. 80 is a perfectly fine number, so we decided to book it, even though it wasn't looking incredibly stable. I didn't want to sit there all night because of the what ifs. By the time we got home, about 45 minutes later, her glucose was already 60! Unless something is wrong, her glucose stays well above 70. I was able to get it up to 90 again before she fell asleep with a melted popsicle/formula mixture and then I hooked her up to the pump and set it to give her 45ml every hour through the night, so her glucose wouldn't have time to drop. She tolerated it well and it was a perfect solution! Her glucose was 111 in the morning. 

New button!

Saturday: We kept her on interval feeds for most of the day and she spent most of the day sleeping...until shortly before we went to the high school for the final run of Oliver! but more on that later... :)

So, I was right after all.

Last week I received a call from the genetics/metabolic clinic. The nurse told me that KayTar's recent test for VLCAD was negative and they were dismissing her from clinic. I was irate. I don't care that her test was negative, I mean, what is one more negative diagnostic test on top of the giant teetering stack of negative diagnostic test she already has in her file? I cared that she was being dismissed altogether. "I don't know." is an acceptable thing for a physician to say, but "I quit." is not acceptable, at least as far as I'm concerned. I understand not having any new leads or any idea what the next step might be, but if a kid has a metabolic issue, then metabolic clinic should continue to follow her case at the very least. Follow up once a year, maybe. However, I didn't say any of this, I just said, "Thanks. Goodbye." and choked back a few hot, angry tears.

Yesterday we had a follow up with endocrine which I feared would be a repeat of that last interaction with metabolic clinic. I was pleasantly surprised; the doctor and I actually spoke to one another, she reviewed her chart, labs results, and got updates about how things have gone for the past few months at home. At the end of all that, it turns out, I was RIGHT months ago, when I said it was ketotic hypoglycemia. It is a diagnosis of exclusion and since we've excluded EVERYTHING imaginable, we are officially calling it idiopathic ketotic hypoglycemia. I wish I could have been right without all these miserable tests, especially the inpatient fast, but I know they had to rule all those other conditions out. The diagnosis itself is just a catch-all and idiopathic is fancy medicalese for "who knows why!", but sometimes that is the best modern medical science can offer. It is better than nothing and being right is somewhat satisfying. Wonder if I can put this on my med school applications? ;) Basically, we will continue to do what we are doing; glucose checks, adding cornstarch to her feeds, frequent feeds, monitor her activity levels, test for ketones, overnight feeds when sick, go to the ER when we can't fix things at home, and follow up with endocrinology in 6 months. Hopefully, she will outgrow it at some point!