GI and Eyes!

I'm so terrible at this blogging thing these days. I remember when I used to write almost daily, just because...now even when I have something to say, it takes me forever to find the time to sit down and do it! Of course, I used to have a lot of readers so I felt more of an outside compulsion to keep up. Now it is mostly for me and it turns out that ME is a little lazy at times! ;) Time to play catch-up once again!

On October 18th, KayTar had her GI check-up. She was 49 inches tall and 50 pounds...if I remember correctly. I do know that she is getting taller SO quickly these days. The doctor was very pleased with her growth and the continued success of the Augmentin for her motility and said we can just keep things as-is until her next check-up...then we might consider upping her daily calories. The Augmentin is working really well on a daily basis and has perhaps made the biggest difference in her stooling habits (keeps things loose and we have soooo many fewer accidents now)...but it is not enough when she gets sick or is recovering. It took us nearly an entire month to work back to normal feed rates after her crash at Mito Camp. But yay! She's back to normal feeds now! She's also fighting with a new bug, so we'll see how long it lasts. I think we may eventually need to move on to something else to help her motility, but I'm at least satisfied with the way it has helped her intestinal motility, even if her tummy still gets very sluggish when sick.

She had her ophthalmology check-up on October 30th. I was SO impressed by her in that appointment. For all of these years, when he would ask if she was having any trouble, I had to give my best guess based on my observations, but this time when he asked, she replied, "Well, the numbers on the calendar at school just look like blurry dots from my desk." It was such a little moment, but she seemed so grown up in it! Then they told her they had to dilate her eyes and she lost her mind and had to be restrained while she screamed...so that tempered it a little. ;) She actually said, "Don't do this to me, it is worse than the Cruciatus Curse!" Oh, drama and wizardry! The doctor said he myopia is rapidly progressing and her prescription went from a -3.25 R and -2.50 L to a -4.25 R and -3.75 L...no wonder she couldn't see the numbers! He also recommended bifocals. We got her new glasses in next week and I think they are gigantic and resemble space goggles even though I ordered the smallest bifocal frames, but she loves them and is so excited to be able to SEE clearly!

Almost, but not quite.

 KayTar had a really great week this week. She is back on 8 ounce bolus feeds four times a day and she went all week without extra visits to the nurse for abdominal pain or headaches! Thursday night, I was contemplating writing an email to the pediatrician to tell her how well things had been going...then 20 minutes after I had this thought, KayTar called out that her tummy was hurting. Blurg! I cannot adequately explain how much I want to get her back to baseline. We are getting close, several months and two new meds later, and I'm glad we are getting close...but we just can't get her all the way there.

I called her GI on Friday and spoke to the nurse. Before we just accept that this recurring pain is just part of her new normal, I want to be sure we aren't missing anything we can fix. When she saw the doctor last, there was no pain, just decreased feed tolerance and increased reflux...so I wanted to make sure she had all the information. The nurse spoke to the doctor and called back, but I was out and she spoke to Josh instead. She said to take KayTar off her motility medication for the weekend and start her on probiotics and call to update on Monday. The problem is that KayTar's pain preceded starting the motility meds, and it actually the big reason we went ahead and started the medication, so I already know that isn't the cause of the pain. I'm not going to risk ruining the fact that she is finally on normal feeds again without them having all the information, so I called back, but no one returned my call before end of day Friday. So, I started her on probiotics, but didn't take her off the motility meds...not until I talked to them to clarify the order of events a bit better.

We have had a good weekend. Josh's birthday was Friday and he had the day off, so we spent it together. We spent half of it at the kids' school, eating lunch with the kids and helping out with Jump Rope for Heart, then had dinner with Josh's family in the evening. Yesterday, we went to Turkey Trot at the kids' school, my niece's birthday party, and then Josh and I went out to eat with our best friends for dinner. It was a fun night! We picked up the kids after and shortly after we got home, KayTar grabbed her eye and said she had a headache...and a tummy ache. I gave her Motrin and heated up her rice bag for her tummy. She fell asleep pretty easily spite of it. She said it had been hurting for a while, but she wanted to wait until she got home to tell someone. I reminded her that she needs to speak up as soon as she starts feeling bad, so we can help her feel better sooner.

I hate that she is still having pain for no discernible reason. I hate to think that we may really have lost her baseline for good...which makes the possibility that she has something progressive carry a bit more weight. In truth, I've spent a bit more time thinking about that lately than I'd like. I still have no idea what I'm going to do next semester yet, though registration for classes is open now. But, I'm glad her feeds are back to normal. I'm glad she had a great week at school and only had pain twice. I'm incredibly glad she is still happy, bubbly, bouncy KayTar and she takes these little bumps on stride. That is truly a gift.

Monday morning update: She didn't tolerate her 8 ounce feed this morning. She called down and asked me to turn it off because she was feeling pukey. She only got a little over half of it before feeling yucky. We're trying 8 ounces again for her midday feed at school, so we'll see how it goes. I really need to knock on wood when posting good news of any sort...when will I learn?!

This weekend was sponsored by...

Miralax and Underjams.

KayTar has been having some GI issues again. For a couple of weeks now, she's been going several days between bowel movements (which means her constipation is getting out of control) and we've toyed with her Miralax dose, but haven't been able to work things out easily. She has been waffling between liquidy stool and nothing...so this weekend we upped the Miralax and stayed close to the bathroom! KayTar enjoyed some movies and relaxation time in my bed and we got things moving. I think she is all cleaned out now, the tricky part will be finding a dose that keeps things moving from here on out. She actually had fewer accidents this weekend than she normally does, because things were running like clockwork. I'd send her to the toilet after a feed and she'd take care of business. However, we can't keep things THAT loose for long, because she would be losing too much fluid that way. So hopefully we find the right dose for her!

In other GI news, we increased KayTar's feeds last week and it started out well, but she had some post-feed tummy pain a few times and then she vomited up her entire feed Friday night. She said she was too full. It was the first time she has vomited in a good long while (for her anyway) and the increase in formula volume was the only real change. Saturday morning, we went back down to 6 ounces at a time and she's been okay, mostly. She also threw up last night (a few minutes prior to puking, she was crying because she wanted to sleep in my bed for a third night in a row). I couldn't figure out where her puke bowl was in the dark (it was hiding under the bed) so I had to run her to the bathroom and we didn't quite make it all the way. There was a bit of a trail left behind us. Yuck. She seems okay, though...just part of being KayTar, I guess!

I'm a little disappointed that the transition back to 8 ounce feeds was not a success. While 6 ounces at a time is totally workable and much better than 4 ounce feeds, I just don't like that we've lost ground or that we're looking at a new normal for her. Although KayTar has a history of new issues cropping up on her, this is the first time I feel like we're likely seeing a decline in organ function (well, other than her progressive hearing loss). Her reflux and constipation have gotten worse and her volume tolerance has decreased. It is SLIGHT, so slight, but it is still a step in the wrong direction. Her GI doctor did write a prescription for Augmentin to be used as a prokinetic agent (200mg, 4 times daily) to try to get her back up to normal feeds, but I'm torn about starting that at this point. IF this is progression, then we are likely to see more at some point, and if we pull out the big guns now, there won't be many next steps to take LATER when we might be looking at a more significant decline. We can live with five 6 ounce feeds every day. It is a little more inconvenient, but not too much of a change overall. But if in a year or two years or three years, she starts to tolerate less and less volume, I don't want to be out of options. I'm getting ahead of myself, I recognize that, but these long term concerns are a factor in what decisions we make right now. I think at this point, we're going to keep her at 6 ounce feeds for a couple more weeks and maybe attempt another transition. I'm not sure if time will make any difference, but it is the course of action that seems least risky for her right now.

Weekend Update

The Gas Fiasco
As of 6:30 last night, we have gas service restored! What a trip this has been. Whoa. The new plumbing company we used was wonderful. Friendly service, FAST, and a decent price...all with the added benefit of discussing with a knowledgeable plumber. If you are a local friend and need plumbing work done, call Crown Plumbing! So, the plumbers started work on Wednesday. I came home to find the most beautiful looking trench that I've ever laid eyes on in the backyard, progress! They got the line replaced and the new up-to-code valves installed on Wednesday night, but when they pressured tested the line, gas was still being lost somewhere. We have (well, HAD) two old-school gas wall heaters in the bathroom...Josh didn't mention them to the plumber, because the guy from John Moore told him they were not important for this. Turns out, he was WRONG, they were important AND they were leaking gas. Because the new plumber wasn't prepared for working on these, he  had to get additional parts and come back the next day. The heaters were removed and the lines were capped off (and now we are sporting some wicked-fancy holes in our sheet rock) and he retested the line. It was STILL loosing pressure, so he went in search of more leaks. He found on in the cubby in BubTar's closet that houses the furnace. There was a leak at one of the 90 degree turns in the line, so he fixed that...then he found another...and ANOTHER! Three leaks up there, one in each of the bathrooms, in addition to the line itself detaching. Even though they were probably minuscule leaks without full pressure in the lines, it is still a little unsettling to discover! He fixed everything on Thursday morning. Thursday afternoon, a city inspector came out and said it was in working order and they would release the utilities so the gas company could return our meter and turn our service back on. That evening, a worker from the gas company came out and refuse to install our meter because the trench was not filled in yet. It seemed like a total BS excuse since the neither the plumber nor the city inspector mentioned that was a problem. Alas, what can you do? So he left and we still had no gas. Friday morning, the plumber had the diggers come fill back in the trench and we called the gas company back. I stayed home on Friday until it was time to take KayTar to therapy, at which time Josh came to sit at the house just in case they showed. They did show, but got called out on an emergency and said they couldn't say when they would be back. I just picked the kids up after therapy and brought them home to wait. It was Friday afternoon and we did not want to go another weekend without gas! He showed up at about 6pm and got us fixed right up. He said lines are snapping left and right in our city and he had been called out on 3 just that day! He also said that when people ask him what plumber to use he says, "I can't tell them who they should use, but I tell them who they shouldn't use and that is John Moore! Call John and pay more!" He also said the guy that had come out the night before will do ANYTHING to get out of working and there was no reason he couldn't have reattached the meter with the trench open. Siiiigh. More incompetence. It is all good, though, because we have gas again...after 16 days without it! A hot shower in my own home has never been as luxurious as it was last night! :)

KayTar + Nexium
KayTar started Nexium 2 days ago and it has been encouraging so far! The first day, she ate 2 pieces of bacon, most of her Pringles, an entire hot dog (hold the bun) and asked for a second at lunch, 3 mini-popsicles, and 2 still-frozen nuggets at dinner! This morning she has already had 2 pieces of bacon, a jar of baby food peas, 4 pepperoni, and a mini-popsicle. She told me that she hasn't thrown up in her mouth for 2 days! :) I'm feeling very hopeful about this medication switch!

Insurance Drama
Last Friday, I spent almost the entire day on the phone.The insurance issue with the new clinic and her current health plan has reached an impasse and we are going to have to switch. I first called the state to inquire about how I would go about changing health plans, since we never have, and I was assured that it was a simple phone call and we would be squared away. But before I made the switch, I called EVERYONE to be sure they all take the prospective new plan. I talked to probably 25 customer service reps...every clinic, doctor, pharmacy, HHC, therapist that KayTar receives services from was on my list. They all accept this health plan, so I called the state back. This time I was told we were no longer able to switch, because we had been on our current plan more than 90 days. We could appeal this decision in writing, but even if approved it wouldn't go into effect until after the first of October (at the earliest). Siiiiigh. I told her what the previous rep had told me and she said that was not the case. So I said okay, and then called back to try to reach the original rep. Except, you can't actually do that. They give you rep ID numbers, but they are useless because they do not have extension or someway to connect to do a specific rep. So I used this third rep as a tie breaker of sorts (or a tie creator? seems like the phrasing here is wrong, but you get the idea), and she said the same thing as the second rep, so I submitted a letter in writing...now we wait! As a little bonus to this story, I received a letter in the mail yesterday saying that the kids might lose insurance coverage at the end of October. I promptly had a series of small heart attacks while dialing the state HHSC to find out what was going on...only to be told that the letter was sent in error and we would be receiving a corrected letter in about 2 days. WHEW!

KayTar + head pains
Poor KayTar is having a lot of headaches/pains lately. She had at least 2 last week and one every day this week. We were talking about it a couple of days ago and she said, "I have head pain EVERY day." but she said it with a DUH tone of voice, like this was obviously something I should already know. They are not incapacitating and often she just wants an ice pack, but it still doesn't sit right with me. She complains of pain over her right eye on her forehead and in her eyes themselves. She has an ophtho check on the 27th, so if it is a glasses issue, we should know then. Otherwise, I'm not sure what is going on! Maybe sinus pain from allergies? We have her on Benadryl and Claritin, and her allergies seem well-controlled. Maybe headaches from transient desats? I honestly don't know. We see pulmonology on October 11th (since we had to push it back due to the insurance drama) and we see the new geneticist that day, too. Hopefully one of her doctors will have a helpful suggestion!

KayTar + OT
Yesterday was KayTar's first OT appointment in over a month! She was doing feeding therapy and with the gut changes and decreased feeding tolerance, we just took a break while we went through testing for that. Because it still isn't under control, we are taking a break from feeding OT and switching to more traditional OT for her fine motor strength and coordination. We have known for sometime that she has weaker hands than her peers, but we just have not focused on it until now. The therapist did testing with KayTar yesterday and WHOA, her hands are weak! She was tested with a hand gripper equipped with some sort of pressure guage, and she scored a 6 with one hand and a 7 with the other...normal for her age is 28-29! That is significant weakness. That combined with the inefficient way she grips her pencil and the EXTREME force she uses to push the pencil to the paper, it is no wonder she fatigues so easy during writing assignments. Until we are able to get her back to "regular" feeds, we will be doing all fine motor work in OT, and if we get her gut issues under control, we will add in some texture/feeding work, too...just not as much as before. Oh, and the OT really noted her new breathing pattern, too, and commented it was not nearly that bad the last time she had seen her. She wants the pulmonologist to make sure things are okay with her airway before we start back with the feeding stuff, too.

The GI Plan.

KayTar had her follow-up appointment with her gastroenterologist yesterday. Dr. R is one of my favorite doctors and not JUST because she has let me shadow her service before! She is always interested in KayTar as a whole child, not just a GI tract. She talks with both of us and even allows KayTar to have a bit of stage time to tell jokes/do impressions or whatever she happens to think up. I have never felt rushed in one of these visits, which was especially appreciated this time as we had a bit more to discuss than usual, since KayTar's GI system has been the biggest troublemaker lately.

First, we discussed the gastric emptying scan. She confirmed it was abnormal and that KayTar is having motility issues. I asked if the half-emptying time is volume-dependent (meaning that it always takes her stomach 73 minutes to just move 1.5 ounces out) or not (meaning that 4 ounces would move through in the same time frame if it was half of the stomach's contents), and she said in MOST people, it is not volume-dependent and the stomach should half-empty in the same time threshold whether there was 3 or 8 ounces in there to start. However, she qualified that with a "BUT, this is KayTar, soooo I can't say if that is the case here or not. Her motility may worsen with increased volume." Basically, the scan was enough to confirm the clinical symptoms we were observing in her sudden volume intolerance and increased reflux, but it can't give us more precise information.

Then, she gave us the new plan. She is switching KayTar's PPI from Prevacid to Nexium, which we will start tonight. Then, in two weeks, she wants us to try to transition KayTar back to her normal formula volume. If it works, great! If not, then we will start giving KayTar a dose of Augmentin prior to every feed as a prokinetic to help induce more normal motility in her gut. If we are still unable to get her back to regular feeds, we will just continue with this smaller/more frequent feeding schedule, and follow up in January. She said she likely will not order any further testing until summer (at the earliest) because she doesn't want to disrupt KayTar's school schedule and the next tests are rather unpleasant. She said that if her overall intake and growth continue to be good, we may put it off until it is really necessary to put her through. Hopefully one or both of the new medications will help get her back on track, but if not, I'm fine with the plan she has laid out for KayTar.

Finally, we discussed what might have triggered this change. She said it may be a post-viral phenomenon from her relatively minor viral infection in June...but probably not. She said it may just be "a KayTar thing", which is the category most things fall into. She also said it could be disease progression (what disease? we don't know!), but that said she'd like to ignore that last one and that I probably would, too. She is definitely right about that. Losing ground is one of the hardest parts of any disorder/disease, I'm sure, but it is especially hard when you don't even know what disease is causing it to happen and if it might be preventable if you DID know what you were up against. So, for now, we are just going to keep on trucking and not worry too much about that aspect of it. We'll try the new medications and then go from there, depending on what the results are. The new feeding schedule isn't the worst thing to ever happen, in fact, she hasn't vomited once at school this year (knock on wood), but it is more disruptive for her and for the people caring for her, whether at school or at home....so if we can get her back to "normal" (or maybe even up to 6 ounces every 3 hours), I think everyone would be pleased!

Completely unrelated photo ;)

Gastric Emptying Scan

KayTar had her gastric emptying scan today, or as she describes it, "I was taped to a table and shoved in a machine FOR HOURS!"

She was NOT a fan of being restrained. She let out a few peals of her patented, eardrum-rupturing KayTar screams as they taped her down, but she calmed pretty quickly with some help from Spongebob on the monitor. She couldn't use her hands, though, so I had to do things like rub her eyes or scratch her nose or wipe her tears. :(  We rigged up her Gee so she could rub her lips on it as needed for comfort. Once she resigned herself to her fate, which happened fairly quick, it was an easy enough test.

  

We didn't get any results at the appointment, but our fabulous pediatrician called me with them this afternoon. A normal result is half-emptying in 60 minutes, KayTar half-emptied in 73 minutes, which is only slightly longer than normal. However, she was only given 3 ounces...so it took 73 minutes to empty out 1.5 ounces, which seems like a long time to move such a little amount. We've never done one of these before and the pediatrician doesn't have much experience interpreting the results, so she can't say if the rate of emptying is volume specific or stable across the board...so I don't know what to make of it until we see her GI on the 13th. These tests are the sort you take with a grain of salt anyway, so we'll see what her thoughts are before changing things up. For the time being, her 4 ounce feeds every 2 hours seem to be well-tolerated so we're sticking with it. Oh, and they saw her refluxing again in this scan, too. We may need to add in another medication to help control that. I'm on pins and needles for her appointments the next few weeks; we follow up with her mito doc next Tuesday, have our first visit with pulmonary the following Monday, and follow up with GI the day after that. Here's hoping we learn something new and useful from all of this testing!

G-button Check, Check!

KayTar had her g-button check this morning. It was an easy and interesting test, the best kind! I wasn't exactly sure what to expect, since this was our first go at this and there isn't much information about there about g-button checks under fluoroscopy on Dr. Google. Here's how it went. First, KayTar gowned up and laid on the table and they took a plain old x-ray. Then, the radiologist came in and they pushed in barium via her g-button and we watched it on screen. KayTar thought it was so cool to see her spine at first and then her stomach and a bit of her intestines. We even saw her esophagus as she refluxed the barium. I found that part impressive! Her own little barium geyser. She has been diagnosed with and treated for reflux for a while, but it has definitely gotten worse lately...so it was good to get internal conformation of what we are externally observing in her. She recently went from being on Prevacid once daily, sometimes not even that was needed, to complaining often of throwing up in her mouth and tasting her per g-button meds frequently, even after upping her Prevacid to twice daily.

The other interesting tidbit was that the radiologist pushed in 3 ounces of barium in and he told me that was about all her stomach could hold and he wouldn't recommend giving her much more than that at a time in a feed. I told him what we were feeding her previously, 8 ounces over 30 minutes, and he looked a little skeptical and said that wouldn't be tolerable for her. She has never had a study like this before, so I have nothing to compare it to...but we do know she WAS tolerating that feeding regiment for a long time. For now, we're continuing the small feeds and (mostly) no meat, because it seems to be working thus far. It was sad at dinner tonight, though, because we had sausage and she wanted it SO bad. She licked the spatula like a lollipop and then did the same to a little piece of sausage. I finally caved and let her eat one little piece of it (one small round sliced pieced from a smoked chicken sausage). She was just dying for it and I'm a softy!

The next step is the gastric emptying scan to hopefully find out why she is only tolerating a small volume, probably next week if the pediatrician can get us a slot. Her MRI is next week, too. It will be our last week of summer and I think the clock might just run out on us before we get this latest puzzle figured out and put together!

KayTar took this shot of her guts during the test and told me she was going to tell 

BubTar that the black (barium) was blood to "freak him out".

 

She took this shot of me in the little x-ray machine mirror.

 She asked me to take this one of her from the point of view of the x-ray.

PS: Her basic metabolic labs are back and are perfect! Glad to hear her nutritonal status is on track so we can rule that out. Looks like we are looking at mechanical issues.


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It's a Start.

KayTar's gut still isn't behaving itself. She didn't tolerate her full feed volumes the night before last, yesterday morning, or last night. She only got about 50% of what she needed yesterday. It is happening more and more often, so something has to change.

First, I made her a pediatrician's appointment for Wednesday to be sure there isn't anything fixable happening that we're missing...and I figure we'll discuss the breathing and fatigue issues while we are there, too.

Second, I changed her feeding schedule today to half-sized feeds every 2 hours, instead of full-sized feeds every 4 hours. So far, so good, with the exception of her "belly burning" for a while midday. It is a pain, though. Hooking her up every two hours is a lot more disruptive than every 4 hours, and it takes longer overall. She got feeds at 9am, 11am, 1pm, 3pm, 5:30pm, 7:30pm, and she will get one at 9:30pm, and 11:30pm to get it all in. Of course, she'll be asleep for the last two.

Third, we're not letting her eat any meat. This one is a bit of a long shot and going to be the hardest to implement if we have to continue it. KayTar is *almost* a total carnivore when it comes to what she is willing to eat by mouth. She doesn't eat A LOT, but a huge percentage of what she does eat is fatty meat. If her gut is indeed having trouble processing things, then meat, especially meat high in fat like bacon, is probably just compounding the problem, as it is tougher to break down than a lot of other foods. If she is chowing down on meat and it isn't breaking down and moving through in a timely manner, it may be gumming up the works for other things (like her Pediasure), causing an upset stomach and vomiting. I haaaate telling her she can't eat something when she wants to, but we have to figure this out, so for the next few days, meat is a no-no. If everything goes well with the half-sized feeding schedule, then we will add meat back in and see if things go awry again. The pediatrician may have additional suggestions, so we'll see what she thinks and go from there. Hopefully this little belly starts behaving itself!

Brain Dump.

Last night I had insomnia. Actually, I've been having it a lot this summer. Not sure if it because I'm used to a much more busy and exhausting schedule during the year or if it is because I have some worries bouncing around in my head. Several years ago, my boss told me that she kept a notebook by her bed and when she couldn't sleep because she was thinking too much about something, she would write it down and then she could sleep. I tried it and it worked, but I got out of the habit of keeping a notebook by my bed since then. Last night I promised myself I would write this post in the morning as a mental compromise of sorts. Then I turned over, because when I was a kid who had bad dreams, my dad told me that there was a bookshelf in my head and if I didn't like the book that was open, I could turn over and the books would shift and a new book would open. Since then, I always turn over if I have a bad dream or need to think about something else. It usually works. Anyway, on to the list!

1. KayTar's breathing. Yup, she is still breathing strangely. Yup, it is still bothering me. We're having the same issues with getting in to see the pulmonologist as we did trying to see her mito doc. Insurance says she is in network, the facility says she can't be. Blurg. The other thing that is bothering me is that we won't see the pulmonologist until after school has started. I won't how we need to handle this while she is at school, if she needs accommodations in PE, ect. It might not be a significant issue at all, but we don't really know that yet and I don't like not having a clear idea of how to proceed.

2. KayTar's gut. We just upped her Prevacid because she was almost daily complaining of "throwing up in her mouth a little" or tasting her per g-tube meds. It seems like the increase is helping, as she's only complained of the throw up thing once since then. However, she had another off day with her tummy on Wednesday, not tolerating her normal feeds, vomiting for no apparent reason. I don't know why this is happening. Usually in the past, she has trouble with feeds when she is sick or very congested from allergies, but neither of those are the case right now. Then on Wednesday night, she woke up from sleep, crying, wrenching, and shaking a lot. Her glucose was fine, but the nausea was persistent and she wanted Zofran. No idea what that was about either. She is still having fecal incontinence issues, too.

3. KayTar's nutrition/formula. The gut issues led me to recalculate KayTar's nutrition requirements, because it has been a while since we evaluated that and I wanted to see how far from the mark she is on her bad days. Unfortunately, I discovered that she isn't getting her caloric DRI on days when she gets her full feeds. Thus far, it doesn't seem to be affecting her growth, but it doesn't give her much give for these low days or for when school and the Parade of Pathogens starts up again. I discussed it with the pediatrician via email yesterday and we decided to try Pediasure 1.5 Cal for her. She can get her DRI in less volume than she is currently getting, and on bad days, she won't be as far from the mark. Perfect, right? Then I did a little digging and found that it is generally less tolerated and kids often drink even less when on it. If her gut is already having some issues with tolerating her normal formula, I'm a little worried the switch might make things worse. Or it could be perfect. I don't know. I guess we will find out, though. The other underlying concern is that KayTar's neurological episodes are currently a thing of the past (we just passed the 2 year mark in July! YAY!), but the last time we switched formulas, from Pediasure to the generic kind, they reemerged. Anytime we mess with her formula, that always comes to mind. We hope that staying within the Pediasure brand will prevent that, but there is really no way to know. The truth is, the formula switch and the reemergence of her episodes might have been coincidental...but there is no real way to know. It is something that we think about, though.

4. KayTar's energy. We have had a great summer; two little vacations, swimming many days, some time with friends, and so on. However, KayTar has spent most of the summer in my bed. Literally. And on vacation, she spent most of the time in her vacation bed or mine. Not all of it, but a lot. It started at the end of last school year, she would come home, climb in my bed, do her homework, and rest until bedtime. This summer, she has spent progressively more time resting. If we go swimming or to the movies, she is fine, but gets back in bed when we get home usually. If we do something with walking, like the mall, she becomes fatigued pretty quickly. Most days she crawls into my bed when Josh leaves for work and is still there when he gets home in the evening. It is fine right now, we are all home and can accommodate her, but in a couple of weeks, school is going to start and I don't know how she is going to handle the transition and increased demands.

5. School. As I mentioned before, school generally means the Parade of Pathogens for KayTar. She's had a VERY healthy summer (knock on wood) with only ONE febrile illness, but school means exposure to a lot of adorable germ-infested small people. I'm worried about how increased infections and increased energy demands are going to affect all of these other issues. We are not going to have her breathing issues or her gut and nutrition issues worked out by then. I do hope it goes smoothly, I mean, she did well enough last year in Kindergarten...but it seems like a lot of these issues just started cropping up at the tail end of the year and have progressed a bit over the summer, so I'm still a little nervous about how she will handle it this year.

So that's that...hopefully with all of this written out, I can get a good night's sleep tonight! 

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Enema of The State

That is both my favorite Blink182 album and the phrase that has been stuck in my head since we purchased a children's Fleet enema for KayTar's manometry testing prep. I was dreading the enema, as the last time KayTar got constipated, we had to use a liquid glycerin suppository and she haaaated it. It actually made her vomit. Needless to say, I wasn't stoked about having to go one step above that to prep for her test. It went really well, though, she didn't mind at all! She also had to be NPO (except for clears) as of 3am, so I set an alarm for 2:30 so I could give her a feed with cornstarch at the last minute and another for 3am so I would go turn it off. She had a sip of water around 6 when she was technically supposed to be 100% NPO, but she had a scratchy throat, so I made a judgment call.

The test itself went well. At one point KayTar said, "THIS is what I was nervous about?!" It appears that her muscles and nerves are communicating appropriately, but we discovered that she didn't know how to squeeze her sphincter shut. She was pushing/bearing down whether they asked her to push or squeeze, no matter how they tried to explain it. They taught her how to do it in about 5 minutes and verified that she was doing it properly with the sensors and she said, "Wow! I've never done that before!" so I'm thinking that might be a big part of her fecal incontinence issues. Knowing how to hold it in is an important skill if you ever want to make it to the toilet! She was so pleased and she practiced her new skill all the way home!

After the testing, we went to the kids' school library for summer library time! The kids had a great time, checking out books, playing in the sand table, and listening to stories. Towards the end of our time there, KayTar wanted to be held, so I picked her up and she laid her head on my shoulder. I thought she was just tired from our early morning, but when we got home she felt warm (even warmer than a car trip in the Texas summer should make someone), so I took her temp. 102. Dang. She laid down in my bed to read while she got her tube feed, but she was out in a few minutes. It is the second day of summer and she's already sick! Oh well, at least we don't have to worry about absences! The pool will still be there when she's better.

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Appointment Round-Up!

We had a triple header yesterday! It has been a while since we've had multiple appointments on one day, but that's just how it worked this time. KayTar needed to see the ENT and have audiology testing done before her ARD meeting next week, but we got a surprise confirmation call about a GI appointment on the same day...it worked time-wise so we kept them all. It was a surprise because those pesky burglars stole my iPod which had KayTar's appointment schedule for the next YEAR on it, so I'm sure we'll be getting several more of those surprise appointment confirmations as the year goes on. I use Google calendar now, synced with my iPad and iPhone, in case the devices are stolen again, I won't lose all that information.

GI:
KayTar is still having fecal incontinence, which is a nice way of saying she almost always has stool leakage prior to going to the bathroom, which is our sign to put her on the toilet and ask her to go. We put her on the toilet at the same time every day to try to avoid that leakage, but there is a limit to how well you can control someone else's bowels...know what I mean? We give Miralax, we put her on the toilet at the same time to avoid accidents, but it isn't 100% effective, because her colon isn't 100% predictable. She is in undies, but we have to use pantyliners so we aren't changing/washing 3,000 pairs of underwear per day. Anyway, the GI thinks it is time to do manometry testing (link is not to our hospital, but the information is good) this summer, to find out if this is behavioral or if she truly doesn't have nerve signaling from that particular area. KayTar overheard me explaining the procedure to Josh on the phone and said, "What?! They want to put a balloon in my butt?! Am I going to float up to the ceiling?!" We're trying to decide if she is mature enough to handle it this summer or if we should wait until next year. I asked the GI if this continued fecal incontinence is common in kids like KayTar and she said, "Well, there aren't really any kids like KayTar to go by!" Isn't that the truth! KayTar was ON for that appointment. She really likes to perform for our GI doctor and we're lucky to get the chance to discuss any sort of business, honestly. Somehow, we found time to talk about the #2 situation in-depth and then touch base on her frequent vomiting, bizarre diet, elevated LFTs and reflux. We're not changing anything at this point, just updating information. She said the elevated LFTs might be from something mild, like a virus, or part of her overall presentation, but we don't want to do a liver biopsy just yet. We're seeing a new doctor in June who will likely re-order all sorts of labs, so if they are still elevated then, we might need to deal with it. I really like her GI doctor because she is always interested in KayTar as a WHOLE person, not just the GI-tract, so we also go over any changes to the whole picture since her last appointment. She is one of the few doctors who does this and I appreciate it.

After GI, we tried to meet up with Heather and her kids who are in town for medical testing/appointments, but it was lunchtime rush hour in the hospital so by the time we got food (There were like 6-7 doctors in front of us at Subway and KayTar said, "What is this, the doctor store?" LOL.), found an elevator with room for me and KayTar's stroller, and made it to the parking garage, we didn't have enough time left before they had to be off to their next appointment. KayTar and I had a quick lunch and went to the bookstore to kill some time before the next appointment. KayTar read part of a Flat Stanley book, Knuffle Bunny Free, Quackenstein, and The Stinky Cheese Man while I listened/borrowed Borders' Wi-Fi to check my email. We grabbed a coffee (for me) and a milk (for her) and headed off to the next stop.

Audiology:
KayTar HATES the sound booth testing. The white noise is too loud, the whistling is too whistley, the booth is too cramped. She did fine, though. Left ear is non-functional, right ear still looks perfect. Hooray!

ENT:
Is it just me or are ENT appointments totally useless? He basically looked at her and said, "Yup, those are ears, and that is a nose, and that is a throat." Oh well, we had to do it so we can get her otological eval for her ARD.

In the waiting room, KayTar made friends (doesn't she always?) with a mom and baby. The mom was very nice and chatted with KayTar with great interest and once KayTar hooks someone like that, I can't reel her back in...so I just went and sat on the floor near them. The baby was ADORABLE. Oh my gosh. He seemed to really like KayTar, too, which she loved. She's after me to give her a little sibling, but that's not happening! At one point, KayTar said, "Look (baby's name), you have ten fingers! One, two, three, four, five, six, seven, eight, nine, ten!" But the mom said, "Oh, look! How many fingers does he have?" Turns out he had polydatctyly and actually had TWELVE adorable, pudgy baby fingers. KayTar thought this was awesome and said, "Ohhhh, he's a SPECIAL baby...I just have ten." Sometimes I have to worry about saying inappropriate things, like "Why is that lady so wrinkly, did she stay in the bath too long?" or "That man is so FAT!" but I really never have to worry about how she'll react to differences like these...she's sees the best in all of it. Twelve fingers just means Kindergarten math will be easier for him. ;) Later she kept asking me, "Are you SURE I didn't used to have twelve fingers?"

After all our appointments were done, we headed over to the Ronald McDonald House (which KayTar thinks is AWESOME and is a little bummed we live too close to stay there when we need to see the doctors) to see Heather and her family.We had a great time and KayTar is just OVER THE MOON about her new teenage friends (seriously, the whole ride home was "My teenage friends..." this and "My teenage friends..." that.) and was tickled beyond measure that they all have g-buttons like her. We actually met Heather through our pediatrician, who evidently networks in the ladies' room for us, LOL! Dr. H was at the RM House with her mom a month or two ago and met Heather...her kids reminded our doctor of KayTar, so she got her contact information and her doctor's info, and I'm sure glad she did. For me, it is an exceedingly rare thing to talk to another parent who understands even half of what I'm talking about in reference to KayTar, so meeting someone who understands all of it, is miraculous. Her kids have mito and we'll be seeing their mito specialist in June. I'm really looking forward to having a fresh pair of eyes look over KayTar's case! Anyway, Heather and her family are so sweet and I'm very glad we had the chance to spend some time together...so is KayTar, who was doodling Abby's name this morning before school. Haha! She's already making plans for when they come back in June.

By Kindergarten...

KayTar isn't eating. It doesn't really matter since we have the ability to give her enteral feedings and she gets all of her necessary nutrition that way, but we still do notice and it--ahem--eats at us.

She has never eaten many carbohydrates, but that list is quickly shrinking. She eats no fruits, no pastas, no cereals, no cookies, no breads...well, on rare ocassions she will eat a couple of bites of garlic bread. Up until recently, she would eat chips, soda, icing, french fries, ice cream, croutons (with all of the brown removed)...not exactly HEALTHY, but still food that she was willingly eating. Most of those are out now. Here's the BIG one, she won't drink her Pediasure by mouth anymore. She is frequently complaining that foods are too sweet.

She favors proteins almost exclusively, most of the foods she will eat are greasy meats...bacon, breakfast sausage (that has been cooked and put back in the freezer to cool), chicken nuggets (straight from the freezer), tuna with mayo, pepperoni. She'll also eat powdered parmesan cheese and stage 2 baby food peas and corn and sweet potatoes. That's about it. But in the last week or two, she isn't eating any of that on a regular basis. Today, she ate a snack size bag of chips. Yesterday, she ate a few bites off of croutons at Olive Garden along with a bit of parmesan cheese. The day before that, she ate 1 frozen chicken nugget. The only thing she is taking with consistency is her glass of Miralax water each day.

I'm not sure WHY she isn't eating many of her "safe" foods anymore or why she is complaining about the sweetness of things. I don't know why she won't drink Pediasure anymore. It seems like she's having a bit of a flare up of her GI symptoms right now...her constipation is suddenly causing intermittent problems and her feeding issues have greatly intensified. She's been surprisingly healthy recently, no fevers or URIs (knock on wood), no issues with severe ketosis or hypoglycemia. There doesn't seem to be a cause for this little flare up, but there it is nonetheless.

When we started all this, years ago, at our first GI/feeding disorders specialist visit the doctor told us, "The goal will be to have her eating normally by Kindergarten." At the time, it seemed reasonable...actually, it seemed a little ridiculous. "OF COURSE, she'd be eating normally by Kindergarten, that is years from now!", we thought smugly. Except that Kinder starts in 3 weeks and she's about 98% g-tube fed, and her food list is shrinking, not growing.

Josh and I were discussing this in the car a couple of days ago and he said, "Well, has anyone ever been able to predict the way KayTar would behave?" And she pipes up from the back seat, "Hey! What do you mean? I ALWAYS behave myself!" That sums it up pretty well, I think. Life with KayTar is often fraught with medical issues and unpredictable outcomes, but there are plenty of smiles and laughter along the way.

Still.

I got the results of KayTar's LFT recheck labs yesterday. THIS time her ALT is mildly elevated and her AST was at the high end of normal. LAST time her AST was mildly elevated and her ALT was within normal limits. Aye yi yi. More abnormal and nonspecific results!

However, I was doing some reading and it sounds like this is a typical laboratory finding in kids with GSD 0, which genetics is currently working her up for. Interesting. Of course, none of her doctors put this together.

Endocrine called Gastro (quick aside for you non-medical folks: gastroenterology also handles hepatology, hepatology focuses on the liver, LFTs are liver function tests) about the results, since they were still abnormal. Gastro wants them checked a THIRD time and will order further testing if they are still abnormal. Anyone want to place a bet?

Today I called Genetics to let them know about the test results, in case they need to tack some other test on to the retest panel. All of the clinics run off the same system, but unless someone CC's them specifically, they won't look at any labs but the ones they ordered until her next appointment, so if I want labs to be condensed into as few sticks as possible, I should probably let them know.

I'm feeling...something...today. I'm in a little bit of a funk. It's not because KayTar's labs were abnormal, I mean seriously, they are ALWAYS abnormal. I'm not worried. Maybe it is because I still CARE that her labs are abnormal, that there is still a little part of me that is always hopeful that this will be the set of off-kilter labs that breaks the case, that someone will pay enough attention to connect the dots and figure this out. Doctors look at constellations of symptoms to make a diagnosis and by this point, we're looking at an effing GALAXY full of them. Four years worth of them. This Sunday is my birthday and all I want, still, after all of this time, is for someone to look up into that metaphorical sky and finally recognize what it is that they are looking at.

More medical stuff....

We spent 8 hours at the hospital today. KayTar's tree nut challenge was today. Although she HATED the cashew butter and nearly barfed, she toughed it out and did it! She passed the test and now has no feeding restrictions...except for the 100,000 restrictions she puts on her own diet!

Because she had a minor episode of lip swelling a few weeks ago that seemed to be unrelated to food, the allergist wanted to run some labs (TSH, T3, T4, free T4, C3, C4, CBC w/diff and ESR). So after the 4 hour tree nut challenge, the nurse put EMLA cream on both of KayTar's antecubitals and we headed down to the outpatient lab to check in. There was a wait (both for labs and for the cream to do its job), so we went to the cafeteria to have Subway as I had promised KayTar. She likes the Spicy Italian, hold the bread, veggies, and dressings. We ate, then went back to the lab. The tech tried to get blood from both arms, but no luck. After the first try without blood, KayTar said, "I'm not bleeding. Well, I guess I'm dead." KayTar's one good vein was still bruised from Tuesday's labs. A second tech came in to try, but no dice. They sent us back upstairs for more EMLA...on her hands this time. Then we killed another 30 minutes before heading back to the lab. While we were wandering around, a nice volunteer gave KayTar a brand new little Beanie Baby bird out of the blue. She was pretty pleased. Two more sticks (one in each hand) and they finally got blood! 4 sticks total, plus some fishing around. Thank goodness for that EMLA. It was a long day. She more than earned her Little Mermaid game for the DS.

In other news, still NO POOP. I'm starting to get a little anxious about it. Since her KUB, 2.5 more days worth of stool has accumulated in there. I have a feeling this resolution is going to be fairly horrible for everyone involved. Ugh. I'm going to put a call into the gastroenterologist tomorrow morning to ask if we just stay the course or need to take additional action. We shall see.

The one about poo.

If you are squeamish or just don't love reading about poop, skip this one. ;) I keep up with a lot of KayTar's medical issues here because it makes for easy future reference, constipation included.

Back on July 7th (the date of which I only know because of the blog), KayTar had a middle of the night bout of abdominal pain. The next day we doubled her Miralax dose and there was a poop-a-palooza. At BubTar's basketball practice, she pooped her undies, a pull-up, and her pants. Then at home she pooped on the toilet...then the tub. Aye-yi-yi! It was all very loose, so we skipped Miralax the next day, and then went back to normal.

Then on the 17th, she passed a large, hard stool of impressive diameter. I think we were centimeters away from reentering the fecal midwifery zone. In the middle of the night, she started having very loose stools which continued into the next day. I gave a 1/2 dose of Miralax. On the 19th, she had no stool in spite of the regular Miralax dose. I put a call into the gastroenterologist that morning, because I had a sneaking suspicion we were dealing with a blockage (or several) that the looser stools were leaking around, but without x-ray vision, it is rather tough to parse it all out. I left a message explaining the situation and requesting a KUB, but nobody called me back until yesterday morning. I took her in for the KUB and we got the results this morning.

Her colon and rectum are full of stool, not to the point of impaction, but definitely in need of a good clean out. Rather than her typical dose of 1.5 tsp of Miralax QD, we are doing 3 tsp BID for a few days. Looks like we will be staying home, battening down the hatches and preparing for the Shit Storm of the Century. Unfortunately she has her tree nut challenge tomorrow, which means we'll have to take this show on the road. She has been struggling with encopresis for a while now, which is why she is back in pull-ups. Hopefully after the clean out, that will improve once we get her maintenance dose correct. I guess there is nothing to do now but wait and see how it goes....literally AND figuratively.

Ouch!

We had a decent 4th. Josh and I spent the entire day writing papers (for the same class), but in the evening we sent the kids to my parents' house and we out with friends. Traffic, fireworks, food, drinks, and a rollercoaster. Fun night! Monday I had a trig exam, last night was lecture, and tomorrow is my final. Joy. We've had three kids attempt to spend the night in the last week and all of them got homesick and went home. Poor BubTar (2 of the 3 were his buds) took it like a knife to the heart, he's so sensitive. I tried to explain it has very little to do with him (the kids were already trying to make new plans with him as they were leaving), but he takes things so personally. It was sad.

After our little boarder left last night, I set up the pump to slow feed KayTar and Josh and I settled in to watch Hell's Kitchen and an episode of Louie. The feed finished, I unhooked her, and went back to watch more TV. 15-20 minutes later she started fussing. I'd pause the TV and she would get quiet, I would hit play and she would start up. I started to wonder if she was being controlled by the remote as well. I went up to check on her and she was writhing a little and moaning, I asked what was wrong and she said she was too full, so I called down and asked Josh to bring up a bowl just in case. Too full usually means puking. She didn't puke, though, and the pain got worse. Usually, there is no pain when she is too full. In fact, for all of her gastro issues, she never has pain. I vented her tube, though, and pulled out some of the feed with a syringe, in case she actually was too full or too gassy. It didn't help and her pain got worse.

We brought her downstairs and by that time she was crying and shivering with pain. I checked a rectal temp and it was totally normal. I asked her to touch the place where it hurt the most with one finger, but she couldn't articulate it. She kept moving her finger from place to place and then said, "I don't know! Just tell me the answer!" I guess it was generalized pain. I asked her if she wanted to be held or to sit in her baby chair or lay in bed and she got very agitated and said, "I can't tell you! You just decide!" We tried to set her in her baby chair to rest, but she couldn't get comfortable. Josh held her on the sofa, and he patted her back in the soothing, absentminded way we all seem to do when holding a baby. KayTar sharply told him, "Stop that! It is making me hurt worse!" A few minutes later he cracked a little joke about her needing to toot and she said, "I AM NOT KIDDING!" She loves a good fart joke, so she's feeling pretty rough when she can't appreciate one. She never has pain like this, even though she has a history of constipation, so I was a little concerned. She settled down, though, and slept through the night and has been fine today. She fell asleep around about 1:45 in the morning and I stayed up for a while longer, waiting for her to call out again, but she didn't.

This morning, she didn't tolerate her entire morning feed, but otherwise she seems to be 100%. I've given her some extra Miralax with water today, in case it is constipation, but I'm not really sure what caused the pain. I emailed our pediatrician (I bet she regrets giving me that address sometimes, but she never lets on, LOL.) and she said that it isn't likely to be intussusception or appy with this presentation, but to go in if it comes back again. I sure hope it doesn't come back, though, because I've got a trig final tomorrow and spending today at the doctor or ER for abdominal pain will not be a good thing!

On a totally unrelated note, I think she has aspirations to be the next Billy Mays: