Settling In

Well, I last left you on the edge of your seats (or so I like to imagine) waiting for the arrival of a FOURTH oxygen regulator and with KayTar's sats misbehaving at school. Since then, we regulator has arrived (though not on Thursday when I spent my whole day waiting for it...nope, I had to spend most of the following Friday waiting for it, too!) and it has been working, for the most part. We did have an issue with it losing its seal on the first day she used it at school, but KayTar got out the T-handle and shut the valve ON HER OWN! I didn't even teach her how, she just learned by watching. She called it "the axe method" because she thinks the T-handle looks like a mini-axe. What a big kid she is turning into! Since then we have been taking it off the tank after every use and putting it back on when she needs it and haven't had anymore issues (KNOCK ON WOOD!). We shouldn't have to take it off, except to refill the tank, but whatever...if it is working, I'm happy.

As far as her sats...meh. She is below the pulmonologist's cut off more often than we'd like and nearly every day in PE. The plan this week has been to put her on oxygen for 30 minutes to an hour before PE regardless of sats (as long as it doesn't put her at 100%) to give her body a break and perhaps increase its ability to meet her needs during the 30 minutes of PE. In addition, if her sats are low at her lunchtime check, we just put her on oxygen after lunch and keep her on until PE. If her sats are low in PE, then she gets oxygen at that point and keeps it on through music. So far, it does not seem to be making her sats any better DURING PE, but my hope is that giving her a body a break before/after that exertion will minimize any ill effects from it. She enjoys PE and I do think it benefits her to stretch her abilities (they encourage her to participate, but always allow her to self-modify)...this week she walked a MILE (or so she told me, 7 stamps=1 mile and she earned 7 stamps). KayTar walked a MILE! I probably would not have thought it possible and I definitely would not have allowed/asked her to push it like that in real life, but she did it (and besides her oxygen issues, she handled it well)...which is why I think there is a benefit to her participating in PE. I don't want to pull her out because of the low hanging sats, but putting her on oxygen during that madness (there are a TON of kids in PE) isn't feasible either. So for now, we're going to keep plugging along like this, tweak some things, and see how it goes. The pulmonologist might have a different plan for us when we see her in November, but we'll cross that bridge when we come to it.

It is a relief to finally have reliable equipment for her (though, I'm totally scared to try the regulator on the other 2 tanks we have because I'm afraid it will break!) and it feels good to be settling into a routine and have some rough form of a plan in place. It isn't perfect and we'll have to tweak it many times, I'm sure, but the worst part of new medical needs/new equipment is all of the unknowns. It feels like the answer to every question becomes "I don't know!" and I hate living like that. Once we get to a place where we have some idea of what direction to head and what our day to day might look like, I feel much better about it. Now we know that on most healthy days, KayTar will likely need oxygen at least part of the day, and on most sick days, KayTar will probably need oxygen most, if not all, of the day. We may not know ALL of the answers, but I can definitely settle for knowing one or two!

Regulators.

I am at home today, waiting on a FOURTH regulator from the home health company, as we have had THREE malfunction on us since last week. THREE!

The first one worked great initially. We used it on the E tank for about 3.5 weeks without any issues. Then we got the little M6 tank, put the old regulator on it, and sent it to school with KayTar...and it blew. It kept blowing. The HHC replaced it.

The second one worked great when we first put it on, but within an hour of being on the tank...it blew! And kept blowing. The HHC replaced it with an ADULT regulator, thinking maybe the pediatric regulators were faulty. The RT told me that we were the only ones having issues with these new regulators. Great!

The third one seemed to work great initially. I tried it on both tanks and it felt VERY secure. I sent it to school on the M6 tank...and it blew! I thought that maybe I hadn't secured it well enough (because it made a pretty tight seal and I bruised my hand with putting it on/taking it off the tanks the night before), because eventually the nurse did get it to run for KayTar at school for a while after reseating it a few times.When we got it home, I cracked the tank and turned on the regulator and KA-whoooosh! Grrr. We've been trying to get it to work, because I just can't believe we have had so many faulty regulators...but nothing is working. I even tried it on the E tank, but it KA-whooshed and it was like 10 times louder. I seriously JUMPED back.

I've been reading about regulators and trying to figure out if we might be doing something that is causing this to happen, but I can't seem to find any answers! However, I did realize that the only thing that changed when we went from Working Regulators to Non-Working Regulators was that we started using the little M6 tank. The tank itself looks fine. I don't see any imperfections. But I think possibly somehow that tank is causing these regulators to break..it is the only theory I can come up with that makes any kind of sense. If it was something WE were doing incorrectly, this would have been happening since the beginning...but we used her E tank for 3.5 weeks without any problems. If it was really faulty regulators, I would believe one...maybe even two...but THREE? In a row? It just doesn't seem likely. So I'm at home today, waiting for the RT to come by and either swap out a new regulator with the intention of ONLY using it on the E tank...or with a new regulator and a new M6 tank. It is almost 2 and I haven't heard from her yet. The tank and regulator are at school with KayTar in case she needs it (with the hopes the with reseating, she can get enough O2 without it blowing), so I need to go get it when the RT is headed this way. Hopefully she calls in advance and doesn't arrive when I am out picking KayTar up...like on Monday! Most of all, I hope we can get this figured out for good...so we can have a reliable portable oxygen system for KayTar!

She was patting her tank like she was burping a baby and
said, "My baby has gas!! Get it? Because oxygen IS a gas?!"

PS: While I was writing this, the nurse emailed me and said KayTar had low sats in PE and she was attempting to start the O2, but it wouldn't make a seal at all...so she had to go without. AGH! We have to get this figured out!

Oxygen! Part 2

I finally talked to the pulmonologist's nurse on Thursday. She said that Dr. J wants KayTar on oxygen when her sats hit 95% or below. I asked her the same question in about four different ways, hoping to get a different answer, but NOPE! 95% is when she needs to go on oxygen, no matter how I phrased it. The truth is that I was pretty deflated by this news. We've been checking her sats both at home and at school this week and she is RARELY over 95%...in fact, even with my low-ball order of at/below 92%, she still has been on oxygen twice at school this week! When I heard the official orders, I felt kind of like this...sideways and all. LOL!

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I understand the orders, I do. If KayTar's body is already struggling to make ends meet and running sub-optimally, then allowing her to sat low and experience chronic, even slight hypoxemia, is not ideal...especially when we have the ability to fix it for her. I get it. I just feel a little fussy about it! Initially I was worried about how KayTar would feel about it because when she first started overnight oxygen, she said, "I'm glad I don't have to wear this at school, people would think I'm weird!" but when I talked to her about the fact that her needing to wear oxygen in class might be more than hypothetical, she said, "That'd be okay...and I could build my muscles from carrying the bag!" I've obviously got a lot to learn from that kid, because I was not feeling so swell about it!

We didn't want to rock the boat at school too much, but the (best) nurse (in the universe) offered to check her sats a little more frequently on Friday, in case the two little snapshots (before lunch/during PE) of her oxygen levels were not entirely telling...but still, at 2/3 checks she was below the cut-off and she ended up on oxygen in the afternoon. I don't know what it means or what the best way to proceed is, honestly. Maybe her sats are running low due to the infection she had LAST week or maybe they are always like this. Maybe we should just keep her on oxygen all day at school when she is in the classroom and PE (and let her go off of it for lunch/specials), since she is low so often and it isn't feasible to monitor her frequently. Maybe she just needs a boost a couple of times a day. Maybe, maybe, maybe! At this point, I don't know what our plan for Monday will be (oh, and to complicate this further, the regulators on the small tank seem to be malfunctioning!)...but I've at least accepted that we do need a plan! So far today, her sats have been good at every check...so I am still holding out a little hope that this is a temporary set-back due to the respiratory infection she is just getting over and maybe she will just need additional supports when recovering...but if that isn't the case, we'll deal with it.

The tough part is that when we "lose" something with her (or add supports/machinery/whatever, depending on how you look at it) it is usually permanent...so unfortunately, I have FEELINGS about such things which distract me from the important things in life, like the fact that she is here with us and she is HAPPY and she is "healthy" and we have the ability to support her in these ways here at home or at school and we have precious people in our lives who help us put her needs first and care for her when she is out in the world...so many blessings!! So instead, I'm choosing to feel like THIS (okay, maybe not quite THAT happy, but you get the idea)!

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Oxygen!

KayTar has been on overnight oxygen for almost a month now and I think it is going really well. She started off being fairly bothered by the cannula, I had to cut the prongs down a little and use bandaids to secure to her face (tightly) so she would leave it on her face AND so it didn't wiggle and bother her. Then last week, she told me that she didn't think  I needed to tape it anymore and she has left it on perfectly since then! We switched out cannulas last night and I didn't need to cut the prongs down this time! From a compliance standpoint, she's doing great with it!

She wakes up easier in the morning (even school mornings!) and seems to be getting better quality sleep. Last year, she got her feed in her sleep, Josh dressed her while she slept, and he carried her downstairs and she slept some more on the couch. For the most part, she is awake by the time her feed finishes this year. This morning she was a little sleepier because she it is her first day back after a week out and she still isn't feeling 100%. Nighttime alarms (when healthy) have also been reduced since starting oxygen. She was originally prescribed 1/4 liter per minute for nighttime use, but a lot of times it is insufficient to keep her sats up. When she is healthy, she is using about 1/2 liter at night and satting about 99...but when she was sick, we got up to 1.5 liters and she was still satting 95-96 overnight. Obviously, when she is sick she needs more respiratory support than we knew prior to having a monitor here at home, but we are glad to be able to give her what she needs now! She has had some fairly low desats while sick, like down to low 80s/high 70s, and generally her sats are just lower overall, especially when sleeping. From a symptom management standpoint, she seems like she is doing great with it, too.

When the pulmonologist ordered the oxygen, she did so mainly for nighttime use, but also gave us the go ahead to use it as needed during the daytime. She said to keep her oxygen sats between 96-99 and be careful not to let her sit at 100% if on oxygen. Beyond that, it is kind of our call at this point. Prior to this recent illness, we hadn't used it during the day at all, but while she has been sick, we've used it on several occasions when we notice she is satting lower than she should be (usually under 95). At home, it is a really easy call to make. We have the oxygen available, she is just sitting around here, there is NO reason not to hook her up and give her a little boost. But she is back to school today and I'm SO uncertain as to what the right call is where her oxygen is concerned. I brought the pulse ox up to school and her tiny little M6 tank (which we need to get a pulse regulator for) just in case. I told the nurse that if she is at/below 92, go ahead and start her on 1/2 a liter. It is lower than we would let it sit at home, but at school there are more issues to consider, especially social issues. Oxygen is such an OBVIOUS sign that someone is sick and I don't really want her to have to draw that kind of attention unnecessarily. I know, I know, a g-button and feeding backpack don't exactly SCREAM normalcy, but it is subtler to me...or we're just both used to it and the necessity is undeniable. I also know that a wheelchair doesn't exactly announce HEALTHY CHILD COMING THROUGH either but again, KayTar clearly needs it when we are out and about AND she loves it. That makes it a non-issue for me. She isn't totally comfortable with the oxygen yet (though she is very accepting of it at home when it is needed and she has even asked for it at times) and when she first started using it, she said, "I'm glad I don't have to wear this at school, people will think I'm WEIRD." It is a harder call to make knowing that she feels self-conscious about it. In the end, her physical health is the most important factor, but it is easy enough for me to tell myself that she was managing before we started monitoring her and before we had oxygen to give her, maybe not managing well, but managing nonetheless...so is it worth it to make her wear it at school? And how low is low enough for it to be worth it?? So many things to consider! But for today, we set the bar at 92. I also put a call into the pulmonologist to get her opinion on what the cutoff should be and I'm waiting on a call back. I feel MUCH better about putting her on the oxygen during the day if it is the DOCTOR'S call, not mine. Sometimes it is hard to willingly put more on her little plate when there is so much there already! Luckily today her sats were good when checked and it was a non-issue. She is doing a lot better and I don't really expect she will need that kind of support this week, but it will be good to have some more detailed information from the pulmonologist, so I'm hoping she gets back to me soon!

PFTs and Echo

KayTar's testing did not go so swimmingly on Monday. The pulmonary lab uses a different mouthpiece than our pulmonologist's clinic and KayTar started gagging straight away when she put it in her mouth. I was able to talk her through it, though, and with some encouragement she was able to to make it through the first few tests. Unfortunately, after the first try on a test where she had to hyperventilate and then attempt to exhale against a closed valve, she puked. Puking means the end of testing in the pulmonary lab, so that was that. We did not finish the testing without bronchodilators and we didn't even start the testing that is repeated after bronchodilators are given. I doubt we got anymore information than a typical in-office PFT...and that it will likely be re-ordered for her and she will have to be NPO beforehand. It also means we finished testing WAY ahead of schedule and had a huge gap between 10am when we finished and 1pm when her echocardiogram/bubble study was scheduled.

We went over to check in early, in case they had an opening...but they never did. We went to get lunch in the cafeteria and oh my gosh, do I ever LOVE KayTar's wheelchair. It is AMAZING! She can't push herself a LOT, but she can push herself some and it makes such a difference! In the cafeteria, instead of trying to push an unruly stroller and carry food and a drink, I just said, "KayTar, follow me!" and she could! If there was a tight corner, I could use one hand to help and the chair would move with ease! Just little things like being able to hold the elevator doors for her while she rolls in or opening a clinic door for her are SO much simpler. She loves the autonomy, too. I'm SO glad we opted for a wheelchair and not a medical stroller, she is very proud of it and it makes everything a lot easier for both of us. If you have a kid with limited mobility or even limited energy/endurance and are looking for a seating solution, I highly recommend the wheelchair route if they have any ability to self-maneuver. It makes more of a difference than I ever would have imagined and I'm SO glad that Heather gave me that advice when we were making our decisions. After lunch, we just hung out in the waiting area, hoping for a turn!

It was FINALLY our turn and KayTar was delighted to discover the orders had been changed so she did not need to have an IV. She said, "Yes! I wished it away!" Initially the orders were for a bubble study, but the cardiologist called the pulmonologist to see if she would change it to a normal echo because that should be sufficient to diagnose the problems she is looking for (which is what my wise friend Amber had told me! Mito moms always know the right answers!). The echo itself was no big deal, we just relaxed for about a half an hour while the tech looked at her little heart. I know nothing about cardiology, so I had no idea what I was looking at. I can tell you she has a heart and it was beating, but beyond that I'm clueless. I tried to get the tech to tell me if things looked mostly normal, but she was tight-lipped. Darn! I'm not sure when we will get the results, hopefully this week, though. I expect it to be normal. None of her docs have ever been concerned about heart problems and I think the pulmonologist ordered this as more of a rule OUT than a rule IN sort of test. It will be nice to get the results, though.

The end of testing days is always our favorite part, or as we like to call it New Monkey Time! :) This time around, I had asked KayTar if I could surprise her with a special sock monkey I found online and she initially said yes, but after such a long day of testing, she wanted an immediate reward and decided to go to the gift shop. We walked in and there was the SAME monkey I had picked out for her and it was her first choice, too! I love it so much! It was meant to be, I guess! On the way home, she said, "I just love today!" Not many people feel so fondly about a day that starts with puking and is spent at the hospital, but that is just how KayTar sees the world...the glass is ALWAYS overflowing, never half-full or half-empty!

Changes!

It has been a big week at the 'Tar household. Last Wednesday, KayTar received her awesome new wheelchair and her pulse oximeter. Yesterday, she received her oxygen concentrator and started on nighttime O2. A lot of changes and new routines to get the hang of. It might be a little sad and overwhelming if we hadn't been working towards these things for so long now (it was LAST summer when we started noticing KayTar's weird breathing...or lack thereof, and she has been too big for her stroller for AGES), instead I feel a lot of relief and maybe just a touch of new equipment anxiety. I'm relieved that insurance has readily agreed to cover what she needs and thankful we have time to adjust to the new stuff before the school year starts. I'm glad we have some new supports in place to help her feel better and have more restful sleep and more daytime energy, hopefully.

The wheelchair has been a very simple transition. KayTar loves it, so I love it. It takes more time to put it together and break it down to get it in and out of the car (which isn't exactly fun in this August heat), but the ease of use for both me and KayTar makes it WELL worth it. It takes about 4 steps to break it down, remove the seat cushion, remove the wheels (just have to press a button), flip up the anti-tips and the foot rests, and pull up on the handle in the middle of the seat. To put it together, you just reverse the process. Simple.

The pulse oximeter has been pretty simple, too. KayTar likes the probe on the big toe of her right foot at night. We only get 4 probes a month, so we've learned that after the first couple of days, we need to wrap it in a bandaid to keep it secure. I bought her some cute girly bandaids so her glowing toe can look cool. She doesn't have any complaints about using it. It has alarmed on several occasions, but she has resumed breathing on her own each time. It alarmed earlier this week on vacation and when I stood up to go check it, I discovered my leg was TOTALLY asleep and I collapsed. It was a spectacular fall. For the most part, she is satting a little lower than the pulmonologist wants her to...but not super low. We are going to do some spot checking at school (especially during PE) to see how she is managing there, too.

Oxygen is going to be the trickiest adjustment. She was very compliant during reading time and when I was cuddling with her at bedtime, but every time I went to check her, the cannula was out. It was a combination of it getting knocked out when she moved and her moving it in her sleep. I found it on her forehead and chin several times and once on her blankey. It is going to take some trial and error to figure out a good way to keep it in place! She said she didn't feel any better this morning, but I suspect you actually need to keep the cannula in to get the benefits of oxygen therapy! ;) We have a Respironics EverFlo compressor and an Ultrafill system to fill her portable tank if needed. It is pretty quiet and didn't seem to put off a lot of heat, which is a relief! BubTar did say that it sounded like Darth Vader was using the toilet (we are keeping it in the bathroom near their rooms)!

Monday is her echocardiogram, bubble test, and full pulmonary function testing at the hospital...then we may know a bit more about this respiratory stuff. In other news, her tummy has been giving us daily trouble recently (vomiting, feeling too full, stopping feeds early, venting out LOADS of air)...I don't know if we overdid it on vacation and things will normalize or if it is just something we will continue to deal with at this time of year. It is much less stressful this year, though, since we now have experience with dealing with it!

****

Oxygen update: I cut the ends off of a bandaid and used them to secure the cannula near her nose and threaded the line up her pajama shirt and it worked! I don't know if it will be a long term fix, but she wore her oxygen all night last night! Of course we had to color coordinate with her pulse ox bandaid! :)

Sleep Study #2 Results

We got the results of KayTar's sleep study earlier this week, but we don't know what to do with them! We're having some issues being turfed between the pulmonologist/sleep doc and the neurologist. The main problem is that she had 31 central apneas (where the brain fails to signal the body to breathe) that lasted up to 30 seconds and were associated with desaturations to the mid/low 80s. Also, her oxygen saturation was only 95% when awake and 93-94% when asleep, which is lower than I'd like even if it is not hugely abnormal. Her lowest oxygen saturation was 83% with 23 desaturations of 3% or more. Other subtle abnormalities were her sleep efficiency, 71.1%, 111 arousals, and she spent a tiny amount of time with her EtCO2 above 50. Her AHI/CAI are 4.5 overall, but 5.7 in NREM.

I'm a little frustrated right now from the lack of a response I'm getting from her physicians, because I'm a bit concerned about the results of this study. 6 months ago, her study looked significantly better. She had only 15 central apneas that were only 8-16 seconds in duration and her oxygen levels never dropped below 91%. Her average oxygen saturation was 97%. Her AHI was 2.0. All of it was worse this time and it has only been 6 months since the last study. She is also "forgetting" to breathe still during the day and we have to remind her. It doesn't really make sense to me that someone can forget to breathe, but when she is concentrating on something, she does. I wonder if it is related to the central apneas.

Between the daytime symptoms she has, the "forgetting" to breathe, the lower average oxygen levels overall, the increased and prolonged central apneas, and the desaturations...I am really hoping that the pulmonologist will recommend treatment this time, at least on a trial basis. I also really want to be able to monitor her oxygen levels at home with a real pulse oximeter. I know other patients of our pulmonologist who have been treated with fewer significant issues than KayTar is exhibiting, so I hope that once we are really able to connect with her and review all of this (soon, I hope!), she will have good suggestions for what to do next!

Sleep Study #2

KayTar had another sleep study on Friday night. Overall, I think it went better than the last. We were at a different facility in a different hospital system and even though both are supposed to be geared towards children, this one did a MUCH better job with KayTar. At the last facility, they were not very friendly and they were very forceful and rushed in getting KayTar hooked up and into bed. This time, our tech was WONDERFUL. During the 12 hours we spent there, she gave KayTar a roll of pink Coban wrap with hearts on it, a pink light-up wand, a Little Miss Princess notebook, an Avengers pen/necklace, stickers, a popsicle for breakfast, and she hooked up her gorilla and made plans to get a name badge holder for KayTar for the next time she has a sleep study. She was great with KayTar, very friendly and patient, and let us stick to our usual bedtime routines even though it meant KayTar went to sleep later than usual. The facility itself was also much nicer, instead of a single hospital bed in the room, there was a very comfortable double bed. KayTar said, "Oh my gosh, they gave us a hotel room!"

Once the girls (KayTar and her gorilla) were hooked up and in bed, KayTar was allowed to read for a half hour, as usual, and I followed the tech into the hall to ask her to watch for/make note of any breath-holding KayTar did during reading time (without drawing K's attention to it, didn't want her overthinking it and changing her normal behavior). She thanked me for reminding her because the pulmonologist had mentioned it in her notes. In the morning, she told me she was able to catch some of those spells during reading and she noted it for the doctor. Hopefully, even if we still don't know why it happens, catching it in action will at least tell us if it is something we need to be concerned or not about since she was hooked up to EEG, EKG, and oxygen/CO2 monitors at the time.

As for the sleep itself, it was rough! If you've never had the pleasure of having a "sleep" study (it deserves the air quotes, believe me), it is not very restful. Poor KayTar was so hooked up (she had electrodes on her scalp, face, neck, chest, and legs; bands on her chest and stomach; a transcutaneous pCO2/O2 monitor; and a pulse ox probe on her finger) it made for a very fitful night. She was especially agitated by the nasal cannula and CO2 thermistor and tried to pull it about a thousand times (slight exaggeration). The tech came in to put it back in about 20 times (no exaggeration) and I had to tell her to leave it alone more than 50 times (no exaggeration). In the morning, I asked the tech if they were able to get adequate information with all of her waking and removal of the cannula and she said they did, so that is good. If they got good info, one mostly sleepless night will be well-worth it!

I could only glean limited information about how it went. I know that her transcutaneous CO2 was above 50 for all of the sleep time I observed (normal is below 45, but some docs don't treat until it is over 50 for a certain % of the night). I could tell she was about to wake up because it would go down to 44-45 right before she woke up and could tell when she was back to sleep because it was back to the 50s. During her last study, she was between 44-49 most of the night, but they were only measuring EtCO2 with the nasal cannula, so if she was blowing off CO2 from her mouth, it was not measured. The transcutaneous monitoring gives a more accurate picture, so I'm glad this lab uses it! The other data I have is her transcutaneous pO2 and it doesn't really mean much to me, because I can't find much information about the norms. Most of the night, her pO2 was between 40-70. The only information I could find about pO2 said it should be between 80-100, but that was where an ABG was concerned and not transcutaneous monitoring, so I don't know what to do with the information for sure. I couldn't see her oxygen saturations, so I don't know anything about those numbers yet. I won't know anything about central apneas until I hear from the pulmonologist, but I do know she stopped breathing once because she started kicking and I thought she was fussing and tried to calm her down and the tech told me she was still sleeping and to let her be. Later, she told me that she had stopped breathing and the kicking was an attempt to wake up/start breathing. Based on the limited information I have about her CO2 levels, I won't be totally surprised if we have to start some kind of night-time treatment for her after this (in which case, the little miss will have to adjust to having something in/around her nose at night). We'll see what Dr. J thinks of her study (hopefully soon!) and go from there.

Hooray for Dr. J!

KayTar saw her new pulmonologist for the first time today. We've been WANTING to see this doctor for about 6 months, but had to wait for the kids' insurance plan to switch over. She has a reputation for being thorough and really good for kiddos like KayTar who don't always fit into the regular little boxes. Our main concerns were:

1. KayTar's weird halting/breath-holding breathing pattern. (Even BubTar tells her "KayTar, breathe!" when he hears her doing it. It isn't just something we see at home, her OT has asked us about it several times, because she does it frequently during therapy.) 

2. KayTar's possible desats that we've seen on pulse ox. I know that these little fingertip pulse oximeters are not great and it may be inaccurate desats, but I feel like it deserves some consideration.

3. KayTar's sleep study results which I felt were kind of brushed off by the other pulmonologist we saw. (I think his official advice was "Don't worry about it and stop checking the pulse ox.")

I was not disappointed today. She WAS very thorough, perhaps more thorough than I was expecting! As a side note, I guess that KayTar now carries a label of myopathy in her file, which I didn't realize until she said, "With her having a myopathy, it is important to take this seriously." Good to know! I guess we've moved from unspecified metabolic disorder to unspecified metabolic myopathy? Not a huge leap, but interesting!

She ordered x-rays of KayTar's chest and lateral neck today to be sure there are no structural abnormalities (in the heart, lungs, trachea, ect.) contributing to the weird breathing business (which she referred to as apneustic breathing), which we had done today after the appointment.

She ordered labs (VBG, basic metabolic panel, IgE, CBC w/diff, and a few tests for aspergillosis) which we will have drawn tomorrow since she has another specialist appointment tomorrow and they may want labs, too...we don't want two sticks when we could get it all done with one tomorrow! Some of the labs are just for ruling things out (aspergillosis) or general information. The VBG is to check her CO2 status, because she had some mild hypercapnia on her sleep study. If it comes back with elevated CO2 levels, then we may have to get an ABG (eeek!). I'm kind of hoping we can figure things out without taking that step.

She is also requesting that KayTar's neurologist adds brain stem imaging to her upcoming MRI (I haven't written about this yet, but I'll get to it soon) because that type of breathing can be caused by a lesion in the brain stem. I don't think she really thinks this is the problem, but again, she is thorough. She said that if this is the problem, usually they breathe this way all of the time and there are times that KayTar does not breathe in this pattern.

We follow up with her in 2 months and will move forward depending on what, if anything, is discovered in this first round of testing. She also wants a repeat sleep study in her sleep lab later this year, because they have redundant measures of CO2 levels which will paint a better picture of KayTar's actual levels during sleep and because the pulmonologist herself is who reviews the data that is collected. All in all, it was a good first appointment. I feel like our concerns were heard and are being taken seriously even though KayTar is somewhat in a gray area with these vague and unusual kind of respiratory symptoms.

Rainy day reading gear on the way in.

Princess Ladybug has a PFT.

Someone spotted a homeless sock monkey as we passed the gift shop and suckered me into it. ;)

Sleep Study Results

We saw the pulmonologist on Tuesday to get KayTar's sleep study results.

She was asleep for 86.9% of the study. (norm is greater than 90%)
She had 189 arousals or 20.9 per hour. (norm is less than 14 per hour)
Her O2 saturation was usually 97% with desats to 91%.
Her EtCO2 was as high as 52 mmHg, but usually 45-49 mmHg (norm is below 45)
She had 15 central apneas lasting between 8-16 seconds.
She had 3 obstructive hyponeas.
She had a slightly slow occipital rhythm on EEG.

So, she had some abnormalities...but nothing too dramatic, and nothing to current pulmonologist wants to treat. It is a little frustrating...on one hand, we are glad she had no major abnormalities...but on the other hand, we are tired of living in the gray area! It is also frustrating because I've talked with a few parents whose kiddos see the pulmonologist who specializes in these metabolic/mito kids (the one we were supposed to see) and she treats at lower thresholds, because these kids tend to be more affected by these subtle changes, so kids with more normal results than KayTar are being treated. I'm especially concerned about her EtCo2 being mildly, but consistently elevated all night. That, plus the fact that she holds her breath so much of the day, makes me worry a bit about hypercapnia. Seems like there is a chance that headaches and fatigue could be stemming from that and I'd like to rule it out for sure before we ignore it as a possibility. The truth is that if nobody truly knows what is going on inside KayTar's body, they really don't know if these slight changes are causing problems for her. Alas, getting a doctor to return a phone call or email these days evidently requires an act of God...so I'm not going to hold my breath that anyone will actually follow-up on this. Pun intended. ;)

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In other news, KayTar has had a couple of good weeks, school-wise. She hasn't really needed to make any extra trips to the nurse during the day (with the exception of an odd earache complaint), so that is good. But she is still having issues at home...headaches a couple of nights this week and more abdominal pain tonight. I've been playing phone tag with the GI office since Friday before last...10 days now. I've spoken with a couple of nurses in the meantime, the one I mentioned in the previous post, and a different one who seemed at least familiar with KayTar's case and was reading comments that I could tell actually came from her GI. She basically asked what I felt was appropriate and I said that I just want to make sure we aren't overlooking a physical problem before writing this off as her new baseline. We decided to order a KUB and get a referral to a motility specialist. The last thing she said was, "Let me call [clinic location] to get that KUB scheduled. I'll call you right back." That as WEDNESDAY. Sooo, we're getting nowhere quick. I'm really hoping someone gets back to me this week, though, so we might have a shot of figuring this out for her.

Sleep Study!

KayTar's sleep study was last night and it went better and worse than I expected. It went better in that she didn't have any major allergic reactions to the adhesives, so the no-Coban/limited adhesives plan worked. It was worse in that there wasn't much sleep happening during that sleep study. She slept enough for them to get the data they needed, but it was not a restful night and she moaned a lot and woke up confused a few times. She usually sleeps right through the night, even when we've been inpatient, but it is tough to get rest when you are hooked up like this! EEG, EKG, nasal cannulas, bands around the chest to measure expansions/contractions, sensors on her legs and chin, pulse oximeter...yowza!

That being said, I'm certain she got more sleep than I did. I was not feeling my best in the first place, but when you add in the freezing room, hard mattress and pillow, sleeping fully clothed with a hoodie on, and a kiddo who isn't sleeping...well, it was not a recipe for successful snoozing. We got through it, though, and it was definitely not the worst test we've ever been through!

The techs would not tell me anything, so we have to wait 3-4 days for the report from the doctor. I'll call on Monday to check on things, because I'm very curious about how it looked on their end. I know from watching the one monitor in the room that her ETCO2 stayed in the 45-49 range for the most part and I saw her RR drop below 10 a few times. Of course, I didn't watch the monitor all night...I did attempt to catch some Zzz's, though they proved to be quite elusive. KayTar and I spent the day here recuperating and I am SO looking forward to a good night's sleep in my own amazingly comfy bed tonight.

Double Header.

KayTar is doing much better! She is still on Suprax, but she was doing well enough to attempt the day on Thursday. She made it through the day, although her glucose was a bit lower than usual (probably from the extra stress from the pneumonia). Friday was a in-service day, so she had extra time to rest/heal over the weekend. Monday was a totally normal day (right down to the daily headache)!

This zebra art car parked in front of us.

Since it has become clear that it will be months before we get the insurance issues sorted out so she can be seen at the facility that houses her mito doctor, and her soon-to-be geneticist and pulmonologist, we decided to see another pulmonologist in the meantime. Our pediatrician recommended someone (who happened to be the same doc that a friend recommended, too) so I called yesterday to get an appointment and they fit us in today, right after her already scheduled ophthalmology appointment. Perfect! It is so nice when we can take one trip out to the medical center and knock out more than one appointment...especially when we don't have to kill a chunk of time between them.

"Take a picture of how strong I am!"

First up was ophthalmology and the dreaded eye drops. Oh, how she HATES the eye drops! She did pretty well this time, though. Yes, there was screaming, but it was like 5% of her normal eye drop screaming level, so we're putting it in the wins column. Her prescription has changed some since last time, enough to justify new glasses, but not enough to be causing the daily headaches. Drat. He said it is RARELY vision that causes these recurrent headaches, but it is good to rule it out altogether. KayTar got some of those super cool post-dilation shades and we were off to our second stop.

Gloves! She was ecstatic they gave her some!

The pulmonologist seems great. He wasn't dismissive, which is what I was worried about. KayTar isn't a typical kid, even in terms of her systemic dysfunctions, so she needs doctors who can look a little beyond how normal kids function and how normal diseases progress. Those kind of doctors can be hard to come by! I told him about her weird breath-holding breathing pattern (and showed him a video) and told him about the transient desats. First, he started to say that those fingertip pulse oximeters are sometimes in accurate on little kiddos (which I agreed with) and that you really have to pay attention to things like the waveform and heart rate, too...and I politely interrupted to tell him that I had been paying attention to those factors, too, and it seems to be accurate. I had a video of it on my phone, so I showed it to him and he agreed that it seems accurate on all fronts. He doesn't think it is related to the weird breathing (so maybe that IS a tic?), but he thinks the desats are worth looking into. She had a pulmonary function test in the office today, which was good, and she is being scheduled for a sleep study in the near future. He said if it is happening when she is awake, it is probably happening as much or more while she sleeps, so hopefully that will give us some information. He's also going to look at her previous chest films to be sure nothing was missed. I think it is a solid plan and I'm glad we're looking into this a little more closely, it has been a nagging worry for months now.

When all was said and done, KayTar declared it the BEST DAY EVER (she's probably had a couple thousand best days ever by now) because she got disposable sunglasses from the ophthalmologist, and gloves and a nose clamp (from her PFT) from the pulmonologist. She's madly in love with that nose clamp because, "I can finally hold my nose without having to use my hands!" We're very lucky that she is so easy to please. :)