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

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

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

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

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

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

If I should die...

A couple weeks back, I was driving across town to purchase a special Christmas present for KayTar. Between school being downtown and KayTar's docs being in the medical center, I'm on the freeways nearly every day of the week, usually without incident. But on this day, that wasn't the case. I was in the process of changing lanes when the car in front of me (in the lane I was leaving) slammed on his brakes, so I had to hit mine so I didn't clip him on my way out. I'm sure I've done this loads of times without any issue, but for some reason THIS time, my car started fishtailing across THREE lanes of traffic on I-45. I was definitely not in control of the car and did not know how to fix it. I remember thinking, "When you are hydroplaning or skidding, you are supposed to turn into it..." but I couldn't remember what to do about fishtailing! But then the car was okay again and somehow, I never got hit. It could have definitely ended badly with the way my car was careening around. My first thought after I had control of the car again was, "OMG, there are so many things about KayTar's care that exist ONLY in my brain!" If something had happened to me, poor Josh would have been lost. He is very involved in her daily care, don't get me wrong...but I do all of the remembering. I know her schedule and when she needs feeds and meds and when her doctor appointments are and who her doctors are and who writes what prescriptions and what home health companies handle what and where all her records are...and and and!

As soon as I got home, I got to work on compiling some pertinent information for Josh. A lot of it was already fairly accessible, as I keep and online copy of the start of her medical binder on Google Drive and a physical copy by my bed, but it wouldn't have helped him any since I've never mentioned it to him or shown him where I keep it. The medical binder already had the following info (and the physical copy also has her latest notes from clinic, sleep studies, audiograms, ect):

-List of physicians/therapist/insurance case worker and all contact info
-Copy of her insurance card and SS number
-List of all meds, what she takes them for, when she takes them, and if they are PRN or daily, as well as her formula and oxygen information
-Equipment and supplies list (and I added what HHC handles what equipment and when to contact them, as well as how to find her glasses script info online)
- An updated Visual Guide to KayTar

In addition to this, I wrote up a walk-through of an "ideal" day in KayTar's life, a non-sick, run-of-the-mill sort of school day:

5:00am Nexium
6:15am Give 7 ml Augmentin and start feed ((dose) 240 ml @ (rate) 480ml/hr) of 8 ounces of Elecare 3 tsp Miralax, and 1 tbsp cornstarch
6:45am Feed over. Get dressed. Turn off O2. Brush hair and teeth. Pull hair into ponytail. Give 2 puffs of Advair. Socks/shoes on. Prep school feed (8 ounces Elecare, 1 tsp of cornstarch and water). Make sure glucometer bag is packed and is in the front pocket of feeding backpack. Pack lunch. Write note for her jacket pocket.
7:20am Leave for school. Bring backpack, lunchbox, jacket, pulse ox, and feeding backpack. If it is Monday, bring O2 tank and supplies (check with Mrs. H about the Augmentin that stays in the clinic to see if/when she needs more.). Drop pulse ox, feeding backpack, and oxygen in clinic.
11:00am She gets her glucose check and tube feeding at school.
3:05pm Dismissal. Pick up pulse ox from clinic either before or after dismissal time. On Friday, bring home O2 tank and supplies.3:30pm Give 7ml of Augmentin and start another tube feed. Do homework. She does her handwriting work on the paper that I have stored on my computer. [My Documents->My Pictures->September 2011->Hi-Write Paper] Most things she can do on her own, but she often needs help remembering the steps for ABC order. We do it this way (if you don’t understand from reading it, just read it to her and she’ll show you what it means): Write the alphabet on the paper. Go down the list and circle the letters of the alphabet that the words start with. If there are multiple words with the same first letter, then she writes 2, 3, 4, ect. near the circled letter. Then she goes down the alphabet and stops at every circled letter, finds that word, gives it the proper number. After all the words are numbered, then she puts them in order on her blank paper.
6pm Shower or Bath. Pajama time. Braid hair. Teeth brushing. 2 puffs of Advair.
7pm Give 10ml of Benadryl and dose of Nexium. Reading time.
7:30pm Bedtime and cuddles. Start oxygen at ¾ liter.
8:30pm Mix Elecare; 27.5 ounces of water + 22 scoops of Elecare. Lay out clothes for next day (both kids).
9pm 7ml of Augmentin and tube feed.

This is an ideal day...we both know it doesn’t always go like this, though! :) On the weekend, it all gets shifted around. Feeds are typically 4 hours apart, so set an alarm until it becomes second nature. Be sure to check her oxygen saturations periodically, if she is BELOW 96, start her on ¾ liter of oxygen and increase until sats are appropriate.

I also wrote a "troubleshooting" guide for if she is sick, kind of the thought-processes I go through when deciding how to treat her when sick. It won't cover everything, but these are the most frequent things we encounter:

Ketones/Hypoglycemia:
*When KayTar is sick, periodically check her urine for ketones. If positive, check every urine until they resolve and occasionally thereafter.*Check glucose in the morning when sick and when she is symptomatic during the day (lethargic, nauseated, dizzy/wobbly, “legs don’t work”). If lower than 70, give 2 ounces Gatorade. Recheck in 15 minutes. Give 2 more ounces of Gatorade. Continue until glucose is appropriate. If below 50, go to ER at Children’s Memorial Hermann and give them her protocol letter.
Coughing/Congestion:
*First line of treatment is Benadryl (10 ml every 4 hours) and Albuterol (2-4 puffs if coughing is not bad, a nebulizer treatment if it is bad, every 4 hours.*If the cough is not responding well to that, you can give her 10ml of Triaminic Long Acting Cough (dye-free). The other OTC cough meds make her manic, beware! If you ever have to use a different brand, do not give it after 2pm!*If coughing does not resolve after a viral illness or if she gets better (no more fever) and then spikes a new fever 1-3 days later, take her to see Dr. H...she either needs oral steroids or has pneumonia. This is her pattern.*When sick she needs increased oxygen support, make sure to increase her oxygen dose so her sats are in range and keep her on 24 hours until she is doing better.
Gut Stuff:
*Give 5ml of Ondansetron every 6-8 hours for vomiting.*If she feels too full after a feed, draw some out with venting/decompession tube.*If she is not tolerating formula feeds due to illness, slow the rate down (try 120ml/hr). If she still is not tolerating it, try slow drip Gatorade instead (a rate of 60ml/hr and a dose of 240ml will run in 8 ounces of Gatorade in 4 hours. Try to get 2 of these in during the day. Then run the pump overnight at 30ml/hr with a dose of 240 ml and she will get 8 ounces over 8 hours to keep her glucose up. Try formula at this rate the next day. When running formula over long periods of time, hang an ice pack with it. Continue to work the rates up from there until she is back to (or close to) normal, which is rate of 480ml/hr with a dose of 240ml.
Fever:
*I do not usually give meds for fever unless it is at/above 102 or she is bothered by it.*First line of treatment is 10 ml Motrin every 6 hours. *If Motrin does not break the fever or if it spikes back up prior to the 6 hour mark, you can give 10 ml of acetaminophen every 4 hours. Write down what time you give which med, it gets confusing in the middle of the night. If you know she needs both meds to keep the fever down, try to get into a schedule of alternating them every 3 hours.
*If she is feverish and ketotic/hypoglycemic and not tolerating feeds...or if she stops urinating or isn’t cognitively herself...take her to the ER at Children’s Memorial so they can manage her.

I put all of it on Google Drive and shared it with Josh. I also showed him how to braid KayTar's hair...you know, the important stuff. ;) It isn't everything and I don't think I can EVER cover all of the bases, but I feel like I got enough of the important stuff down in writing to make it considerably easier for everyone if something were to happen to me. I probably should have done it ages ago, in all honestly, but thinking about and preparing for your own death just feels sort of grim. Realizing how quickly and unexpectedly something terrible could happen was the little push I needed to finally take care of this!

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 Diagnosed!

We had an appointment with Dr. K (KayTar's mito doc) last Tuesday and got some kind of big news. KayTar is now officially the closest she has ever been to being diagnosed! Dr. K said that her muscle biopsy from four years ago is 100% definitive for a metabolic myopathy and since we've ruled out basically all other types of metabolic myopathies and the report from her recent visit to the metabolics/genetics says that they, too, think that it is mitochondrial disease...that is likely what we are dealing with. She wrote orders for her protocol letter and a prescription to get her started on CoQ10 to help with energy and ability to fight through infections a little more successfully. She said that she definitely looks and functions like a mito kid, we just need to get confirmatory testing back before she puts the official, always and forever, label on her. So, it is still a bit of a waiting game, but now we are waiting with some supports in place at least! If the Transgenomic testing comes back with a known disease-causing mutation, then that'll will be that. If not, we'll move on to exome sequencing. If we don't get an answer there, we may redo her muscle biopsy...but that is quite a way down the road. For now, I'm just happy that she is being treated as though she has it and that her team is in agreement that it is the right direction to go.

We actually received her protocol letter last Thursday (they are QUICK over there!)...and wow. It certainly drives home the seriousness of the condition. In case you don't know, a protocol letter is a letter we take with us in emergent situations so time is not lost when she is in need of medical supports, such as IV fluids. It is a fast pass through the ER waiting room and into a treatment room, and I can see why! It says things like, "any person with a mitochondrial disorder can go from walking and talking to intubated and in the ICU in a very brief time period" and "There is no specific treatment for mitochondrial disease and the disease is often, but not always, fatal." and "Any change in KayTar's condition should be taken seriously as she can quickly become fatally ill." Nothing quite like seeing your worst fear typed up in official black and white on a medical document as a likely outcome! Frightening realities aside, we are thankful to have the letter in hand. In fact, last Saturday when she crashed at camp, we should have taken her to the hospital...but at that point, I didn't know which hospital to take her to or what to even tell them. At least now, we know where to go and what to tell people so that she will get the care she needs as quickly as possible. It was a rough recovery and she missed a week of school, but she went back yesterday and is doing pretty well considering!

Mostly for my own future reference, I want to discuss what exactly happened at camp and why we were so concerned. KayTar was sick and struggling, she kept spiking a fever, she wasn't tolerating her normal feeds (puked ALL over herself and the camp clinic at one point)...but she pressed on and pushed herself to have fun at camp. We usually discourage this because we know how it will end...but it was camp and she never gets these opportunities, so we let it slide. She had fun all day and then at dinner, she passed out mid-sentence. We call it "emergency shutdown mode", but she basically powers down and aside from those automatic brain functions (breathing, pulse, ect.), she is out. We moved her from the cafeteria to her cabin, from her wheelchair to her bed, changed her clothes, discovered she had lost control of her bladder (bad sign), and put her to bed...without so much as a stir from her. Then when we decided it was time to go, we packed her up, picked her up, rode to the clinic in a golf cart, put her on a sofa in the clinic, moved her to the car...all without any response. We drove home for 2 hours...no response. My anxiety was building this entire time and I just knew we would need to drop the boys at home and go straight to the hospital...but a minute away from our house, 5 hours after she powered down, she opened her eyes and said, "Are we going home?" WHEW! We were still worried, but not unconscious-child worried, which is a different class of worry altogether. Like I said, it was a rough recovery...she had bladder/bowel control issues for a few days, was on nearly continuous feeds, requiring 24 hour oxygen, and was really, really run down...but now we are *almost* back to normal! And for the record, she asserts that camp was 100% worth it all...and I happen to agree with her!

Sometimes Mountains Move.

Last Wednesday, as I was in line to leave the parking garage at school, I read an email from our case manager at Transgenomic (the lab with the $17,000 test that might be able to diagnose KayTar). It said that she had spoken to our insurance and they had denied our appeal again, for the THIRD time. But she offered me an interest-free payment plan, you know, in case the interest was what was holding us back...not the $17,000! It was extremely frustrating and although I am not a crier (I hate crying), my eyes welled up with tears and I had to choke back quite a bit of emotion that was threatening to escape through my eyeballs. I vented my frustration to a couple of people, but otherwise kept it to myself. It was depressing and dwelling on it was not going to be helpful for me, so I made a decision to talk to KayTar's mito specialist about an alternate route to diagnosis and to find out if MDCP (Medically Dependent Children's Plan, something we are trying to get K into, but that is another post) might cover it...and then I decided to let it go and choose joy. I wasn't super HAPPY about choosing joy, but at some point, I just had to accept that letting myself wallow would not make me feel better and it would not change the outcome. I would have to wait and see what came next and decide to go for another round of appeal when I had more information. And I prayed.

I don't talk about my faith much here, but it is an integral part of who I am. I pray about things in our life and for people we care about and things going on in their lives, too...but in praying over our life, I rarely ask Him to change circumstances or fix things. I believe that He allows us to be challenged for a reason and if He wanted to intervene, He could and would. He knows my heart better than I do, I'm sure, and He doesn't need me to tell Him what I desperately want or need. I don't pray for KayTar's healing, I pray for wisdom for those caring for her, strength for Josh and I, joy for her, diligence to fight through the battles we face, and so forth. He created her just as she is and I don't pray away the struggles that come with it, I do my best to pray through them. Sometimes when I am too tired to pray for strength, I just simply pray that He would move someone else to pray for me. I know that mountains have a purpose and it isn't my job to tell Him where they belong, even when I am tired. He knows. So on Wednesday, I did not ask Him to fix it...instead, I told Him I was exhausted. I told Him that I was tired of jumping hurdles and just wanted to lay down in the middle of the track. I maybe flopped onto my bed to illustrate my point. I asked for strength and wisdom in moving forward, because dramatically tossing myself down in the middle of the metaphorical track was not a realistic choice, and I thanked Him for the encouraging people He has placed in my life. There was more, but that was the meat and potatoes of it. I felt kind of better. Less wallowy, more ready to find some joy and move forward.

Today, my cell phone rang. It was an out of area number and I answered it because I thought it might be related to Mito Camp (this weekend!). Instead, it was a manager at Transgenomic. She said that unfortunately, there was a lab error resulting in KayTar's test sample being ruined and they would need another sample. We've had this happen before, so it was not a real shocker. I said something like, "Well, we're having such trouble getting insurance to approve it anyway---" and she interrupted me and said, "Yes, I know you've been working with J to get it covered, but whether or not that happens, we will be running KayTar's sample immediately after receiving it and there will be no charge to you either way." And I said, incredulously, "ARE YOU KIDDING ME?!" She was very polite and said, "No ma'am, I've noted it in the chart. There will be no charge for the testing."  Then some very different emotions attempted to escape through my eyeballs again and I tried to suppress the urge to jump around until I was off the phone. It was miraculous! I don't know if the test will give us answers, but today it doesn't really matter. There was a mountain in the way and then it was gone! There are billions of more important things happening in the world today and every day, bigger prayers that beg to be answered, and I can't explain why He chose to move this mountain...for me...today. I just know that He did. Sometimes mountains move, and it is unbelievably breathtaking when it happens.

Bet you can't do this!

So KayTar has this little trick that her knee can do, wanna see?



Interesting, right? "It's like my knee is doing push-ups!" I'm not exactly a medical professional, but I'm pretty sure that is not the direction a knee is supposed to move on purpose. Anyway, I don't know how long she has been able to do this for, but sometime in the past couple of months, I would feel her popping it while we were cuddling at night. She also says that she does it in class when she feels bored (why twiddle your thumbs when you can dislocate your knee?). Then she showed her music teacher and class (poor Mr. N! he is a little sensitive about weird medical stuff), and he had her show the nurse, too. We kind of wrote it off as just one of those weird KayTar things (and musculoskeletal issues are soooo far down on the triage list around here), but as it was discussed more, I thought it might be at least a mention to her OT on Friday.

The verdict is: Nope! Not normal! And yup! It will cause damage and make her more prone to injury!

She said that if she keeps moving it that way, within a year or two she will start to have pain and she is likely to tear a ligament (ACL/PCL) and need surgery/bracing. She is going to talk to the PT and we'll start work on getting it tightened up and hopefully avoid pain/injury/surgery. Glad I asked!

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!

First Fever!

The germs have landed! Man, those little buggers are quick. Friday evening we drove out to my parents' country house to spend a couple of nights. Everyone had a good time (and I took a LOT of photos that I really love), but on the last day, KayTar started coughing...then she felt puny...then she wanted her oxygen in the daytime...then her throat started hurting. Uh-oh! I was hoping that it was allergy-related so I gave her a dose of Benadryl, as she helped with the hay early in the day, but it didn't quite seem like allergies.

We headed home not too long after and had to stop on the drive to give her a dose of ibuprofen for her throat and hook up/turn on her oxygen (per her request). The requests for ibuprofen continued through the day and night when her previous dose wore off, so it was clear we were dealing with an illness and not allergies...and then the fever started yesterday. Last night it was up to 103.5 with ibuprofen...poor baby was so hot and her heart rate was on the high side, too. Her usual overnight oxygen dose is 1/4 of a liter and I had her on a liter and her sats were still lower than they usually are on 1/4 and dipped much more frequently. Nothing serious, but definitely showed her little body is working harder than usual!

She is missing her first day of school for the year today. She is a little congested, has a junky cough, sore throat, and fever. Pretty sure it is just our first viral visitor of the year...hopefully it won't hang around too long and she can get back to the business of being a second grader!

Letter to the Teacher 2012

Hi! My name is KayTar! I'm so glad to be in your class this year! My mom helped me write this letter to tell you a little bit about me that might help our year go more smoothly.

I am an excellent reader. I’m almost done reading the Harry Potter series and I’ve read it all by myself! I learn very well from written word, especially because I don't always hear verbal words or instructions as well. When I watch TV at home, I like the captions to be on, so I can follow what is happening. I think it would help me a lot if my reading skills were used in class to help me keep up with what is happening in our room! Sometimes I get distracted or forget what I am supposed to be doing, so my other teachers have provided written instructions for classroom tasks and schedules and it helps me a lot.

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I can't hear with my left ear. I used to wear a hearing aid, but my hearing got worse and it stopped helping. Because I don't hear as well without it, make sure you speak clearly to me. If you are helping me do something, stay on my right side. It is hard for me to filter out background noises and I might have a harder time focusing, especially if the room is noisy. This is another reason it helps me to have written instructions. My audiologist wants me to use an FM system in the classroom this year.

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I wear glasses to help me see clearly. I am nearsighted, so my glasses help me to see things that are farther away. I like to be very close to what I am looking at usually, because my eyes work best when I am up close.

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I am a little slower than my friends and I get tired easily if we are walking long distances so I might need extra help. I use a wheelchair for long distances, so if we go on a field trip I will need it. Sometimes I fall down, because I'm not always steady on my feet, especially when I am distracted. I might have a difficult time keeping up with the class when we have a fire drill and I might need 1 on 1 help from a grown-up when that happens. The muscles in my hands get fatigued and weaken easily, so I have special modifications in this area. My doctors think I have a disease that causes me to be weaker than my friends and causes me to run out energy much quicker than they do, called mitochondrial disease. It may seem like I am being lazy at times, but my body just cannot always keep up. Even when I seem to be keeping up on the outside, sometimes my body is slowing down on the inside because it can’t keep up with all of the energy demands, so my mom and dad try to make sure I don’t burn too much energy on “outside” tasks so my body can use more energy for “inside” tasks and keep me feeling better. If I get very run down or tired, I may need to rest in the nurse's office or have my glucose checked. [The nurse] is very familiar with my medical needs and can help you make decisions about how best to care for me at school. I get tired much quicker when I'm even a little bit sick and there may be times I need to sit out of PE during the year. If I ever complain of being dizzy or wobbly or say my legs aren't working, I need to see the nurse right away.

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I am very sensitive to heat and cannot be outside for very long in the heat of the summer months. While it is so hot out (above 90 degrees), it is best if I do not do recess or outdoor activities in PE and I will need a quiet indoor activity while my class is outside. Sometimes being out in the heat causes me to run a fever, even if I'm not sick. It also makes my stomach have a harder time working properly and can make me feel badly in other ways, too. Sometimes if I play too hard even when the temperature is nice, it might make my stomach feel sick.  It doesn’t always happen immediately, but my tummy gets very slow when it is too tired and it makes me feel bad.

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Some days throughout the year, I am very light sensitive and can't bear to be out in the sunlight. I have magic lenses in my glasses to help, but some days they don't help enough. If my friends are going outside and the sunlight is bothering me a lot, I might need to go sit in the nurse's office or with another class while my friends play outside. Other days it doesn't bother me as much and I enjoy playing outside with my friends very much!

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I get migraine headaches and if I ever complain of head or eye pains, or changes to my vision, send me to the nurse immediately for medication, even if it doesn't seem to be keeping me from participating. It is important I get my medicine at the beginning, so they do not get much worse.

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I have asthma and if you notice me coughing a lot or coughing so hard that I gag/vomit, I need to be given 4 puffs from my Albuterol inhaler with my yellow spacer mask. I have some other breathing problems and I wear oxygen at nighttime.

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I don't like to eat much, so at breakfast or lunch I might choose not to eat. My mom will send something that I like every day, just in case. The textures of some foods might make me gag, either by sight or touch. If it gets too close to me I might push it away from me to protect myself. Sometimes just thinking about these things might make me feel sick. I have a g-button and a special feeding backpack that give me all the things I need to be strong and healthy. I also need [the nurse] to check my glucose to be sure my body is getting enough of what it needs. There are times when my tummy just doesn't work right and I vomit even when I'm not sick. [The nurse] knows how to help me and will call my mom if she thinks I may need to go home, but many times I finish the school day even if my tummy is acting funny. If I say I feel too full or like I might throw up, send me to the nurse quickly and she can help me feel better by venting my tummy. I also need to keep my water with me at my desk so I can take small sips and get enough fluids during the day to help prevent headaches and other symptoms from getting worse.

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My lips and tongue have gotten VERY big a few times and my doctors aren't sure why this happens,  if you notice my lips swelling, or if I say, "My tongue hurts." it means I'm having an allergic reaction. Take me to [the nurse] immediately. I need to have 2 teaspoons of Benadryl per my g-button. If I have trouble breathing or stop breathing, I need to be given my EpiPen Jr. Call my mom immediately if I have signs of an allergic reaction.

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Like the rest of my GI tract, my colon doesn't work quite right and I have to take medicines to help it out, because of this, sometimes I have small bathroom accidents. [the nurse] will have supplies to handle this if it is an issue. When I go see her before lunch, she will remind me to try to use the bathroom to help prevent accidents. Rarely, I may need to wear pull-ups when I am having more trouble with this, but I don’t want my friends to know! I am on new medications that are helping this problem more, so I hope we won’t have to worry about it much this year!

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I know it sounds like I have a lot of things going on, but most school days go really well for me! I think EVERY day is the best day ever and I'm so excited to be in second grade this year. I love learning and I know I will have lots of fun in your class!

Your new student,
KayTar

You can find the letters for previous years here: 2011, 2010, 2009, 2008. 

[As an aside, I find it REALLY strange to call the nurse "the nurse" because she is SOOO much more than that to us these days!]

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!

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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.