We are in the New York Times today!

Thursday afternoon after we got home from ice skating with some of our favorite folks (one of the fun summer things we've been up to lately), the doorbell rang. I said, "Who is it?" fully expecting it to be the neighbors saying our chickens had escaped again...or maybe a proselytizer of some sort. But the answer was, "Manny Fernandez. I'm a reporter with the New York Times looking for Kyla [Last Name]." I was shocked! Reporters from the New York Times do not show up on my doorstop every day out of the blue!

As you probably already know, the Supreme Court upheld the Affordable Care Act that day and it is a big news story. The reporter had been in our area because there is a high rate of uninsured here and he had seen an earlier article in the local paper about us and tracked me down. I spoke with him for a few minutes, but because it was just me and KayTar here, I couldn't do a whole lot of talking. I asked if he would email me his questions and I would respond that way and then we could touch base about anything else the following day and that is exactly what we did. Yesterday, the reporter and a photographer came back and we talked a bit more and got some photos.

I felt very comfortable with the interview and what I said, but I'm always nervous about how what I say might be edited and perhaps not stay true to what I intended to communicate, so I was anxious to see it this morning and woke up much too early to check on it. I *think* it came across really well. I definitely didn't read it and think "That's not what I meant!" Whew! It is online and in print today (page A11). KayTar is SO excited to have a picture of her kissing a sock monkey in the New York Times. When she heard the reporter and photographer were coming yesterday, she said, "I thought I was going to be a nobody, but I guess I'm going to be a SOMEBODY!" Haha! She cracks me up! She's ALWAYS been a somebody in my book!

Here are the links to the article and the audio blip from me.

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.

Living Well

I recently ordered/read Living Well with Mitochondrial Disease by Christy Balcells. At this point in time, KayTar is not diagnosed as having mitochondrial disease, although it is thought to be the most likely suspect for her medical issues. She is simply categorized as having a unspecified metabolic myopathy for now...however, I found a LOT of the information in the book applied to KayTar, diagnosis or no.

The book gives a great overview of what mitochondrial disease and good explanations (both simple and complex in different sections of the book) of the biochemistry of it. It gives a good overview of systems that tend to be affected (though, I noticed respiratory was not listed and I know many mito kids have issues with that) and what symptoms present most commonly. It spanned infancy to adulthood and diagnosis to palliative/hospice care. The only fault I can find with the book is that there was not a lot of information for what to DO about symptoms...gastroparesis is mentioned, but the testing, medications, and treatments for it are not explored in detail, for example. The book would likely be much too long if it went into all of these little details, but I think a more in-depth symptom management guide might be a helpful companion book to this one, if the author feels inclined to write another! The two portions that were most helpful for me were the section on hydration and creating/keeping a medical binder.

Appropriate levels of hydration are very important to people with mitochondrial disease...or with any metabolic deficiencies...or any of us as humans, really. It is MORE important when something isn't working quite right, though. According to the Holliday-Seger Formula for Maintenance Fluids that is provided in the book, KayTar should be getting 1540ml of fluid daily (based on her 22.2 kg weight) on a healthy, not overly hot day that she spends indoors. She only consistently gets about 960ml per day from her feeds/formula and I didn't realize she was getting sub-optimal fluid until now! She really struggled last summer and I wonder now if some of that was compounded by the heat and deficit in her fluids. So a few days ago, I decided to start tracking her PO intake a little better. I put a sippy cup of water in the fridge with 8 ounces in it and told her to get her drinks (as much or as little as she wanted) from that cup through the day instead of paper cups in the bathroom, ect, because I was going to measure how much she was drinking. She was VERY excited about it for some reason, so the intake her first day was WAY better than I thought. 10 ounces! I think the novelty of the situation had a bit of an impact...she said it was "like a video game". The second day she drank about the same PO, but she had to miss a whole feed because her gastroparesis was acting up (insurance is giving us crap about getting her prokinetic refilled). Yesterday was a more normal day, only 2 ounces PO. The novelty has officially worn off! Over 3 days, her highest fluid intake was 1260ml and her lowest was 1020ml. In case you don't speak in mls, she is roughly 8-16 ounces short on fluid every day. So we're going to have to figure out a way to bolus her with Gatorade throughout the day, perhaps it will help prevent the slump she was in last summer. (As an aside, I've been using the new MitoAction app to track her fluids, meds, symptoms...it isn't perfect, but it is super handy!)

The book recommends keeping a medical binder in both virtual and hard copy form...a good recommendation no matter what kind of medically complex kiddo you have! Page 1 should be a contact list, all physicians (all contacts for them, clinic, cell, fax, email...whatever you have), and 3 emergency contacts. Page 2 should be a copy of the front and back of current insurance card (and medical record #, if you happen to know it). Page 3 should be a current list of all medications (daily and PRN) with strengths, dosages, frequency. I used Google Docs for all of this because it is accessible from anywhere and I also printed a hard copy. The next sections are a bit trickier for us, so I had to make notes to get things from physicians at our next visits. The section after Medications should be Diagnosis. We don't have one! If you are like us, the book recommends getting copies of pertinent testing and results (that are applicable to the possible dx) and a H&P from the referring doc or specialist that is investigating. I also stuck a copy of The Visual Guide to KayTar in there. The following section is Emergency Protocols. We don't have any right now, but I took a photo of the pediatrician's RX from KayTar's last MRI stating she needs D5 1/2NS rather than any other fluid when sedated, fasting, or in need of IV fluids, due to her metabolic disorder. It will be a placeholder until I get a standing order written up from the pediatrician on her letter head. After that comes Notes from Specialists, with most recent copies of clinic notes and test results. Then the final section is Hospitalization records, where you can keep discharge summaries. It seems to be a pretty thorough system (and is covered in a more detailed manner in the book) and I have some collecting to do before ours is in working order!

The book was definitely worth the purchase price (Amazon has the best price!) and I have obviously found information that is applicable to us now out here in No Diagnosis Land and information that might be applicable to us in the future as well. While it isn't as thorough in some areas as I would have liked, it gives a very good overview and a lot of excellent general information that applies to mitochondrial disease and conditions like it. It is written in a very reader-friendly way and I would feel comfortable passing it along to family members, friends, or people who work with KayTar to give them a better idea of what is going on with her...they wouldn't need to read all of it, of course, but some of the sections would be very informative for them regardless of how medically-savvy they are.

Deadville, Population: Lice

(KNOCK ON WOOD)

So we have still been dealing with lice. We did 1 treatment with RID, 1 treatment with Nix, picked through her hair frequently for 1-2 hours, used the fancy new comb, continued to treat the environment (washing her bedding every 1-2 days, vacuuming and spraying the couch and common areas), retreated her hair with Nix on Monday...and I was STILL finding live lice/nymphs at every check. We were making some progress (and keeping it from spreading), but not because of the medicated treatments. I emailed the pediatrician on Tuesday night to see when she recommends using the prescription grade stuff and she said NOW would be the time.

So yesterday she called Ovide in for us. Guys, it is SERIOUS stuff. It is the equivalent of the atomic bomb in the war against lice. It is malathion (an organophosphate) in a mixture of isopropyl alcohol, terpineol, dipentene, and pine needle oil. It says it is a lotion, but it is a clear liquid in a glass bottle (I think it might digest plastic if in contact too long). It is SMELLY. Oh my gosh, does it ever stink...like diesel fuel or turpentine...or diesel fuel AND turpentine. And it is flammable. I was a little reticent to use the hardcore stuff on KayTar, because she is so sensitive (especially where her skin and respiratory system are concerned), but the pediatrician said she has used it on her own kids and it has been effective in one treatment without any issues. I trust her judgment where KayTar is concerned and we were ready to be DONE with lice. (At the library on Monday, where they KNOW US because we are always in there, she announced in her precious and never quiet voice, "I wish I didn't have LICE!" There was simultaneous head-turning. It was mortifying!) So, we did it. Did I mention it smelled terrible? The whole house smelled. It had to stay on her head overnight, 8-12 hours, so we put an old towel over her pillow. Aside from the noxious fumes, it did not bother or irritate her, and she adjusted to the odor quickly enough. This morning we washed it out and I combed through her hair and didn't see ANYTHING living up there. This is the first day I haven't found something alive! Josh and I are going to recheck it tonight (we can be more thorough with 4 hands and 2 sets of eyes) and I'm fervently hoping we don't find any critters up there.


Things I have learned from this experience:

1. Lice are becoming increasingly resistant to OTC products. They call them SUPER LICE! I call them terribly annoying. If you see LIVE ADULT lice after a treatment with an OTC product, try a different one. If you still see LIVE ADULT lice after the second treatment, talk to your doctor about a prescription. If it the treatment was effective, there shouldn't be live adults left. Also, with Nix, don't use any conditioner or conditioner containing shampoo, it will decrease efficacy...so will swimming in a chlorinated pool.

2. The non-toxic suffocation methods are not effective at killing lice because lice can close their spiracles (breathing holes) and become dormant until the suffocation hazard passes. These methods can making combing through easier, but won't actually kill anything.  

3. You don't have to go crazy with cleaning the environment. Washing/drying (on highest heat) all clothes/bedding/towels that can be washed that the "infested" person has used should be sufficient. If it can't be washed, just dry it on high for 30+ minutes. If it can't be dried, then vacuum it well, regularly. No need to keep things bagged for weeks if you can wash/dry or simply dry them. We did frequently re-wash/re-dry KayTar's bedding and vacuum her floor and the couch and her carseat often, too. We washed all laundry on hot during this fiasco.

4. Nit-picking is VERY time-consuming and most effective when 2 people work together on it. 4 eyes and 4 hands are considerably better than 2. Nits are almost impossibly tiny. 

5. The Nit Terminator comb is worth the $10 on Amazon, but still not as good as eyes and fingers. 

6. Environmental transmission is somewhat unlikely. Lice eggs do not hatch without the incubator-temps of the human scalp. Live lice die within 24-48 hours of leaving the head. Head to head contact is the most likely source of infestation. 

Lice still give me the creeps, but not nearly as much as they did on day 1! The title of this post is a direct quote from KayTar. She also said, "I hope my head has enough tiny coffins for them all!" I love that kid.

Light Bulb Moment.

A few weeks ago, the light in the bathroom burned out. After the kids were in bed, I went in the bathroom to take my shower and suddenly remember that the light had burned out, so I asked Josh to change it (he can reach it without standing on something). He obliged, and as he stood there doing me a favor, I was struck by a rare opportunity. You see, our bathroom light is on the ceiling and has a glass fixture over it that is held in place by several small screws. To remove the cover, it take two hands...one to unscrew the screws and another to hold it up so it does not fall and break. So there my sweet husband was with two hands above his head, concentrating on the task at hand with the butt-end of a new light bulb in his mouth, wearing a t-shirt and his boxer shorts...and I thought, NOW is the perfect time to pants this guy. So I silently crept up behind him, gave his shorts a little tug, and ran! And poor Josh, he had no choice but to stand there and finish what he was doing, pants-less. When I came back in, there he was...still diligently working on the light fixture. I was, of course, laughing so hard that I was crying which made him start laughing, so now he was pants-less, hands above his head changing a light bulb, holding another light bulb in his mouth while laughing hysterically, and trying not to drop anything. It was a priceless moment, one that will bring a smile to my face and laughter to my heart for many years to come. Oh, and in case you are wondering, yes, I eventually helped him with his shorts...and no, he has not retaliated...YET! ;)

Everything is better with KayTar.

As I mentioned in the last post, we captured a louse in a Ziploc bag. Thursday night, after her bath, KayTar checked on him and discovered that our wee prisoner of war had died.

K: Oh, he was so cute! That's sad.  

Me: Uh oh, you aren't getting sentimental about the lice, are you? 

K: My head is probably like a really fun amusement park for them! 

Me: I bet it is a great place to raise a family; free food, lots of forest growth-- 

K: Great schools!! I think because they are drinking my blood, they are getting some of my brains. I bet they are really good at reading. There is probably a little [My] Elementary up there!"

It was SO hilarious. As much as I hate that she has lice, this proves that she can make ANYTHING entertaining! I told my mom the story and she said, "I can just imagine the little lice coming to the front of her hairline when she gets out the Goblet of Fire to read at night so they can read along." which is an image that is equal parts endearing and repulsive! KayTar can find the good in just about anything...even lice!

An Lousy First

After 6 years of having kids in school (and 4 additional years of daycare), it has finally happened. One of the 'Tars has the dreaded LICE. I wasn't going to even write about it because it seriously creeps me out, but all of our firsts, from the great to the terrible, are documented here, so it only seems fitting to immortalize this milestone with the written word, creeptastic as it may be.

I noticed KayTar was scratching her head quite a bit the last week of school, so I asked our favorite nurse to give her a once over. I hadn't seen anything when I looked, but she has a lot more head checking experience under her belt and I trust her eyes more than my own in this case. I waited anxiously to hear back and was SO relieved when she gave me the all clear. Except...on Saturday morning, I spotted one in KayTar's hair. AGH! And another. Then another. It was SO icky. I know that lice have nothing to do with hygiene or cleanliness, I do. I know they actually prefer clean heads because it is easier for them to latch on to non-greasy hair. I know that they don't transmit disease and are more of a nuisance than anything actually harmful. But regardless of that, there is something so squicky about them. Yuck! I've had to hand deliver bowel movements, treat pus-filled MRSA infections, clean vomit and feces more times than I can ever count, but THIS grosses me out the most. Thankfully, everyone else had clear heads when checked...but we're watching for itchy heads, that is for sure!

I stripped all the beds. I collected all pillows, towels, laundry, stuffed animals (oh LORD, the number of stuffed animals that girl owns!), and other washable upholstery...washed it on hot and dried it on hot. I learned from several reputable sites (AAP and CDC, for example) that if you can dry something on hot for 30 minutes, you don't have to leave it bagged for 2 weeks like most people think. We vacuumed and sprayed the carpet, couches, and other things that didn't fit in the washer and then treated KayTar with Rid and then picked through her hair. Even with the recent haircut, OH MY GOSH, she has a lot of hair. It is a huge task to search through it, takes almost 2 hours. The nit combs that come with the medication are worthless, you HAVE to use your fingers to get eggs out. I could really benefit from having 3 hands temporarily. I ordered a special "professional grade" comb yesterday, it is the one the local nit-picking salon uses...it had great Amazon reviews. We'll see how it actually works. I've been redrying her bed stuff daily on hot and rechecking her head every 1-2 days. I was happy to learn that nits that are no longer on someone's head (like if a strand of hair falls off with one attached), the eggs will not hatch...they need the body warmth to survive. Lice and their eggs cannot survive very long without a human head, so environmental transmission is unlikely. Monday, I found another live louse, so we treated her again with Nix this time per the instructions in the AAP article. Yesterday, I found another live one. I know that it is just missed eggs that are hatching and we just need to keep nit-picking and getting rid of them as we see them, but I really just want them to be gone already. Give me a metabolic or neuro problem any day over this...I feel much more capable of handling it!

KayTar is, of course, handling it like a champ. If life were fair, she'd get to skip over this ordeal since she has more than paid her dues in other ways...but nope, it is just one more thing for her to tough out. And as always, she is doing it with smiles, laughter, and a good attitude. At first, though, she was pretty grossed out, too, and blamed me a little for finding it, "If you had just let me get my milk and not wanted to do my hair, this never would have happened!" LOL! But then yesterday, as I was checking her hair AGAIN, I let out a exaggeratedly whiny, "I don't wanna do this anymoooooore!" and she said, "Well Mama, do you want to have to strip the beds and rewash EVERYTHING?" I mumbled, "No..." and she said, "Well, then we are just going to have to finish this." Yes, ma'am! I also captured the louse we found yesterday and put it in a Ziploc baggie so she could see it and she asked me if we could squish it. I told her they don't die very easily like that and it would die all by itself because it can't survive long without her head. She said, "Oh! So the lice are like Lord Voldemort and I'm like Professor Quirrell?" (reference to Harry Potter and Sorcerer's Stone). She is so clever. And then we had a good laugh because Voldemort was surviving on the back of Quirrell's head...just like a louse! Let's just hope these lice don't keep coming back like Voldemort did!

First [Real] Haircut!

On Friday, I took BubTar to get his hair cut for summer and while he was getting his done, KayTar had a sudden urge to get her hair cut, too. She wanted it SHORT, too. She had never had a real haircut, only little trims at home, so SHORT made my heart stop...so we compromised on keeping it long enough for a ponytail. It was still QUITE a haircut!

It went from this:

To this:

WHOA!

♥ Such a beautiful girl, I have! ♥

I took the one on the right and was reminded of this one from years ago!

I think they both look pretty awesome with their new 'dos!

First Day/Last Day

Catching Up

Yikes! It has been a while since I've written anything. Life has been pretty calm (and maybe even a little boring at times) so that might have something to do with it. Time to play catch up...again!

BubTar did, in fact, improve rapidly after he started antibiotics. He's been back to normal for quite a while now. Yay! KayTar never caught whatever he had, double yay!

KayTar had her follow-up with her neurologist at the Headache Clinic on the 21st and the doctor was very pleased with how she was doing, so we don't have to go back for a year! She said she wouldn't usually let someone go that long between visits, but she knows that I'm the type of parent who will make sure to contact her if there are any major changes in the meantime.

We got KayTar's liver labs back and they are still slightly off. Her AST was 43 (37 is the upper limit of normal) and her ALT was 66 (with an upper limit of 55). I thought they had been elevated at every check for a year, but I looked back through the blog and it has been TWO years. Time flies. Her GI nurse was out last week, so I haven't touched base with her about it yet. After 2 years of elevations, I don't know if they will want to investigate it further or just continue keeping an eye on it.

Today was KayTar's pulmonology follow up with Dr. J. I really, really like Dr. J, she is kind and thorough and I appreciate that! KayTar's imaging studies and labs from last visit all came back normal, but the mycoplasma panel was forgotten somehow. We had labs drawn for that today as well as labs for iron, BUN, creatinine, TSH, folate, B12, lactate, and pyruvate. Most of the labs are to investigate KayTar's new weird symptoms that started a few weeks back...she woke up complaining that her legs had been trembling and it woke her up. She vomited, along with it the first few times. I felt her legs trembling one of the times and it was like when you have a high fever and get the shakes from it, but only in her legs. Nobody has been very helpful with this particular symptom...the pediatrician wanted to order an EEG, but it still hasn't been ordered, and the neurologist just kind of said, "Huh." So I was glad that Dr. J is looking into it a bit deeper. I am wondering if it might be anxiety-related, but Dr. J seems to think that if it was anxiety, it probably wouldn't be starting during sleep and waking her, it would be happening more when she was already awake. So we'll see what these labs say and she will also be having a repeated sleep study (in Dr. J's lab this time) to see what we see.

The insurance has denied the request for KayTar's nuclear mitome testing, so we are starting the appeals process. The reason they listed truly cracked me up, "Additionally, after history, physical examination, pedigree analysis, genetic counseling, and completion of conventional diagnostic studies, a definitive diagnosis remains uncertain." No sh!t, Sherlock! Why do they think we want to run a highly specialized genetic study on her? Because all other avenues have failed to provide A DEFINITIVE DIAGNOSIS. Geniuses!

School is almost out! We are in the last week...can't wait for summer vacation to start! :) Hopefully, I will be a better blogger over the summer.