Miss Vocal

Mira was talking up a storm today. When I mean 'talking' of course, what I really mean is vocalizing. Mira is non-verbal and non-communicative - she can express when she is hungry or upset by crying, but outside of that, it is often difficult to understand her intentions or needs, when she cannot express them through words or gesturing. This works both ways - when she is vocalizing in a positive manner. it is equally difficult to understand what is provoking it. What was ironic about all of her positive energy today, was the fact that she didn't want to spend any time in her chair. She started this morning by just babbling consistently, all morning long and up until lunch. She did this every time we put her in her bed, but whenever we transferred her to her chair, she stopped talking and started to get a little fussy. So, back in her bed she would go, and within a few minutes, was back to her chanting and kicking in her bed. This pattern continued pretty much all day. She did get a little irritable later in the afternoon, but nearly three bottles later, she perked up and was ready to race around the house. Just your typical late afternoon hunger pangs I guess. Right before dinner was the only time during the entire day, that she actually enjoyed being in her chair for a bit. We raced for about 20 minutes, then more vocalizing back in her bed. Again, I'm not sure why she was babbling so much today, but it certainly was nice to hear her be in a positive mood - she sure had a lot to say.

Strong Finish to the Week

Despite a rough start to the week, Mira slowly got back into the groove of being back at school and finished strong these past two days. She only had one brief fussy spell yesterday afternoon, otherwise has been vocal and active. Mira has been enjoying racing around the house in her chair around dinner time, especially tonight when she became super excited. It has been getting increasingly cold here, with the temperature supposed to drop down in the single digits tomorrow, so it looks like we will be trying to find creative things to do inside for the weekend. I tried putting the headphones on Mira last night, but she didn't seem too interested.

Positive Rebound + A Musical Experiment

Despite having a little bit of a rocky start, Mira had a much better day today. She woke up and was a little fussy after breakfast and as she was waiting for the bus, but she got over it pretty quickly. The report back from school was that while she wasn't overly enthusiastic about certain activities, she was tolerant about doing them. She didn't have any fussy spells during the day, nor on the bus ride home, which is more than we could hope for, considering the awful last 2 days she has had. She was very quiet the rest of the afternoon, taking a long nap before finally getting up for dinner. She even enjoyed racing around the house for awhile, which even prompted some babbling and vocalizing at times.

I tried a little experiment with Mira tonight, right before bedtime. I took Jonah's over-the-ear headphones (ones he often uses with the iPad) and put them on Mira, to have her listen to some quiet classical music. I figured Erik Satie piano music would be relaxing enough for her to handle. I assumed she would try pull the headphones off, as she doesn't care for people messing with her hair or have things on her head, but much to my surprise, she kept them on. After she had the music playing, she tilted her head back and started looking around the room a little. I played a few songs for her, maybe 15-20 minutes worth, and she was as quiet as can be. She wasn't really doing much, but she seemed to enjoy it or at least wasn't complaining about it. She let out a few yawns in her chair and quickly went to bed.

Rough Day Back at School

I'm really not sure if Mira was trying to tell us that she wasn't ready to go back to school after being off for several weeks, but if today was any indication of her disapproval, all I can say is 'message received'. Mira continued to be fussy and irritable this morning, ending up back in her bed after only a few minutes in her chair. Apparently, she was briefly content at school in the morning, but it quickly faded and she was crying the rest of the day. When Sarah got her off the bus, she had been wailing the entire way home. She wasn't happy about anything this evening either, thus she ended up going to bed around 6:30pm, probably exhausted from working up such a storm for the second day in a row. No seizures at home or at school, but plenty of irritability. Some days you just have to throw your hands up in the air and let it happen, because it all seems pointless to try and find relief.

Hold on to Your Hats, It's Going to be a Bumpy Ride

After three solid days of being in a positive, happy mood, the wheels fell off the wagon today, with the horses running off in separate directions over the hillside, never to be seen again. Mira was a complete mess all day today. She started off with breakfast looking happy and content, but after having a huge TC (tonic-clonic) in her bed mid-morning, everything took a turn. She began crying and wailing in her bed the rest of the morning and the entire afternoon. Nothing would calm her down - no amount of bottles, walking, or resting in her bed would help her decompress. The wailing subsided, albeit very briefly (as in 15 minutes) by the time I got home from work and she had a few bottles for dinner, enough to give her (and everyone else) a breather from the crying. Minutes later, she was back at it, tears streaming down her face, wailing at max capacity. We again tried walking, but she detested it, so we put her back in her bed, which completely set her over the edge, spiraling into an extended frenzy of crying and wailing. She went solid for about an hour, until we finally gave her a half tablet of Xanax, as Trazodone does nothing for her in these situations. She continued on for another 30 minutes and has just now, finally stopped. It's now 9:00pm and I'm sitting here thinking, what the f*^k happened?

This has always been, and I have no doubt continue to be, a bumpy ride. Mira had such a great stretch of awesome days and this feels like a cold bucket of ice water over your head. We didn't make any dietary, vitamin, or medication changes in the last 3 or 4 weeks. She isn't constipated. She doesn't seem overly tired. So what is it? Do we chalk it up to 'just being Mira' or 'welcome to catastrophic epilepsy' or what? Either way, going through this journey is so completely and utterly exhausting sometimes.

18,453

Mira wanted nothing but to keep moving yesterday. We went for a walk around the neighborhood and down to the store, which did burn quite a few steps. However, a majority of the time was spent yesterday doing laps with Mira in her chair, inside the house. I have figured out how to do a sort of extended, modified figure 8 in the house, by going from the kitchen, to the 'foyer' (which is basically where our dining room was, prior to the addition) into the living room, then back into the kitchen. We call it 'the track'. We have hardwood floors throughout the house, which makes it easy for us to push her around and make pinpoint turns, while avoiding cabinets, furniture, and low windows. Yesterday, we raced around for hours, since Mira was really enjoying it - she was vocalizing, kicking her feet, clapping her hands, and at times, even laughing. I didn't want to stop the momentum, so we just kept on walking. She had another TC-free day, with only a few visible lighting jerks - she was also in a great mood again, with the exception of bath time in the morning, which isn't unusual. Once that was over, she was happy the rest of the day.

Great Start for 2016

Mira has been doing very well to start off the year. She hasn't had a big TC (tonic-clonic) since last Tuesday, when she was having these awkward movements in the morning, that eventually led into 2 involved TCs. For the past three days however, she has only has the same periodic 'lightning' jerks - I don't know what else to call them. They don't look like her typical myoclonics (that she has had for years), as her body doesn't shift to the right and her head doesn't drop at all. They only last a second and they are very random. She is having maybe a dozen or so a day that we actually witness. She stopped having those morning myoclonics, which is awesome and hope it continues! Overall, since we have eliminated the toy and have (most likely) reached a plateau with the fluoxetine, we are going to sit back and see where this takes us. So far, she has gone from having 1-2 TCs a day to 1-2 a week, which is a substantial improvement. Her mood (knock on wood) has improved somewhat as well - I had posted earlier that she is having more good days than bad, which is still holding steady. Today in fact, she happily sat through lunch at one of our favorite restaurants, with the entire family, not making a peep. Go Mira!

The Year in Review

I have never been one for resolutions, but at the end of 2014, I did conjure up the idea that 2015 was going to be the 'year of simplification', and ironically, the idea held true all year long. Most of the time, the idea of simplifying efforts and focusing only on the important issues happened as a result of circumstances, rather than a conscious effort that I had to periodically remind myself of at specific calendar milestones.

Late in 2014, I had decided to dissolve my LLC at the end of the year, which I had established back in 2011. Trying to juggle residential projects on the evenings and weekends became very overwhelming at times - with the economy gaining momentum, in turn fueling homeowners' desire to expand or renovate. In our area, the houses are older (typically 50-75 years) and smaller, which has produced a steady surplus of work for me over the years - at times, more than I could handle. The original notion behind the LLC was to do projects for families and patients with special needs, but in retrospect, only a small fraction of work was actually coming from that idea. As the company moved forward, a majority of projects came from non-special needs referrals from past projects, the neighborhood, friends, and family. I ended up finishing off my last renovation in early 2015 and officially closed the books to focus on my day job. This was a huge step toward simplification.

Later this year, I also stepped down from my position on the board of directors for Bleeding Kansas, which is a non-profit group organized for the purpose of getting medicinal cannabis legalized here in the State of Kansas. Unfortunately, trying to devote additional time to the cause had become too much and I frankly felt guilty, for not being able to contribute to the level of other members on the board. There are some extremely devoted people here in the state, who are politically tied-in and driven to see that legalization becomes a reality. I am forever grateful for their efforts and they deserve someone who is capable of devoting as much to the cause as they are.

In terms of medication simplification, Sarah and I have always tried to have a 'less is more' attitude toward Mira's pharmaceutical trials, thus we tried a Lyrica wean at the end of 2014 and into 2015. If you read back in some of my prior posts in January of this year, you will come to realize just how much of a disaster it was. Mira was miserable, so we ended right back where we started. We did however, successfully wean Vimpat over several months in the fall, just finishing it up at the end of Novemeber. We still hold steady with this idea of keeping the pharmaceuticals to a minimum, as Mira has a horrendous track record with them. Ultimately, in exchange for the Vimpat, we are just getting settled in with fluoxetine, which right now, seems to be helping quite a bit. Yet, I do think her recent improved demeanor is a result of a combination of changes, which I will get into later.

The loss of her toy has been the most significant change we have made all year. Call it simplification, call it necessity, or whatever you like, the fact that we had to take her toy away from her permanently, has been bittersweet. It personally makes me sad, when you consider the fact that she has played with that toy nearly every day for the past 8+ years. We have heard that noise and those lights, nearly every day in the house since 2006. We have had a long relationship with it, from hunting high and low on Ebay countless times to snag reserves, to conjuring up new ways to keep it strapped to her easel, to burning through literally hundreds of Duracell AA batteries. In browsing prior posts, it appears we had been flirting with the idea of taking it away, since April of this year. It finally became so overwhelming for her, triggering seizures every time, that it had to be put on the shelf, permanently. Another strange and sadly nostalgic step toward simplification I suppose, but a step nonetheless.

While we did pursue a few testing avenues for Mira, most of it was ongoing and residual efforts from her being in a few genetic studies from years prior. We did move forward and received results from some testing from a CEP (Comprehensive Epilepsy Panel) through GeneDx, which yielded a heterozygous mutation in her TPP1 gene, which is apparently irrelevant, since she still has one functioning gene. I have read that even heterozygous issues are sometimes implicated in epilepsy, even following up with the idea with her neurologist, who is communicating with the genetic specialists at Children's Mercy. I also had the idea, back in February, that Mira should be tested for GAMT and a few other isolated metabolic screens throughout the year. Her levels turned out to be right on target on all of the tests, thus we put all of those ideas to rest as well. Other than routine neurology and endocrinology visits, this year has been fairly quiet on the testing front. Mira is still enrolled in the whole genome study at the hospital, but there won't be anything to report, unless they identify an issue in the future. Should something relevant to Mira's situation surface, they will queue her DNA up for testing, but it is all happening behind the scenes and we would be notified accordingly.

Simplicity does have its limitations. Throughout 2015, we have continued to battle with Mira's irritability and that's not not to say that we have been trying or that we have completely surrendered. I did a substantial amount of research on just irritability, particularly in regards to chemicals in the brain and how they affect depression and pain, which led me to serotonin. I have always had this idea that Mira's fussy demeanor has something to do with a deficiency of some sort in the brain, thus went down a rabbit hole of research regarding Pyridoxine Deficiency (for the third or fourth time) and thought it seemed logical to do a trial of B6, about mid-year. Pyridoxine is supposed to raise serotonin levels in the brain - just one of 150+ different functions in the body. We did see some fantastic results, about 45 days into the trial, where Mira became consistently vocal and animated, even keeping seizures to a minimum. My optimism eventually waned as she slipped back into her familiar neurological patterns. I still think there is something to this however and after discussing options with her neurologist, we opted to put her on an SSRI, which ended up being fluoxetine. It's too early to tell whether it is effective or not, since it has only been a month.

So what has changed over the past year? At first glance, looking at everything at a global level, it is difficult to tell. Mira still has seizures, she is still irritable, and we still don't have any solid options remaining in regards to helping her in either one of those departments. The State of Kansas is no closer to legalizing medicinal cannabis (thanks to one single person, Mary Pilcher-Cook, who is a atrocious senator and a horrible religious hypocrite), which would create a potential avenue for us to explore. We still continue to contemplate moving to a legal state, while serious at times (heavily researching Colorado and even taking a trip out there) to half-joking comments (such as, 'f*^k this, let's just pack up and move everyone to Los Angeles so we can all be outside most of the time') when Mira has a rough week. I still continue to read, research medical options and ideas, write the occasional letter to The White House, and hope that something, someday, might help Mira have a better quality of life.

However, if you look at everything on a micro level, Mira has had more good days than bad, at least lately (meaning over the past few weeks). In perusing old posts, I found it alarming just how many poor days Mira has had over the course of the last 12 months - how many wailing, inconsolable days she has had. It has always been a viscous cycle of seizures, sedation, and irritability, but a few things are starting to change, just within the past month. Since we have permanently retired her toy, Mira has had more positive days than usual - I haven't seen any morning myoclonics for the past two weeks, which is something. I am convinced that there isn't one single, solitary medication, therapy, diet modification, or natural remedy that will control Mira's seizures, but rather a combination of changes that will help. Over the past two weeks, we have made some fairly drastic changes with her - removing the toy, fully weaning her off of Vimpat, and added an SSRI. The optimistic side of me is hoping that some magic combination might help - the pessimistic side of me continues to chant a familiar mantra, which is 'the more things change, the more they stay the same'. Only time will tell.

Thank you all for continuing to read and follow Mira's blog. It is nice to know that we are not in this boat alone and that we have wonderful family, friends, and support around the world. I wish you all of the best for 2016.

Mostly Happy, with a Chance of Seizures

Something strange has been happening with Mira for the past several days - she has been happy, nearly the entire time, since I last posted. Up until this morning, she hasn't had a big seizure (which I will get into later), but what has been most noticeable, is her quiet and subdued demeanor. She hasn't necessarily been overly energetic or excited, just content and going with the flow. Most days, Mira has objected to something every day (often multiple times or all-day long), whether she is hungry, tired, or whatever the issue may be - she would certainly vocalize her opinion. The past few days however, she hasn't really fussed once, about anything. It has been a little surreal. We have been running around shopping, eating out, and even headed down to Union Station to see the trains, and Mira didn't complain a single time. Even with all of the noise, transfers in and out of her chair, and having her sleep schedule thrown off some, she has been very chilled about it all. She has been a real trooper these days. She did wake up this morning and had some strange repetitive movements - they didn't look like seizures, but they eventually led into a big tonic-clonic. We couldn't exactly tell what they were, as she kept groaning and sort of almost laughing at times, but she kept doing it every 4 or 5 seconds. The cycle of moaning and flexing/tensing her muscles repetitively, went on for about 45 minutes, then ultimately went into a big seizure. We have seen similar episodes (particularly this year we have seen an increase) but this episode seemed different than the others. She ended up having another big seizure right after lunch. Other than the 2 tonic-clonics, she is still having these random, quick lightning jerks throughout the day. They last just a second, almost like startle reflexes - she is having maybe 10-20 a day that I can see.

Uneventful, Yet Very Positive and Active Day

Mira did another 180 degree turn again today, staying happy as can be all day long. Not 24 hours prior, she was wailing at the top of her lungs and today, she was all smiles. She has been all over the map for the past week, so we never know what to expect when she gets up in the morning. She was full of energy from the second she woke up, flapping her arms, vocalizing, and kicking her feet. She took a few catnaps throughout the day and each time, woke up scanning the room and kicking her feet. Mira even withstood some errands in the afternoon and getting rained on, as we had to sprint back to the car on our last trip. In her chair, she kept doing this cycle of leaning forward, putting her legs up on the front of her seat, then slowly slouches down under her lap belt, which usually gets her wedged down so that the belt ends up around her stomach. We typically leave her chest harness off about 98% of the time, only using it when she is on the bus or in a really crowded place where we are concerned about her lunging forward and hitting something. Even when she has the harness on and the lap belt is nice and snug, she can still manage to slouch her way down toward the end of her seat. It's much easier to keep the belt loose, so that we don't have to unlatch it, pull her back up, then redo it every time. Either way, she still has to be re-positioned back in the chair - she only does this when she is really active, like today. Her volatile demeanor these days is definitely keeping Sarah and I on our toes. We shall see what tomorrow brings!

Merry Christmas!

If you would have asked me yesterday how I thought our Christmas was going to unfold, I would have most likely pasted together some optimistic adjectives, based on Mira's spectacular day. She was happy and content, focusing on people at times that came in the room, and even braving an after-dinner van ride to go and see Christmas lights on the Plaza. She was alert and happy the entire day, not fussing once. Today however, she was singing a completely different tune. Much like earlier in the week, she was wailing, irritable, crying, and just not wanting to do anything. She spent most of the day in her bed, since she hated being in her chair, every single time we tried to put her in it. We tried to keep her involved in the festivities, which mainly involved cooking, but she wanted no part of it. She didn't sleep much, but did have a serious appetite throughout the day. No seizures or any seizure activity all day, but plenty of crying.

The boys were up at 6:00am and were ripping open presents within 30 minutes. Having to sit an entire half-hour always seems like an eternity to have to wait when you are a kid. I need to have at least one cup of coffee in my system before I can properly function, and the boys were eager to help me moving in the right direction. Mira was not interested in being a part of the gift opening, voicing her disapproval very loudly right off the bat, so she ended up back in her bed. We tried throughout the morning to rouse her interest in something, whether it be racing around the house, listening to music, or having a bottle. It was far too cold to go for a walk, so unfortunately, Mira's choices were limited. Despite Mira's fussing, we managed to have a nice Christmas. Sarah replicated much of her spectacular Thanksgiving meal, while substituting a small chicken for a turkey. Turkey is overrated anyway, so we mixed it up. Once you have had a taste of stuffing in November, it gets you craving it again by the time Christmas rolls around. Thankfully, there are plenty of leftovers - the gift that keeps on giving. We ate mid-afternoon and were basically semi-comatose within an hour, all sprawled out on our respective couches and chairs. I hope everyone had a nice holiday or a great day in general, wherever you were today!

Seizures and Bad Timing

It became apparent what had been bothering Mira for the past few days and it was definitely something neurological. She continued her pattern of becoming irritable soon after being out of bed, but today, she erupted into a big tonic-clonic right after breakfast. She followed it up with another one about 2 hours later. We let her recover after the first one, so we figured we would try and get her out of the house to run a few errands, but as I was transferring her from her chair into the van, she went into an involved seizure and for the first few seconds, I didn't realize she was actually having one. She started curling into a fetal position the second I lifted her out of her chair. Having a 70+ pound child in your arms who suddenly goes rigid and shifts their weight all to one side, completely throws off your equilibrium. It was just bad timing, as she lunged forward into the seizure, the instant I was lifting her to put her into her seat in the van. It caught me off guard - not so much so I thought I was going to drop her, but enough to have me work hard to bring her back to baseline and get her situated in the van, just temporarily so she was stable and sitting somewhere. For a second I considered just turning around with her and putting her back in her chair, but my first reaction was to pick the path of least resistance. This second seizure lasted about 45 seconds. Needless to say, Sarah and I divided our errands and conquered them individually, while one of us stayed home with Mira.