Thursday, December 31, 2015

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.

Tuesday, December 29, 2015

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.

Saturday, December 26, 2015

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!

Friday, December 25, 2015

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!

Wednesday, December 23, 2015

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.

Tuesday, December 22, 2015

A Whole New Level of Cranky

We aren't exactly sure what was going on with Mira all day today, but she was extremely irritable. This morning she was fine for a brief moment in her chair, but her mood turned sour for the rest of the morning. An hour later, it progressed into a full-on wailing at the top of her lungs, with intermediate whimpering and sobbing, for the entire morning. She finally quieted down right before lunch time, then took a nap for over four hours. Yes, a four hour nap. She finally woke up before dinner, had a repeat performance of wailing and crying for another hour or so, then when back to bed. She has been drinking plenty of fluids all day, doesn't have a fever, and no other visible symptoms, but obviously, something was seriously bothering her. She isn't constipated and her hands and feet were fine. No big seizure activity at all - some isolated 'lighting' jerks on occasion, but nothing else. She wanted nothing but to be back in her bed - no racing around the house or even walking around.

Sunday, December 20, 2015

The Winds of Change


Mira had a much better day today - a drastic change from yesterday. She didn't have a ton of patience after breakfast, but then again, that is pretty much the norm for her. She drank a fair amount and wanted to be back in her bed. Apparently, she didn't get enough sleep, because she ended up taking a 2-hour nap shortly thereafter. We only went for one walk today, which Mira was super excited about - kicking her feet and vocalizing most of the way down to the store. It was incredibly windy outside, so we didn't end up going out after lunch, but Mira didn't seem to mind. She was content hanging out with us watching a little football and cleaning up the house. She was really only fussy in the morning for a bit and then once late after dinner, but both times, it was very brief and probably because she was tired.

Saturday, December 19, 2015

Just Keep Walking

Mira has been irritable again today and the only remedy for her crankiness is going for walks. She was in a decent mood this morning for a few hours, but right after lunch, she ramped up the irritability. We went for our first long walk, down to the post office and the grocery store, then came home and she took a nap. After she woke up, she was cranky again and wanted nothing to eat, so we went out on another excursion, just around the neighborhood. We seem to do a fair amount of walking on the weekends, to try and break up her boredom or whatever is ailing her. It's always fun to track steps on my phone - after finally checking it late today, it seems today was an active day. Counting some errands this morning, I ended up at around 20,000 steps. Fortunately, the weather was tolerable and we were able to get outside and actually go for a few walks. It was a little chilly at 50 degrees, but we bundled Mira up and she was very enthusiastic about being outside in the sunshine. I'm crossing my fingers for similar weather tomorrow.

Friday, December 18, 2015

Rough Week Ending

Mira has been really off the last few days, riding the roller coaster up and down, irritability wise. She has been having really big seizures at school too - one Wednesday and one on Thursday, both in the afternoon, both around the same time. Despite not having the toy, she is still having seizures, which isn't a surprise, I suppose. We haven't seen any involved tonic-clonics at home for nearly a week, but obviously, her toy is not the primary issue. She still finds a way to have seizures. The last three days, she has come home from school either sleepy or irritable, sometimes both. Today was her last day at school until after the new year, but it wasn't a good day - she was fussy off and on for the entire morning and by the time she got home, she was a wreck. She must have been having stomach woes because she is having a ton of BMs lately too. Just a whole lot of poop - not sure where all of this activity is coming from (or whether it's the most riveting topic of discussion for her blog), but it's just reality. I hope this isn't a preview of what to expect for the next 17 days at home, as we are all destined to lose our sanity. There are some days I wish we lived somewhere warm, where we could at least occasionally shatter the inevitable cabin fever we will all soon experience, by getting outside and take Mira for a walk or something, just to get outside in the fresh air. Kansas City weather doesn't always cater to our needs - 25 degrees one day, 60 and raining the next day. Time to move to southern California for awhile.

Tuesday, December 15, 2015

Toy Withdrawal

Mira has been very up and down today. She was irritable when she woke up - no seizures and not even any myoclonics that I could see - just fussy. She has had a few 'lightning' jerks throughout the day, but hey were very brief and random. Her teacher said she had  good day at school - she was even giddy with excitement when it was time to go out for recess. She tolerated the Kidwalk at school and overall, had a decent day. This afternoon at home however, she has been on a roller coaster. At times she was wailing, with tears streaming down her face. She definitely misses her toy and is going through withdrawal not having it for the past few days. It's going to be a long road ahead.

Sunday, December 13, 2015

Rain and Sleep

It has been raining heavily here all day. which usually means one thing - our basement will flood, which it did, in full force today. We live in a 60+ year old ranch house, with foundation walls that leak like a sieve. It is a steady flow, never more than just surface water in a few corners of the house, but enough to be a headache. We put an addition on the house back in 2009, which doubled the size of our current footprint, but we built it with a crawl space foundation, in order to save money. It has not leaked a drop. Our existing foundation however, leaks through the walls, the floor, and everywhere in between. It has become a recurring problem when it rains heavily, which there isn't much we can afford to do about it right now, accept channel the water from the corners to the basement floor drain, and keep everything protected and off the floor. The basement is not finished, so there isn't much down there but storage bins. After 16 years of dealing with this, 2016 might be the time to fix it, if we can afford it. Being an architect, I sort of know what our options are from a structural standpoint - it just comes down to the level of invasiveness we want to take on and how much money do we want to pour into trying to remedy this.

With the rain comes sleep. Mira spent nearly the entire day, sleeping. Today was Day 1 without the toy and Mira voiced her objections a few times, but for the most part, she was completely out of it and in her bed. She woke up after a late lunch, had a few bottles, then went back to sleep. She did the same thing around 6:30pm, stayed up for about an hour, drank a few more bottles, then wanted back to bed. She wasn't up for racing around the house or for just hanging out in the living room with us.

Saturday, December 12, 2015

The End of an Era


All good things must come to an end. I was happy to give an update earlier with Mira not having any seizures for almost a week, mainly due to her not having her toy. As the week progressed, Mira became increasingly irritable, until we reached a breaking point and ultimately, gave it back to her. Much to my amazement, she didn't have a seizure the first two times we returned it to her. That was short-lived. The past several times we have re-introduced the toy, her myoclonics became more pronounced with each exposure, and in turn, prompted us to limit her time with it, the moment we felt things were becoming too intense for her. Everything came to a head this morning, when we gave her the toy and she instantly erupted into a huge seizure. Following the seizure, she was completely off for the rest of the day - clammy hands and feet, sweating, and tons of drooling. Unfortunately, the pattern has become so consistent and established over the past 6 months, that she can no longer play with the toy at all. We are forced to retire it permanently. I cannot believe we have reached the point in this journey where we have to keep something from her that has brought her so much entertainment and joy for the past 8+ years.

Wednesday, December 9, 2015

Caving to the Fuss

Typically, I am starting off every post with Mira's daily seizure count and frequency, but for now, I have nothing to report in terms of seizures. Mira has not had a major seizure since last Friday and not since we started the fluoxetine. She has had some brief morning myoclonics, but nothing more. However, with every medication alteration over the past 10+ years, there always seems to be a negative side to every positive checked box. Mira has been very irritable over the past three days - fussy at school and fussy at home. My first reaction was to chalk it up to the fluoxetine, but I think (and hope) it is something else. I think she misses her toy. As I posted on Sunday, there is the expectation from her that once she is settled in her chair, that toy needs to arrive in front of her on a silver platter (aka her tray) within the first five minutes or there will be trouble. She loves that toy.

Having gone 5 days without the toy and having to deal with a lot of fussing and anxiety, we relented late this afternoon and gave it back to her. I was expecting her to immediately launch into a seizure, but rather unexpectedly, she played non-stop for almost 90 minutes, without so much as a twitch. Mira was balling her eyes out and the second Sarah turned on the toy, her crying came to an immediate, abrupt stop. She stared at it with her mouth open as though she couldn't believe what she was hearing. Focused and determined, she gazed at it for a solid 30 seconds before reaching out to start playing. Five days is the longest stretch she has gone without that toy since it was first introduced to her in therapy, more than 8 years ago. I hope this isn't an isolated reintroduction with it, but history has proven that the toy is a consistent seizure trigger for her, which is the reason we took a break from it in the first place. After playing for 90 minutes solid, I gave her a bath, put her back in front of it, and she continued to play for another 20 minutes, but she was visibly worn out from playing. We will see if giving it back to her will help tomorrow. For tonight, it was the toy for the win.

Sunday, December 6, 2015

Interesting Day

Today was day 2 of starting the fluoxetine and day 2 for keeping Mira's toy on the shelf, thus it was an interesting, mixed day. Mira woke up having some subtle myoclonics, but they didn't last very long - only while I was getting her changed and dressed. She was in a fine mood for about 30 minutes in her chair for breakfast, but then became pretty fussy. I think there is this pattern that she has been accustomed to for so long, thus an expectation that she is going to have her toy in front of her. Disappointment sets in once she realizes it isn't coming. So, I put her back in her bed for about an hour, then a cycle of fussing, drinking bottles, racing in her chair, and going for walks in the neighborhood began, for the next 6 hours. Finally, about 4:00pm, she completely clammed up. She was in her chair, as calm as can be, just looming around the room. It was really strange. She was scanning the room, rubbing her hands together, not making a peep. She sat while we cooked and ate dinner, still not making a sound - wide awake and alert, just not objecting to just chilling out in her chair, hanging out with us. After dinner, I gave her a bath, which usually sends her into a tailspin of irritability, but she continued to be quiet as can be, still not opposing to anything she was subjected to, even the hair washing, which she typically gets really upset about. It was certainly an odd day, like a light was flipped on with her late in the afternoon. She went from needing to be constantly entertained, to being extremely chilled, just going with the flow. Other than the brief myoclonics this morning, she didn't have any other seizures the rest of the day, which makes 2 consecutive days without a tonic-clonic, which is fantastic! If she can't get used to not having the toy and if we can try to keep her busy with other distractions, while perhaps crossing our fingers with the fluoxetine, I hope we might see a significant reduction in seizures.

Saturday, December 5, 2015

Fluoxetine

We started Mira on fluoxetine today, which we thought was logical choice, seeing as we could keep an eye on her over the weekend. It is a once a day dosage and has a very long half-life, yet we were told that she would fairly drowsy the first few days. It takes a few weeks in the system to reach its potential efficacy. Starting her on fluoxetine was going with the hope that an SSRI might help her irritable disposition, which is something we deal nearly every day with Mira. I suggested trying it with her neurologist at her last appointment, to address her mood,  but also based this idea on the studies and trials I had read, which used SSRIs for seizure control - fluoxetine in particular. There are numerous abstracts regarding Dravet and SSRIs as well and if anyone is interested, you can search for them through PubMed. One of the more current (very generalized concept on it) is located here.

Mira did not have any seizures today, mainly because we kept her toy from her all day. She wasn't too happy about that, as she a few irritable spells and wanted to spend as little time as possible in her chair, after realizing she wasn't going to play with it. Each time we took her out of her bed, she lasted less than 15 minutes in her chair before she got upset. She did spend a fair amount of time napping and sleeping, but nothing out of the ordinary, especially for the weekend.


Friday, December 4, 2015

The Toy Hiatus

Jonah spent another day on the couch, but slowly broke out of his shell by the afternoon, even wanting to play some hide and seek after dinner. He didn't eat much all day, but finally had a reasonable amount to eat at the table. Eli continued to have chills and sweating throughout the day, taking several showers to try and cool off, when he was warm. By the end of the day, he seemed as though he was turning a corner as well. I hope this round of viruses is short-lived and really hope Mira stays clear of it. Mira was the only one who went to school for a full week, while the boys ended up getting another extended weekend, right after a 5-day stint for Thanksgiving break.

Speaking of school, Mira had a decent day at school again today, which was a nice turn of events from her irritable disposition earlier in the week. We have given some serious thought about abandoning her toy permanently, as it is triggering a seizure, every single time now. Six months ago I would have never through we would be at this crossroads, taking away the one thing that she truly engages with consistently. Unfortunately, it has become a real problem. This morning, she became fussy, probably because she was bored in her chair, so I tried the toy, and of course, she had a massive tonic-clonic within the first 10 seconds. It's too much of a gamble and it's heartbreaking to keep it from her, but it is too much stimulation and consistently a trigger for her now. We are going to take an extended hiatus from it and see where this all goes.

Thursday, December 3, 2015

1 out of 3

This week was fairly uneventful, up until this morning. After dinner last night, Jonah kept complaining that his stomach was bothering him, but couldn't elaborate much on exactly what was bothering him. At the same time, Eli was complaining that his head hurt and wasn't feeling well. They both went to bed at their respective bedtimes, then each woke up this morning to different ailments. Jonah had an upset stomach at some point during the night, as he threw up on the side of his bed, yet didn't bother to wake us up to help him - he just went back to bed. Eli woke up to chills and a fever of 102.3. Needless to say, they both stayed home from school, resting on the couch and taking it easy. Jonah hardly ate anything all day, but was able to catch up on a fair amount of Curious George episodes before dinner, then had another vomiting episode before he went to bed. We kept him up for awhile to make sure his stomach was a little more stable and to express to him the protocol for feeling ill in the middle of the night - please wake us up! Eli continued to shiver from chills on the couch, but his appetite remained intact, as he downed an entire pizza in less than 10 minutes at dinner. Ironically, Mira was the only one who went to school today. No fever, no vomiting. However, as history has proven, it's just a matter of time before the rest of us catch whatever stomach and/or flu-like bug(s) that are now floating around the house. I am crossing my fingers I don't get hit, as I have to travel to Canada again the beginning of next week.

Monday, November 30, 2015

Back to School

As I suspected, Mira had a pretty rough day at school today. She was not happy this morning getting up and her poor temperament continued all day. Her teacher said she was upset most of the day and was not interested in doing much of anything, except fussing. Her appetite remained strong, but she is obviously having a hard time adjusting to school after a 5 day break. She continued to be irritable up until dinner time, thus we gave the toy a try, which of course, was a mistake. We took it away after 30 seconds, when she started having a strong myoclonic. I raced around the house for a bit in her chair, which took the edge off for a few minutes - she then proceeded to get very drowsy, almost falling asleep in her chair when we finally stopped. Just a typical up and down day for Mira. It might take her a few days to get back into a groove.

Sunday, November 29, 2015

Drowsy and Irritable

Mira has been fairly drowsy today, yet periodically voicing her disapproval of certain things throughout the day today. She woke up this morning and was very cold, clammy, and reserved. She managed to not have any tonic-clonics the entire day, probably because we didn't put her toy in front of her once today, which added to her grumpiness. We tried once with the toy yesterday and she immediately went into a strong myoclonic within the first 5 seconds, so we have avoided it since then. Most of her day today involved taking naps, in her chair, in her bed, and at times, when she was having a bottle. She became irritable this afternoon before dinner and just wanted in her bed. Unfortunately, she needed to get up and have a bath, which she was very unhappy about. After bath time, she had another bottle and was back asleep within 10 minutes. Something tells me she isn't going to be happy about getting up early tomorrow morning for school, after being off since Tuesday.

Friday, November 27, 2015

Still Quiet

We had a very quiet and relaxing Thanksgiving yesterday, which was great. Sarah spent a ton of time on Wednesday preparing most of the food (with the exception of the turkey and stuffing) so that we would have a minimal amount of prep and actual cooking time on Thursday. Everything turned out to be so incredibly delicious, especially the stuffing - Sarah is the master at it.

Mira continued to be quiet most of the day yesterday and today. She has been having these periodic laughing episodes, which is music to our ears, but we have no idea what is provoking them - they are very random. She just laughs a little, usually in her bed - by the time we go in to see what she is up to, she stops. They only tend to last a few seconds, but again, always nice to hear her laughing. She continues to have about one tonic-clonic a day, but we have been keeping her toy exposure to a minimum. She didn't seem to miss it too much today, although she did get a little cranky this afternoon. We tried it, she had a coupe of strong myoclonics, so we immediately removed it. I was able to get a picture of her and it was perfect timing - I got the feeling she was looking at me at one point.

Today was Mira's last day of Vimpat and while we did see a steady increase in seizures the past coupld of weeks, the seizure activity seems to have come back down to a more 'normal' range. Sarah and I are going to discuss when to start the fluoxetine.

Tuesday, November 24, 2015

Very Quiet

The past couple of days, Mira has been really quiet and reserved. She has been averaging at least one big seizure at some point during the day. This morning she had one about 15 minutes before the bus arrived, which made her extremely tired all day at school. She had a similar day yesterday at school, without a big seizure. Both days she has been having some smaller seizures and head drops throughout the day, based on her teacher's note, but no major tonic-clonics. We have been avoiding her toy as much as possible, as it has been backfiring every time we think it is safe to give her - her lone seizure we witnessed today, was triggered by her toy. We thought she was going to get upset this evening when we didn't put it in front of her, but she was extremely quiet the entire time. She kept dozing off in her chair and when she wasn't napping, she was just quietly staring into space.

Sunday, November 22, 2015

Slight Improvement

Today was better than yesterday, if for nothing more than not having to administer trazodone. Mira didn't have near the number of twitches and seizures just 24 hours ago, but she did have a massive tonic-clonic about 1:00pm this afternoon. We managed to run some errands before the seizure, which Mira was excited about. Just getting out and about in Target was the highlight of the day. After the seizure, she spent the next few hours in her chair, recovering, as seen in the picture above. She slowly came around late afternoon, but she continued to be groggy the rest of this evening. I suppose the most interesting part of the day was this morning, when Mira was vocalizing quite a bit, just repeating this sort of moaning sound every few seconds. It went on for over an hour. It wasn't seizure activity, since she was scanning the room and flapping her hands every so often - I think she just had a lot to say.

Saturday, November 21, 2015

Rough Trazodone Day

Mira had a bad morning. She started twitching during breakfast, with some periodic 'lightning' jerks, that almost look like an extended startle reflex. Those progressed into some heavier myoclonics, then into a series of pre-tonic-clonics. She kept having this mixture of startles, myos, and near TCs for a solid hour. She had breaks between all of the seizures and her breathing was never altered between each one, but every 5 minutes or so, she would have some combination of seizure activity. This clustering of repetitive seizures is something we have become all too familiar with this year, this being at least the 4th or 5th time since April.

I think this activity has been going on for a few days now, we just hadn't witnessed it this heavily until this morning. I noticed a lot of eye fluttering and eye shifting yesterday during dinner, where she would turn her head, locking her gaze onto her left side, and her eyes would literally shift back and forth a few times. All of this would last about 5-10 seconds - all of it was obvious seizure activity, just a variation we have not seen for awhile.

All this being said, ended up giving her trazodone about mid-morning, because the seizures just would not stop. It kicked in about 45 minutes later and she was finally able to get some rest. She ended up sleeping for several hours, even only on a half-dosage. When she woke up, she was in a miserable mood - wailing and crying, so back in her chair she went to have something to eat. I get the feeling that some of the medications just give her a sort of pharmaceutical hangover, where she wakes up groggy, with a headache, and nauseous. She pushed back three bottles within 15 minutes and started crying to be back in her bed.

Overall, she has had a rough day. This morning, she stepped down on the Vimpat and is now 3/4 of the way through the wean. She is at such a low dosage, that it is probably not doing much of anything. As I had stated in my last post, we are definitely seeing an increase in seizures, but we want to wait until she is fully done with this medication before we start something else. We have literally completely run out of options with AEDs, as she has tried every available one on the market, thus we are trying something different once she is fully weaned, with a low dosage SSRI.

Thursday, November 19, 2015

The Balancing Act

Mira continues to have a consistent flow of seizure activity. She averaged 1-2 tonic-clonics every day this week and had hundreds of myoclonics, even without her toy near her. The irony is, with her weaning the Vimpat (she is almost 3/4 of the way through it) while she seems to be having more seizures, she is much more engaged at school. Her teacher said she is making great eye contact and is very focused at times. I have abandoned all hope that we will ever find a positive balance with medications, seizures, and a consistent level of engagement. It is obvious that with less pharmaceuticals in her system, she is more alert, but we are definitely seeing an increase in seizure activity, at least over this past week. She had a nice bath this evening and didn't complain once, but afterward, really wanted to go to bed.

Sunday, November 15, 2015

Irritable Day

Mira has had some bouts of irritability today. I think I jinxed it yesterday by saying that she has been quiet and subdued the past few days, since today, she was definitely off. She had an average morning - a bunch of myoclonics, a bottle, then wanted back in her bed after about an hour. Then she had a quick nap, waking up in a foul mood - wailing and crying, with tears streaming down her face. After a bath and a couple of bottles, her mood improved briefly. We then went for a walk, which Mira uttered her only laugh of the day, when the wind kicked up and blew her hair around for a few seconds. Later this afternoon, she was again really cranky and nothing satisfied her - she went to bed early and now sounds much happier.

Saturday, November 14, 2015

3,Then 2, Then 1

After Mira's really rough day earlier this week, having 3 multiple tonic-clonics and myoclonic episodes, I suppose she 'improved' slightly be the end of the week, by having only 2 big seizures on Thursday and only one yesterday. She had been fairly consistent with having at least one intense tonic-clonic per day and today was no exception. Mira has been very lethargic and tired all day today, napping at every opportunity. I had to wake her up on both outings today, first to the park for a while, then to run some errands this afternoon. Both trips generated a few moments of excitement for her, but overall, she has been very subdued and quiet. The drool she is producing is non-stop - she is like a faucet. We have resorted to her wearing a hand towel all of the time and when it isn't on her lap, we are constantly wiping her face. She has been drenching her clothes, especially when she is 90 degrees upright in her chair. She just reached the half-way point with the Vimpat wean and really, nothing has changed - it is difficult to gauge her seizure activity sometimes overall, but it doesn't seem any worse than it was a few weeks ago. Her demeanor is strange during the wean - I would expect that she might become more irritable and upset, but what we have witnessed is the exact opposite. She is withdrawn, silent, and tired all of the time. It's actually as though she is more sedated coming off of the medication.

Wednesday, November 11, 2015

3 is a Terrible Number

Mira could not last more than three seconds with her toy today. She was up early and full of energy this morning, but having so many myoclonics before I even got her out of her bed. One right after the next, for a solid 15 minutes, Mira endured so many seizures, all before 6:45am. I waited for another 15 minutes or so after they stopped, right up until the time she started to get fussy and really wanted her toy. I put it on her board and within three seconds, she burst into another string of myoclonics. I went through this scenario, after another 15-20 minute wait, and again, she erupted into another strong myoclonic within three seconds of having her toy in front of her. That was the end the toy for the day. To top it all off, at school, Mira had three intense full-blown tonic-clonic seizures, all within about 90 minutes. She had the first two were about 15 minutes apart, then she had another about an hour later. Her teacher said she was completely out of it the rest of the day. Three seconds; three myoclonic episodes; three tonic-clonics. Mira was so spent at the end of the day, she just fell asleep in her chair. She is exhausted and overwhelmed with seizures this week.

Monday, November 9, 2015

Back to 'Normal'

After Mira had one great day this weekend, she slipped back into her pattern of having at least one tonic-clonic a day, if not more. The past 2 days, she has woken up and had an intense seizure within 20 minutes of being in her chair. The one she had this morning set the tone for her for the remainder of the day - she was completely worn out and spent much of the day sleeping it off. She could barely stay awake through dinner, having zero energy before bed. The Vimpat wean doesn't appear to have much of an impact on her mood or her seizures, but she still has a long way to go.

Saturday, November 7, 2015

Signs of Energy

Mira had a positive day, which was a nice break from the past few weeks she has been having. She had some fussy spells, but we didn't see any seizures after this morning, and she was able to get some quality, uninterrupted stints with her toy. During one of her brief irritable spells this afternoon, we went out for a walk around the neighborhood, and Mira was very enthusiastic about it - she was moving a ton and kicking her feet the entire time. She even enjoyed racing around the house some -  not getting vocal so much, but at least showing signs of energy. She had an enormous appetite today for some reason too, finishing off multiple bottles at almost every sitting. She never refused a bottle until right before bed, when I think she was finally tired out and just wanted to sleep.

Friday, November 6, 2015

No Relief all Week

Mira has had a rough seizure week. She is consistently having 1-2 involved tonic-clonics a day, typically in the morning and late afternoon. This is on top of her steady stream of myoclonics that she has every day. She has been getting zero relief and still unable to play with her toy for any significant amount of time. She had one good stretch earlier this week for almost an hour, but every other time, she just has continuous myoclonics from it and we have to quickly take it away. We are continuing with the Vimpat wean, which has been a little rocky so far. Wednesday she had an awful day at school, with her teacher reporting she was very irritable all day. Earlier that morning, she had an enormous seizure, a long day at school, then had another intense seizure before dinner. As I've said before, it feels as though if she isn't having a seizure, she is recovering from one. Mira is also becoming so much less active at home. She doesn't seem to enjoy racing around the house at all and hasn't vocalized in weeks. She has become much more passive too, not really mustering the energy to do much of anything. Today she seemed very tired, slouching in her chair after dinner, just wanting to go to bed early - she just looks exhausted.

Tuesday, November 3, 2015

500,000 of Your Closest Friends





So in case you haven't heard, the Royals won the World Series, for the first time in 30 years. Just minutes after I hit the 'post' button on Mira's last blog entry on Sunday night, the Royals mounted yet another miraculous comeback in the 9th inning, then completely sealed the Mets' fate with 5 more runs in the 12th. It was truly an unbelievable season for the Royals, after a stellar year, just one season ago when they fell 90 feet short of winning the World Series against the Giants.

In anticipation for the celebration today, most, if not all school districts cancelled school for the day, which I think was a very wise decision. Had any buses tried to come within 10 miles of downtown, they would have been trapped. With a parade starting at noon, followed with a rally at Union Station at 2:00pm, the volume of traffic and fans completely shut the city down this morning. I didn't even attempt to go into work, as I had heard that Northbound I-35, which leads into the west side of downtown, was a parking lot starting at 9:00am this morning. People were literally abandoning/parking their cars on the side of the highway, since there was nowhere to go and traffic was at a complete standstill for hours. People were comparing this surreal scene to something straight out of The Walking Dead. Some had a 3-4 mile walk from the highway to Union Station, based on the pictures I saw. Sarah had considered taking the boys down to the parade and I was going to stay home with Mira, but she quickly reconsidered after discovering the early traffic reports. Around 500,000 people consumed the city - I have never seen anything like it. It was an astounding turnout for the Royals.

Sunday, November 1, 2015

Strange Days

We started the Vimpat wean yesterday morning and while we wouldn't expect to see any differences for another week or so, Mira has been a little off for the past 2 days, but really, she has been off for the past several weeks. She has been very quiet this weekend and sleeping quite a bit. The only time she is even remotely animated is when we are either going for a walk or running errands. Even when we are out and about, she takes every opportunity to sneak in a catnap, with her ongoing exhaustion clearly evident throughout the day. She took an extended 2 hour nap this morning, right after breakfast.

On a positive note, she was able to play with her toy some on both days, without having a tonic-clonic. She had a number of strong myoclonics throughout the day, even when she wasn't anywhere near her toy. When we were on our walk earlier today, she had a few random events, where she would have a myoclonic, then came back to baseline, reaching for her toy, as though it in front of her. I think she is so conditioned to having seizures in front of her toy, it's just a natural reaction for her. It's terrible and saddens me to see her do this - she has been having seizures for over 10 years now and her post-myoclonic reaction is to reach for her toy, after these isolated heavy myoclonics.

The other thing we have noticed over the past few weeks is Mira's drooling. She is almost like a faucet most of the day - we don't think it is sedation per se, since she has been at the same dosage medication wise, for a long time. In fact, we are anticipating less sedation, since she is actually moving down on the Vimpat. Overall, she is moving less, certainly vocalizing less, and drooling more, and we have no idea why. These are strange days. Chalk it up to daylight saving time, the colder weather, or simply exhaustion from relentless seizure activity. 

I am going to finish watching the Royals-Mets game. I'm not too optimistic about this one, with the Royals down 1-0 and the Mets have the bases loaded with nobody out. I have a feeling the series is coming back to Kauffman on Tuesday, which is fine by me.


Thursday, October 29, 2015

A Perpetual State of Exhaustion

Mira continues to be quiet and really out of it. Ever since the weekend, she has become increasingly withdrawn, having a dazed expression and seemingly unmotivated to do much of anything. Every time we try to put her toy in front of her, it provokes a seizure. For awhile, it was every other time, but now, it is just a given - it just simply triggers a seizure, every time. It feels as though if Mira isn't having a seizure, she is recovering from one. It is just a constant cycle of seizure activity, being post-ictal, and then a slow recovery, only to lead into another seizure. This cycle is keeping her in a perpetual state of exhaustion. We haven't made any adjustments to her medications yet, as we were waiting until the weekend to start. We want to be able to keep a close watch on her when we start the Vimpat wean.

Saturday, October 24, 2015

Quiet + Sleepy

Mira has been taking a lot of naps over the past few days. With the weather changing and it getting so dark so early, I think it is throwing her sleep patterns off. She has been waking up during the night too, which isn't unusual, but it it is causing her to sleep more during the day. It's difficult to say how often she is getting up and how long she is actually staying up. We ran a few errands around lunch time and Mira was dozing off and on, mainly whenever we were sitting still. She wasn't crabby during the errands, but had a number of brief fussy spells this afternoon. We have not made any medication changes as of yet, since we are waiting for the pharmacy to fill her revised Vimpat prescription. She will be on a liquid form of it during the wean, which we will most likely start midweek. We are going to wait until she is completely off of the Vimpat before we start the fluoxetine. We don't want to have more than one variable moving at a time.

Wednesday, October 21, 2015

Neurology Appointment

We had our standing neurology appointment today, which we have had on the books for almost 6 months. The clinic is so busy, that we typically have to set our future appointments 4-5 months out, right when the schedule officially opens. Trying to predict whether or not you might have a conflict that far in advance is pretty much impossible. You just have to make sure you don't cancel, otherwise, you are back in the queue, which means you probably have another 4-6 month wait to get back in. Did I mention the neurology clinic is busy?

We have been going to the neurology clinic for over 10 years now, seeing 4-5 different neurologists - we have been seeing our current neurologist for the past 4 years and we are very comfortable with him. He is direct, respectful, and knowledgeable. I have learned not to have any expectations with what we will discuss, as we often go sideways in the conversation. For a few years, it seemed each appointment ended up completely different than I imagined it would go, thus I just quit trying to speculate. Sarah and I had a few things we wanted to address at this appointment, including the efficacy (better yet, non-efficacy) of Vimpat, the potential of putting Mira on a SSRI (to potentially manage her irritability), getting into ophthalmology (since Mira has not had a vision screen in years), and to discuss the idea of a gluten-free diet.

Taking her off of Vimpat is a simple concept. We don't feel like it is doing anything for her seizures and the less medication she is on, the better. We dare not even consider taking her off of Lyrica - we have learned our lesson the hard way, twice. Both times it was a disaster. Weaning Vimpat will hopefully go smoothly. She will taper down over the next month and should her situation worsen, we will do the exact opposite and try moving her up. She is on a fairly low dosage so there is the potential to move up on it, yet based on the fact that she has failed so many trials, with a plethora of different medications (and dosages), with varied mechanisms of action, the chance of a Vimpat increase actually helping, is extremely slim. Her neurologist reinforced this to us and we understand the percentages. All pharmaceuticals are a gamble either way.

We also discussed putting Mira on a SSRI, which is a Selective Serotonin Reuptake Inhibitor, to try and make a dent in Mira's ongoing irritability. We have always battled her mood and instability, so trying a different approach is worth a shot. Her neurologist was very open to the idea and has a host of patients that use SSRIs to help, primarily with anxiety and not so much epilepsy. We collectively decided we are going to ease her onto a low dosage of fluoxetine. Her neurologist was able to witness her crankiness during the appointment, when Mira cried for the last 15 minutes, while we tried to carry on a conversation over her wailing and tears.

The final issue we discussed was the idea of putting Mira on a gluten-free diet. The response from her neurologist was that there is no evidence whatsoever that a GF diet helps with seizure control. He sees a lot of patients that are either currently trying it or have tried it in the past, but nearly all them have had limited success. Most try it for 6-12 months, seeing positive results after a few months, but then the efficacy of the diet tends to fade after awhile. Despite the lack of results and studies done on gluten-free diets and epilepsy, he said there is no harm in trying it. As I noted in my last post, Mira is basically on a GF diet, minus the protein powders. I was able to find a plant-based powder that is gluten-free, soy-free, chemical-free, and contains no artificial ingredients, which you would think would taste awful, but it actually tastes great. I mixed a scoop with a glass of rice milk and I thought it had an unexpected sweetness to it. The smell is very earthy, but the taste is fairly sweet, not grassy. Mira chugged three bottles of it yesterday and doesn't seem to disagree with the taste.

Mira is having a pretty typical week - same amount of seizures, same irritability. With all of the changes in medications that are coming up, we will be keeping a close eye on her. All the kids have a 4-day weekend, with parent-teacher conferences the rest of the week.

Saturday, October 17, 2015

Glutamine, Glutamic Acid, Gluten + the ALDS


This has been an interesting weekend to say the least. After getting some disappointing news at work about midday on Thursday, I was pleasantly surprised at the end of the day, by winning a pair of tickets to the first game of the ALDS at Kauffman Stadium. Sarah and I were lucky to find a sitter on such short notice (thanks grandma Helen) so we were able to go. The weather was great - a little brisk at 47 degrees when we left the K, but no rain. The seats turned out to be awesome, 6 rows back behind the 3rd base dugout. Even better was the fact that the Royals dominated the game, posting a 5-0 shutout, then followed it up today by having an unbelievable 5-run 7th inning to beat the Blue Jays this afternoon, taking a 2-0 lead in the series. This team, just like last year's lineup, continues to amaze me.

After spending a lot of nights either reading or trying to meet work deadlines, the Royals game was a welcome break. I know I have mentioned this in the past, but many of the best ideas and research I tend to follow up on, come from other parents, and sometimes, from people very close to me. Sarah, my wonderful wife, mentioned a few months ago, after an appointment she had with a new chiropractor, mentioned Celiac Disease (CD) for something to consider for Mira's seizures. The conversation with this new chiropractor took a detour at some point and ended up being a discussion more about Mira than Sarah, ironically. Sarah has mentioned CD in the past before and being my stubborn self,  had dismissed the notion at the time, that there was any connection between Mira's seizures and CD, based on what I had read in the past.

Needless to say, after Mira's continual woes with seizures over the past month, I went down the CD rabbit hole a few weeks ago. It is actually pretty simple in terms of CD - just avoid gluten. With a wide range of symptoms and degrees of gluten sensitivity, one can manage the symptoms by going on a strict gluten-free diet. One of the issues we consistently deal with is Mira's diet. It has always been a delicate balance of weight gain, constipation, and nutrition, usually with the first two items being of greatest concern.

Years ago, we started supplementing Mira's diet with Pediasure. For any of you out there who have looked at the price of a can of Pediasure, you would be shocked. It is outrageously expensive, considering the primary ingredient is sugar. Even supplementing with a single can a day, becomes very expensive. The main issue we had with it was that it caused significant constipation. We probably spent 6 months or so supplementing Mira's diet with it, back when she was around 2 or 3 years old, then used it periodically for the next few years. The cost and the intestinal issues it caused forced us to look at other options.

What we found to work better for getting calories in her, was protein powder. There are literally hundreds of different formulas for protein powders, for which I have always had a love-hate relationship with all of them, knowing what ingredients they contain. Some are better nutritionally than others, but overall, the ingredients are constant - whey protein isolate, soy isolate, or some combination thereof. mixed with varying levels of other amino acids and vitamins. Over the years, we tried striking a balance with Mira's weight by adding small amounts of powder to her bottles, which we have realized, after years of doing this, adds to her constipation woes. I love the fact that they provide her with calories to keep her weight on, but hate the questionable ingredients and additives. Many of these powders contain massive amounts of sugar, fructose, sweeteners, and often a host of other ingredients I can barely pronounce.

A couple of years ago, we stopped giving Mira these powders and over a period of 6-9 months and she lost a considerable amount of weight, so much so, that her neurologist and pediatrician raised some concerns, and suggested we see a dietitian, which we did. The recommendation was to put her back on some sort of calorie booster, which happened to be protein powder. We put her back on it, her weight came back up to a normal range, and here we are, another year or so later, still having tons of seizures. Again, I have always had my concerns about these powders, so I went back to Sarah's original idea of Celiac Disease and started reading all about gluten, glutamine, and glutamate. I have always questioned what these essential and non-essential amino acids do to the nervous system in particular - a few weeks ago, I started really diving into what each amino acids in a typical protein powder profile actually does.

It didn't take long to see what glutamine and glutamic acid (GA) do to the nervous system and what rolse they play in epilepsy. One of the most frightening articles I read however, was how glutamine is processed within the body and how it transports ammonia through the central nervous system (CNS). Basically, glutamine enters the mitochondria, wreaking havoc on the mitochondria functions within it - basically, it's toxic and based on this article, is proposed to ultimately cause hepatic encephalopathy. Since glutamine can be produced from glutamate by the body, it is considered nonessential. Introducing additional (or excessive) amounts of GA into the body can cause neurological flux, especially if there is a problem within the cycle. Glutamate cannot cross the blood brain barrier, so it processed in the CNS to create glutamine. Disruptions or defects in this cycle of processing within the CNS can be a cause of epilepsy, Alzheimer's, and other neurological issues/diseases.

Protein powders are loaded with GA, to the tune of 3-5 grams per serving, which is an astounding amount for the body to handle and again, if there is a defect in this cycle, it can have terrible effects on the CNS. Reading all of these articles and trying to understand the connection between glutamine, glutamate, and gluten has been eye-opening. If Mira does has an issue with getting additional amounts of glutamine and her body cannot properly process it or is processing it in the CNS and there is an issue, it could be causing significant neurological issues.

I'm trying to decipher how all of this may or may not affect Mira and her diet, but the bottom line is we should try avoiding glutamine and gluten as much as possible, to see if it might help her. There isn't any harm in trying Mira on a gluten-free diet. She is basically on it already, minus the protein powder. It has to go. The second issue to be concerned with is to get calories in her, without resorting to gluten. A gluten-free diet isn't all that restrictive in terms of food choices - we could increase calories through everything from coconut oil to sweet potatoes, or any other fruits and vegetables that might be heavier in calories. As a trial, I avoided the powder all day today and Mira did not have any tonic-clonics. She had a few myoclonics this morning and this afternoon, but nothing major. She was very vocal before bed, doing her ba-ba-ba song for about 10 or 15 minutes as we raced around the house in her chair. I haven't heard her vocalize that much in awhile.

We are still doing the B6, but I would love to hear from parents out there that have tried a gluten-free diet and if it had any effect on seizure control. If anyone has any additional information on Celiac Disease and epilepsy, I would also like to read more. Thank you Laura, for all of the information and of course, thank you to my amazing wife, who always has rational, positive ideas, while I am off deep in the weeds.

Tuesday, October 13, 2015

Lackluster Day

Mira had so many myoclonics this morning - just a barrage of seizure activity. We had to avoid the toy completely the entire day. When she woke up, she was twitchy, cold, clammy, and completely out of it. She had zero energy at school, taking naps off and on throughout the day. Her teacher said she wasn't interested in doing much of anything - she wasn't fussy, just tired and just looked sedated. She continued to show no energy the rest of the afternoon and through dinner. She drank a few bottles and had plenty to eat at school, but she was really unresponsive the entire day.

Monday, October 12, 2015

Rough Seizure Days



Mira had a rough weekend. Saturday she was reserved and quiet most of the day, not showing a lot of energy nor interest in much of anything. We ran a few errands and it was obvious that she was not feeling well. She wasn't having too many myoclonics and only had one bigger tonic-clonic the entire day. On Sunday however, someone flipped a switch in the morning and Mira was consistently irritable from the time she woke up, until the late afternoon. We went out to visit some friends down south, which broke the cycle of whatever was bothering her. She managed to get some uninterrupted time with her toy while we were visiting, however, it was short lived. Afterward, her toy continued to be a trigger about 50% of the time we tried it. We end up just hovering over her for the first 5 or 10 minutes we give it to her and if we see her myoclonics becoming more intense, we just take it away. Many times though, she explodes right into a seizure as soon as it is in front of her. 

This morning, I knew a big seizure was coming on, as soon as she was out of bed. She was happy, smiling, and full of energy, without so much as a twitch, which is often an indication that she is going to immediately have a seizure, especially when this happens in the morning. As expected, she immediately had a seizure when she was in her chair and for the next hour, she was unable to even keep her head up for more than a few seconds. It's all a terrible cycle these days with her seizures. Her drooling has drastically increased over the past few weeks too - probably due to the constant seizures, which are causing her to be very lethargic and listless for a good portion of the day. She ended up having another tonic-clonic before dinner, which caused her to be twitchy, cold, and only mildly responsive the rest of the evening.

Saturday, October 10, 2015

Controlled Substances

After battling seizures for over 10 years now, I definitely have a love-hate relationship with AEDs (antiepileptic drugs). Mira has tried so many over the years, that it all has just become a wash with what medication has what supposed mechanism of action or which one has the least side effects. One thing is for sure, no AED, by itself or in combination with any other AED, has ever stopped Mira's seizures. My love for the potential for medication is that they actually do help some children out there that are battling epilepsy. They may not work for Mira, but at east they are helping other kids.

My hate with AEDs is the method in which they are developed, run through clinical trials, marketed, and ultimately, used on children, often with little or no clinical research in regards to efficacy or safety. The pharmaceutical industry is consistently misguided in their efforts, often capitalizing on suffering patients and families like ours, all in the sake of profit. I won't digress into the latest news on OxyContin, nor again list the frightening side effects of Lyrica, Lamictal, or any of the other 15+ medications Mira has tried (or is currently taking) to help with her seizures. Most pharmaceuticals have a 'proposed' mechanism of action, but the fact that they clearly state in their prescribing information pamphlets that 'the method in which (insert medication name here) works has not been fully elucidated and is suggested to work in the following manner', is truly frightening. Not even the manufacturer, researcher, developer, nor the marketer knows how a particular AED actually works, they just assume and so long as it is documented that it is unproven, that is enough in the eyes of the FDA. It's all a guessing game, often with efficacy rates for seizure control only slightly better than a placebo. Pharmaceuticals are forced through clinical trials with this same approach: 'Well, it's slightly better than you taking nothing and it actually can create horrific side effects that are much worse than you taking nothing in the first place, but well, it will be fine, because we aren't really sure how it works anyway, so let's just see what happens. If something bad does actually happen, we will be held blameless, because well, we told you so right here in writing'.

All of this comes to light several times every, single month, whenever we have to get one of Mira's prescriptions filled. Today, in fact, we ran into a dilemma. Mira has been taking Vimpat for the past 18 months or so, which has had a negligible effect on her seizures and little effect on her irritable disposition. We continue to question why she is on it and in fact, it will be a topic of discussion in our neurology appointment coming up in a few weeks. The dilemma was that we only have 2 pills remaining and didn't realize she didn't have any remaining refills. The neurology clinic is obviously closed on the weekend and refuses to accept calls after hours. The only choice you have is to page the on-call neurologist through the main line and have them contact you, should there be a critical issue you need to deal with.

Is getting a prescription refill a critical issue? Normally no, since most pharmacies understand the importance of maintaining 'therapeutic' levels of medications in the system (especially AEDs) and can advance a few days worth to get you by until you can contact the prescribing physician. Unfortunately, you cannot do this with Vimpat, since it is a controlled substance. That's right. Straight out of the DEA's endless list of controlled substances is my daughter's Vimpat. Along side of your friendly neighborhood narcotics PCP, cocaine, LSD, and heroin, are the commonly marketed AEDs tranxene, clonazepam, clorazepate, diazepam, lorazepam, phenobarbital, and pregabalin.

Needless to say, the pharmacist cannot legally advance even one pill of a DEA controlled substance,  thus we had no choice but to call the hospital nurse line, who in turn called the on-call neurology nurse, who in turn contacted the on-call neurologist, who ultimately called in a prescription to our pharmacy, just so we could get a few days worth of Vimpat until Monday.

Why is my 10 year old child so easily prescribed a DEA controlled substance, with a questionable mechanism of action and nearly zero efficacy in terms of seizure control? Welcome to the American pharmaceutical industry. Why would giving her an oil extract from a plant put me in jail and have my children and family taken away from me? You can thank Senator Mary Pilcher-Cook for reinforcing that hypocritical, greed-infused logic here in the State of Kansas.

Wednesday, October 7, 2015

Same Trigger

Mira's toy continues to be hit or miss this week. She has some days where she plays solid with it for awhile, with no seizure activity whatsoever. Hours later, it will be the exact opposite - her toy is an instant trigger, sending her into an huge tonic-clonic. It's about 50/50 over the past few days. She has been doing well at school, with reports coming back that she is content and ironically, no seizures.

Saturday, October 3, 2015

10 Second Window

The picture above is from a few days ago, before Mira starting having her toy turn into a consistent seizure trigger. The past 2 days, Mira hasn't lasted more than 10 seconds with her toy in front of her, without erupting into an intense seizure. The window of opportunity to remove it before she has the seizure closes extremely quickly and we seem to miss it. We have tried 4 or 5 times since Thursday evening, at various times of the day and every time, the result is the same. We hate to keep it from her, since she loves it, but I feel as though we have to take a sabbatical for a while, at least until her seizures start to come back to baseline. She seems to be doing alright the rest of the time, when she isn't in front of her toy. A few random myoclonics here and there, but nothing to the degree when she starts playing.

Ironically, Mira has been doing a fair amount of babbling and vocalizing - unprovoked of course, but great to hear nonetheless. We even went through an entire dinner out, including a 30 minute wait to be seated, in a very crowded and noisy restaurant. Mira was amazing during all of it. Not a single whimper or cry and she was even smiling a bit at times. She finished off a couple of bottles during dinner and was content just kicking her legs and moving around in her chair. We couldn't have asked for anything more, except the boys not acting like, well, boys.

Thursday, October 1, 2015

Ready for Relief

It's been a long week for everyone. Mira has been having more seizures than usual, with today being probably the best day she has had all week, only having a few myoclonics this morning. She didn't have any tonic-clonics and has been in a positive, yet quiet mood most of the day. She had a fair amount of time with her toy this afternoon and she wasn't interrupted by her usual stream of head drops and seizures. It was nice to see her having some relief after a stressful week of seizures.

I have been putting in a fair amount of overtime at the office over the past two weeks, which hopefully my schedule will be returning to normal soon. With travel and juggling three different projects, September proved to be a tiring, blur of a month. It will be nice to be at home this weekend and not have to worry about deadlines, at least for a bit.

Mira has school pictures tomorrow, which is always a guess as to what sort of mood she will be in when it's her turn to be in front of the camera. Some years she is completely out of it, either slouched over or taking a nap. Other years she is just irritable, posing for the lens wearing the longest frown you can imagine. Whatever tomorrow brings, just know that she will be wearing the beautiful outfit she can, thanks to Sarah's great taste in fashion. 


Tuesday, September 29, 2015

More Heavy Seizures

Mira has been in a fairly decent mood the past few days, but she has been having a ton of seizures. She is still having countless myoclonics, especially in the mornings, and she is averaging 1-2 intense tonic-clonics every day this week. She has been having good days at school - some brief fussy spells, but for the most part, she is happy during the day. Her teacher has been reporting that she isn't having a whole lot of seizure activity at school, but we continue to see so many at home. She was very vocal and happy in her bed after school, but as soon as we got her in her chair, she started having continuous, strong myoclonics, that immediately progressed into a an intense tonic-clonic. I couldn't get her tray out from under her quick enough and the seizure had 'locked' her against the tray for the frist 5 or 10 seconds. I had to tilt her back in her chair, which took some of the weight off of the tray, and was able to unlock it and remove it, all during the first part of the seizure. Mira cannot catch a break these days and is getting zero relief from these constant seizures.

Sunday, September 27, 2015

So......Many......Myoclonics

Mira is having a ton of myoclonic seizures today. Her toy is the trigger with some of the clusters she is having, but other times, they are just coming from out of nowhere. She woke up and had some time with her toy, which immediately sent her into a cycle of steady myoclonics. The first 10-20 were intense, but they gradually eased up some, so she wasn't interrupted every 20 or 30 seconds by them. She even had a few clusters before she was even out of her bed.

We were out the door early to participate in the Whisper Walk, which Mira was enthusiastic about, for at least the first 45 minutes or so, then everything started to break down. I don't think we were walking fast enough or not creating an exciting enough experience for her, since by the end of it, Mira was irritable and ready to go home. The boys were the same - whining, sweating, and ready to be back in the van. All of the kids internal timers went off simultaneously, peaking at around 90 minutes of being out in the fresh air and sunshine. Apparently, that was all they could muster.

Again this afternoon, Mira continues to have more myoclonics, but intent on playing with her toy and doing nothing else. She is refusing to drink and wants nothing but to try and play uninterrupted. For now, at least she isn't crying - fingers crossed it will continue.

Friday, September 25, 2015

Great School Day

After ramping back up on the pyridoxine, Mira seems to be feeling so much better. She is still having some big tonic-clonics (averaging at least one a day) but her mood has improved drastically. She had a rough day Wednesday at school, but for the past two days, she has been doing really well. Her teacher said she was all smiles today, vocalizing, and doing a considerable amount of hand-clapping, which is really a stimming exercise for her. When Mira is active and excited, she will clap her hands two or three times in a row very quickly, then rub her ears with both hands, then rub her palms together, usually in that order. She will do this routine repeatedly, sometimes up to 10 or 15 minutes. She hasn't been doing it much for the last several weeks, until today.

We went for a nice walk after dinner, which got Mira excited for about 10 or 15 minutes - all arms and legs were going like windmills, until she exhausted herself after a few blocks. She must have been pretty busy and active all day at school, since her energy level was down by the time we got back to the house. Then again, she had a fairly intense seizure about an hour or so beforehand, which she seemed to recover from quickly at the time, but her stamina just wasn't there. I think it was overall a busy week for her and she may just be ready for a day to just relax.

Tuesday, September 22, 2015

Up + Down + Pyridoxine

After Mira's episode on Friday, she has been very up and down, but mainly down. She was pretty irritable over the weekend, falling back into her cycle of not being able to stay happy for more than a few moments. On top of that, she was having a ton of myoclonics, all weekend long. Ironically, her irritability lately seems to coincide with her weaning down on the pyridoxine, at least it feels that way. For weeks we were getting positive reports back from school, telling us how well Mira was doing and how she was content most of the time, which is atypical for Mira, yet the B6 was not doing anything for her seizures for the past month or two. So I guess rocking the boat, with moving her down on the pyridoxine for a few weeks was not the best move - it isn't doing much for her seizures, but it is helping a ton with her moodiness. Pyridoxine raises serotonin levels in the brain (to what degree in comparison to say an SSRI, I have no idea) so possibly it is helping her just generally improve her mood. So, as of yesterday, we moved back up to where we were a few weeks ago and already, she is more vocal and happier. She did very well at school too - the first positive report in a while. I am going to look into this possible serotonin connection further. There is an interesting investigation of the role serotonin plays in epilepsy located here.

Saturday, September 19, 2015

Another School Episode

Mira had another episode at school yesterday, where she was having multiple tonic-clonics within a short period of time, which prompted the her teacher and the school nurse to call us at home. We decided to pick her up again from school and take her back home, again contemplating whether or not we should administer Diastat. This is the second time in the last three weeks where we have had to pull her from school because of her seizures - this also marks the fourth or fifth of these episodes she has had in the last three months, which is a significant increase since this summer alone. Prior to this year, she has maybe had 2 or 3 in the last 8 or 9 years - the fact that she has had 5 in the last 4 months is disconcerting to say the least. We are pretty baffled as to why she is having so many of these episodes. We decreased her pyridoxine dosage well below a therapeutic range and we haven't made any other medication or dietary changes for nearly a year. Her weight has been steady over the past year or so as well, which probably rules out any potential need for an increase in her current medications. She is still taking Lyrica and Vimpat.

During her episode at school, Mira either bit her tongue during one of the seizures or she has lost another tooth. Her teacher notified us immediately that she saw some blood in her mouth right after the seizure, which leads me to believe she bit her tongue during one of them. We couldn't get her to drink anything until late in the day, as she continued to be postictal for hours after all of the seizures. We couldn't feel anything on her tongue, but she is missing one of her upper left teeth. She finally started to drink something and ended up discharging a lot of dried blood that was in her mouth, but we still couldn't figure out where it was coming from.

Yesterday was an exhausting day for her. This morning she seems to be feeling much better.

Tuesday, September 15, 2015

Trigger Toy

Mira has been having a number of big seizures over the past 2 days, all of which are being triggered by her playing with her toy. This morning she had a huge tonic-clonic, only after playing for around 30 seconds. She then had another right before dinner, with the culprit again being the toy. We have never been able to track her seizures and when she might have one, but what we do know is that once her toy starts becoming an issue, you have to have her take a sabbatical from it for a few days. She can go weeks without it causing her one bit of seizure activity, then turn around and have a week of it being a constant trigger. It's sad because she really enjoys playing with it and even gets upset when she is expecting it to be in front of her, only we can't give it to her, because we know it will provoke an intense seizure. Mira managed to have a great day at school, despite the seizure this morning. Her teacher said she was smiling and happy most of the day, even content being in her Kidwalk.

Sunday, September 13, 2015

Chewy Hands

Mira has been having a decent day today - only one brief fussy spell, but the rest of the day she has been smiling and happy. One of the things we are noticing, today in particular, is that she constantly has her hands in her mouth. A finger, her thumb, and sometimes 4 of her fingers at a time, in her mouth, which is creating a ton of drool. Her hands and arms have been covered in it today. Not sure if it is a stimming or sensory thing, but she usually isn't so consistent about it as she has been today. We will keep an eye on her teeth and gums to see if it might be a tooth that is bothering her - I doubt this is the case, as she doesn't seem to be in any pain. In terms of seizures, she has only had a couple of myoclonics all day and only when her toy was in front of her. We tried giving it to her on several occasions throughout the day and every time, without fail, it brought on some seizure activity. So, we are just avoiding it altogether.

Saturday, September 12, 2015

The Giggles

The remainder of the week proved to be fairly typical, in terms of Mira's seizures. After having her all-star weekend, the myoclonics resurfaced in full force, being fairly consistent throughout the rest of the week. I ended up having to travel to Nova Scotia early Thursday morning, not getting back until late last evening, which left Sarah to hold down the fort. Both of those days, Mira had a steady stream of myoclonics in the morning, but not so much at school. Her toy is still often the trigger for her these days, so we tend to limit her exposure to it, especially if she is twitchy and has cold feet. We have been using her feet and hands as sort of a temperature/seizure potential guide when giving her the toy, which seems to limit the likelihood that her myoclonics will progress into a full tonic-clonic. I have to admit though, that it feels like a guessing game most of the time,

Mira has been having a some spontaneous, unprovoked laughing/giggling episodes over the last few days, which is very much out of the ordinary, Sarah told me that a few times, usually late in the afternoon, she would just start laughing and cracking herself up - completely random events. I was able to get some solid giggling out of her just a bit ago - she was staring at the ceiling in her bed, just smiling away, periodically laughing and scanning the room.

It's a beautiful weekend here, ironically 15-20 degrees cooler than it was in Nova Scotia, so we are going to try and get outside and enjoy the weather. It's always a good opportunity to air out the house too. 

Tuesday, September 8, 2015

Three Great Days - One Bad One

After a fantastic Labor Day weekend and seeing a minimal amount of seizures, Mira woke up having a constant barrage of myoclonics. They were relentless this morning - she must have had 100 or so within an hour. It was inevitable that she was going to top it off with a huge tonic-clonic, which happened this morning, right after I went to work. Sarah said that she barely ate anything all day afterward, with the TC just wiping her out. Despite having a rough morning, she showed a little bit of energy late in the day, but her mood was again wiped out by another huge tonic-clonic right after her bath. Hopefully, tomorrow will be a better day for her.

Monday, September 7, 2015

A Nearly Perfect Weekend

Mira had another solid day yesterday, although less vocal and she did have 3 or 4 very slight myoclonics throughout the day. All things considered, she had a fantastic stretch of being nearly seizure free. We spent a ton of time walking, as Mira seemed to not want to stop being in motion. The weather over the past few days has been sweltering, with really high humidity, so most of the time, we were braving the temperatures and walking around the neighborhood. Jonah went with us on several occasions, sweating up a storm on his bike, as we walked until Mira was satisfied. She has really only been fussy this weekend when she was bored, especially at night. Both Saturday and Sunday nights, we tried putting her to bed at her usual time, but she refused, fussing until we put her back in her chair and either went for another walk or tried her toy again. The toy worked for a bit, but walking the neighborhood was her obvious preference. Yesterday evening, the walk drained her energy, as it was still in the 90's at 8:00pm. Unfortunately, it is now pouring rain here, so getting out to burn off some energy is going to have to happen indoors. There's only so much room in the house to race around in her chair, but we have managed to maximize the indoor 'walking track' and we have hardwoods throughout the entire main floor, which makes it easy to maneuver around obstacles.

This morning she is again full of energy and her vocalizing has returned. I saw a a host of subtle myoclonics (maybe 10-15) right after breakfast, although none of them were intense - most were very random and barely perceptible. I'll take it for now - three mornings without her standard clustering of myos is awesome. Despite the isolated myoclonics over the past 2 days, it's safe to say that Mira has won the seizure battle this weekend. Final score: Mira 2 Seizures 0.

Sunday, September 6, 2015

Great Seizure-Free Day

Mira had an awesome day yesterday. She woke up smiling and full of energy, never seeming to slow down the entire day. In the morning, she might have had what looked like some very subtle myoclonics, but frankly, it was difficult to tell, since she was moving around quite a bit. Much of her time was spent babbling and kicking her feet, as shown in the video above. She did this numerous times throughout the morning, shaking the entire kitchen at times. She was also very talkative, babbling for 45 minutes straight at one time while we were walking around the house. She had her cranky moments, I think which stemmed from her being bored, as she always seemed to get mad in her bed. In fact, we tried to put her to bed at her usual time after dinner, but Mira wanted nothing to do with that. She became very agitated, so we put her back in her chair to play with her toy for another 30 minutes, then raced around the house until she tired out.

Outside of the questionable myoclonics in the morning, I didn't see any seizure activity all day long. Her toy wasn't a trigger, she didn't have any head drops, ticks, and certainly not any tonic-clonics. Her happiness yesterday was directly related to her lack of seizures. It was obvious that she was so awake and focused, with nothing in her way to distract her. This morning so far, is turning out to be a similar to start to yesterday. Again, more questionable myoclonics, coupled with a lot of babbling and wanting to move around. It is so refreshing to see Mira have a solid day where she is not interrupted by constant neurological interference.