Saturday, December 31, 2016

The Year in Review

The more things change, the more they stay the same. In the overall picture of Mira's journey with epilepsy, 2016 was much like a lot of years prior. Fortunately, there weren't any big surprises and throughout her fight with seizures, much of Mira's days did not alter the course from years past. She still battled with the same issues she has always dealt with - uncontrolled seizures, irritability, episodes of dystonia, constipation, and maintaining weight. This year was no exception. Every day is an adventure with Mira and nothing is predictable.

I have to go back and look at all of my posts from the beginning of the year, just to jog my memory, as things are such a blur by the time December rolls around. Ironically, we are experiencing the same things we were almost 12 months ago, just in a slightly different fashion. The discovery of how poor Mira's vision was, was perhaps one of the biggest events of the year. Our relationship with the ophthalmology clinic at Children's Mercy has now been established, as we will be monitoring Mira's vision much more closely now. We have several appointments in early 2017, including an exam with ophthalmology, and another with a vision specialist through the school district, who offers an annual clinic for children with special needs.

Mira had numerous bouts with heavy seizure days - times when she either had multiple seizures, or dystonia, or both at the same time. A few of those days were particularly worrisome, where she was having countless seizures at home and at school, all within a short period of time. Perhaps one of the worst experiences Mira dealt with this year though perhaps, was when she had a UTI, back in February, where she was dealing with some serious pain. We had never heard her cry and scream with such force as we did then. Fortunately, it came to pass fairly quickly with antibiotics, but it sort of set the pace for the beginning of the year for her having a lot of seizure-filled days and dystonic episodes, especially in February and March.

A biopsy in May ruled out yet another potential diagnosis for Mira - the fact that she doesn't have NCL or any type of storage disease that might have been discovered. The actual procedure I think was the most traumatic part for her, but she was able to get through it ok. We have tried to minimize the amount of testing Mira has been subjected to and the biopsy seemed to be one of the more invasive tests she had to go through lately - she has had numerous EEGs, MRIs, spinal taps, and countless blood draws, but all of these are occurring much less frequently in the past 5 or 6 years. I can't remember the last EEG she has had, which is probably a good thing.

I can assure you that there are a few things we will leave behind in 2016 and at the top of the list are bananas. After Mira's horrible constipation bout with a single banana, we have all been forever scarred by the trauma, that one horrible piece of fruit can bring. Bananas are now off the menu. A couple of weeks ago I would have thought that we would finish our the year having not seen or heard the toy, as we nearly went through all of 2016, before giving it back to her. So far, it has been our savior, redirecting Mira's irritable disposition, albeit only temporarily sometimes, but enough to give everyone a break. Tonight in fact, she played with it for a solid hour while we were out at a friends' house, helping Mira combat some of her frustration and fussiness up until that point. She has been having some tough afternoons and the toy helps reel our sanity back in. One final issue we hope to leave behind in 2016 is Mira's relentless eye rubbing. We started off the year with her constantly rubbing her eyes, to the point where they were raw and red. For some reason, the amount of eye rubbing has reduced to almost zero over the past few months, even in the bright sunlight. Glasses on or glasses off, daylight or evening, it all seems to have disappeared - we haven't made any changes so I am unsure why she has stopped, but am hopeful that we can permanently leave that behind in 2016.

It's funny to read my last post at the end of last year. My mantra still holds true - the more things change, the more they stay the same. 2015 was the year of simplification and we did exactly that, but ironically, not much has changed with Mira's seizures or irritability in 2016. It didn't matter what we did or didn't do throughout these past twelve months, as nothing really seemed to change dramatically. I have no idea what 2017 has in store for us, but I am sure it will undoubtedly be an adventure. I wanted to thank everyone out there that has continued to read Mira's blog over the past 10 years. I hope everyone has a wonderful, prosperous, and seizure-free 2017.

Monday, December 26, 2016

Myoclonics

Mira has been having a ton of myoclonics and episodes today - she had a cluster of myos this morning (look a lot like classic infantile spasms, minus the eye fluttering) that went on for 30 minutes. We haven't seen that activity for a few weeks, so it caught us off guard a little. She continued to have a lot of startles throughout the day, at all times, with no real pattern to anything. Her exposure to the toy has been minimal today, since we had been seeing so much seizure activity. A few times today, she startled so hard that she knocked the bottle out of her mouth and sent it flying across the floor - it scares the crap out of me when that happens, and makes us walk on egg shells every time she has something to drink. We went for a walk this afternoon, which Mira was super excited about, but she continued to have these episodes while we were out - arm stiffening, going completely rigid, and favoring her left side, by slowly locking her position in that direction - her arms completely extended and completely unresponsive. These episodes happened all day long, with no apparent trigger. The two times she actually had her toy in front of her, we didn't see anything, so we don't think there was anything sparking seizure activity with her as a result of playing with it. If there's one thing that has been consistent about Mira's epilepsy, is that it has always been inconsistent - you never know what to expect, every single day.

Sunday, December 25, 2016

Merry Christmas!


Our day started at around 5:45am, when both of the boys woke us up, ready to open presents! Not surprising - Eli has been wanting to build his own computer for months, earning and saving money so he can slowly buy all of the parts he needs. His hope was that Christmas was going to get him one step closer to that goal, by hopefully getting additional cash to buy more parts. Jonah
has been talking about Minecraft toys and telescopes for a while as well. They both were not disappointed, as Santa delivered the goods this year. Mira got a ton of outfits, a new winter coat, a new Gap zip up jacket, and a huge pink cozy blanket. This is the third one that we have bought for her, since the boys tend to take her blankets and run off to their respective rooms with them. This one is all hers.

Mira has been having a decent week, up until today, where she became irritable most of the afternoon. The toy helped alleviate some of her crankiness, but her time with it has been fairly limited. She seems to be interested in it for less and less time - she tries to shove it off her tray after less than 10 minutes each time. We have been on quite a few walks, including today, to help break up the day and to take advantage of the spectacular weather. We went from below zero temps last week, to 62 degrees today - it has been crazy around Kansas City and the midwest for that matter. Overcast and breezy for Christmas made it feel like Northern Florida.

We won't be doing much this week but relaxing after a great day with family, cooking, and presents. Hope everyone had a Merry Christmas!

Sunday, December 18, 2016

Cautiously Optimistic

Mira woke up in a great mood this morning, as opposed to yesterday, where she was wailing and crying, most likely since she wasn't feeling well. Today was much better. She was energetic and smiling a lot all day. She kept clutching at her sweatshirt and her pants, pulling on them at random times throughout the day, but otherwise, she has been acting as she always has. Sarah and I have been cautiously giving her the toy to play with - only 2 times today, each for about 20 minutes. On both occasions, she was the one who wanted to stop playing, by trying to push it off the tray. Yet, after watching her both times, she didn't have a single twitch or jerk. However, she did have a few spasms in her bed, unrelated to her playing with the toy. I'm being cautiously optimistic about her having it, so we will continue to closely monitor her every time she is interacting with it.

Needless to say, I went on a quick hunt to have a backup toy for her and was extremely fortunate to find 2 of them on Ebay, both at a reasonable cost. I snatched them up quickly. We are talking about a toy that was produced only for one year, back in 1999, so trying to find this particular toy is a challenge. We have at least 7 or 8 of them at home, and all but one of them are non-functioning. The speakers go out, the board gets fried, or they just wear out. I felt I was extremely lucky yesterday, finding a few, 2 of which were listed under $50 each, when I saw a third one here on Amazon, for the ridiculous price of almost $300! There is some seriously overpriced ones out there, so I will continue to keep my eyes out for when the reasonable on come around.

Saturday, December 17, 2016

The Return of the Toy


Sarah and I have been debating 'the toy' as of late. As you may recall, we had to take Mira's toy from her about a year ago, as it was provoking relentless seizures every time it was in front of her. We felt terrible having to keep it from her, as it is the only toy she has consistently interacted with, and the fact that she had such a long history with it. Unfortunately, it slowly became a seizure trigger for her late last year, to the point it was directly causing her to erupt into a tonic-clonic the final 3 or 4 times we tried it. We have been talking about giving it back to her for the past few weeks, simply because we think she is really bored around the house. While she hasn't been in an irritable mood lately (the past 2 days being an exception because she is congested and has a fever) we felt that her options for entertainment are very limited, especially with the weather being so bad outside. It's currently 7 degrees, snowing, and the temperature is supposed to dip down to -3 degrees tonight. Obviously, going for a walk outdoors isn't an option and with Christmas vacation just around the corner, it inevitably is going to struggle finding things for Mira to do around the house. Thus, we tried the toy this afternoon, after a solid year of not having it in front of her.

Watching her like a hawk, we strapped it on her easel, slid the toy in front of her, and turned it on. It was like she had never missed a beat with it. She immediately grabbed the corner 'bulb', started smacking it with her palm, and pressed her face right up into it. Now that she has her glasses, hopefully she can actually focus on it much more easily. For the first few minutes, she would not take her eyes off of it. Her mouth was wide open, her expression (pics above) was priceless. She interacted consistently with it for a solid 20-30 minutes and didn't have so much as a twitch. No eye-fluttering, no seizures, no myoclonics. We watched her the entire time. It was a huge relief, but it certainly doesn't mean she won't have one from it in the future. This was only the first reintroduction - we put it in front of her again late this afternoon and she was equally enthusiastic about it, although she became bored after 20 or 30 minutes, and tried to shove it off her easel. Just like old times.

Needless to say, we are going to take this slow, as Sarah and I have some trepidation about it still. It makes us nervous with her in front of it, knowing what she went through late last year with it. Unfortunately, since last year, another one of the toys stopped working, so we are down to our last functioning toy. We are going to need to be on the lookout for a backup. If anyone spots one on Ebay or anywhere, please let me know!

Thursday, December 15, 2016

Great Stretch, Minus One Bad Day


Mira has been doing great this past week, with the exception of one rough day last Friday, which I will get into later. She has been in a positive mood, with a minimal amount of fussing, which is very odd for her to have such a long stretch of non-irritable days. Mira has been getting up full of energy (and lots of dragon noises) and we haven't been seeing any strong myoclonics in the mornings either. She continues to be surprisingly calm and quiet in the evenings, often taking long naps once she is off the bus and back in her bed. Her appetite has been very consistent - she seems to be enjoying the meal plan we have her on and she certainly appears to be putting on some weight, which is fantastic. Really, the only thing that we seem to be battling these days is the weather. It has been bitterly cold the past week or so, with temperatures hovering in the mid to low teens most days. It's supposed to dip into the single digits over the weekend, which means coming up with some creative things to do indoors. Mira has some super sweet pink mittens, so at least she can be fashionable in the cold.

In terms of her bad day, Mira had a multiple seizure day last Friday, having 2 at school and another one later in the evening. It completely wiped her out last weekend, where she was clammy, rigid, and lethargic (picture above), which is a fairly typical bout of dystonia and/or just being post-ictal from all of the seizure activity. However, those were the only seizures she has had in the past 10 days or so - again, her myoclonics have been negligible and she hasn't had any 'altered' days, other than the one last Friday.

On a side note, our van is slowly falling apart. Mira's side door a few days ago and is unable to fully open, which means we have to switch her car seat to the other side and have Jonah go through the same side. I absolutely detest reinstalling and adjusting Mira's car seat and it always feels like a major undertaking. It has so many attachments and belts, that it ultimately ends up being a 1-2 hour driveway event, at a minimum. Seeing as it's so frigidly cold, I might be trying to pull the van into our tiny single car garage to try and work on it. The door has been malfunctioning over the past week, and combined with the 7 month old battery dying last weekend, the 10 year old van is showing its age. We think all of the power door issues on Mira's side is what is draining the battery, so we will have to get it looked at next week to try and get it resolved. Add it to the list of things we weren't expecting to shell out money for over the holidays.

Tuesday, December 6, 2016

The Parental See-Saw

Parenting has a habit of being a ride. A bumpy, often tumultuous ride at that. The only analogy I can come up with a that it's like siting on a see-saw. The day-to-day activities of doing the same, seemingly predictable, motions - up and down, never really side to side so much. Night and day, Work and school. Driving back and forth. Preoccupied with the thousand other things you are trying to manage. Sometimes, you aren't paying enough attention to the person on the other end of the see saw, and they push their legs more forcefully off the ground, so you will take notice, as your rear end slams against the soil on the opposite end. Other times you are sky high, inches away from sliding off the seat, the threat of plummeting to the ground, ever near. You have to be constantly aware of everything that is happening on either end of that board, as one simple move sends everyone flying.

It has definitely been a see-saw the past week or so around our house. Mira has had some very positive moments, where she is doing constant 'dragons' and is as happy and energetic as can be. She has also had some fussing spells that seem to last for hours. Jeckyll and Hyde. Sarah and Jonah both have been hacking at a cough that won't seem to go away, with Jonah adding an on and off fever for the past week or two. Up and down. Eli has had his moments as well - typical teenager issues that we all remember dealing with at one point or another. All the kids have been taking a turn on the see-saw with Sarah and I tag-teaming it on the opposite side - sometimes it feels like all three of them are pushing off the ground as hard as they possibly can, just to see what happens. The parental see-saw is exhausting.

Mira actually had a very positive day at school. She was very attentive, tolerated the Kid-Walk for a solid half-hour, and was smiling all day. Her patience ended on the bus ride home, where the driver and para said she wailed the entire trip back. She did the same exact thing yesterday. By the time I got home from work, she was again all smiles - she even let loose a few 'dragons' during her bath. Her bus showed up early today, which I was unprepared for - this after being 15 minutes late yesterday.

No one can seem to find an equilibrium this week. The see-saw ride continues.

Thursday, December 1, 2016

The Thanksgiving Drive

Last week we made our second trip out to Kentucky, with a stop on the way out in St. Louis to visit my family for the evening. Embarking on a road trip or spending any significant amount of time in the car with Mira is always a gamble. We never know what the highway will bring. During our neurology visit the week before, our neurologist recommended a small dosage of valium while we were on the road, to help ease Mira's anxiety in the van. After our first trip out to Kentucky last summer, I think all of us lost a little bit of our collective sanity, having to listen to Mira's crying for a solid 225 miles on the out to KY. It was painful, thus we felt a little more empowered this trip, having some ammunition before we embarked on a 9-10 hour excursion in the car.

We weren't very optimistic heading out toward St. Louis, as we didn't get 3 miles from the house before Mira started crying. We stopped, fueled up, and gave Mira a dose of valium, which didn't seem to do too much - she continued to cry off and on for most of the ride. Tolerable, but certainly not ideal. We spent the evening and headed out early the following morning, with 4 hours under our belts and another 5+ hours to go. Mira did a little better, but ended up crying, somewhere around Louisville, and continued pretty much until we got out of the van. Once she was inside and able to stretch her legs, she was in a much better mood. In fact, she was extremely happy for the rest of the afternoon.

However, for the remainder of our visit, Mira was very irritable. Every day, Thanksgiving included, she was just not happy and nothing would keep her happy for more than a few minutes. Just one of those roller coaster weeks. We had to split up on a few outings each day - I ended up taking Mira back to the house on several occasions, while the rest of the crew went out to lunch. Mira would have not lasted more than a few minutes in the restaurant, which makes a stressful outing for everyone. She wasn't much happier once we were back out of the van either, so we were never sure what was really bothering her.

Regardless, we had a pleasant Thanksgiving break, loading up the van early Sunday morning to head back to KC. This being only our second attempt at driving straight through for 9+ hours, I was frankly cringing at the prospect of having Mira's irritable temperament continue the entire drive back. Much to everyone's amazement, Mira went an entire 600 miles without making a peep! She was truly amazing - she was wide awake and didn't sleep a wink for the drive, but rather was content just looking out the window or just happily enjoying the ride. She did break down that last 20 miles, but I was in shock that she went solid for almost 9 hours without so much as a whimper. We did give her a dosage of valium before we left KY and another about 5 hours into the drive, which seemed to do the trick.

Unfortunately, she has been a train wreck most of this week - she has been irritable every day since, finally settling down today and has become extremely sleepy this afternoon. With all of the travel and visits, her sleep patterns have been thrown off and I think she is just worn out. Ironically, in terms of seizures, we haven't seen any the last week to ten days. She hasn't been having any myoclonics in the morning and certainly no tonic-clonics to speak of, at home or at school. With all of her fussy days, we typically see a spike in seizure activity, but again, it has been eerily quiet in terms of seizures since my last seizure post back on November 9th.

Monday, November 21, 2016

Neurology

I know I've said it before, but Sarah and I have learned to not have any preconceived notions about how Mira's neurology appointment will end up going. In the past, we think we are going in to talk about one thing and end up getting spun 180 degrees and discussing something completely different. We have resigned to the fact that we have no idea what to expect - we have some questions and discussion points lined up, but typically end up only briefly touching on those, if at all.

However, her appointment on Thursday was actually one of the more straightforward meetings we have had over the years. Mira's weight certainly came up, despite putting on 4 pounds over the past month, she is still in the lower 20th percentile for weight. The diet we initiated late last month is starting to show - she is gaining weight and it is helping, but she has a ways to go. Her neurologist no longer seemed concerned about it - we have taken the necessary steps to boost her calories and we all think we are headed in the right direction with her. In terms of medications, we didn't make any changes, as her seizures seem to have decreased some since our last appointment back in May. Overall, Mira's demeanor has improved over the past few months, despite having some awful days here and there. That being said, we didn't think it was necessary to change anything from a medication standpoint.

We did have a discussion about pyridoxine, which was interesting. I have read extensively about it over the years and we have done trials with Mira in the past. I explained to her neuro that she does respond very positively to high dosages to pyridoxine - we have not discovered exactly why, but the only two clinical diagnoses that I am aware of that respond to high dosages are pyridoxine deficiency and homocystinuria, neither of which Mira has a genetic marker or indicator for. Yet, somehow she responds positively to it, thus we have been keeping her on dosage of it for a while. I tried weaning her off of it a few months ago and she was a complete irritable mess. There are concerns of peripheral neuropathy, but it is very rare. I am much less concerned about the side effects of pyridoxine, than of the countless pharmaceuticals she has tried over the years. Her neuro certainly wasn't against it and admitted that we were more of the experts on it than he was, but he was intrigued to understand and research more about it. It was refreshing to walk out of the appointment not having any major concerns, scheduled additional testing, or unanswered questions. I have a lot of respect for her neurologist - I think he is very open-minded and honest about everything - he always has been.

Wednesday, November 16, 2016

Neurology Tomorrow

It's been a fairly quiet week here since my last post. Mira has been in decent spirits, mixed with occasional bouts of crankiness, which are usually redirected through a long walk outside She hasn't really had any major tonic-clonics, but has been having days of twitching and random myoclonics, but nothing significant. We finally heard back from the seating clinic, after almost two weeks. Based on the conversation that Sarah had with them, and me trying to contact them with no return calls, I am not optimistic that the communication has improved much over the past 4 years. New name. Same issues.............potentially. I will refrain from forming any opinions right now, as it is very early in the process, but again, there's history here that cannot be ignored.

We have a scheduled neurology appointment tomorrow morning at CMH and I am certain the topic of discussion will revolve around Mira's weight. At our appointment earlier this year, it is was pretty much all we talked about, and I have a suspicion that it will surface again tomorrow. It is always a struggle with Mira and we having been taking steps to increase her calorie intake, but it is difficult. Ironically, we don't really have anything specific to discuss with her neuro - we don't plan on making any medication changes, as we feel we have reached a consistent state with Mira that no medication, therapy, diet, or vitamin will ever fully heal or remedy. I am not anticipating much insight or an updated forecast of what to expect next. Mira's weather pattern hasn't varied much over the years: consistent myoclonics, mixed with occasional tonic-clonics, with gusting winds of irritability, and a chance of intermittent dystonia and lethargy.

Wednesday, November 9, 2016

Rocky Road Home

Mira has been having trouble in the afternoon this week, which is not uncommon. The bus ride home has been especially difficult for her. Whether it's the end of the day or she doesn't like being on the bus that long, she makes it known that she is not happy. The past few days she has come off the bus crying, just wanting to be in her bed. Tuesday afternoon, she came home and crashed in her bed for 2 1/2 hours straight. Daylight savings this weekend must have thrown her for a loop or something.

She has been tolerating the increase in calories over the past few days, enjoying the flavorful shakes we have been making for her. We are slowly integrating a new meal replacement powder for her too, one that has more calories (without the sugar) and a lot more flavor than her current one. We don't want to introduce too many changes at once with her, as it may mess with her regularity or digestion in general, however, she does need to start increasing those calories.

In terms of seizures, she has been having a few near tonic-clonics at school, although they aren't lasting very long. She had 2 brief ones yesterday, but apparently they didn't affect her too much. Otherwise, we haven't been seeing much activity at home, which is great.

Sunday, November 6, 2016

Maintaining Weight

One of the challenges we have always faced with Mira is trying to get enough calories in her throughout the day. While she doesn't have a G-tube and is able to eat by mouth, her diet is fairly limited, reduced to a consistently recognizable, nearly all-liquid diet. This makes it a challenge to give her bottles that have enough calories - balanced calories that aren't all sugar, carbs, or straight up protein, which is really hard on the kidneys. She is able to eat some foods by spoon, mainly yogurts, puddings, and pureed foods that are soft enough for her to eat without chewing. We supplement with protein powders and vitamins, but they never seem to be enough for her to keep the weight on.

While Mira continues to grow in height, she hasn't been able to keep up through her diet to maintain a healthy weight. This became evident during her weigh-in this past Thursday in the seating clinic. She had lost another few pounds since her last appointment, yet continues to increase in height. We have seen dietitians in the past through Children's Mercy regarding her weight and frankly, they haven't been very helpful or insightful visits. These dietitian appointments and follow-ups tend to be more of an exercise in calculating what she is actually currently eating, but offer little assistance in how we can get more calories in her and help us implement a balanced diet to get her weight up. That being said, we end up doing our own research and generating recipes that Mira can not only tolerate from a taste standpoint, but also making sure it is balanced nutritionally.

After realizing that her weight has again dropped over the past few months, we started putting together a reasonable meal plan this weekend, one that will hopefully get her to start gaining some weight. Mira has always enjoyed yogurts, rice milk mixed with protein powders, and squeezable fruit and vegetable packets. Unfortunately, these alone aren't enough to hit her daily target caloric intake, especially the fruit and vegetable packets, which tend to only tend to be in the 75-100 calories each, at best. We are trying to come up with creative ways to add avocado, coconut oil, peanut butter, yogurt, pudding, rice milk, malted milk, and other higher calorie foods to her diet, which is challenging. Long ago, Mira used to enjoy sweet potatoes and macaroni and cheese, and other more firm foods, but since she can no longer tolerate these, it has been difficult to have her drink anything like that, in a pureed form - they just don't go over as well when she is drinking them through a bottle. This weekend however, she has really enjoyed some of the smoothies we have put together for her, which I might add, always need to be warm - Mira will refuse to drink anything below room temperature, which also creates a challenge when everything has to be a warmer temperature.

If anyone has recipe ideas that might work for someone like Mira, I would love to hear them!

Saturday, November 5, 2016

An Afternoon in the Seating Clinic

Mira was having a fantastic week, all the way up until Thursday. Our busiest day turned out to be a rough one for Mira. She was fine when she got up Thursday in the morning, but had a huge tonic-clonic about 8:45am at school, then had another one about an hour later. These 2 seizures completely wiped her out for the rest of the day. Unfortunately, it was very bad timing, as we have had Mira's seating clinic appointment on the books for 3 months now, and we had to take her in that afternoon. She was having a significant bout of dystonia - her legs rigid, her torso and back damp from her body temperature being out of whack, and very altered overall. We couldn't get her to do anything but slouch in her chair for the entire appointment. Poor kid - she wasn't fussy at all, but suffering with the aftershocks of the earlier seizure activity. 

As far as the appointment went, it was fine. Perhaps there was enough water under the bridge from our last experience with the DME company (it had been 4 years) but it was still mildly awkward. The irony was, that we had originally gone in to the clinic to order a lightweight stroller for Mira, one that we could lift easily in the back of the van, and we walked out ordering a stroller and a new chair. We did not realize that it had been over 4 years since we actually took possession of the chair she is in now, and after discussing all of the issues we had been dealing with over the past 6 months, we decided to get the ball rolling on getting her a new 'permanent' chair. The clinic said appointments are 6 months out, so it just made sense to just move forward with the process now, while we have everyone in the same room. Her new chair will be similar to the one she currently has, only the frame will be longer to accommodate her future growth. Height wise, she has maxed out the seat and frame of her current chair, with zero options to expand it any further. This is the first step in this whole process and I sincerely hope it does not get derailed down the road, as we have run into so many times before with this company.

Mira has been doing much better over the past 2 days - her appetite is back and her dystonia has subsided. She has been very quiet and sleeping a lot.

Monday, October 31, 2016

This Week + The DME Company

Mira has been doing really well over the past week or so. She has been minimally irritable and we haven't seen a tonic-clonic since her big one last Sunday. This past weekend we went on some long walks outside, since the weather has been so pleasant, and Mira was loving every minute of it. She was consistently making this humming noise and kicking her feet the entire time. Yesterday at bath time, she was even smiling some, instead of her usual cry-a-thon. It's been a nice week for her.

On a side note, her tilt mechanism on her chair broke about 2 weeks ago. When something goes wrong with her chair, we tend to have the same dilemma - do we call the only durable medical equipment (DME) supplier here in Kansas City, which we have had numerous terrible experiences with over the past 10+ years, or do we try and fix it ourselves? I could not bring myself to even consider picking up the phone to deal with said DME company and seeing how we are going to be forced to deal with them later this week (which I will get to in a moment) I figured I wanted to limit our interaction with them as much as possible. Avoidance is the key, so I started down the path of repairing her chair myself, which I have done in the past.

So I took the tilt mechanism apart on her chair, figured out why it was malfunctioning, researched the part number online, and ordered the part direct from the supplier. It was quick and painless to do, only the part took 10 days to get here. I dismantled her chair this evening and installed the new tilt mechanism. It works like a dream and I enjoyed a drink, not only for fixing her chair, but for successfully avoiding any communication with the equipment company. I will gladly shell out $114 for a part and install it myself, than deal with the DME again. Can you tell I dislike them?

Unfortunately, we are forced to deal them this week. We have an appointment (one we scheduled 3 months ago) to get Mira fitted for a lightweight stroller and we really have no choice but to go through the seating clinic. This appointment will inevitably will create another forced marriage with the DME, due to their monopolistic practices. Their customer service is atrocious and over the years, they have bought out every other DME company in the area, creating a mega-company that specializes in poor communication, terrible service, and questionable accounting practices. Having not had to deal with them for the past few years and still very jaded, I truly hope their business model and customer service has improved.

Saturday, October 22, 2016

The Many Faces of Mira






It's been a busy and hectic past few weeks - I have been traveling quite bit, with trips to Massachusetts and New Brunswick taking up most of my work weeks. Sarah left for Los Angeles this morning and will be back on Tuesday, so in a sense, we have been passing the baton to try and stay on top of things. Mixed in between all of the travel, we have had conferences for all three kids, at three different schools, all at different times throughout the day on both Thursday and Friday. Mira has been having some off and on days - mostly on, where she has been in a positive mood, but she had an awful day last Sunday to keep it all interesting. She was really irritable all day last Sunday and we couldn't figure out why, until about 10:00pm, where she had a massive seizure in her bed. The aftershocks from the seizure went on for another 30 minutes. It was an awful one - no wonder she was in such a bad mood, as she was probably dealing with the pre-seizure activity all day. This week has been much calmer, with the shortened week because of conferences, Mira has been taking advantage of the down time. We have been taking it easy today, just running errands and trying to stay close to home. Today, Mira was making some seriously silly faces - I took these pics all within a span of 30 seconds. Serious face, dragons, laughing, contemplating, and so on - holding her ears for some reason the entire time!

Sunday, October 9, 2016

Living in the 1850's


We ventured out on Saturday and went to the Indian Festival, which is an annual celebration of Native American culture and food, which just happens to be just a few miles from the house. Sarah actually grew up just blocks from the festival, which she attended as a kid, but we had actually never been as a family, until this year. It turned out to be a lot of fun and we had perfect weather along with it. Jonah was able to spray a fire hose, go on a horse and wagon ride, and eat massive amounts of kettle corn. Even Eli was mildly entertained, I suppose by the kettle corn alone. Mira had a great time too - she really enjoyed being out in the sun and we did a serious amount of off-roading in her chair, as most of the tents and displays were out in the fields or grass, which made navigating her chair a workout for me, especially when going uphill. 



Walking through the historic house they had on display and experiencing the wagons, houses, and other period artifacts had me thinking about children with disabilities, of all things. I imagined trying to raise a child with Mira's issues, in that era - with the barest of essentials, the lack of therapies, and wheelchairs, and just the general living conditions that people endured back then. I couldn't imagine trying to raise a child with special needs with so very little and in such harsh conditions - we are all very accustomed to having everything so accessible and available, that it really put things in perspective. Pushing her up the hill seemed so trivial afterward. We had great weather, no mud, an actual wheelchair to guide us through, and food in our stomach as we worked our way around the festival. In 1850, living in a tiny one-room structure, with a leaking roof, outdoor plumbing, with no diapers, no safety beds, and no access to medication would have been brutal for a child like Mira - simply surviving the winter would have been the priority. 

Thursday, October 6, 2016

Feeling Better

Mira has been doing a little better over the past few days, although she had a pretty rough day at school earlier this week. She had one of those days where nothing made her happy - both at school and at home. However, she had a much better day overall today. We had some relatives visiting in from Vermont today and Mira was as calm as can be, for over an hour in her chair while she sat with us in the living room just talking. That's a rare event, considering late afternoons are typically not her favorite time. She did great today though!

Sunday, October 2, 2016

Late Afternoon Crumbling

After a stellar couple of days in a row (all part of a couple of good weeks) Mira slowly crumbled today. This morning started off great - she was full of dragons and very happy. We went for a walk this morning and another one right after lunch, which Mira enjoyed - she was kicking and making this rhythmic humming noise every 5 seconds (she has been doing this weird humming noise a ton lately - I can't really describe it) until we got back home. She took a nap this afternoon, but woke up in a really sour mood. She was wailing and crying non-stop for about an hour, all through bath time and through a couple of bottles for dinner. Once she was back in her bed, she settled down, but she was really ramping it up for awhile - tears streaming down her face, grimacing facial expressions, etc. Not sure why she was so worked up, because she hasn't done this for the past few weeks that I can recall. She is quietly resting now. Hopefully, she will have a smooth day at school tomorrow.

Saturday, October 1, 2016

The Dragon

Mira has been in a great mood all day today. We started off by running a bunch of errands in the morning and Mira was content the entire time. We dumped off all of our stuff from the store and headed out for a walk right after lunch and Mira continued to be full of energy. She was having some subtle myoclonics during our walk, but otherwise, seizures have been minimal. She took a brief nap after we returned home, then we did some racing around the house and again, Mira was as happy as can be. Throughout each of our outings and even when we were just hanging around the house, Mira was consistent with one thing - vocalizing. We call it her 'dragon' noise, where she sticks her tongue out and makes this heavy breathing sound, much like a fire-breathing dragon. She was doing it constantly today - it is a sign that she is happy and in a good mood and she did it so many times today that I lost count. She was just in a solid mood all day long. She was even doing repeatedly around bedtime. Something interesting also happened late this afternoon. At one point, I offered her a bottle around dinnertime and she purposely pushed it away and shook her head no, side to side! It could have been coincidence, as I could not get her to do it again, but it was certainly something I have never witnessed before. She has always pushed away bottles when she doesn't want them, but she has never done this with her head side to side to indicate no. I couldn't believe it - again, it could have been unintentional, but it really did seem deliberate and purposeful.

Thursday, September 29, 2016

A Special School Moment

Despite her episode over the weekend, Mira has been doing extremely well over the past week. She has been in a positive mood lately (in fact, for the past few weeks, with some minor exceptions) and has been very attentive and focused. At school this morning, her teacher presented Mira her name card in circle group, which she does every day, but today was different. Mira reached out with both hands, took the card, and dropped it in the basket that was sitting on her tray. Her teacher and paras were shocked and they all erupted and said 'yay Mira' and started clapping. Mira actually smiled with all the clapping! Everyone was so amazed at her reaction, that they clapped again, and Mira reciprocated once again by smiling! It was one of those rare moments where Mira had an unsolicited reaction to something. It has always been extremely difficult to communicate with her, as she rarely has a purposeful reaction to any situation or stimuli. Such an amazing day for her - I wish I would have witnessed it!

So what have we been doing differently these past few weeks? If you go back and read my post earlier this month on pyridoxine, (or for that matter, any of my posts on pyridoxine) - that would be a good place to start. For now, I want to enjoy Mira's moment today and hopefully not jinx everything by possibly attributing it to the supplement therapy we started her on at the beginning of the month. More soon...........


Sunday, September 25, 2016

Great Saturday - Off Sunday


We have been trying to get into the habit of getting out every weekend - anywhere; somewhere, just to get out of the house. The boys are always fairly pessimistic about going out on the weekend, as they would rather just be plugged into a screen the entire time, which is what we have been trying to avoid. They of course need their downtime on the weekends after being cooped up in school all week, but they also need to have some diversification of activities. So, this weekend, we went down to the annual Plaza Art Fair, which is a fairly big event that shuts down a large portion of the Plaza here in KC. The weather was perfect and despite Jonah's incessant griping about walking, the heat, his hunger pangs, and whatever else he could complain about, we managed to have a decent outing. Mira was in a great mood the entire time, in fact, she has been doing really well the past few days - that is, until last night. If I have said it once, I've said it a thousand times - epilepsy sucks. She was doing awesome all day yesterday - not a single fuss or whimper, was full of energy, and had a decent appetite. After our Plaza outing, another walk, and a brief stop in the park (I realized that she is waaaaaaaaay too tall for the swing in the park now - see picture above) yesterday afternoon, she went to bed, very uneventfully. However, about 9:00pm, she had a massive seizure in her bed, which we heard from the living room (our house is very small, so we can hear everything from almost anywhere) and rushed in to find her completely rigid and clammy. She continued all day to have a continuous episode of dystonia, complete with odd, rhythmic movements in her arms and torso, cold, clammy feet, turned inward, and her being in a very altered state, mentally. She would barely drink anything this morning and had a completely dry diaper. She continued to be dry up until this afternoon, which we finally started to see signs that she was coming out of these dystonic events, which she has had in the past. By later afternoon, she started kicking her feet more, finished off a couple of bottles (albeit very slowly) and finally had a wet diaper. Today was just a polar opposite of yesterday - epilepsy robbed her of having an entire solid weekend.

Sunday, September 18, 2016

Art Outing



It's always a challenge trying to find things to do as a family, especially on the weekends. During the week is always hectic, with the kids in school, but we still manage to have the occasional dinner outing or open house at school, but they are few an far between. Last weekend we took the kids out to a park on the Missouri side and this weekend we decided to try and hit the Nelson-Atkins Museum, here in Kansas City. I had been to the new addition a few times for meetings and such, but had never actually been to the older portion, not seen the extensive historical collection of work there, even after living in the area for almost 20 years. Sad, I know. Sarah has taken art classes here over the years and even the kids have been to the museum a few times, either with Sarah or through school field trips. Needless to say, the kids all did very well - Jonah kept asking when we were going to leave, but at least Eli found enough to peak his interest, especially in the Asian Collection, his favorite room. Mira was vocal and happy throughout the entire excursion, making consistent dragon and moaning noises in every room, usually when we were walking. We went outside and saw the reflecting pool, which Mira particularly enjoyed, because of the long trek over the uneven cobblestones surrounding the pool. Overall, it was a positive outing for everyone.

Mira has been doing well this past week. She did have a couple of tonic-clonics at school on Tuesday, which completely drained her the rest of the day. She slowly turned things around the rest of the week and was in a positive mood the entire time. I had to travel again part of this week to Massachusetts again, but fortunately got out of a trip to Cleveland today and Monday - yet have another trip to Louisiana toward the end of this upcoming week, It's been a heavy travel month, but it it slowly tapering off. 

Mira and I went out for several walks today, which she very excited about. She was very patient at the store, which usually makes her restless and irritable when we are at a standstill. All of the activity must have worn her out, as I had to wake her up just to have dinner and a bath. She went right back to sleep after all that.

Sunday, September 11, 2016

This Week



Mira had a pretty decent week - after her rough weekend of crying and wanting to be in a constant state of motion, she seemed to calm down this week, especially at school. She has been increasingly vocal, mostly when she is moving. We have been doing our fair share of walking this weekend, as the weather has been spectacular both days. Since I was in Boston on Thursday and Friday, Sarah was ready for a much needed break on Saturday, so I took all three kids out to Loose Park, over on the Missouri side. Despite a few eye-rolls from Eli, Mira, Jonah, and I went and fed the ducks and geese for a while, while Eli put his headphones on and ventured off around the park. There is nothing less entertaining than going to a park with your two younger siblings, from the perspective of a 13 year old. I remember that age all too well. He loves his independence. The rest of us had fun feeding the ducks, then hitting the slides, and finally finishing the afternoon off by getting hamburgers at a place down south back on the Kansas side. Mira absolutely loved the park, kicking her feet and making a ton of noise the entire time. The weather was way too nice to be inside.

This afternoon, we tried going up to Westport, not only to hit their annual weekend art fair, but also to have lunch at our favorite Indian restaurant. Neither one of those activities sat well with all three kids, but especially Mira. I think she has mad because she was ready to take a nap, while we were all set to head out the door. Bad timing led to her being fussy at the restaurant, with Sarah and I passing the baton, eating in shifts - one person eating, while the other was outside walking Mira in circles. Some outings are great - others, not so much. They all survived the outing and Mira and I ultimately ended up taking advantage of the cool temperatures, heading out for yet another walk.

Monday, September 5, 2016

Same Amount of Walking - Same Result

Today was very similar to the last few days before - Mira was unhappy and always wanted to be moving. We walked down to the village after breakfast and Mira was making her consistent moaning noise during our entire walk. Even when we stopped in a couple of stores to do some shopping, she continued making that noise, which of course, turned a few heads. She was content once we were back at home, for about 20 minutes. We did some walking in the the house, then more walking after dinner - in between all of our outings, she was mostly fussy. She hated every minute of her bath and only finally settled right before bed. Such a long, long 3 day weekend.

Sunday, September 4, 2016

So Much Walking

After a solid, nearly irritable-free week, the wheels feel off the wagon this weekend. Mira has been very unhappy the past two days, with no particular pattern to her irritability. Yet there has been one thing that seems to lift her spirits - walking or even more accurately, staying in motion. We have been on numerous walks, to the tune of 3-4 a day, in an effort to try and keep her happy. It only works for so long and frankly, it is exhausting pushing a 75 pound chair, a 70 pound child, and a backpack full of whatever we picked up at the store, uphill, often during the hottest part of the day. The second we come in doors and she is motionless for more than a few minutes, she starts crying. It is extremely difficult to stay in a constant state of motion with her and we can only walk indoors for so long (again, thanks to hardwood floors, we have a built-in 'track' that we circle around, from living room, through the 'foyer',  and into the kitchen/dining room, and back again, to complete our loop) before someone gets tired - usually it's me. When she is moving, she has been increasingly vocal, making this consistent, rhythmic moaning noise every 3 or 4 seconds. It's definitely a sign that she is enjoying herself, as her legs are stretched out (as in the pic above) and kicking, almost in unison. We often hear a lot of her infamous 'dragon' noises, which is a definitive marker of happiness for her. We went on a couple additional brief walks this afternoon and continued indoors for a bit, but she soon tired of everything, which sparked a wail-a-thon for about 45 minutes before dinner. She hasn't had any tonic-clonics for the past several days, in fact, her last one was at school early in the week, so I suppose it is boredom or a lack of variety that is frustrating her. It was an exhausting weekend. Hopefully, tomorrow we can find some additional activities to keep her busy.

Tuesday, August 30, 2016

Super Chilled

Mira has been really quiet the last two days. This morning, I had to wake her up for the bus, even after going to bed early last night. Once I had her dressed an in her chair, she was briefly enthusiastic about her bottle, but quickly faded after she was finished, so I put her back in her bed for another 20 minutes before the bus arrived. The second I put her back in her bed, she immediately folded in half (her comfort sleeping position) and fell right back asleep. Not surprising, she had a quiet and chilled day at school, not even objecting to getting into the Kidwalk, nor seemed to mind heading to the library with one of the other classes in the middle of the afternoon. She continued to just want to relax in her bed after dinner and a bath - she wasn't even up for racing around the house much. Some days, she is content just relaxing.

Sunday, August 28, 2016

A Good Week Overall

After having a very irritable weekend, Mira had a much improved week. She managed to have some fussy moments, especially on the bus rides home (which I will get to in a moment) but overall, she had a positive week. I was in New Hampshire part of the week, which had equally hot weather as we have been experiencing here in Kansas City, minus the rain. We have had some serious downpours here over the weekend, which has caused flooding throughout the city, even down at our local shopping district. We are expecting more rain this week. Despite being trapped indoors most of this weekend, Mira has been in a pleasant mood and has been having a minimal amount of seizures - just a lot of myoclonics in the morning mainly.

In terms of the bus rides, I'm not surprised that Mira isn't tolerating the commute to and from school this year, as the length of time that she is actually on the bus has increased dramatically. Thanks to even more budget cuts this year (thanks to our horrible Governor Sam Brownback - please feel free to email him) they have consolidated bus routes and schedules, so that Mira ends up being on the bus for an hour, each way, to and from school. The route the bus takes for the school year requires them to run in completely different directions to pick up every child on the route. Mira is the first one on the route and the last one off on the way home, so she ends up being on the bus the longest. This does not make her happy. We are going to contact the bus company and see if they can rearrange the pickup sequence or see if something can be done.

We are continuing with the pyridoxine, moving her up to about 25mg/kg, which seems to feel like the sweet spot for her for now. I noticed a change in her moodiness once we bumped up. I continue to find articles regarding pyridoxine's benefit to a multitude of ailments, including epilepsy, but am still trying to find an exhaustive source to understand all of the exact functions (mainly enzymatic - there are over 100 individual functions) that B6 actually performs. It is a very complex vitamin and I want to know as much as I can about it and why it has a positive effect for Mira.

Monday, August 22, 2016

Well...........That Was Brief

After Mira's miraculous turnaround last week, she had an incredibly irritable weekend. As quickly as her mood shifted 180 degrees, she spun back around and was right back where she was before. I have no idea what to do sometimes and it feels like everything I try to do to and help, is just one gigantic fail. As soon as I open my mouth, I think i jinx her positive streak. We have so few options these days and it seems nothing helps for an extended period for her. She just has good days and bad - lately is has been a lot of very fussy days, minus the 4 or 5 day stretch we had last week. She was having all sorts of lightning jerks all weekend - no major seizures, but certainly small seizures rising to the surface and causing issues with her. She was fussy this morning before the bus arrived, but apparently at school, she has a decent day. She had a big tonic-clonic this morning, which didn't completely wipe her out, but her teacher said it was definitely an intense seizure. We will keep on the pyridoxine for now and keep riding the roller coaster. It seemed to help break the cycle, albeit very briefly.

Thursday, August 18, 2016

Complete Turnaround - Thanks Pyridoxine!


Mira has been doing unbelievably better these past 4 or 5 days and I suppose we owe it all to pyridoxine. She had been having such a miserable time over the past 2 weeks, that we had to try something different, if for no other reason that to break the irritability cycle she was lodged in. As history has shown, Mira always seems to respond well to higher level dosages of pyridoxine, particularly in the 15-20mg/kg range. I have had many theories about her being pyridoxine dependent (too many to count) but I can assure you, at an absolute minimum, she is pyridoxine responsive. Regardless of how, what, why, and for how long B6 works for her, I am convinced it does work for her, in some capacity. Even if she has never tested positive for the ALDH7A1 gene, you will have a hard time convincing me, after this past week, that she doesn't have some sort of genetic, molecular, mitochondrial, and/or enzymatic dysfunction that responds to pyridoxine. Her turnaround since giving her the equivalent of 20mg/kg, has been nothing but miraculous. I have not witnessed (although others have) her fuss, whimper, or cry for the last 5 days. Instead, I personally have heard nothing but vocalizing, dragon noises, and overall, utter happiness. She also has not had a major tonic-clonic for quite a while. She did have some brief myoclonics in her bed yesterday afternoon, but that has been about it. I don't care if this is short-lived or not - right now, Mira is very happy, doing well at school, and has had a minimal amount of seizures all this week. The driver and para on the bus said she was crying her eyes out yesterday on the ride home, constantly pulling on her chest piece in the process. The second Sarah got her in the house and out of her chair, she immediately stopped crying and was happy as can be. That to me is a sign of expression - her wanting something by communicating, rather than just crying non-stop, with every effort on our part, being an exercise in futility and frustration. The repeated chest piece grabbing is something new. She also has been clutching at her legs, chewing on her blanket, and consistently vocalizing every single day since starting at the this higher dosage. She had been on a dosage of roughly 8mg/kg since we tapered down last September, after being on a higher dosage last summer. We saw amazing results for a few weeks last year, but it slowly faded out within a matter of a few months, as she fell into her old patterns of heavy seizures and high irritability. I'm fully prepared to ride this out indefinitely, knowing there are 2 variables that differ from last year, that may or may not have an impact on her recent success. First of all, she is on a higher dosage of fluoxetine (we raised it several weeks ago, due to irritability) and second, she no longer has her toy, which was an enormous seizure trigger. So, the pyridoxine journey continues and we will again, take it day by day.

Saturday, August 13, 2016

First Day of School


Things really started to final shift for Mira on Thursday, where she wasn't as irritable. Typically when her mood starts to shift like it did so abruptly, we expect some heavy seizures and/or some neurological flux to follow soon thereafter. Sure enough, Mira woke up Friday morning and was clammy and rigid. She wasn't having any seizures per se, but she was definitely altered - one of her episodes of dystonia and rigidity. The unfortunate part was it was the first day of school. It was strange for both Jonah and Mira to start school on a Friday (granted it was only a half-day) but I stopped questioning the districts choices when it comes to school schedules. We decided to send her off to school, since she wasn't having myoclonics or anything, we figured she was simply going to be lethargic, which was exactly what happened. Her teacher said she was very quiet and subdued - not fussy, just tired. She remained in that state for the rest of the afternoon at home. By the time this morning rolled around, any symptoms of her dystonia has disappeared and she was in good spirits for the entire day. Such a nice change of pace for her, after a solid stretch of nearly constant crying, fussy, and restlessness. Jonah was all smiles starting school yesterday - he was very excited to get to walk to the farthest classroom at the every end of the school. He gets to experience all the other classrooms, the library, the music room, and the art room, all before getting to his room - such a journey every morning for him. Eli had his teacher for kindergarten years ago, the only change is that Jonah has her for second grade. On Monday, Eli starts, so finally, all three kids will be in school - Sarah will probably collapse on the floor in complete exhaustion, while either doing celebratory snow angels on the hardwoods, or simply enjoying the silence for bit. She deserves a serious break.

Wednesday, August 10, 2016

What is Going On Lately?


These were the only two images we could get of Mira this afternoon, when she wasn't crying. Her mornings have been relatively decent, but her afternoons have been horrible. She has been extremely irritable this week, around the same time every day - today she was literally screaming, with tears running down her cheeks, fogging up her glasses in the process. We have no idea what is provoking this, but it certainly has escalated this past week. Coincidentally, she has been having dry diapers in the morning - one today and one on Monday. Last time this happened, we ended up taking her in to the pediatrician's office, because she had a UTI. I hope this isn't the case. Fortunately, she has had wet diapers (and soiled ones) the rest of the day, but we have no idea why she is holding it through the night. Constipation certainly isn't the issue either, as things are moving along in that category. I'm not sure if her extreme irritability and the dry diapers are related. It's always a guessing game, as she obviously cannot communicate with us, other than through crying.

Sunday, August 7, 2016

Doing Better

Mira's irritability tapered off somewhat toward the end of last week and into the weekend. Ironically, we have some construction going on at the house - we are having all of the original windows, front door, and garage door replaced, so there has been a fair amount of noise happening during the day. Typically, Mira isn't too keen on loud noises, yet with all of the banging, nail guns, and sawing, she seems to be taking it all in stride. They won't be finished installing for at least another week to ten days, so hopefully her patience will continue while they continue to work. This is Mira's last week of summer vacation, so I am sure she is also anxious to get back into a school rhythm. Her myoclonic clusters subsided for a few days, but she started having them again this afternoon. Overall, she has been in a fairly decent mood this weekend, but did become irritable right before all of the seizure activity started. We only say one near tonic-clonic over the past 4-5 days, when we were out walking on Friday, she slouched forward and became very rigid, but the seizure didn't fully evolve into a TC.

Thursday, August 4, 2016

Unhappy Mira

This has been another rough week for Mira. She continues to have these random clustered myoclonic episodes throughout the day, every day this week. I think all of this neurological activity is going hand in hand with her irritability. Every morning so far this week, she has woken up in a foul mood, crying and fussing until she is distracted by something - a bottle, a change in scenery, a nap, or sadly, more seizures. The cycle of seizures and fussiness, with a few random moments of quiet, is taking its toll on everyone here in the house these past few weeks.We called neurology on Monday about the uptick in her irritability and the recommendation was to increase her dosage of fluoxetine, which we started on Tuesday. It may take several weeks for it to take effect and for her to show any potential reaction to the increase, so we are being patient for now. This has been the most exhausting summer, on so many fronts, and I am so ready for it to be over.

Sunday, July 31, 2016

Myoclonic Clusters



Mira has been having an increasing amount of myoclonic clusters, at all times of the day. When I went to get her out of bed this morning, she started having a series of episodes - curled toes, eye deviations, rigidity, etc. Her breathing is never altered or anything, but it's all seizure activity nonetheless. She had one of these episodes in the middle of the afternoon yesterday, and again the day before. There never seems to be any pattern to it, as with most of her seizure activity in general.

Friday, July 29, 2016

Both Ends of the Spectrum

Mira spent the beginning of the week being in a very cranky mood - she was super irritable during the day on Tuesday and Wednesday, with nothing making her happy. Things have lightened up quite a bit since then, capping the week with Mira laughing and giggling today. We experienced both ends of the spectrum this week. She has been in a great mood today, maybe due to the fact that we have been doing serious amount of walking, all of it in the house. Walking around kept her smiling, so long as we were in a constant state of motion. I think we ultimately wore her out - she is in bed snoring right now.

On another note, my back did not improve after this weekend, in fact, the pain became unbearable, Sarah took me into the ER Monday afternoon, to see if there was something else going on. I scheduled an appointment with my PCP for Tuesday, but the pain was so bad I needed to try and manage it sooner, thus a visit to ER was warranted. They gave me some painkillers, which helped enough for me to get to my doctor the following morning and I was able to get a little sleep. After a steroid injection, a muscle relaxer injection, x-rays, and some additional stronger pain meds, things are feeling much better. I am still very sore, but at least the pain is manageable. I have had this happen before, just from transferring Mira, but had never experienced pain quite like I did earlier this week. As Mira is getting heavier and taller, I need to be more cognizant of how exactly I am picking her up, Transferring her chair into the van is typically what causes one to bend into an awkward position - her chair weighs a ton and is extremely difficult to get into the van with just one person.

Sunday, July 24, 2016

Rebounding

If there is one positive attribute with Mira's episodes (her most recent one last Wednesday - see the post before this one) is that they typically never last more than 24 hours. This latest one was no exception. By the time we put her to bed Wednesday evening, she had already been slowly coming around a bit beforehand and by the morning, she was back to baseline. Her fussiness and irritability has subsided over the weekend, with a minimal amount of seizure activity since. However, randomly this afternoon, she was laying in her bed and started having a series of strong myoclonics, one about every 15 seconds and the pattern went on for about 45 minutes. This is also not an uncommon occurrence these days. Rarely do they happen in the middle of the afternoon, but today they did - typically she has these right after waking up. I suppose today has been a different day in general for her - after having a couple solid weeks of irritability, she was very quiet and content all day today. She did spend a fair amount of time in her bed just chilling out, only getting up to race around the house and to have something to drink. She has loved racing around today (it was miserably hot today (hitting 98 degrees, so going outside for a walk was not an option) even if it was just around the house - she never seemed to tire from it and the only reason we stopped was because it was actually wearing me out. Sarah and I have been taking turns transferring her, as I again strained my lower back, either from transferring her chair into the van yesterday or from sitting awkwardly on the couch, hunched over my computer - not sure which. Either way, today I have had some serious pain that no amount of Advil seems to combat. This happened a few months ago and it look almost 2 weeks to fully get over it.

Wednesday, July 20, 2016

Very Rough Day

Mira had a really rough day today. I went to wake her up this morning and it was obvious that she had just had an intense seizure. She was laying in bed in a sort of post-ictal position, one that is reminiscent of the aftermath of one of her involved tonic-clonic seizures. Her arms were stretched outward and she was still having some small spasms with her arms. Her back was completely soaked and she was staring at the ceiling. I can tell it had just happened, at least within the past 10 minutes. Unfortunately, it is not uncommon for her to have a seizure when waking up, but it is always disconcerting nonetheless. I got her changed, dressed, and in her chair, but based on her expression, you could tell this was potentially going to be an off day for her. It's sometimes difficult to tell immediately after a seizure, so we decided to send her off to school, keeping in mind that with limited summer hours, she was only going to be gone for a portion of the morning.



Sending her off to school was probably not the best decision and I should have read the signs better, as she had another big seizure on the bus. The driver and para told her teacher, who in turn told us. Her day didn't improve much after that, as she was off the entire morning and was having a ton of twitches and jerks at school, throughout most of the morning. We could barely get 1/3 of a bottle in her before her bus arrived, which was red flag that things were not going to go well - Sarah couldn't get her to drink anything the rest of the day. She was either completely rigid or very lethargic, or both, the entire day. We finally managed to get her to drink a full bottle at about 7:00 this evening, which took her about 20 minutes to actually finish. You can tell from the pics above, that her arms were tense, her feet extended and crossed, and she was very clammy - her temperature was completely off. She was extremely quiet and altered, not responding to much of anything visually or any motion. Most of the time she will react to her chair moving, but today she was motionless and void of any recognition with every turn of her chair.

She has had numerous episodes like this in the past and you never know when they are going to happen. Fortunately, she only happened to have 2 tonic-clonics, as she has had as many as 10-12 in a single day in the past when these dystonic episodes surface. We have been keeping a close eye on her, as she has gone through the neurological ringer today. Epilepsy sucks, that's all there is to it.

Sunday, July 17, 2016

Another Rough Week


Mira has had a turbulent week, starting off last Sunday by spending the day having continued jerks and an absolute ton of myoclonics throughout the day, which continued into Monday. I had to fly out to New Brunswick on Tuesday morning and didn't get back until mid-day on Thursday. Mira ended up having an enormous seizure Tuesday night in bed, which Sarah said was really awful. That seizure took a toll on her and she was very lethargic all day Tuesday and part of Wednesday - her teacher said she was out of it at school and didn't want to participate in much of anything. By the time I was home on Thursday, she was back to her typical routine, which has been over the past few weeks, just a lot of continued fussing. There has been something bothering her, neurologically, but there is no telling what exactly it is - she has just been irritable and twitchy. On Friday, she seemed to have a better day, even spontaneously laughing on a few occasions, but overall, has not been happy with much. On a positive note, her fancy new sparkly shoes came in (as she outgrew her last pair of Tom's) and she hasn't been able to kick them off yet, not from a lack of trying. These particular shoes are about the only pair she manages to keep on her feet about 95% of the time and of course, they are stylish. She gets so many comments on them!

Monday, July 11, 2016

Continued Fussing

Mira's fussy spell continued through the entire weekend - just like a newborn baby, she would nap, then wake up and start crying until she had a bottle, or two, or three. This cycle started off this morning and was unrelenting the entire weekend. She did manage to sleep solid through the night both nights, but she would pick right back off where she left off the evening before, crying. It never seemed to subside during the day for more than an hour at a time. We did manage to go for a few walks, but it has been so sweltering hot and humid, that it just felt miserable outside most of the time. Getting outside seemed to break Mira's routine temporarily, but she would soon become either lethargic, irritable, or both if we stayed out too long, meaning more than 15 or 20 minutes. She was off school all last week, which may be part of it - she starts back tomorrow morning, so hopefully, things will turn around for her. She hasn't been having any seizures or anything significant - just her few typical random spasms and jolts every so often.

Friday, July 8, 2016

Cranky Week + Biopsy Results

Mira has been very irritable this week. Blame it on the heat, boredom, or seizures - or a combination of all three, but nothing has been keeping her happy these days. Her patience seems to wear thin very quickly, especially in the morning and late afternoon. But then again, at all times of the day she has been known to vocalize her displeasure. I was off this afternoon (thanks to summer hours) and took Mira out for a walk later this afternoon, as an attempt to redirect her when she was pretty cranky. She did great for the first 10 minutes - happy, kicking her feet, and doing some dragons. Shortly thereafter, she started having some strong tonic seizures and movements, where she stiffens up her upper body and her legs, repeating the cycle of seizures for the next 10 or 15 minutes. We started heading back home after I saw this cycle unfolding, so by the time we ended up near the house, she had stopped, but it quickly wore her out. She was quiet the rest of the afternoon - not irritable, yet not doing much at all but resting.

We did talk to her neurologist earlier this week and Mira's biopsy results came back normal. I guess we can check NCL off of the list - the long growing list of things Mira doesn't have. We were told it was a only a very remote chance that anything would surface from the test, so it wasn't much of a surprise that it came back negative.

Saturday, July 2, 2016

Agendas

Mira started off this month in a sour mood. She did alright this morning for a bit, but we pressed our luck when we tried to go over to Lawrence for lunch. Mira started to get a little fussy in the car, then really ramped it up when we sat down to eat. We only brought three bottles with us, two of which she finished in the car on the drive up, so with only one remaining, she finished off the last one in the first 10 minutes we were sitting down at the table. We were out of tactics and ammunition, so Sarah and I traded off, taking her outside and walking her around, while the other ate with the boys. Sometimes you simply have to pass the baton, and muster through what you hoped was going to be a simple, uneventful outing. It is always hit or miss with Mira and going out, so as far as today went, it was a big miss. That being said, it was incredibly loud in the restaurant and Mira has never been a big fan of noise in general. Then again, she was getting upset long before we actually reached the restaurant, so I have a suspicion it was something else that was bothering her, most likely something neurological. By the time we got back on the road, Mira was happy for a bit, but soon started crying again, until she was back in her element - her bed. She ended up taking a small nap and was in a much better mood the rest of the evening. Some days I feel like we are just better off staying at home and doing nothing, which frankly, is what we do most of the time. It's exhausting going out sometimes, thus we seem to do it less and less as a family now, especially during the summer, when everyone has their own idea of how the schedule is going to unfold for the day. We knew we were asking for trouble when we decided to simply go out to lunch with a surly 13 year old who is impressed by nothing, an 11 year old with severe disabilities, and an 8 year old who is on the spectrum, while they are all simultaneously complaining, in their own way, that they would rather simply stay at home. Three kids - three different agendas. Every.......single........day.

Tuesday, June 28, 2016

Happy Birthday, Mira!



Lots of legs lifts and kicking for Mira today, because she turned 11 years old today! She was full of energy this morning, constantly extending her legs outward (she always keeps them extended when in her chair) but I was able to capture some pics of her doing these windmill circles with her legs, every so often. She is super flexible and she can practically sweep things off of the kitchen counters with those leg extensions. Mira has never been much for parties and cake so we ended up having a quiet celebration here at the house and unfortunately, Mira seemed very tired. Mira has grown so much in the past few years, that looking back on some of her pictures, it's really hard to believe how much older she looks. She has shot up in height over the past 18 months as well. She is getting too tall for her chair, but I am dreading the process of getting her a new chair (it was extremely painful with her current chair) yet soon enough, we are not going to have a choice.

Her seizures have been at bay for the last few days, which has been fantastic. That always puts her in a better mood. Over the weekend, she was having a ton of lightning jerks an spasms all day long, which was making her irritable, especially on Saturday. No big tonic-clonics for a while though. Over the past nearly 11 years, her seizures have always come in waves - silent for a bit, then often erupting a few days later. You just never know what to expect.