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


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.

Friday, September 4, 2015

Friday + Genetics

After the bus accident, the rest of the week has been fairly uneventful. Mira has been in a pleasant mood for a majority of the time, despite averaging one or two tonic-clonics a day. She has been getting positive reports back from school, informing us that Mira is really enjoying herself most of the day - in a good mood all day, with the occasional disapproval of being in the Kidwalk. That's not out of the ordinary. If there is one thing that I have noticed over the past few months of her being on the pyridoxine, it's that Mira's mood has improved. She seems to have more good days than bad, even through all of the seizure activity.

Sarah had a conversation with the neurology clinic today and she got us set up with an appointment next month. Fortunately, we were able to keep seeing our regular neurologist, who we have been seeing for the past 6+ years. He agreed to keep Mira on as a patient, even though she does not have a confirmed genetic mutation that is causing her seizures. Although, she tested positive for a mutation in the TPP1 gene, which I had read can still cause issues despite it being a heterozygous mutation, we were told it probably isn't relevant to her seizures. I don't quite understand it, as the report I read from GeneDx is contradictory to me - one of the things we will discuss with him next month.

Wednesday, September 2, 2015

Bus Accident

Mira had an eventful day today - on the way home from school, her bus was rear-ended by another vehicle on a side street, a few miles from the house. Fortunately, no one was injured and the bus only sustained minor damage. The car that rear-ended the bus however, took the brunt of the collision. Apparently, a car ahead of the bus had slammed on its brakes, which created a chain reaction of the bus stopping short, which someone behind the bus, perhaps following too close, rammed into the back of it. Again, thankfully no one was hurt. Though, if there is one thing for certain, someone's insurance company isn't going to take kindly to their customer rear-ending a school bus. Good luck with your policy rate hike.

Mira came home after the accident on the same bus - there wasn't enough damage to the bus to where they couldn't continue to drive it, but it did take a fair amount of time for the police to file a report and get the paperwork going on the incident. Mira ended up getting home about an hour later than usual. Ironically, she was very vocal when she arrived, babbling and making all sorts of sounds, like she had something to tell. She continued making sounds for another 45 minutes, then had an enormous seizure.

She has been doing alright the past few days - no real changes in seizures or mood, other than this afternoon. She had a great day at school today - lots of smiling and babbling. It's been very humid the past week, so getting outside hasn't really been an option, and as the continued eye-rubbing and temperatures tend to keep Mira indoors.