Tuesday, March 31, 2015

Sunny Days = Sunny Mira

Mira has been doing great the past couple of days - seizures have been minimal (lots of myoclonics in the mornings of course, but no TCs) and she has even been smiling and giggling some. There have been a few moments of irritability, but really only at the end of the day, when she is winding down and ready for bed.

She had loads of energy this morning - wanting to do laps around the house while waiting for the bus. Her positive day continued when she was able to get outside at school and get into the swing. We capped off the day by getting in the swing again next door after dinner. Having some extremely pleasant weather the past two days doesn't hurt either. Feels like all of the stars have aligned for her to have a couple of good days in a row.

It's great to see Mira catch a little of a break and have a string of positive days for once - I hope the trend continues!

Sunday, March 29, 2015

Rough Morning, Improving Afternoon

Mira had another big seizure this morning, about 10:00am, although not as severe as the one late yesterday. She wasn't out for too long from this one, but she still took a sizable nap afterwards. She doesn't seem as drowsy and twitchy as she did yesterday, even smiling a little during a bath a couple of hours ago. Right now, she is relaxing in her chair, kicking her feet periodically as we are enjoying a lazy Sunday afternoon,

Saturday, March 28, 2015

Rough Day for Mira

Mira's day started off innocently enough, but gradually worsened as the day progressed. We ran some errands around lunch time, which Mira seemed to enjoy, but by the time we got home she was irritable and hungry The problem today was whenever we tried letting her play with her toy, she erupted into some amount of seizure activity - each episode worse than the next.

The first couple of times, she started having some intense myoclonics, so we immediately took the toy away from her. We tried a few times, several hours apart. She kept having a ton of twitches the rest of afternoon, when she wasn't napping from the seizures. Then right before dinner, she was pretty fussy, so we put her in her chair and she played with her toy for about 2 minutes. She then quickly and unexpectedly had a massive tonic-clonic seizure. The aftershocks and post-ictal heavy breathing seemed to go on forever. It was terrible to watch.

Eventually, about 5 or 10 minutes after the seizure, she quieted down and just basically crashed in her chair. This one really took a toll on her. She started stirring around about an hour later, but she was still very lethargic. She had a little to drink before bed, but she wasn't too interested in doing much but laying down.

Thursday, March 26, 2015

A Long Week

I have been on the road for the past 3 days - back to back trips to Colorado and North Carolina, getting back late last night. Traveling takes a toll on everyone - work is work, but Sarah has to hold down the fort while I am out. For one person to take care of Mira (and the boys of course), it can get exhausting, especially when it comes at the end of a very long break from school.

Mira had a rough Monday, the last day of spring break for everyone, but she had a much better Tuesday and Wednesday. I think after being off of school for so long, Mira was simply getting bored and needed to be back doing some activities. A change of scenery often helps with her.

We have had a busy week so far and the weekend is about to get busier - welcome to spring!

Friday, March 20, 2015


After all of the craziness and sickness that has occurred over the past week, the storm cloud finally lifted today. Sarah has quickly gotten over her virus and is feeling much better today. Mira too is feeling much improved, after well, evacuating her system. We were actually able to get out and head to dinner, without incident. We also opened every window and were able to air out the entire house, which also seemed to help everyone feel a little better.

Thursday, March 19, 2015

The Anti-Spring Break

Over the years, we haven't had a whole lot of success with spring breaks and this week was no exception. I can remember back about 5 years ago, where all five of us caught the flu and spent the entire week on the couch or in bed, with Sarah and Eli having to drive back from Illinois after Eli spiked a 104 fever. Needless to say, our break was cut short or rather, non-existent. As they made their way back to Kansas City, Jonah, Mira, and I had similar symptoms. Collectively, we all joined up at home and had a miserably sick spring break.

Our 2015 spring break is turning out to be pretty similar. Jonah and Eli both caught a stomach virus at the end of last week, each spending the weekend vomiting and laying on the couch. Fortunately, I only caught a milder version, dealing only with some nausea and minor stomach upset. Today turned out to be Sarah's turn, suffering the same symptoms. We were planning on driving out to Illinois (ironically, the same trip we tried 5 years ago) but we had to cancel, due to Sarah now coming down with the virus. I think we need to be quarantined through the weekend, just to be safe.

Mira so far has avoided getting sick, but she is having her own battles with constipation. We constantly struggle with this, as she is not mobile, she cannot regulate her BMs like the rest of us do. She has been really struggling for days and today it escalated to a point to where we had to intervene. There never seems to be a happy medium with keeping her regular - she is either at one end of the spectrum or the other. Again, I won't go into detail of today's events, but let's just say that I think we are getting her back on course. All of this struggling wore her out today though - she was having a miserably rough time and was wailing continuously all afternoon. She just went to bed a bit ago, after finally having a BM. She was completely exhausted.

So here we are, another spring break in paradise, slowly nursing ourselves back to baseline from various ailments. The word 'break' is certainly an oxymoron this week.

Tuesday, March 17, 2015

Quiet Morning - Irritable Afternoon

Mira continued to be quiet and reserved this morning. She played with her toy for a little while, but again, seemed more interested in being in her bed. She had some brief fussy spells before noon, but nothing out of the ordinary. A similar morning as yesterday.

This afternoon however, Mira ramped up the fussometer and was extremely irritable. From 4:30-5:30pm, she was wailing and crying relentlessly. Refused to drinkg anything and did not want her toy. Didnt car for being in her bed or her chair either, which leaves little option for much else. I think she eventually wore herself out and took a brief nap before dinner. She had a reasonable amount to eat after she woke up, played with her toy some, but appeared very sleepy, just like this morning. She had a usual amount of myoclonic seizures throughout the day.

Monday, March 16, 2015

Sleepy Mira

Mira has been really tired for the past two days. After her big seizure yesterday morning, she has been very lethargic and quiet ever since. Yesterday, she took a series of naps throughout the afternoon, waking briefly each time to stare around in her bed or her chair, only to drift back off to sleep. She hasn't been fussy or irritable at all really - just tired and passive.

She woke up this morning and was very clammy and sweaty. She was having a lot of repetitive movements and twitches for a good hour and again, was very tired and not moving much. We didn't bother putting her toy in front of her, as she was slouching in her chair and completely uninterested in doing much of anything. She managed to drink a bottle after 20 minutes of being up, but really seemed to want to go back to sleep, We put her back in her bed and sure enough, she crashed again.

The lethargy and periodic napping continued all day today. The most energy we witnessed all was around bath time, when she was at least a little animated, but as soon as she was back in her chair, she began to slouch over, returning to her passive state. She briefly played with her toy before bed, but showed very little energy.

Sunday, March 15, 2015

No GAMT - More Seizures

I contacted Mira's neurologist yesterday via email regarding her GAMT panel and he responded that it came back normal. They had tested her levels of creatine, guanidinoacetic
acid, and creatinine, all of which came back within their respective reference ranges. I guess we can scratch that one off the list. I'm not sure whether the genetic panel they ordered passed through insurance or not. If it did, it will be a few months for the results to come back, probably around the time we have Mira's next neurology appointment.

Mira struggled with irritability all day yesterday. As the boys are have been battling a stomach bug all weekend, it is likely that Mira's stomach was giving her issues too. Eli said his stomach was bothering him Friday night and into Saturday morning, which led into Jonah catching the bug, which led to a lot of vomiting all day yesterday. Jonah seems to be doing much better this morning, which is a drastic change from yesterday, when he couldn't keep so much as a sip of water down all day long. Sarah and I ended up cancelling our dinner date, which was disappointing as it was one of only two different occasions this year that we actually had a babysitter available.

Mira had a steady flow of myoclonic seizures all day long yesterday, but heaviest in the morning. It is likely, with the stomach issues floating around and the constant seizure activity, she probably wasn't feeling very well. I got her in the swing yesterday afternoon and for the first time, she didn't care for it - she briefly smiled on her first push, but it quickly faded into more fussing, which was a good sign that she was probably feeling nauseous. I didn't want to press my luck with another vomiting episode in the swing, so I gave up pretty quickly.

This morning she woke up and had a huge seizure about 30 minutes ago and has been quietly resting. Hopefully, the rest of the day will be better. She ends up getting draped in her Hello Kitty snuggie, which at least makes her looks stylish while she is sleeping it off.

Friday, March 13, 2015

First Day of Spring Break

Mira's first day of spring break was up and down, She started off the day in typical fashion, with a whole lot of myoclonics, some of which were very intense and almost led into some heavier activity. She had a consistent number of drops and seizures most of the day today, but fortunately, none of them escalated into anything more.

It was such a nice day out - we opened the windows, aired out the house, and got Mira out to the park, a couple of times. She thoroughly enjoyed the swing today, at least the first time Sarah brought her outside, She took a nap and woke up cranky afterward, which eventually led into an extended Unhappy Hour. At times, Mira was wailing at the top of her lungs - something was really bothering her. Her irritability eventually subsided and we managed to walk down to the Village and get some dinner. Mira actually did great through dinner, not so much as even making a cry, laugh, or anything. She drank a couple of bottles while we ate, despite the service at the restaurant being exceptionally slow.

Going out to dinner is always a gamble - Mira can either be in a reserved mood, like tonight, or immediately start crying and fussing as soon as we sit down at the table. If the irritability bus has kicked into high gear, you best option is to take her outside, then Sarah and I eat in shifts. Sometimes we muscle through it and eat quickly. It just depends and and you never know what is going to happen - tonight was definitely a great night to eat out, which I would have never expected after her fuss-a-thon just an hour earlier.

On the way back from our walk from dinner, we stopped by the park again and got Mira back in the swing. The second round in the swing didn't last very long, as Mira looked very worn out and was slouching in the chair after only 5 minutes. We got her out and ended up just walking around the park some, but she was pretty drained. Something tells me she is going to sleep well tonight.

Wednesday, March 11, 2015

Exhausting Seizure Day

With Mira dodging a day without having a TC, she made up for it today by having 3 of them. The first two were at school, one right before lunch and one afterward. The second one completely knocked her out. Her teacher said she was very lethargic and sleepy the rest of the day. To top it all off, she had a third one right before dinner. She recovered a little more quickly on the last siezure, but overall, she had a pretty rough day.

She was fairly irritable late this afternoon, before and after the seizure. She managed to drink about a bottle and a half, then I got her into the shower. After so many intense seizures today, her hands and feet were cold and clammy, even after the shower. In addition, she ended up in this defensive pose (see picture above) for the rest of the evening. In this pose, she brings her hands up to her sides, as though she doesn't want anyone near her and just has a blank look on her face. She ends up rigid and tense, making it difficult to get her changed and ready for bed.

After her shower, she went straight to bed and quickly fell asleep. It was an exhausting day for her, as all of the seizure activity today took its toll.

Tuesday, March 10, 2015

Finally - No Tonic-Clonics

Mira's streak of having a daily 5:00pm tonic-clonic thankfully came to a halt today. She woke up having her usually clusters of myoclonics, but fortunately, none of them progressed beyond that. The same thing happened this afternoon, where Mira was in a pleasant mood and had a good, long stretch with her toy in her chair.

With the change in time with daylight savings (and the fact that I escaped from work a little early) we were able to get Mira outside in the park and into the swing for a little while. She lasted about 15 minutes, before she quietly started slouching her way down, which her way of saying she's done. Mira was making the quirkiest faces and expressions for the first 5 minutes or so, like she was trying to contain her excitement, gnashing her teeth and smiling at the same time. It was a strange look from her, one that I have never seen before. Regardless, she certainly was happy and energetic.

We tried going for a walk around the park, but she was pretty tired out and getting restless in her chair. She had a good appetite, having three bottles in the last hour before bedtime. Mira stayed awake for another hour once she was actually in bed, just happily stirring around and slowly kicking her feet.

Monday, March 9, 2015

Another Day, Another Big Seizure

Mira seems to be getting into a groove - a very bad pattern of having a big tonic-clonic seizure in the middle of the afternoon. This marks the 4th or 5th day in a row where she has had a TC, almost at the exact same time. She is still having a number of myoclonics in the morning, so not much has changed in terms of having other seizure types and/or seizures at other times of the day. The single TC she keeps having in the afternoon slowly creeps up on her - she starts having a cluster of myoclonics, which eventually escalates into a full-blown tonic-clonic.

She had a decent day at school, but took a number of naps throughout the day. She was quiet this afternoon, with this involved seizure taking a toll on her. She ate a fair amount for dinner and was happy to be back in her bed to rest by bedtime.

Sunday, March 8, 2015

Weekend Hospital Visits

Mira continued her trend of having daily tonic-clonic seizures yesterday. She had been consistently having at least one TC every day for the past 3 or 4 days, but it seems as though it has been going on much longer. I always think her seizures have been slowly increasing over the past few years, but it could just be my imagination. It difficult to gauge.

Saturday was a long day. I took Mira in to get blood draws and a urine sample for a series of tests that neurology had ordered about a week ago. Going into the lab on the weekends is always an adventure and fortunately, we have only had to do it twice over the past 9+ years. After yesterday's experience, I remember why we try to avoid the weekends whenever possible - this week was crazy busy, so we didn't have much of a choice.

Because the regular lab doesn't hold weekend hours, we were sent up to the main lab in a different part of the hospital to get the samples done. The reception 'area' for the main lab was probably never intended to accommodate more than 2-3 people at a time, because it is about the size of a walk-in closet, which makes it difficult to maneuver Mira's chair around. Ironically, the adjacent room, where they actually do the blood draws, is even smaller. Needless to say, with Mira, myself, and 2 nurses in this tiny room, it was extremely tight. A third nurse tried to come in, but literally could not even get into the room with Mira on the exam table.

Mira did great for the first two vials, but she suddenly jerked during the third one and the needle turned in her arm. It had to be extremely painful for her as she started wailing - fortunately the nurse saw it immediately and stopped half-way through the third vial. Poor Mira was really upset for a good 10 minutes afterward. Her catheter to get a urine sample went much more smoothly and Mira even was laughing through it, despite having to wait another 20 minutes to get a different nurse. While the staff is always great and professional, the entire process of getting a simple blood draw and urine sample took over an hour and that was with no one waiting in front of us. The lesson is, never go on the weekend unless you are prepared to wait.

Anyway, Mira was extremely irritable this afternoon for some reason. She had a really rough time from about 4:00-5:30pm, where nothing would make her happy. She finally came around before dinner and was happy and engaged the rest of the evening. We were able to get outside yesterday and today to get Mira in the swing, which she thoroughly enjoyed and this time, without vomiting.

Friday, March 6, 2015

More Big Seizures

On both Wednesday and Thursday, Mira had a couple of intense tonic-clonic seizures, nearly at the exact time both days. In both instances, the seizures happened between 4:30-5:00pm, when she was playing with her toy. I think she starts to wear down around that time every day (thus the Unhappy Hour name) and we tend to see an increase in neurological activity and most often, irritability.

Despite having these intense seizures, Mira had some decent days, especially today. Apparently at school, she was in a really positive mood all day. Seizure wise, today she didn't really have too many head drops or myoclonics, not to mention she broke the cycle of having a big TC at the same time she has the past few days. She even was babbling up a storm after her bath after dinner.

Tomorrow morning, Mira will go in for some blood draws and samples for a creatine and GAMT panel. We have to go early in the morning, as she has to be fasting when she has all of the testing done.

Wednesday, March 4, 2015

Enormous Increase in Myoclonic Seizures

Mira had a massive amount of myoclonic seizures yesterday morning. From the time I was getting her dressed, until about 20 minutes before her bus arrived, she was having a seizure every 5-10 seconds. I was getting really concerned as no change in scenery or activity would stop them. We were a step away from keeping her home and giving her something to try and break the cycle. Over the past 9 1/2 years, we have only had to intervene (usually with Ativan) to stop one of these prolonged episodes.

When she does have one of these 'cycles' of seizures, she is breathing in between them, but when they are happening every 5-10 seconds, she can't really do anything with purpose, because the seizures just overwhelm and distract her to the point where she almost immobilized. Most days, in the morning in particular, she tends to have clusters of seizures, but very rarely to the extent and intensity that we saw yesterday. The cycle of seizures finally started to fade out after about 30-35 minutes.

The only change we have made over the past week is temporarily weaning and stopping her creatine supplementation. Mira has to give blood draws toward the end of the week and we want her to be at a baseline level when they run the GAMT testing. Having elevated creatine levels could render a false negative if she has adequate levels in her system. They are supposed to do an amino acids profile and blood work for a few other tests as well. Whether or not all of this recent spike in myoclonic activity coincides with stopping the creatine is purely speculation at this point.

Mira was fairly irritable at school yesterday and last night. She had an enormous appetite and ended up drinking almost 4 entire bottles before going to bed. I was afraid that some of her dinner was going to come back up after having so much to drink. She kept wanting more and more after the first 2 bottles, like she hadn't eaten anything all day. This morning she had a lot less myoclonics, but still was off and on irritable before the bus arrived.

Monday, March 2, 2015


Mira was pretty excited to get back to school today, at least for a while. She didn't have a ton of patience this morning while waiting for the bus and the fact that it was 20 minutes late, didn't help her cause either. She was happy once she was outside and heading toward the bus. The report back was that she had a decent morning at school.

Apparently, she was having a fussy spell around lunch time, until her teacher brought out the big drum. Mira loves to kick her feet on the drum and is a sure fire way to get a smile from her. They were able to snap her out of her irritable mood with the drum and she managed to have a good rest of the afternoon.

This evening, she was a little quieter than usual and was was having a number of myoclonics in her chair around dinner time. She had a decent appetite, but wasn't all that enthusiastic about playing with her toy for more than a few minutes. We put her back in bed and she ended up staying awake for another 45 minutes, just relaxing quietly.

Sunday, March 1, 2015

Rough Start, Better Afternoon

Mira started off the day with a heavy tonic-clonic seizure. She was in her chair for about 20 minutes and without warning, she went straight into it without any myoclonics beforehand. She had already had something to drink and her meds, so it was atypical for her to have one like that. She rebounded from it fairly quickly, within about 20 minutes or so.

The rest of the morning was much better for her - no seizure activity and she was in a pleasant mood, all things considered. She took a fairly long nap after lunch and woke up hungry and a little irritable, but it didn't last long. After a couple of bottles and some quality time with her toy, she remained in a positive mood the rest of the evening, even through her bath.

I managed to catch her babbling a little but during this extended time with her toy, but was cut short about 20 seconds in when she decided she was done. As you can see at the end of the video, when Mira is done with her toy, she grabs it (always with hr right hand) and tries to shove/throw it off her tray. Since we have it doubled-strapped with Theraband, it makes it difficult for her to fling it off. She now has managed to collectively push the entire tray, plexi stand, and the toy completely off. She has some serious upper body strength.

Mira had some solid fussy spells yesterday, but overall, it's been a pretty decent weekend, despite being trapped indoors because of all of the snow.