Sleep. Eat. Fuss. Sleep. Sleep a Little More

Mira was incredibly sleepy the entire weekend, which is not surprise. She had 9 visible tonic-clonic seizures within an hour or so on Friday afternoon. She was completely wiped out the rest of the evening and weekend, sleeping at every moment it seemed. Saturday she remained a little clammy and had a few sporadic head drops and spasms, but no major tonic-clonics. Sunday she had a bit more energy, but again spent most of the day resting.

This morning she woke up and was back to her old self - happy for a bit in her chair, then fussy, then dozed off, then went off to school. Her teacher reported that she was back in her old rhythm in the class as well - dozing off at times, eating well, fussy at times, intertwined with a few moments of being happy.

Today officially marks 3 months of starting the pyridoxine. As I posted earlier, it has been somewhat of a bust I guess, but we are going to ride it out at a lower dosage. The theory being, that if she was going to follow the same patterns she did in 2007, we would see a drastic reduction in seizures over the next few weeks, which occurred after about 3 months of taking it before. I don't have a very positive feeling about it, but it won't hurt to keep waiting. Based on her dramatic seizure episodes (multiple tonic-clonics and clustering) that she has had over the past few months, she does seem to be following a similar trend that she did years ago, but it could all just being random seizure activity. The next few weeks I suppose will tell.

Terrible End to the Week

Mira's seizures are back with a vengeance. Over the past few weeks, they have been slowly escalating back to where they were, where she was averaging around 40-50 myoclonics a day, along with 3-4 tonic-clonics a week. We had seen some improvements back in July, but she has slowly slipped back into her familiar patterns. Her seizure activity has been pretty steady over the past several years, with slight variations - she always has her occasional positive stretches, with an equal amount of bad ones.

Yesterday however, she had an extremely rough day. She seemed fine when we were getting her ready for school, but by mid-morning at school, she had already had 2-3 tonic-clonics, which her teacher thought was a little out of the ordinary. By the fourth one, she summoned the nurse to get involved and promptly called us. Over the next 45 minutes, she had another 7 tonic-clonics, all ranging from 30 seconds to a minute long, repeating every 5-10 minutes. Her breathing was fine in between all of the seizures, but the fact that she has so many in such a short time frame, was disconcerting and frightening. This was again, a very similar episode, if not worse, than the one she had on her birthday, which marks a significant increase in these episodes this year.

Sarah talked to her teacher and she decided to pick her up at that moment and get her home, then we would consider Diastat to potentially break the clustering. Keep in mind that Mira has never gone into status epilepticus, which is very serious, requiring an immediate response, usually involving a dosage of Diastat (which the school has on hand for Mira) and a prompt call to 911. Not that having clusters of tonic-clonics within such a short period of time isn't concerning, it's the fact that Mira was steadily breathing in between all of them. Sarah had her back at home quickly and back in her own environment, keeping a close eye on her in her chair. She continued to have a constant flow of subtle myoclonics, twitching, and dystonic movements for the next hour or so. After all of the activity subsided, she was sweaty, clammy, and only mildly responsive. We were again on fence for this episode about giving her Diastat, waiting on the sidelines to see if the tonic-clonics resurfaced. Fortunately, they stopped. It was a stressful day and we have been watching her like a hawk since. She has been doing a ton of sleeping so far.

Ironically, I have been re-evaluating this B6 trial, thinking that she should taper down, since she has not had a positive reaction to it, at least the past few weeks. I don't want to completely abandon it, but we are going to definitely taper down. As I stated before, I was very optimistic around 6 weeks in, when Mira seemed to making some great strides, being very vocal and keeping the seizures at bay and/or minimized. It has since fallen apart, thus we are going to begin to slowly taper her down to 100-200mg a day, which is a mild clinical therapeutic dosage, that is less likely to cause potential issues. I am not sure at this point whether the dosage we had her at is making her seizures worse, but it certainly hasn't been helping over the past month. We will wean her own to this lower dosage and keep monitoring. Yesterday was so exhausting for her, but this morning she at least seems to have more energy. She is still a bit clammy and her temperature is off, but at least the major seizure activity has been sidelined for now.

Backwards

Mira has really slipped back to baseline. She has had 2 big seizures over the past two days and countless myoclonics, at all times of the day. Ironically, she has been in a fairly pleasant mood most of the time (with the exception of bath time) but the seizures are relentless. She also has been having decent days at school, not being as engaged as much, but at least content. We have been staying at the same dosage on the B6, but there has been little positive change since the middle of July, in fact, she seems to have gone backwards in many ways. Next Monday will be around the 3 month milestone for starting the B6, but as I stated in my last post, I have pretty much lost hope with it. It was certainly worth a try and will continue it for awhile, since I feel as though it has helped her mood some. That being said, I suppose there has been one positive aspect - completely ineffective in terms of seizure control, but an improvement in her overall demeanor.

Pyridoxine Update

Mira is almost 3 months into her trial with pyridoxine and my optimism level has plummeted to nearly zero. Over the past few weeks, the myoclonics have consistently returned and her pattern of tonic-conics has also settled back in. I was feeling really positive about all of it about the middle of July, which was around the anticipated mid-point of my theory of her having some level of pyridoxine deficiency/dependency (PD), would start to take effect. Mira was becoming increasingly vocal, her seizures, at the time, seemed to be diminishing (even having a stretch of seizure-free days) and for a while, we weren't seeing any morning myoclonics. For that matter, we weren't seeing ANY myoclonics, all day long. As you may recall in one of my earlier posts, I had a theory, based on Mira's history of seizure freedom, that she may be PD.

She has been holding steady at around 25mg/kg, which again, is around a standard trial dosage for PD. I had tried raising it this past week, but it severely affected Mira's sleep patterns. It made her miserable during the day and kept her up all evening. Dropping the dosage back to where she was, seemed to be a good balance about a month ago. Now, I'm not optimistic about any of it. We are going to continue to move forward, indefinitely, but with the rising realization that this was yet another therapy that will ultimately, do nothing for her seizures.

Until you have a child with such a catastrophic syndrome or disease, you will most likely never understand the yearning and desire to ease your child's suffering. The relentless onslaught of debilitating seizures and stagnant development for the past nearly 10 years, has truly been devastating for Mira, making life challenging for her of course and for our entire family. It has taken me years to try and work toward a point of acceptance, but it is still, after continuing on this journey, very difficult for me to do. Accepting this mantra of 'it is what it is' or 'it could be worse' is something that I still cannot fully digest, but need to embrace, in order for me to better function as a parent to my other 2 kids, my wife, and my career. Acceptance of Mira's condition, without a definitive cause and to not have access to an effective therapy to ease her suffering, is something I am truly trying to accept. It just takes time.

Sleepy Day; Sleepy Week

Mira finished off the week today by having another sleepy, tired day. She had a fair amount of energy this morning, but the constant flow of myoclonics seemed to take the energy right out of her. She perked up a little when the bus arrived, yet the report back from school was that she continued to be tired most of the day. This afternoon, she took another power nap, then when she woke up and tried to play with her toy, but the relentless onslaught of myoclonics again kept her from focusing on much of anything. We went out to the park for a bit and then walked around the neighborhood, which invigorated Mira. She was kicking the second we got out the door and even babbled some during our walk. The nice thing about our stroll around the block was that Mira did not rub her eyes once - an amazing change from the past dozen times we have been to the park, where she rubs her eyes almost constantly.

It has been an exhausting week for Mira, with a full day of school everyday - no wonder she has been so tired. Fortunately she has the weekend to get caught up on rest and sleep. Starting school is always an adjustment - Eli and Jonah have been equally drained this week.

Rough Times Call for Trazodone

Mira had a miserable Monday evening. She was very irritable and restless most of the evening, so we put her to bed a little earlier than usual. She woke up about 11:00pm and just started crying and wailing. We tried everything for the next three hours, but nothing helped. She wasn't even fully awake, but she was seriously upset about something. She refused to drink anything too. Finally, about 2:00am, we gave her a half dosage of trazodone and crossed our fingers. It took about 30 minutes to kick in - either that or she had truly exhausted herself until she fell asleep. Either way, she slept soundly after that and we had to wake her from a deep sleep in the morning to get her off to school.

Her days since the Monday night episode haven't been much better. She has had at least 3 really intense seizures since then - 2 just this evening, on top of the dozens upon dozens of myoclonics. The clammy hands and feet have returned, while she continues to sit motionless in her chair. She is completely out of it. The only thing she is responding to is the occasional bottle.

Volatile

Mira started her first full week of school this week. We still haven't received a report back from Friday or today, since Mira's notebook didn't come back home with her again today. The first couple of weeks, without fail, everyone seems to be working out the kinks. Mira has a couple of new students in her class and on the bus, in addition to having new paras at school, so I have no doubt that everyone is going through an adjustment period, getting settled in with schedules. Mira's side support from her chair also was left at school, so we are anxious to get that installed back on her chair - her teacher realized it was left behind and gave us a call so we wouldn't panic. Even the simplest pieces from Mira's chair are incredibly expensive to repair or replace.

Mira has been in an energetic, yet volatile mood today. She had a lot of energy this afternoon and was very adamant about playing with her toy. She played solid for about 45 minutes, then proceeded to get frustrated with everything afterward. A lot of up and down, happy then sad at the drop of a hat. We tried the park for awhile, then her toy again, then a round-robin of bottles. She finally just went to bed, where she fussed for a few minutes, then fell fast asleep. Seizure wise, she hasn't really had any all day, other than a few myoclonics this morning.

Saturday

Mira has been slowly coming out of her tired stretch, with each passing day showing a bit more energy. I spent Thursday and Friday in Louisiana, getting back late last night, which left Sarah to get the kids acclimated and readjusted to school. The bus schedule seems to be all over the map the first few weeks, with the driver and para trying to work out their new routes and get accustomed to new kids. Mira's first full day of school was yesterday and we haven't heard the report back from her teacher, as her notebook was accidentally left behind in the classroom.

Today she was in a pretty decent mood, even lasting through an entire restaurant sushi meal. That is somewhat of a rarity, especially after a tiring week of getting adjusted to the first week of school. She took a small nap this afternoon and woke up fussy for a bit, but was over it quickly. We went for a couple of walks around the neighborhood, which she (and Jonah) enjoyed a ton - Mira was vocal and kicking all the way down the block both times. After Mira was over her fussy spell, she played for a bit and then became very vocal. She was making these extended screeching noises, which are difficult to explain without actually hearing them. She remained in a positive and energetic mood the rest of the day. Not a ton of myoclonics today or for that matter, for the past few days, just a few here and there. No clusters and no major tonic-clonics.

I am very grateful when I hear from parents of children suffering from seizures, that offer support, compassion, and information. I have found over the years, that the parents are often much more informed when it comes to research, potential therapies, and the latest news in regards to epilepsy. Like most parents, they are consistently searching for any information they can find that might potentially help their child. I don't mean this to sound condescending and certainly do not intend to discredit any medical professional, but we as parents realize that neurologists are only human too and have their limitations. They typically see hundreds of children in clinic and trying to remember the particulars of any one patient can be difficult, if not impossible, based on such a limited interaction, which for us, is every six months. Parents on the other hand, witness our children struggle with neurological issues, every single day, thus we know our child better than anyone else, thus we become the experts.

It's this exchange of information between parents that I am so thankful for, as I am often given relevant ideas that might help Mira. I wanted to thank Laura for contacting me and giving me such a fantastic synopsis and overview on MTHFR - it was much appreciated!

Sleepy First Day of School

Mira continues to be very sleepy, even at her first day back at school, but thanks to Sarah, she sure looked fashionable. Her first day back was only a half day, so it certainly couldn't have been that difficult of a day at school to wear her out so much. I think this is just a continuation of her lethargic stretch, which has been going on for three days now. Her teacher said she was very sleepy this morning and she continued to be equally tired at home this afternoon, napping at every moment she could. The only time I saw her muster up any sort of energy was after her bath, where she kicked her feet for a few minutes, but 5 minutes later, was almost falling asleep again in her chair. No tonic-clonic seizures today, but another consistent string of myoclonics all day long, mostly whenever her toy was in front of her. Her feet and hands were less clammy, especially this evening, but not completely dry. Hopefully, Mira will perk up tomorrow and start showing some energy.

Is the Honeymoon Over?

After having some very positive stretches last month, Mira seems to have fallen back to baseline over the past few days. She had a succession of days, a few weeks ago, where she was vocalizing a ton and was full of energy, even having times where her seizures were nonexistent or very minimal. It was all looking very promising, even up until the weekend. The last few days however, have been the exact opposite. She has all but clammed up and hasn't babbled for 4 or 5 days now and her seizures have come back in full force.

She woke up Monday morning having a steady stream of intense myoclonic seizures, one after the next, every 10-15 seconds. It went on for 30 minutes or so. A few hours later, she had 2 big tonic-clonic seizures that completely wiped her out for the rest of the day. Her temperature swings have been drastic too - clammy, cold hands and feet for the last few days. She woke up this morning, much like yesterday, with more myoclonics and another tonic-clonic this afternoon. Today she spent almost the entire day sleeping. She seems completely out of it - very quiet, spacey, and consistently sweaty when she has been in her chair. The brief times when she actually was awake and in her chair, she was rigid and defensive, just like in the picture above - legs stretched outward, fists clenched, and arms up and at her sides.

Tomorrow she starts back at school, only for a half-day, but at least it will hopefully begin to get her back in a rhythm. I hope the last few days are just a blip on the radar and not a reversion back to where we were a few months ago. As I go back and reread Mira's blog entries, I am reminded that while we have had a few honeymoon periods on some meds/vitamin trials, the positive runs unfortunately, never seem to last. I want to remain hopeful with this pyridoxine trial and we will keep moving forward, but it's days like this, where what little remaining optimism I have that anything will consistently help with her seizures, tends to diminish even further.

The Long Trip Home

We are finally back home, after a very long drive across five states, from Northern Kentucky back to Kansas City, all in one day. We hit the road early this morning and even with gaining an hour (from Central to Eastern time zones) we still managed to stretch a 9-hour straight shot into an 11+ hour drive with stops. This was the first time we had made this trip with Mira and after the grueling 225 mile cry-a-thon on the way out, we weren't too optimistic that this was going to be an easy return trip. We had the luxury of stopping overnight in St. Louis on the way out but on the way home, we had decided long ago to try and make the trip in one day.

Much to our surprise, Mira did very well. We were about 100 miles in when she started getting restless and fussy, so went from her toy, to bottles, to stopping to get her out of her chair and walking her around whatever gas station parking lot we were at to refuel. This seemed to keep her happy for almost 5+ hours! She had a few brief fussing spells here and there, but for a majority of the trip, she was content and/or napping. Toward the end, the last 100 miles or so, she just became tired of being in the car, slowly ramping up the crying until we pulled in the driveway. Who could blame her - at that point, we were all exhausted from being in the van so long.

We had a great trip - we had fun day at the water park, did some shopping, relaxing with family, and were able to get to downtown Cincinnati and the Riverwalk yesterday. Thanks so much to Aura, Ware, Addy, and Truman for letting us invade your home for the week and for entertaining us! It wasn't without some work. Having Mira out of her element is often a challenge, particularly for transfers and diaper changes. At home, most of this is frankly easier and less taxing on Sarah and I, since we have the luxury of having her elevated bed, which alleviates picking her on and off the floor for changes. After a few days away from home, my back and knees were looking for an alternative means of transfer for her - it wears on you. I was however, able to catch up on quite a few naps in between all of the lifting and activities.

Mira continues to do well on the B6, with her seizure activity slowly diminishing, at least this week. Since the end of July, she has had only one tonic-clonic, which is pretty amazing, considering she was having 2-3 a week just a month ago. She is still having the occasional myoclonics, particularly in the morning, but they are few and far between, Yesterday, while were down on the riverfront, she had a few very quick sort of 'lightning' jolts every so often - almost like startles, more than anything. I haven't really seen anything like it and they only lasted a second. Other than that, we have seen a minimal amount of seizures over the past 10 days.

Vacation

I won't sugarcoat it - traveling with Mira is challenging and exhausting. Mira has never been on a plane, as there are too many variables to take into consideration to have her confined to a seat for even a couple of hours. Considerations that would have never entered my mind, until having a child with significant special needs made such ideas necessities, rather than mere 'what if' scenarios. What if she cries and wails the entire flight? What if she has a diaper blowout? What if she has an enormous seizure? What if she gets altitude sickness? What if, what if, what if. Some of the same issues you would consider when flying with an infant or a small child perhaps, the only exception being is that Mira is almost 4'-9" tall and over 70 pounds. You physically cannot deal with a diaper change at 30,000 feet in a tiny bathroom that barely fits one average size occupant.

That being said, flying is simply not an option for us. So, when considering a trip, we are limited to places we can drive - reasonable travel distances and reasonable destinations. The same considerations with flying surface of course, but at least we can try and manage them while on the road. It is still a challenge, but at least we have options. So this week, we embarked on our longest journey in the van with Mira thus far - a two stage trip to St. Louis and Northern Kentucky.

On the trip from Kansas City, we had the option to break our final destination into two legs, stopping in St. Louis,which is a mere 4 hour drive from KC. That was the easy one. St. Louis to Kentucky was over 6 hours. The drive for everyone across Missouri was uneventful. Mira has been on this trip before and not having to stop once, made it a fairly easy commute. She was fussy for a few parts of the drive, but certainly manageable, with bottles mixed with intermittent toy distractions. The toy rigging involves someone (usually Sarah) sitting in the middle row, in the middle seat, holding Mira's toy strapped to a lapseat cushion, having her play until she tires of it and wants something else. When we did manage to get to St. Louis, she was tired of being in the car and started ramping up the crying. She could not fully decompress and it took her a solid hour or so to finally quiet down and fall asleep.

Mira must have had a rough night, because what followed the next morning, was a full venting of all of Mira's frustration, tiredness, constipation, and whatever else was not agreeing with her on the trip thus far. We hit the road as early as we could, with Mira getting increasingly fussy. Then, as we headed toward Kentucky, she proceeded to cry for the next 225 miles. Yes, that's 225 miles, roughly 3 1/2 hours, listening to her cry, wail, and whimper. Three stops, one about every 30 minutes, to try and soothe her with bottles, her toy, and eventually, Benadryl, did nothing for her. No seizures, no twitching. Just crying. We were all set to give her Trazodone, which we use in cases like this, where she simply cannot decompress and find her happy place. Small problem with that idea - we forgot to bring it with us. We stopped for ear plugs, then another stop for earphones to double up on the ears, and another for the Benadryl, was enough to create the feeling that we were either not going to mentally survive this trip or were actually never going to arrive sanely in Kentucky. We would need to stop at the closest highway asylum and check ourselves in. It was brutal. Eli, Jonah, Sarah, and myself were all completely spent and we only a little more than half way there.

Finally, the clouds parted and Mira stopped. For the last 2+ hours, she was quiet as can be, only waking up from her catnaps to try and glance out the window. Unfortunately, we had to rig a beach towel over the window, as Mira had broken off the pull shade on her side of the van and since she was relentlessly rubbing her eyes, we had to cover the window to keep her wrists off of her upper eyelids. She literally rubbed her right eye raw, until we stopped (for a forth time) to conjure up the makeshift window shade to keep her from doing it. It stopped about 95% of the rubbing, but she now has a blister above her right eye.

Arriving in Kentucky was like hitting an oasis. Mira was so happy to be out of the van, we were happy to not have to listen to her wail for the last 2 hours of the trip, and we knew we didn't have to drive anywhere else for a bit. At least not today. Where we are staying in Kentucky too, is fantastic - acres of room for Mira to spread out and get comfortable. The lower floor of the house is almost as big as the entire footprint of our house altogether. We are so grateful for Sarah's sister, husband, and their family, to have us invade their house, after just relocating here a few weeks ago. I know it has been a stressful move for them and to have guests so soon after getting (re)settled in so recently, we are probably adding to the chaos of just having relocated across the country, twice in the last six months.

Seizure wise, Mira has been doing very well this week. At the end of July, she had a very similar day that she had on her birthday - lots of myoclonics and temperature issues. Since then, she has only had one tonic-clonic, yesterday morning about 6:00am. Today she has been having more jerks and myoclonics than usual, with her toy seeming to aggravate and provoke some seizure activity. We have been limiting her toy because of it.

Sunday

Mira is having a decent follow-up day to yesterday, with slightly more fussy spells. She woke up this morning full of energy, babbling up a storm and wanting to zoom around the house as much as she could. Her energy level started to taper off by mid-morning. She must have worn herself out, because she spent most of the afternoon napping. We tried going outside earlier in the afternoon, since it was pleasant in the shade, yet we didn't last long as Mira would not stop rubbing her eyes. We can't seem to get over this repetitive wrist-rubbing immediately after stepping outdoors. I tried pinning her arms to her side for a few moments, hoping to break the cycle and get her distracted, but the second I released, she was back to it. She literally would not stop until we were back inside. I just don't get it - no contrasting light, no wind, no allergies, and while it's hot, it certainly wasn't uncomfortable.

After our brief stint out on the deck, Mira decided she wanted an extended nap. She woke up in a foul mood just now, but her toy made everything better. She keeps trying to shove it off her tray (as in the picture above) but then goes right back to playing with it. We didn't see a ton of seizures again today - a few myoclonics this morning, but nothing really thus far this afternoon.

Great Saturday

Mira has been having a fantastic day today. She has been in a great mood all day, with only one very brief fussy moment, which a few bottles took care of. On another bright note, her constipation issue has resolved and to put it as delicately as I can, things are flowing as they should be. I will just leave it at that.

She has been fairly vocal today too. She was reciting her number one hit 'ba-ba-ba-ba' a few times this morning, but has been working on a new tune this afternoon. Mira sort of stiffens her jaw and leaves her mouth open, while making this repetitive humming sound. It's a new one I haven't heard consistently, until today. She has also been kicking up a storm with her feet all day, so much so that we put some padding down on her footrests again. The entire floor shakes when she is hammering her feet on the pedals.

She took a big nap through dinner and then played with her toy for a bit before bedtime. She didn't have any seizures from about 10:00am on. Before that, she was having a bunch of repetitive myoclonics in her bed for about 10-15 minutes.