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.