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.