Thursday, December 1, 2016

The Thanksgiving Drive

Last week we made our second trip out to Kentucky, with a stop on the way out in St. Louis to visit my family for the evening. Embarking on a road trip or spending any significant amount of time in the car with Mira is always a gamble. We never know what the highway will bring. During our neurology visit the week before, our neurologist recommended a small dosage of valium while we were on the road, to help ease Mira's anxiety in the van. After our first trip out to Kentucky last summer, I think all of us lost a little bit of our collective sanity, having to listen to Mira's crying for a solid 225 miles on the out to KY. It was painful, thus we felt a little more empowered this trip, having some ammunition before we embarked on a 9-10 hour excursion in the car.

We weren't very optimistic heading out toward St. Louis, as we didn't get 3 miles from the house before Mira started crying. We stopped, fueled up, and gave Mira a dose of valium, which didn't seem to do too much - she continued to cry off and on for most of the ride. Tolerable, but certainly not ideal. We spent the evening and headed out early the following morning, with 4 hours under our belts and another 5+ hours to go. Mira did a little better, but ended up crying, somewhere around Louisville, and continued pretty much until we got out of the van. Once she was inside and able to stretch her legs, she was in a much better mood. In fact, she was extremely happy for the rest of the afternoon.

However, for the remainder of our visit, Mira was very irritable. Every day, Thanksgiving included, she was just not happy and nothing would keep her happy for more than a few minutes. Just one of those roller coaster weeks. We had to split up on a few outings each day - I ended up taking Mira back to the house on several occasions, while the rest of the crew went out to lunch. Mira would have not lasted more than a few minutes in the restaurant, which makes a stressful outing for everyone. She wasn't much happier once we were back out of the van either, so we were never sure what was really bothering her.

Regardless, we had a pleasant Thanksgiving break, loading up the van early Sunday morning to head back to KC. This being only our second attempt at driving straight through for 9+ hours, I was frankly cringing at the prospect of having Mira's irritable temperament continue the entire drive back. Much to everyone's amazement, Mira went an entire 600 miles without making a peep! She was truly amazing - she was wide awake and didn't sleep a wink for the drive, but rather was content just looking out the window or just happily enjoying the ride. She did break down that last 20 miles, but I was in shock that she went solid for almost 9 hours without so much as a whimper. We did give her a dosage of valium before we left KY and another about 5 hours into the drive, which seemed to do the trick.

Unfortunately, she has been a train wreck most of this week - she has been irritable every day since, finally settling down today and has become extremely sleepy this afternoon. With all of the travel and visits, her sleep patterns have been thrown off and I think she is just worn out. Ironically, in terms of seizures, we haven't seen any the last week to ten days. She hasn't been having any myoclonics in the morning and certainly no tonic-clonics to speak of, at home or at school. With all of her fussy days, we typically see a spike in seizure activity, but again, it has been eerily quiet in terms of seizures since my last seizure post back on November 9th.

Monday, November 21, 2016


I know I've said it before, but Sarah and I have learned to not have any preconceived notions about how Mira's neurology appointment will end up going. In the past, we think we are going in to talk about one thing and end up getting spun 180 degrees and discussing something completely different. We have resigned to the fact that we have no idea what to expect - we have some questions and discussion points lined up, but typically end up only briefly touching on those, if at all.

However, her appointment on Thursday was actually one of the more straightforward meetings we have had over the years. Mira's weight certainly came up, despite putting on 4 pounds over the past month, she is still in the lower 20th percentile for weight. The diet we initiated late last month is starting to show - she is gaining weight and it is helping, but she has a ways to go. Her neurologist no longer seemed concerned about it - we have taken the necessary steps to boost her calories and we all think we are headed in the right direction with her. In terms of medications, we didn't make any changes, as her seizures seem to have decreased some since our last appointment back in May. Overall, Mira's demeanor has improved over the past few months, despite having some awful days here and there. That being said, we didn't think it was necessary to change anything from a medication standpoint.

We did have a discussion about pyridoxine, which was interesting. I have read extensively about it over the years and we have done trials with Mira in the past. I explained to her neuro that she does respond very positively to high dosages to pyridoxine - we have not discovered exactly why, but the only two clinical diagnoses that I am aware of that respond to high dosages are pyridoxine deficiency and homocystinuria, neither of which Mira has a genetic marker or indicator for. Yet, somehow she responds positively to it, thus we have been keeping her on dosage of it for a while. I tried weaning her off of it a few months ago and she was a complete irritable mess. There are concerns of peripheral neuropathy, but it is very rare. I am much less concerned about the side effects of pyridoxine, than of the countless pharmaceuticals she has tried over the years. Her neuro certainly wasn't against it and admitted that we were more of the experts on it than he was, but he was intrigued to understand and research more about it. It was refreshing to walk out of the appointment not having any major concerns, scheduled additional testing, or unanswered questions. I have a lot of respect for her neurologist - I think he is very open-minded and honest about everything - he always has been.

Wednesday, November 16, 2016

Neurology Tomorrow

It's been a fairly quiet week here since my last post. Mira has been in decent spirits, mixed with occasional bouts of crankiness, which are usually redirected through a long walk outside She hasn't really had any major tonic-clonics, but has been having days of twitching and random myoclonics, but nothing significant. We finally heard back from the seating clinic, after almost two weeks. Based on the conversation that Sarah had with them, and me trying to contact them with no return calls, I am not optimistic that the communication has improved much over the past 4 years. New name. Same issues.............potentially. I will refrain from forming any opinions right now, as it is very early in the process, but again, there's history here that cannot be ignored.

We have a scheduled neurology appointment tomorrow morning at CMH and I am certain the topic of discussion will revolve around Mira's weight. At our appointment earlier this year, it is was pretty much all we talked about, and I have a suspicion that it will surface again tomorrow. It is always a struggle with Mira and we having been taking steps to increase her calorie intake, but it is difficult. Ironically, we don't really have anything specific to discuss with her neuro - we don't plan on making any medication changes, as we feel we have reached a consistent state with Mira that no medication, therapy, diet, or vitamin will ever fully heal or remedy. I am not anticipating much insight or an updated forecast of what to expect next. Mira's weather pattern hasn't varied much over the years: consistent myoclonics, mixed with occasional tonic-clonics, with gusting winds of irritability, and a chance of intermittent dystonia and lethargy.

Wednesday, November 9, 2016

Rocky Road Home

Mira has been having trouble in the afternoon this week, which is not uncommon. The bus ride home has been especially difficult for her. Whether it's the end of the day or she doesn't like being on the bus that long, she makes it known that she is not happy. The past few days she has come off the bus crying, just wanting to be in her bed. Tuesday afternoon, she came home and crashed in her bed for 2 1/2 hours straight. Daylight savings this weekend must have thrown her for a loop or something.

She has been tolerating the increase in calories over the past few days, enjoying the flavorful shakes we have been making for her. We are slowly integrating a new meal replacement powder for her too, one that has more calories (without the sugar) and a lot more flavor than her current one. We don't want to introduce too many changes at once with her, as it may mess with her regularity or digestion in general, however, she does need to start increasing those calories.

In terms of seizures, she has been having a few near tonic-clonics at school, although they aren't lasting very long. She had 2 brief ones yesterday, but apparently they didn't affect her too much. Otherwise, we haven't been seeing much activity at home, which is great.

Sunday, November 6, 2016

Maintaining Weight

One of the challenges we have always faced with Mira is trying to get enough calories in her throughout the day. While she doesn't have a G-tube and is able to eat by mouth, her diet is fairly limited, reduced to a consistently recognizable, nearly all-liquid diet. This makes it a challenge to give her bottles that have enough calories - balanced calories that aren't all sugar, carbs, or straight up protein, which is really hard on the kidneys. She is able to eat some foods by spoon, mainly yogurts, puddings, and pureed foods that are soft enough for her to eat without chewing. We supplement with protein powders and vitamins, but they never seem to be enough for her to keep the weight on.

While Mira continues to grow in height, she hasn't been able to keep up through her diet to maintain a healthy weight. This became evident during her weigh-in this past Thursday in the seating clinic. She had lost another few pounds since her last appointment, yet continues to increase in height. We have seen dietitians in the past through Children's Mercy regarding her weight and frankly, they haven't been very helpful or insightful visits. These dietitian appointments and follow-ups tend to be more of an exercise in calculating what she is actually currently eating, but offer little assistance in how we can get more calories in her and help us implement a balanced diet to get her weight up. That being said, we end up doing our own research and generating recipes that Mira can not only tolerate from a taste standpoint, but also making sure it is balanced nutritionally.

After realizing that her weight has again dropped over the past few months, we started putting together a reasonable meal plan this weekend, one that will hopefully get her to start gaining some weight. Mira has always enjoyed yogurts, rice milk mixed with protein powders, and squeezable fruit and vegetable packets. Unfortunately, these alone aren't enough to hit her daily target caloric intake, especially the fruit and vegetable packets, which tend to only tend to be in the 75-100 calories each, at best. We are trying to come up with creative ways to add avocado, coconut oil, peanut butter, yogurt, pudding, rice milk, malted milk, and other higher calorie foods to her diet, which is challenging. Long ago, Mira used to enjoy sweet potatoes and macaroni and cheese, and other more firm foods, but since she can no longer tolerate these, it has been difficult to have her drink anything like that, in a pureed form - they just don't go over as well when she is drinking them through a bottle. This weekend however, she has really enjoyed some of the smoothies we have put together for her, which I might add, always need to be warm - Mira will refuse to drink anything below room temperature, which also creates a challenge when everything has to be a warmer temperature.

If anyone has recipe ideas that might work for someone like Mira, I would love to hear them!

Saturday, November 5, 2016

An Afternoon in the Seating Clinic

Mira was having a fantastic week, all the way up until Thursday. Our busiest day turned out to be a rough one for Mira. She was fine when she got up Thursday in the morning, but had a huge tonic-clonic about 8:45am at school, then had another one about an hour later. These 2 seizures completely wiped her out for the rest of the day. Unfortunately, it was very bad timing, as we have had Mira's seating clinic appointment on the books for 3 months now, and we had to take her in that afternoon. She was having a significant bout of dystonia - her legs rigid, her torso and back damp from her body temperature being out of whack, and very altered overall. We couldn't get her to do anything but slouch in her chair for the entire appointment. Poor kid - she wasn't fussy at all, but suffering with the aftershocks of the earlier seizure activity. 

As far as the appointment went, it was fine. Perhaps there was enough water under the bridge from our last experience with the DME company (it had been 4 years) but it was still mildly awkward. The irony was, that we had originally gone in to the clinic to order a lightweight stroller for Mira, one that we could lift easily in the back of the van, and we walked out ordering a stroller and a new chair. We did not realize that it had been over 4 years since we actually took possession of the chair she is in now, and after discussing all of the issues we had been dealing with over the past 6 months, we decided to get the ball rolling on getting her a new 'permanent' chair. The clinic said appointments are 6 months out, so it just made sense to just move forward with the process now, while we have everyone in the same room. Her new chair will be similar to the one she currently has, only the frame will be longer to accommodate her future growth. Height wise, she has maxed out the seat and frame of her current chair, with zero options to expand it any further. This is the first step in this whole process and I sincerely hope it does not get derailed down the road, as we have run into so many times before with this company.

Mira has been doing much better over the past 2 days - her appetite is back and her dystonia has subsided. She has been very quiet and sleeping a lot.

Monday, October 31, 2016

This Week + The DME Company

Mira has been doing really well over the past week or so. She has been minimally irritable and we haven't seen a tonic-clonic since her big one last Sunday. This past weekend we went on some long walks outside, since the weather has been so pleasant, and Mira was loving every minute of it. She was consistently making this humming noise and kicking her feet the entire time. Yesterday at bath time, she was even smiling some, instead of her usual cry-a-thon. It's been a nice week for her.

On a side note, her tilt mechanism on her chair broke about 2 weeks ago. When something goes wrong with her chair, we tend to have the same dilemma - do we call the only durable medical equipment (DME) supplier here in Kansas City, which we have had numerous terrible experiences with over the past 10+ years, or do we try and fix it ourselves? I could not bring myself to even consider picking up the phone to deal with said DME company and seeing how we are going to be forced to deal with them later this week (which I will get to in a moment) I figured I wanted to limit our interaction with them as much as possible. Avoidance is the key, so I started down the path of repairing her chair myself, which I have done in the past.

So I took the tilt mechanism apart on her chair, figured out why it was malfunctioning, researched the part number online, and ordered the part direct from the supplier. It was quick and painless to do, only the part took 10 days to get here. I dismantled her chair this evening and installed the new tilt mechanism. It works like a dream and I enjoyed a drink, not only for fixing her chair, but for successfully avoiding any communication with the equipment company. I will gladly shell out $114 for a part and install it myself, than deal with the DME again. Can you tell I dislike them?

Unfortunately, we are forced to deal them this week. We have an appointment (one we scheduled 3 months ago) to get Mira fitted for a lightweight stroller and we really have no choice but to go through the seating clinic. This appointment will inevitably will create another forced marriage with the DME, due to their monopolistic practices. Their customer service is atrocious and over the years, they have bought out every other DME company in the area, creating a mega-company that specializes in poor communication, terrible service, and questionable accounting practices. Having not had to deal with them for the past few years and still very jaded, I truly hope their business model and customer service has improved.

Saturday, October 22, 2016

The Many Faces of Mira

It's been a busy and hectic past few weeks - I have been traveling quite bit, with trips to Massachusetts and New Brunswick taking up most of my work weeks. Sarah left for Los Angeles this morning and will be back on Tuesday, so in a sense, we have been passing the baton to try and stay on top of things. Mixed in between all of the travel, we have had conferences for all three kids, at three different schools, all at different times throughout the day on both Thursday and Friday. Mira has been having some off and on days - mostly on, where she has been in a positive mood, but she had an awful day last Sunday to keep it all interesting. She was really irritable all day last Sunday and we couldn't figure out why, until about 10:00pm, where she had a massive seizure in her bed. The aftershocks from the seizure went on for another 30 minutes. It was an awful one - no wonder she was in such a bad mood, as she was probably dealing with the pre-seizure activity all day. This week has been much calmer, with the shortened week because of conferences, Mira has been taking advantage of the down time. We have been taking it easy today, just running errands and trying to stay close to home. Today, Mira was making some seriously silly faces - I took these pics all within a span of 30 seconds. Serious face, dragons, laughing, contemplating, and so on - holding her ears for some reason the entire time!

Sunday, October 9, 2016

Living in the 1850's

We ventured out on Saturday and went to the Indian Festival, which is an annual celebration of Native American culture and food, which just happens to be just a few miles from the house. Sarah actually grew up just blocks from the festival, which she attended as a kid, but we had actually never been as a family, until this year. It turned out to be a lot of fun and we had perfect weather along with it. Jonah was able to spray a fire hose, go on a horse and wagon ride, and eat massive amounts of kettle corn. Even Eli was mildly entertained, I suppose by the kettle corn alone. Mira had a great time too - she really enjoyed being out in the sun and we did a serious amount of off-roading in her chair, as most of the tents and displays were out in the fields or grass, which made navigating her chair a workout for me, especially when going uphill. 

Walking through the historic house they had on display and experiencing the wagons, houses, and other period artifacts had me thinking about children with disabilities, of all things. I imagined trying to raise a child with Mira's issues, in that era - with the barest of essentials, the lack of therapies, and wheelchairs, and just the general living conditions that people endured back then. I couldn't imagine trying to raise a child with special needs with so very little and in such harsh conditions - we are all very accustomed to having everything so accessible and available, that it really put things in perspective. Pushing her up the hill seemed so trivial afterward. We had great weather, no mud, an actual wheelchair to guide us through, and food in our stomach as we worked our way around the festival. In 1850, living in a tiny one-room structure, with a leaking roof, outdoor plumbing, with no diapers, no safety beds, and no access to medication would have been brutal for a child like Mira - simply surviving the winter would have been the priority. 

Thursday, October 6, 2016

Feeling Better

Mira has been doing a little better over the past few days, although she had a pretty rough day at school earlier this week. She had one of those days where nothing made her happy - both at school and at home. However, she had a much better day overall today. We had some relatives visiting in from Vermont today and Mira was as calm as can be, for over an hour in her chair while she sat with us in the living room just talking. That's a rare event, considering late afternoons are typically not her favorite time. She did great today though!

Sunday, October 2, 2016

Late Afternoon Crumbling

After a stellar couple of days in a row (all part of a couple of good weeks) Mira slowly crumbled today. This morning started off great - she was full of dragons and very happy. We went for a walk this morning and another one right after lunch, which Mira enjoyed - she was kicking and making this rhythmic humming noise every 5 seconds (she has been doing this weird humming noise a ton lately - I can't really describe it) until we got back home. She took a nap this afternoon, but woke up in a really sour mood. She was wailing and crying non-stop for about an hour, all through bath time and through a couple of bottles for dinner. Once she was back in her bed, she settled down, but she was really ramping it up for awhile - tears streaming down her face, grimacing facial expressions, etc. Not sure why she was so worked up, because she hasn't done this for the past few weeks that I can recall. She is quietly resting now. Hopefully, she will have a smooth day at school tomorrow.

Saturday, October 1, 2016

The Dragon

Mira has been in a great mood all day today. We started off by running a bunch of errands in the morning and Mira was content the entire time. We dumped off all of our stuff from the store and headed out for a walk right after lunch and Mira continued to be full of energy. She was having some subtle myoclonics during our walk, but otherwise, seizures have been minimal. She took a brief nap after we returned home, then we did some racing around the house and again, Mira was as happy as can be. Throughout each of our outings and even when we were just hanging around the house, Mira was consistent with one thing - vocalizing. We call it her 'dragon' noise, where she sticks her tongue out and makes this heavy breathing sound, much like a fire-breathing dragon. She was doing it constantly today - it is a sign that she is happy and in a good mood and she did it so many times today that I lost count. She was just in a solid mood all day long. She was even doing repeatedly around bedtime. Something interesting also happened late this afternoon. At one point, I offered her a bottle around dinnertime and she purposely pushed it away and shook her head no, side to side! It could have been coincidence, as I could not get her to do it again, but it was certainly something I have never witnessed before. She has always pushed away bottles when she doesn't want them, but she has never done this with her head side to side to indicate no. I couldn't believe it - again, it could have been unintentional, but it really did seem deliberate and purposeful.