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.