A Perpetual State of Exhaustion

Mira continues to be quiet and really out of it. Ever since the weekend, she has become increasingly withdrawn, having a dazed expression and seemingly unmotivated to do much of anything. Every time we try to put her toy in front of her, it provokes a seizure. For awhile, it was every other time, but now, it is just a given - it just simply triggers a seizure, every time. It feels as though if Mira isn't having a seizure, she is recovering from one. It is just a constant cycle of seizure activity, being post-ictal, and then a slow recovery, only to lead into another seizure. This cycle is keeping her in a perpetual state of exhaustion. We haven't made any adjustments to her medications yet, as we were waiting until the weekend to start. We want to be able to keep a close watch on her when we start the Vimpat wean.

Quiet + Sleepy

Mira has been taking a lot of naps over the past few days. With the weather changing and it getting so dark so early, I think it is throwing her sleep patterns off. She has been waking up during the night too, which isn't unusual, but it it is causing her to sleep more during the day. It's difficult to say how often she is getting up and how long she is actually staying up. We ran a few errands around lunch time and Mira was dozing off and on, mainly whenever we were sitting still. She wasn't crabby during the errands, but had a number of brief fussy spells this afternoon. We have not made any medication changes as of yet, since we are waiting for the pharmacy to fill her revised Vimpat prescription. She will be on a liquid form of it during the wean, which we will most likely start midweek. We are going to wait until she is completely off of the Vimpat before we start the fluoxetine. We don't want to have more than one variable moving at a time.

Neurology Appointment

We had our standing neurology appointment today, which we have had on the books for almost 6 months. The clinic is so busy, that we typically have to set our future appointments 4-5 months out, right when the schedule officially opens. Trying to predict whether or not you might have a conflict that far in advance is pretty much impossible. You just have to make sure you don't cancel, otherwise, you are back in the queue, which means you probably have another 4-6 month wait to get back in. Did I mention the neurology clinic is busy?

We have been going to the neurology clinic for over 10 years now, seeing 4-5 different neurologists - we have been seeing our current neurologist for the past 4 years and we are very comfortable with him. He is direct, respectful, and knowledgeable. I have learned not to have any expectations with what we will discuss, as we often go sideways in the conversation. For a few years, it seemed each appointment ended up completely different than I imagined it would go, thus I just quit trying to speculate. Sarah and I had a few things we wanted to address at this appointment, including the efficacy (better yet, non-efficacy) of Vimpat, the potential of putting Mira on a SSRI (to potentially manage her irritability), getting into ophthalmology (since Mira has not had a vision screen in years), and to discuss the idea of a gluten-free diet.

Taking her off of Vimpat is a simple concept. We don't feel like it is doing anything for her seizures and the less medication she is on, the better. We dare not even consider taking her off of Lyrica - we have learned our lesson the hard way, twice. Both times it was a disaster. Weaning Vimpat will hopefully go smoothly. She will taper down over the next month and should her situation worsen, we will do the exact opposite and try moving her up. She is on a fairly low dosage so there is the potential to move up on it, yet based on the fact that she has failed so many trials, with a plethora of different medications (and dosages), with varied mechanisms of action, the chance of a Vimpat increase actually helping, is extremely slim. Her neurologist reinforced this to us and we understand the percentages. All pharmaceuticals are a gamble either way.

We also discussed putting Mira on a SSRI, which is a Selective Serotonin Reuptake Inhibitor, to try and make a dent in Mira's ongoing irritability. We have always battled her mood and instability, so trying a different approach is worth a shot. Her neurologist was very open to the idea and has a host of patients that use SSRIs to help, primarily with anxiety and not so much epilepsy. We collectively decided we are going to ease her onto a low dosage of fluoxetine. Her neurologist was able to witness her crankiness during the appointment, when Mira cried for the last 15 minutes, while we tried to carry on a conversation over her wailing and tears.

The final issue we discussed was the idea of putting Mira on a gluten-free diet. The response from her neurologist was that there is no evidence whatsoever that a GF diet helps with seizure control. He sees a lot of patients that are either currently trying it or have tried it in the past, but nearly all them have had limited success. Most try it for 6-12 months, seeing positive results after a few months, but then the efficacy of the diet tends to fade after awhile. Despite the lack of results and studies done on gluten-free diets and epilepsy, he said there is no harm in trying it. As I noted in my last post, Mira is basically on a GF diet, minus the protein powders. I was able to find a plant-based powder that is gluten-free, soy-free, chemical-free, and contains no artificial ingredients, which you would think would taste awful, but it actually tastes great. I mixed a scoop with a glass of rice milk and I thought it had an unexpected sweetness to it. The smell is very earthy, but the taste is fairly sweet, not grassy. Mira chugged three bottles of it yesterday and doesn't seem to disagree with the taste.

Mira is having a pretty typical week - same amount of seizures, same irritability. With all of the changes in medications that are coming up, we will be keeping a close eye on her. All the kids have a 4-day weekend, with parent-teacher conferences the rest of the week.

Glutamine, Glutamic Acid, Gluten + the ALDS

This has been an interesting weekend to say the least. After getting some disappointing news at work about midday on Thursday, I was pleasantly surprised at the end of the day, by winning a pair of tickets to the first game of the ALDS at Kauffman Stadium. Sarah and I were lucky to find a sitter on such short notice (thanks grandma Helen) so we were able to go. The weather was great - a little brisk at 47 degrees when we left the K, but no rain. The seats turned out to be awesome, 6 rows back behind the 3rd base dugout. Even better was the fact that the Royals dominated the game, posting a 5-0 shutout, then followed it up today by having an unbelievable 5-run 7th inning to beat the Blue Jays this afternoon, taking a 2-0 lead in the series. This team, just like last year's lineup, continues to amaze me.

After spending a lot of nights either reading or trying to meet work deadlines, the Royals game was a welcome break. I know I have mentioned this in the past, but many of the best ideas and research I tend to follow up on, come from other parents, and sometimes, from people very close to me. Sarah, my wonderful wife, mentioned a few months ago, after an appointment she had with a new chiropractor, mentioned Celiac Disease (CD) for something to consider for Mira's seizures. The conversation with this new chiropractor took a detour at some point and ended up being a discussion more about Mira than Sarah, ironically. Sarah has mentioned CD in the past before and being my stubborn self,  had dismissed the notion at the time, that there was any connection between Mira's seizures and CD, based on what I had read in the past.

Needless to say, after Mira's continual woes with seizures over the past month, I went down the CD rabbit hole a few weeks ago. It is actually pretty simple in terms of CD - just avoid gluten. With a wide range of symptoms and degrees of gluten sensitivity, one can manage the symptoms by going on a strict gluten-free diet. One of the issues we consistently deal with is Mira's diet. It has always been a delicate balance of weight gain, constipation, and nutrition, usually with the first two items being of greatest concern.

Years ago, we started supplementing Mira's diet with Pediasure. For any of you out there who have looked at the price of a can of Pediasure, you would be shocked. It is outrageously expensive, considering the primary ingredient is sugar. Even supplementing with a single can a day, becomes very expensive. The main issue we had with it was that it caused significant constipation. We probably spent 6 months or so supplementing Mira's diet with it, back when she was around 2 or 3 years old, then used it periodically for the next few years. The cost and the intestinal issues it caused forced us to look at other options.

What we found to work better for getting calories in her, was protein powder. There are literally hundreds of different formulas for protein powders, for which I have always had a love-hate relationship with all of them, knowing what ingredients they contain. Some are better nutritionally than others, but overall, the ingredients are constant - whey protein isolate, soy isolate, or some combination thereof. mixed with varying levels of other amino acids and vitamins. Over the years, we tried striking a balance with Mira's weight by adding small amounts of powder to her bottles, which we have realized, after years of doing this, adds to her constipation woes. I love the fact that they provide her with calories to keep her weight on, but hate the questionable ingredients and additives. Many of these powders contain massive amounts of sugar, fructose, sweeteners, and often a host of other ingredients I can barely pronounce.

A couple of years ago, we stopped giving Mira these powders and over a period of 6-9 months and she lost a considerable amount of weight, so much so, that her neurologist and pediatrician raised some concerns, and suggested we see a dietitian, which we did. The recommendation was to put her back on some sort of calorie booster, which happened to be protein powder. We put her back on it, her weight came back up to a normal range, and here we are, another year or so later, still having tons of seizures. Again, I have always had my concerns about these powders, so I went back to Sarah's original idea of Celiac Disease and started reading all about gluten, glutamine, and glutamate. I have always questioned what these essential and non-essential amino acids do to the nervous system in particular - a few weeks ago, I started really diving into what each amino acids in a typical protein powder profile actually does.

It didn't take long to see what glutamine and glutamic acid (GA) do to the nervous system and what rolse they play in epilepsy. One of the most frightening articles I read however, was how glutamine is processed within the body and how it transports ammonia through the central nervous system (CNS). Basically, glutamine enters the mitochondria, wreaking havoc on the mitochondria functions within it - basically, it's toxic and based on this article, is proposed to ultimately cause hepatic encephalopathy. Since glutamine can be produced from glutamate by the body, it is considered nonessential. Introducing additional (or excessive) amounts of GA into the body can cause neurological flux, especially if there is a problem within the cycle. Glutamate cannot cross the blood brain barrier, so it processed in the CNS to create glutamine. Disruptions or defects in this cycle of processing within the CNS can be a cause of epilepsy, Alzheimer's, and other neurological issues/diseases.

Protein powders are loaded with GA, to the tune of 3-5 grams per serving, which is an astounding amount for the body to handle and again, if there is a defect in this cycle, it can have terrible effects on the CNS. Reading all of these articles and trying to understand the connection between glutamine, glutamate, and gluten has been eye-opening. If Mira does has an issue with getting additional amounts of glutamine and her body cannot properly process it or is processing it in the CNS and there is an issue, it could be causing significant neurological issues.

I'm trying to decipher how all of this may or may not affect Mira and her diet, but the bottom line is we should try avoiding glutamine and gluten as much as possible, to see if it might help her. There isn't any harm in trying Mira on a gluten-free diet. She is basically on it already, minus the protein powder. It has to go. The second issue to be concerned with is to get calories in her, without resorting to gluten. A gluten-free diet isn't all that restrictive in terms of food choices - we could increase calories through everything from coconut oil to sweet potatoes, or any other fruits and vegetables that might be heavier in calories. As a trial, I avoided the powder all day today and Mira did not have any tonic-clonics. She had a few myoclonics this morning and this afternoon, but nothing major. She was very vocal before bed, doing her ba-ba-ba song for about 10 or 15 minutes as we raced around the house in her chair. I haven't heard her vocalize that much in awhile.

We are still doing the B6, but I would love to hear from parents out there that have tried a gluten-free diet and if it had any effect on seizure control. If anyone has any additional information on Celiac Disease and epilepsy, I would also like to read more. Thank you Laura, for all of the information and of course, thank you to my amazing wife, who always has rational, positive ideas, while I am off deep in the weeds.

Lackluster Day

Mira had so many myoclonics this morning - just a barrage of seizure activity. We had to avoid the toy completely the entire day. When she woke up, she was twitchy, cold, clammy, and completely out of it. She had zero energy at school, taking naps off and on throughout the day. Her teacher said she wasn't interested in doing much of anything - she wasn't fussy, just tired and just looked sedated. She continued to show no energy the rest of the afternoon and through dinner. She drank a few bottles and had plenty to eat at school, but she was really unresponsive the entire day.

Rough Seizure Days

Mira had a rough weekend. Saturday she was reserved and quiet most of the day, not showing a lot of energy nor interest in much of anything. We ran a few errands and it was obvious that she was not feeling well. She wasn't having too many myoclonics and only had one bigger tonic-clonic the entire day. On Sunday however, someone flipped a switch in the morning and Mira was consistently irritable from the time she woke up, until the late afternoon. We went out to visit some friends down south, which broke the cycle of whatever was bothering her. She managed to get some uninterrupted time with her toy while we were visiting, however, it was short lived. Afterward, her toy continued to be a trigger about 50% of the time we tried it. We end up just hovering over her for the first 5 or 10 minutes we give it to her and if we see her myoclonics becoming more intense, we just take it away. Many times though, she explodes right into a seizure as soon as it is in front of her. 

This morning, I knew a big seizure was coming on, as soon as she was out of bed. She was happy, smiling, and full of energy, without so much as a twitch, which is often an indication that she is going to immediately have a seizure, especially when this happens in the morning. As expected, she immediately had a seizure when she was in her chair and for the next hour, she was unable to even keep her head up for more than a few seconds. It's all a terrible cycle these days with her seizures. Her drooling has drastically increased over the past few weeks too - probably due to the constant seizures, which are causing her to be very lethargic and listless for a good portion of the day. She ended up having another tonic-clonic before dinner, which caused her to be twitchy, cold, and only mildly responsive the rest of the evening.

Controlled Substances

After battling seizures for over 10 years now, I definitely have a love-hate relationship with AEDs (antiepileptic drugs). Mira has tried so many over the years, that it all has just become a wash with what medication has what supposed mechanism of action or which one has the least side effects. One thing is for sure, no AED, by itself or in combination with any other AED, has ever stopped Mira's seizures. My love for the potential for medication is that they actually do help some children out there that are battling epilepsy. They may not work for Mira, but at east they are helping other kids.

My hate with AEDs is the method in which they are developed, run through clinical trials, marketed, and ultimately, used on children, often with little or no clinical research in regards to efficacy or safety. The pharmaceutical industry is consistently misguided in their efforts, often capitalizing on suffering patients and families like ours, all in the sake of profit. I won't digress into the latest news on OxyContin, nor again list the frightening side effects of Lyrica, Lamictal, or any of the other 15+ medications Mira has tried (or is currently taking) to help with her seizures. Most pharmaceuticals have a 'proposed' mechanism of action, but the fact that they clearly state in their prescribing information pamphlets that 'the method in which (insert medication name here) works has not been fully elucidated and is suggested to work in the following manner', is truly frightening. Not even the manufacturer, researcher, developer, nor the marketer knows how a particular AED actually works, they just assume and so long as it is documented that it is unproven, that is enough in the eyes of the FDA. It's all a guessing game, often with efficacy rates for seizure control only slightly better than a placebo. Pharmaceuticals are forced through clinical trials with this same approach: 'Well, it's slightly better than you taking nothing and it actually can create horrific side effects that are much worse than you taking nothing in the first place, but well, it will be fine, because we aren't really sure how it works anyway, so let's just see what happens. If something bad does actually happen, we will be held blameless, because well, we told you so right here in writing'.

All of this comes to light several times every, single month, whenever we have to get one of Mira's prescriptions filled. Today, in fact, we ran into a dilemma. Mira has been taking Vimpat for the past 18 months or so, which has had a negligible effect on her seizures and little effect on her irritable disposition. We continue to question why she is on it and in fact, it will be a topic of discussion in our neurology appointment coming up in a few weeks. The dilemma was that we only have 2 pills remaining and didn't realize she didn't have any remaining refills. The neurology clinic is obviously closed on the weekend and refuses to accept calls after hours. The only choice you have is to page the on-call neurologist through the main line and have them contact you, should there be a critical issue you need to deal with.

Is getting a prescription refill a critical issue? Normally no, since most pharmacies understand the importance of maintaining 'therapeutic' levels of medications in the system (especially AEDs) and can advance a few days worth to get you by until you can contact the prescribing physician. Unfortunately, you cannot do this with Vimpat, since it is a controlled substance. That's right. Straight out of the DEA's endless list of controlled substances is my daughter's Vimpat. Along side of your friendly neighborhood narcotics PCP, cocaine, LSD, and heroin, are the commonly marketed AEDs tranxene, clonazepam, clorazepate, diazepam, lorazepam, phenobarbital, and pregabalin.

Needless to say, the pharmacist cannot legally advance even one pill of a DEA controlled substance,  thus we had no choice but to call the hospital nurse line, who in turn called the on-call neurology nurse, who in turn contacted the on-call neurologist, who ultimately called in a prescription to our pharmacy, just so we could get a few days worth of Vimpat until Monday.

Why is my 10 year old child so easily prescribed a DEA controlled substance, with a questionable mechanism of action and nearly zero efficacy in terms of seizure control? Welcome to the American pharmaceutical industry. Why would giving her an oil extract from a plant put me in jail and have my children and family taken away from me? You can thank Senator Mary Pilcher-Cook for reinforcing that hypocritical, greed-infused logic here in the State of Kansas.

Same Trigger

Mira's toy continues to be hit or miss this week. She has some days where she plays solid with it for awhile, with no seizure activity whatsoever. Hours later, it will be the exact opposite - her toy is an instant trigger, sending her into an huge tonic-clonic. It's about 50/50 over the past few days. She has been doing well at school, with reports coming back that she is content and ironically, no seizures.

10 Second Window

The picture above is from a few days ago, before Mira starting having her toy turn into a consistent seizure trigger. The past 2 days, Mira hasn't lasted more than 10 seconds with her toy in front of her, without erupting into an intense seizure. The window of opportunity to remove it before she has the seizure closes extremely quickly and we seem to miss it. We have tried 4 or 5 times since Thursday evening, at various times of the day and every time, the result is the same. We hate to keep it from her, since she loves it, but I feel as though we have to take a sabbatical for a while, at least until her seizures start to come back to baseline. She seems to be doing alright the rest of the time, when she isn't in front of her toy. A few random myoclonics here and there, but nothing to the degree when she starts playing.

Ironically, Mira has been doing a fair amount of babbling and vocalizing - unprovoked of course, but great to hear nonetheless. We even went through an entire dinner out, including a 30 minute wait to be seated, in a very crowded and noisy restaurant. Mira was amazing during all of it. Not a single whimper or cry and she was even smiling a bit at times. She finished off a couple of bottles during dinner and was content just kicking her legs and moving around in her chair. We couldn't have asked for anything more, except the boys not acting like, well, boys.

Ready for Relief

It's been a long week for everyone. Mira has been having more seizures than usual, with today being probably the best day she has had all week, only having a few myoclonics this morning. She didn't have any tonic-clonics and has been in a positive, yet quiet mood most of the day. She had a fair amount of time with her toy this afternoon and she wasn't interrupted by her usual stream of head drops and seizures. It was nice to see her having some relief after a stressful week of seizures.

I have been putting in a fair amount of overtime at the office over the past two weeks, which hopefully my schedule will be returning to normal soon. With travel and juggling three different projects, September proved to be a tiring, blur of a month. It will be nice to be at home this weekend and not have to worry about deadlines, at least for a bit.

Mira has school pictures tomorrow, which is always a guess as to what sort of mood she will be in when it's her turn to be in front of the camera. Some years she is completely out of it, either slouched over or taking a nap. Other years she is just irritable, posing for the lens wearing the longest frown you can imagine. Whatever tomorrow brings, just know that she will be wearing the beautiful outfit she can, thanks to Sarah's great taste in fashion.