Saturday, January 31, 2015

The Baseball Life

There are some weeks that are just like hitting a line drive into the right-center gap and having to turn what appears to be an easy triple, into something much more. Looking back, this was one of those weeks.

Monday starts off with very brief moment of silence, as you've just cranked the ball deep and you know the center fielder has about a 2% chance of snagging it. Coffee in hand and Mira on the bus, you leave home cautiously, not sure if you should maintain a respectable pace toward first or should be going with burners fully ignited right out of the starting gate. You consider your options, as the boys are finishing breakfast and getting backpacks prepped.

Then Sarah and I look at our calendars and we soon realize what lies ahead. Expressions turn to a mixture of self-inflicted mental exhaustion and mild panic. Your smashing drive to deep right center has taken flight. The right fielder, who was playing tight to the line (because you are a left-handed hitter) has virtually no playable chance and is undoubtedly out of the picture. He's hustling a diagonal toward the gap, but even with a quantum leap or perhaps a jet pack, the laser you just hit is bound for the warning track and he knows it. His potential fielding percentage for this particular catch is spiraling downward with every stride.

The center fielder however, continues the pursuit. With cleats kicking and grass clippings scattering, his upper body is leaning toward the bullet that was sent. He is relentless in his running, but the distance is too much. If only he had positioned himself another 3 steps to the right, he might have closed the gap and made this a routine fly ball. 

It's only Monday. Usually by this point, you are only 45-50 feet down the line, because well, you were making an assumption that this might be an easy week. Ease into it slowly – better save some energy. But as the picture unfolds, it's time to strategize how you are not only going to quickly accelerate, but do it with pinpoint accuracy around first, without compromising speed. You open your calendar. Neurology calls, prescription refills, endocrinology appointments, conference calls, legislative emails, unfolding travel itineraries, and IEP scheduling all come into play as you are rounding the first bag.

Monday is over and the right fielder has a face full of turf and is trying to decelerate from a failed Superman dive. He gave it a respectable effort, with the ball clearing his Rawlings by mere inches. Need to watch the replay to see exactly how far. One day down and a long road ahead. The right fielder is now redirecting momentum, trying to circle back to the ball, which is now destined for a single bounce toward the right-center wall.

Tuesday and Wednesday hit and you are already playing catch-up at work, home, and school. You are hustling second, eyeing the turn. A brief glance into the right corner, you realize the ball just took a bad bounce, in your favor. This redirection changes everything. Worst case scenario, you've now bagged a stand-up triple, with the remote possibility of getting more. Energy reserves are on standby as it's mid-week now. Dinner time is approaching fast and your Sunday procrastination of going to the grocery store comes back to haunt you. You have little to cook, so you need to paste something together in a hurry, because you have a junior high open house to attend in 30 minutes. Carry-out pizza becomes your go to source to solve that dilemma. Laundry can wait. Cleaning can wait. Mira needs a bath. All the kids need a bath for that matter, but eating is now the priority. Bath time will be late.

Between second and third, you are winded and your focus has shifted to the weekend. Yet, the third base coach is windmilling you home. Your autopilot, formerly based on your standing triple, has been disengaged and you need to tap all fuel reserves just to make it around third. You blink and it's Friday mid-day and you are wondering were the week went. The third base coach is still frantically circling his left arm out of his socket. The boys are rowdy because they had a half-day of school. Mira is fussing in her room and nothing will make her happy. The crowd is rowdily standing, spilling their $12 Bud Lights in the commemorative collector cups, as you hope to reward their excitement with your valiant, yet textbook flight path to the plate.

The second baseman snatches the relay and releases toward home. Your helmet is gone with the frantic final push of the last 15 feet. You begin initiate your final descent for the corner as the catcher's anticipation of the impending throw and potential collision causes tension on both sides. With knees blocking the front half and dirt covering the remainder of the gleaming white rubber mat, your left hand sweeps the furthest corner, a fraction before the catcher’s backward flinging mitt smacks the outside face of your forearm. You are safe, because you made it home. It's Friday evening.

Ironically, it’s only the bottom of the first inning in a previously scoreless game, so it is really insignificant with eight innings remaining. It’s the middle of the season and you are 14 games out of first place. But you have the weekend to catch up both mentally and physically, ready to start it all over on Monday. Thank goodness we don't have any solid plans this weekend.

For now, it is 1-0 and we won this inning, anyway. So, in your face last week of January.

Wednesday, January 28, 2015

Sleep and Sedation

Mira has been very sleepy today. She had to be woken up this morning and wasn't too happy about it. She was fine for about 20-30 minutes in her chair, but became irritable after that. Her fussiness continued most of the morning at school, but had she rebounded and had a better afternoon. It reached 70 degrees here in Kansas City and Mira was able to get outside during and into the swing at school. Always a sure fire way to get a few smiles out of her.

This afternoon, Mira looked really exhausted. She took a long nap after she was off the bus and again, had to be woken up for dinner. She slept for almost 2 hours after school. She wasn't as upset as this morning, but started having a ton of myoclonics after being in her chair for only 10 minutes. She was in a fairly pleasant mood during her bath, but continued to have countless seizures while awake for the next hour or so. Once she was back in her bed, she crashed again and was fast asleep within minutes.

I think it's fair to say that the Lyrica increase has kicked in, causing some heavy sedation with her today. She has been on a much higher dosage than she is currently at, so it is the increase that she is getting accustomed to. The delicate balance with irritability, sedation, and seizures is a struggle. We have noticed that she has been drooling significantly more over the past week or so, again most likely a result of the Lyrica.

Tuesday, January 27, 2015

Neurology + Endocrinology

Mondays are always crazy in the neurology clinic. Every parent and caregiver that has had issues over the weekend (us included) start bombarding the nurse voicemail inbox, in a hope to get some input or refills on medications. That is exactly where we ended up. The clinic is great about returning calls and keeping up with everyone - their patient load has got to be overwhelming, based on the number of children each neurologist corresponds or sees in clinic on a given day.

We decided to move Mira up on the Lyrica, of course try and help combat some of the irritability. The increase started this morning and while her temperament was mildly improved, her seizures were in full force today. She had two long tonic-clonics early. Ironically, she had an endocrinology appointment this morning she had these back to back seizures as we were getting ready to go. She continued to have a ton of myoclonics the rest of the day.

Nothing new at her endocrinology appointment. We discussed how long we should continue to do her Lupron shots, which she has been doing for the past 2 years. We will reassess where she is at in another year before we decide to stop.

Tonight, Mira was pretty quiet before dinner - lots of seizures with her toy, but not crying. The best we can hope for right now.

Sunday, January 25, 2015

Back to Baseline

Mira had a rough day yesterday, with a lot of crying and wailing, which put us right back to where she was earlier this week. The cycle continues, as we have no choice but to call neurology tomorrow morning and increase either her Lyrica or Vimpat. Just roll the dice as to which one will help alleviate her irritability level or her debilitating seizures. My guess is the Lyrica, but it is only that, a guess. When we put her on Vimpat late last year, he told us flat out that the chances of it being effective for seizure control hovered around 0%. Such a comforting statistic.

This morning, she has been having non-stop myoclonic seizures, every 5-8 seconds, for the past 45 minutes, that were causing her to grunt and drool with every one. She then erupted into a long, involved tonic-clonic seizure that has now completely knocker her out. This cycle will never end it seems.

Vimpat reminds me a lot of Onfi. Onfi (clobazam) of course is a benzodiazepine, known for causing lethargy, sedation, and is addictive. So addictive that benzodiazepines are listed as a DEA Schedule IV narcotic and Onfi, as well as most mass-markets AED benzos, are listed as controlled substances because of their addictive nature. This is clearly stated in the full prescribing information (page 12, section 9.2) that comes with every pharmaceutical.

I read every one of these pamphlets and most companies attempt to explain the MOA (mechanism of action) within the literature, only they can't. Onfi clearly states that the MOA is unknown, on page 15, section 12. If you have to explain and disclose 26 pages of information for a single pharmaceutical, you have some serious attorneys creating a litigious safety net for your company. You assume the risk because they disclosed it to you.

Oh and by the way, one of the potential side effects of Onfi is Stevens-Johnson Syndrome, clearly stated on page 1, under 'Warnings and Contradictions'. If the side effect of having your skin die and literally fall off of your body doesn't scare you, then I don't know what will. Lamictal (lamotrigine) carries this risk as well and Mira has tried both of these pharmaceuticals, to try and stop her seizures.

Saturday, January 24, 2015

The Extremely Short Honeymoon

As with all pharmaceuticals, the honeymoon started to fade yesterday. After having a wonderful 2-3 days when getting back on Lyrica, yet again, Mira is slowly shifting back to baseline. The smiles went away, the irritability came back, and the seizures have been ever present throughout. We have been down this path so many times and I can assure you, it is incredibly frustrating. With no remaining reasonable options with a potential efficacy rate over 0%, this is as good as it gets, which to me, is unacceptable.

Mira doesn't deserve to deal with these same ineffective pharmaceutical options, over and over again. When you have no remaining medical options left to help your child, this is what happens. Ironically, there are options that need to become available. Mira's neurologist is advocating medicinal cannabis. Yet, based on the testimony of the opposition presented in Topeka this past Thursday, the State of Kansas appears as though it will continue its hypocritical stance on this treatment option for another year. There are fighters in the state and I can't thank them enough for their perseverance in the face of so much absurd opposition. The uneducated and special interests always seem to prevail in killing bills like SB9. In the meantime, children like Mira suffer.

The same cast of characters of opposition, year after year, blow the dust of their dated and misinformed 'data' and lobby the supposed harmful effects of cannabis, presenting a plethora of dangers to the state and the community. The reality is that the perceived threat is to profits and self-preservation. Then throw in the religious card and unnecessarily muddy the waters further. Tired, irrelevant opinions and personal beliefs continue to be the basis of their argument, which has no room in a political arena when it comes to treatment options for the health of my child. It is very simple. State officials should not be making medical decisions for the citizens of this state and opinions should not be the deciding factor in this debate. Patients and medical professionals should be making those critical decisions.

While over 70% of the public is in favor of medicinal cannabis legislation, the remaining 30% includes a select minority of medical professionals (by my count is 1 who I can name specifically) who continue to not only oppose medicinal legislation, but willingly fight it year after year in this state. What doctor in their right mind would deny a scientifically proven medical option for children?

Doctors like Dr. Eric Voth, who has a vested financial interest in Stormont Vail, a for-profit medical treatment facility in Topeka. Every January, Voth embarks on his annual crusade on the behalf of his own financial interests only, while continuing to ignore existing medical science. Associating with a facility whose mission statement includes 'working together to improve the health of our community' is about as hypocritical as it gets. A facility that involves professionals who personally fight for the exclusion of life-saving treatment options is not my idea of improvement, but rather a controlling restriction on what generates revenue. Voth's views on the subject are dated extractions and opinionated at the least, which is truly frightening for a licensed medical professional. The transparency in his advocacy is so predictable and tiresome, yet people listen because it is easier than becoming educated by doing their own research.

As you can tell from my venting, I am tired of the unfounded rhetoric that the opposition continues to promote and many of our supposed state representatives continue to take the path of least resistance. Mainly, I am tired of watching Mira go through this for the past 9 1/2 years and have a state representative seriously tell me as a parent that 'you should continue to explore other options' for your child. What are those options are exactly?

I'll tell you what. I will go into a lengthy explanation of each medication Mira has tried and the risks associated with said chosen pharmaceutical/dietary/therapeutic treatment option and its efficacy in stopping my daughter's seizures. After I am finished, 45 minutes later, you can give me another option. My guess is you won't have any. Unless you are a pediatric neurologist specializing in catastrophic epileptic syndromes involving the mechanism of action of therapeutic agents on voltage gated calcium channels, then chances are, as a parent, I know far more than you when it comes to my child's issues. So, as a paid state representative, stop blocking personal decisions between me and my child's doctors.

Right now, Mira is screaming her lungs out in her bed at 10:30am on a beautiful Saturday morning. Another day in paradise.

Thursday, January 22, 2015

Tonic-Clonics

Mira had a big tonic-clonic seizure this morning about 6:00am. It was a terrible start of the day for her - no one wants to wake up to that. I was up for a few minutes when I heard her make an all too familiar sound - a sort of loud grunt, which I knew was the beginning of a heavy seizure. It lasted for about 30-45 seconds and she was completely out of it for the next 30 minutes. She managed to rebound somewhat over the next 90 minutes before the bus arrived, but she was still pretty quiet and reserved.

She ended up having a decent day at school - not quite as active and engaged as she was yesterday, but still happy and engaged. When she got home, she another involved tonic-clonic seizure, just as bad as the one this morning. She again was very lethargic afterward and took a decent nap. As I have posted before, Mira ends up having anywhere from 5-10 of these a week, sometimes multiple tonic-clonics in one day, as she did today. Unfortunately, it isn't unusual for her. I remember years ago when she had 7 or 8 in one day. I hate epilepsy more than anything and watching her body and mind being tormented is awful. It is the longest minute of your life watching it happen to your child.

She was understandably quiet during dinner and not so enthusiastic about her toy. She still played a little, but was obviously drained by the seizures today. She went to bed a little earlier than usual.

Wednesday, January 21, 2015

180 Degrees

What a difference a day makes. Mira had an awesome day today, which is a complete 180 from where she was a couple of days ago. The report from her teacher at school said that Mira was all smiles today - happy and laughing for most of the day. All of this after only starting back on Lyrica 36 hours ago. Never again will we question if it helps with her irritability. It has had a remarkable impact on her since yesterday - it might be a honeymoon period, as we have seen with other medications, but for now, we will celebrate Mira being in a positive mood!

She had about a dozen myoclonics this morning, certainly down from the hundred or so she had yesterday. Mira took a nap after school and woke up fussy for just a moment, but she snapped out of it within a few seconds after getting her toy in front of her. For the next two solid hours, she played, drank a few bottles, and we raced around the house in her chair. Nothing seemed to tire her. She had a few myoclonic seizures when she first started playing, but didn't really have any visible drops after that.

Right now, she is quietly looking around in her bed, still not really ready for sleep. Today was a welcome change from the last couple of months. Such a positive, remarkable change in such a short amount of time.

Tuesday, January 20, 2015

Welcome Back, Lyrica






We gave this a good run, but taking Mira off of Lyrica has been a complete disaster. This is a medication that she has been on for 7+ years and we decided back in November to wean her off it, as it appeared to have zero efficacy with seizure control. That still holds true. Lyrica does little to nothing for her seizures, but as we discovered over the past 6 weeks, it does a ton for her irritability.

After having another miserable day yesterday, wailing and crying for hours, we gave Mira trazodone. It took an hour to start working to calm her down, this after going for several hours just crying. This is the second time in the last few weeks that we had to resort to giving it to her, as she was having another awful day this week. We have gone to the trazodone more in the past month than the past 2-3 years combined. Mira is exhausted and we are exhausted from all of the inconsolable crying.

Thus, after talking with neurology, we collectively decided to put her back on a low dosage of Lyrica. It's safe to say that this medication change (we have been through a ton of them in the last 9 1/2 years) was an utter failure. Let's take a look at the equation:

Seizures + Irritability = Misery

Take your pick. Mira has uncontrollable seizures, which no medication, diet, or therapy can stop. Add extreme irritability to the mix and you get misery. By putting her back on Lyrica, hopefully at least one of those symptoms will diminish or even drop out of the equation completely. It feels as though you are picking the lesser of two evils sometimes. Ineffective pharmaceuticals or wailing or both.

We started her back on it this morning and by this evening, you could already notice a change in her mood. She wasn't screaming through her bath and her appetite has returned in full force, as she downed three bottles in 20 minutes. I also noticed her putting her hands in her mouth, repeatedly. I'm not sure this is a stimming ritual or what - many girls with Rett Syndrome tend to do this a lot. I hadn't seen her do this over the past few months and she was doing it non-stop after her bath. She was also laughing some while she was doing it.

We are still keeping her on the Vimpat as well. It's also a very low dosage. At this point, we have to just live with the seizures, as Mira always has. She had over 100 myoclonics the first hour she was awake this morning, which is why we are always searching for help to try and stop them. They just overwhelm her some days and she can't do anything. At least now, maybe her demeanor will improve.

Sunday, January 18, 2015

A Better Sunday

Mira had a much better day today. She was mildly irritable this morning, but she got over it fairly quickly and had a solid stretch with her toy after breakfast. She had some very strong myoclonic seizures that I swore were going to unfold into a full-blown tonic-clonic seizure on numerous occasions throughout the day, but they never did. She managed to have around a dozen of these strong myoclonics, but she came back to baseline within 20-30 seconds, most of the time. The seizures all surfaced as sort of longer, drawn out pre-tonic-clonic seizures that lasted about 10-15 seconds. It's hard to describe them.

We were able to get outside and get in the swing for a while. Mira really enjoyed feeling the sun on her face and was worn out after 20 minutes or so in the swing. She was going at a fairly slow pace too, but was laughing hard for the first 30 seconds. She took a long nap afterwards and woke up in a pleasant mood this afternoon.

This evening, she had another good block of time with her toy in her chair, only having a few myoclonics - similar to what she was having in the morning. She ate very well and quietly fell asleep a few minutes ago.

Saturday, January 17, 2015

The Day Begins

Another day of seizures and irritability, right out of the starting gate. Mira is wailing in her bed and it's only 10:00am. Ten minutes in her chair and she was done. She had a half-day of school yesterday and with the holiday on Monday, it is going to be an extremely long weekend at this point.

We will try to get outside, as it is supposed to be a nice day, but I am not optimistic that Mira's temperament will improve. This has been going on for weeks. We have no choice but to call neurology on Tuesday and try something else.

Thursday, January 15, 2015

The Crabby Train Rolls On, Then Stops in the Station for the Evening

Mira has been having repeat days of overwhelming irritability. This week has been pretty consistent with her - never happy for more than 15-20 minutes, doing one thing, or being in one place. This trend has been going on for weeks and we are now considering upping her Vimpat dosage. Her temperament has been consistently poor at school, at home, and everywhere in between. Then, out of the blue, she had a great moment tonight.

Mira had almost an entire hour before bedtime tonight, where she was happy and engaged with her toy - full of energy and only a few brief seizures. We then were able to race around the house in her chair for awhile, which she really seemed to enjoy. After Mira was in bed, she was interacting with me by scratching her right hand on the side of her bed, smiling and laughing when I started scratching in return. We alternated this scratching back and forth for a good 10 minutes, with Mira smiling every time I did after her. It was such an awesome moment to be able to communicate with her on some level, just with some simple sounds and texture.

Afterward, she sat up in her bed, folded herself over (her favorite sleeping position) closed her eyes, and quietly went to sleep.

Sunday, January 11, 2015

Crabby Day

Mira has been pretty irritable today. She woke up crabby and quickly tired of being in her chair after only 10 minutes. Again, another day of off and on irritability, where nothing sustains her attention long enough to keep her from crying. She did have good stretch in her chair this afternoon for awhile, but that was the high point of the day. She had a few myoclonic clusters this morning, but we haven't seen much seizure activity all day, ironically.

Sometimes it feels like a vicious cycle of transfers with Mira. From her chair to her bed, back and forth so many times during the day, because she so quickly swings the fuss-o-meter from one end of the spectrum to the other. It's exhausting for everyone, Mira included, to try and maintain a constant state for more than 15 minutes at a time it seems.

Unfortunately, it was too cold and rainy to get outside again. Today would have been a good swing day for Mira. Hopefully, getting back to school tomorrow will get Mira out of her Sunday blues.

Saturday, January 10, 2015

Kansas Legislation and Reception


As most people are aware, there has been a nationwide movement for the legalization of the use of medicinal cannabis for debilitating syndromes, including epilepsy. If you aren't aware of the potential benefits of cannabis, I strongly encourage you to start reading, researching, and forming your own viewpoint. As I have posted before, there are countless studies that analyze the potential for each of the chemical properties within the plant and their affects on our own internal and biological endocannabinoid system. I believe it is a viable treatment option for Mira and am advocating its usage here in Kansas.

In Mira's case, we have tried everything the pharmaceutical industry and the medical profession have to offer. Over the past 9 1/2 years, some medications seemed to help in subtle ways, but never in terms of seizure control or normalizing her EEG. Other medications considerably worsened her condition and symptoms. Felbatol, acetazolamide, and a host of others were horrible medications for her, causing extreme irritability and increased seizure activity. If you have ever read the side effects and literature associated with all pharmaceuticals, you would be mortified about giving them to your child. Most, if not all mass-marketed AEDs have not been tested on children and it is clearly stated on the pamphlet that comes with your monthly dosage. Pharmaceuticals can be addictive and dangerous. The CDC offers their annual findings on pharmaceutical overdoses and the percentages of those that involve pediatric care.

Pharmaceuticals work for many people and I am certainly not anti-medication nor anti-establishment, but I can assure you that no pharmaceutical has ever worked for Mira. We have assumed the risk with trying different medications, often multiple at a time, but we have lost all hope with trying another one with a very specific, isolated mechanism of action.

Is cannabis the answer for Mira? I don't know but she should have the choice to have access to at as a minimum. I am optimistic about the potential of cannabis to help stop the needless suffering of so many people from debilitating diseases and syndromes. In terms of epilepsy, the efficacy rates for CBD only extracts are very promising and I imagine that an increased scope of CBD/THC access will have an even larger positive impact. Mira deserves to have cannabis as a medicinal option. 

The first step in gaining access legally is through legislation and lobbying. As I had stated in a previous post, I am part of an advocacy group that is supporting a compassionate care bill for the medicinal use of cannabis, Senate Bill 9 (SB9), that needs to be put in front of the Kansas legislature and passed as soon as possible. The bill has died in committee in both 2013 and 2014, but this year we hope will be different. There is solid public support in Kansas and it is just a matter of time before legislation, whether it be medicinal or full legalization, is demanded by the people.

I will be attending a reception in Topeka this Thursday, with several other support groups, including Bleeding Kansas, KC Norml, and Kansas for Change, Inc. to help generate support and exposure for the bill. If you are in Kansas and wish to help support, please join us by attending. The details are on the flyer above and please email me at marcus1988001@yahoo.com if you need additional information. 

Friday, January 9, 2015

TGIF

It always seems to take a week to get back into the swing of things after being off for two weeks. I think overall, Certainly not complaining, as I feel spoiled to get two entire weeks off from work. Mira was happy to be back at school and back doing some activities during the day. Her teacher said she had some fussy moments during the day, but overall was in a positive mood. She is getting used to her new DAFOs at school too, which she was fitted for late last year and we were able to pick up over the Christmas break.

Mira had a pretty similar day as yesterday, although not nearly as many seizures this morning. She had a few clusters when she first got out of bed, but they faded out after a few minutes. This evening, she again played with her toy for a solid hour and was vocalizing almost the entire time. She had a decent appetite afterward too. She wasn't up much for racing around the house and seemed a little worn out by bedtime.

This video is from about a month ago, but it is always great to see her enjoying her toy!

video

Thursday, January 8, 2015

Strange Morning, Great Evening

Mira woke up this morning and was pretty quiet. She started having constant myoclonic seizures before she was even out of bed. She was having a seizure about every 10-15 seconds for a solid 45 minutes this morning. She wasn't crying and tried to keep going back to baseline right after each episode, but I have not seen so many in such a short period of time. The myoclonics slowly faded out about 10 minutes before she got on the bus. She did great at school and didn't have any additional seizures the rest of the afternoon. She refused to drink anything this morning and also refused anything to drink at school. She only drank a single bottle all day, until dinner time.

Before, during, and after dinner, Mira was in a great mood. She played consistently with her toy for almost a solid hour, without so much as a twitch. She was even laughing and giggling at one point. She drank a couple of bottles and went right back to playing. We then raced around the house in her chair for another half-hour or so, in which she became very vocal and animated, kicking her feet and appearing very engaged with everything.

You never know what every day is going to bring. Mira had seemingly polar opposite neurological symptoms today - constant seizures in the morning to looking completely engaged without single spasm this evening. A strange day indeed.

Wednesday, January 7, 2015

Cold, Cold, Cold


Thanks to the arctic blast that has been suffocating the area for the past few days, we have not ventured out much, unless it is essential. Surprisingly, they didn't cancel school today, even when the temperature hovered around 3 degrees most of the day, with a windchill of -20 or so. Mira was bundled up with multiple layers this morning before getting on the bus. We have been keeping her warm at night with these awesomely fashionable socks. Sarah has great fashion sense and Mira is always getting compliments on her sparkly shoes and colorful socks.

Mira has been fussy off and on and school for the past two days - I think it may take her a while to get back into a groove with school. She was very irritable this morning, having a lot of myoclonics and refused to drink anything. She is back into her consistent flow of 20-30 myoclonics at a time, then we don't see any for a few hours. She did the same thing for dinner, but finally decided she wanted to eat right before bed.

I am already tired of winter. We will just be waiting for the temps to rise above at least 20 by the end of the week. In the meantime, Mira will be getting a lot of usage out of her fuzzy sock collection.

Monday, January 5, 2015

Rough Day

Mira had a pretty rough day today - loads of irritability and inconsolable crying. Nothing would suffice and keep her happy for any length of time, especially this afternoon when she had a marathon of fussing. Hardly any seizure activity, which is great, but obviously something else was bothering her immensely.

Part of the problem may be that Mira is very much out of her routine. Tomorrow she goes back to school - having been at home for the past few weeks is maybe getting to her. Boredom? Getting stir crazy? Not enough activities during the day? Not sure, but tomorrow she will be getting back to her usual schedule. One thing is for sure, she will be getting seriously bundled up in the morning, since the forecast is calling for a low of 3 degrees and a high of 9 only degrees on Wednesday.

Sunday, January 4, 2015

The Myoclonic Seizure

I've probably mentioned this before in a previous posts, but our days are often reminiscent of the movie 'Groundhog Day'. This is the third day in a row where Mira has woken up, been very irritable, being cranky just long enough to drink a bottle for breakfast, then went straight back to bed. Like clockwork, was very irritable for most of the morning, then took a really long nap again.

Only today, right before lunch, she woke up, was happy as can be, for about 20 minutes. She was vocalizing and laughing in her bed for a solid 15 minutes, then quieted down and became fussy right afterward. I got her out of bed, had something to eat with her, then she proceeded to have a ton of myoclonic seizures, probably 20-30 within about 15 minutes. Fussy again, then back to bed. Then a quick cat nap. Then up again, because she is irritable an hour later. Repeat again.

She continued to have multiple myoclonic episodes for another 10 minutes, then went into a full-blown tonic-clonic seizure, which lasted about 30 seconds. It was just a matter of time. When she shifts the mood gears, peaking from ecstatic happiness to wailing despair and back again, she usually caps it all off with a big seizure. Then she another tonic-clonic 90 minutes later.

She is now postictal and lethargic, which is typical for her after a draining seizure. She averages anywhere from 4-8 of these tonic-clonics a week, most of them occurring while transitioning out of a myoclonic seizure. It's difficult to explain unless you witness these brief myoclonics. They will scare the daylights out of you, if you aren't used to seeing or hearing them. Mira's entire body will very unexpectedly and rapidly go completely rigid, her arms project outward, while her body slumps forward, and her head always, without fail, turns to the left side.

For those parents who have seen Infantile Spasms (IS) you will know exactly what I am describing. It looks almost identical to a jackknife seizure, but lasts slightly longer. All of this happens in the first half-second, with the total seizure only lasting 4-5 seconds, and all without any warning. Most of the time when she has them, she is in her chair, with her tray in front of her. Because it happens so unexpectedly and with her arms or toy slamming against the tray when it starts, it will undoubtedly get your attention. She is able to slowly transition back to what she was doing within a few seconds after it is over. She rarely goes a day without have one and on a bad day will have 100+ myoclonics a day.

Every day Mira deals with this relentless pattern of myoclonics, tonic-clonics, and bouts of dystonia. Every, single, day she lives with this. Catastrophic epilepsy sucks. Nothing more. Nothing less.

Saturday, January 3, 2015

Same Morning, Better Afternoon


Mira is having a better day than yesterday, overall. She woke up this morning in a pretty foul mood - crying and wailing. Just like a repeat of yesterday, she wasn't happy for long through breakfast and wanted to be back in her bed. She had a series of myoclonic seizures hen she first woke up too, which frustrated her when she tried playing with her toy a little. She ended up back in bed and was irritable for awhile and then took a really long nap. I think all of yesterday and this morning really exhausted her.

She woke up briefly for lunch, had a few bottles, then took another lengthy nap afterward. This afternoon she continues to have a bunch of myoclonics, in her bed and in her chair. She has periodic bouts of crying and fussing, but nothing to the degree of yesterday. With the exception of this morning, this is turning out to be pretty typical day for Mira.

On a positive note, we received one of her backup toys late yesterday, from an Ebay seller that re-listed one for us that hadn't sold previously. It works and is in mint condition too. Thanks to Katie + Jim Thompson for the link!

Friday, January 2, 2015

No Seizures, Tons of Irritability, and the Gawker

Today is turning out to be another roller coaster day for Mira, similar to some of her days last week, but taking it to a whole new level of irritability. She woke up this morning crying and wailing, lasting only a few minutes in her chair after breakfast before the onslaught of fussing restarted. On the positive side, she is eating and drinking fine, but very uncomfortable with something. She continued to be very distraught most of the morning and is still going strong into the afternoon. No seizures, no myoclonics - just lots of crying and irritability. We went out to swing for a little in the park, which Mira enjoyed briefly, but the irritability returned with intensity. Eight hours later, she is still crying off and on. We are not starting the year off on a high note, unfortunately. Today is a trazodone day.

We have pulled out all the stops today, but nothing has worked thus far. Right after lunch, I decided to mix things up and run a few errands with her - she usually loves being in the van, getting out and about. So Mira and I took a short drive down to Whole Foods. She enjoyed the ride, but her mood changed quickly. Once we were in the store, she started wailing once again.

When your 4 month old is crying in the store, most people stare and smile, offering that sympathetic frown to both parent and child. When your 9 1/2 year old, non-ambulatory, non-verbal child is crying at the top of her lungs, people just stare. They stare a lot. Some stare a little more, perhaps because they need to absorb what they are witnessing. Most people practice common sense and willingly exhibit appropriate social etiquette. They smile at Mira, then at Sarah, myself, Eli, Jonah, or whoever is with us at the time. Then they move on. Sometimes they have a nice comment on her shoes or give us a reassuring nod of encouragement.

Then there are people who just stare for an uncomfortable, extended period of time. This is when things get interesting. Sarah and I will call them out on it by simply staring back at them until they make eye contact with either one of us. After the realization that there has been some reciprocation with one or both of us watching them the entire time, there is that awkward moment where they don't know what to do, now that they have been exposed. About 98% of the time, they quickly scan their immediate surroundings and prepare an exit strategy because they seem embarrassed, which is understandable.

Then there are the gawkers. Gawkers are usually children, particularly girls, between the ages of 3 and 6. They love to stare because they are curious. They know Mira is a girl, very close in age to themselves, but she is in a wheelchair. They stare, but they ask questions because they want to know. For an adult, if you are staring for so long, I doubt you are contemplating your organic peanut butter options while locked in a glance with Mira, but rather wondering why our 9 1/2 year old child is functioning at a 3 month old level by crying and screaming. If you want to know, stop staring and ask.

I ran into such a gawker today. After we got through the preliminary round of extended staring and my reciprocating stare at her, the gawker slowly shifted her glance from Mira to me. She stared at me for a solid 3 seconds, then back to Mira (as she is crying) for a couple of seconds, and then back to me again. With such a prolonged exchange, I anticipated and prepared a response for her list of questions. Based on the length and intensity of her watching, I assumed she must have a complex question or at least something deeply profound to say. Completely void of expression, she said nothing. She just stared at me. I gave her a smile and continued to wait for a response that never arrived. She simply turned, while simultaneously raising her eyebrows, and walked in the opposite direction. This uncomfortable exchange of silence and stares only lasted 10-12 seconds, but it was as awkward as it gets.

My initial thought was Mira and I had deeply offended her, creating an undesirable Whole Foods shopping experience, one ripe for a negative Yelp review. Was she offended? Did she want me to try and quiet Mira's irritable disposition? Was my beard too unruly for Prairie Village's hygiene standards? But then I thought, I really don't care. I don't have any idea what was going through that woman's mind and I will probably never know.

The whole gawking experience gets under my skin, but fortunately, it rarely happens. It's been a kind of strange, 'gawkeresque' day I suppose - anything can happen. It's now almost 4:00pm. Thirty minutes after administering some trazodone, we are anxiously waiting for it to kick in, as Mira continues her marathon of crying.

Thursday, January 1, 2015

The New Year


Since 2014 was the year of the seizure, we are hoping for something a whole lot better for Mira in 2015. This past year, Mira struggled with seizures every single day. As I stated in a post about a month ago, her myoclonic seizures increased dramatically this past 12-18 months and while her tonic-clonics seemed to be fairly consistent, they were nonetheless debilitating for her. The intensity of a TC seizure is exhausting for her and terrible to witness. Fortunately, they never last more than 30-45 seconds, but her breathing is intermittent, and post-seizure, she is completely exhausted. She has anywhere from 5-10 tonic-clonics a week. She had 2 TCs yesterday afternoon, about an hour apart, along with tons of myoclonic seizures.

Last year, we also had a number of days of dystonia or dystonic activity. Mira would have periods of what her neurologist refers to as 'non-seizure activity, but neurological in nature'. During these events, Mira is completely rigid, has clammy extremities, and while conscious, is very lethargic and unresponsive to much of her environment. Her movements become repetitive and it is often difficult to get her to drink much. They typically last anywhere from 12-36 hours, with a solid day being more the norm. Mira has had these events for years and they don't seem to be predicated by anything - no triggers, no warning, no diet changes, or anything we can think of. They just happen without warning.

In addition to seizures and periodic dystonia, Mira struggles with irritability. We have no doubt that the constant barrage of neurological interference in her brain (hypsarrhythmia) causes her discomfort, pain, and frustration. She has a point in the day, every day, when she is irritable. It is one of her ways to communicate what she wants, but since she is non-verbal, it is obviously difficult to interpret her needs. She has positive days and she has some truly awful days, where she is inconsolable. Mira goes through bouts where she has a consistent time of the day, where something would set her off and she just check outs. We refer to this time as (Un)Happy Hour, as it ironically occurs in the late afternoon. 

We have tried benzodiazepines in the past, trazodone in particular, but it seemed to lose its efficacy over the years and we abandoned it this past year. Ironically, we have just re-ordered an increased dosage of it this past week, just to have it on hand for those terrible days for her. We would only have to resort to trazodone when she simply can't decompress, usually a couple of times a year. On a typical day, we resort to all of the usual tactics - something to eat, quiet time in her bed, trying her toy, go for a walk (weather permitting) and if all else fails, just wait it out. Now that we have an extra option, an ADA swing in the park right next door, maybe we can celebrate Unhappy Hour less often.

In terms of medications, Mira is completely off of Lyrica, after 7+ years. She had some rough withdrawal days over the past month and I truly hope we are out of the woods with it. Ironically, her seizures have been constant throughout this weaning period. Pharmaceuticals have had little to no effect on her seizure activity over the past 9 years and we have lost all hope with them. She is currently on a small dose of Vimpat (lacosamide) right now and we plan on continuing a trial with it, but keep in mind, this is the 22nd medication we have tried. The chances of a marketed pharmaceutical working for her for seizure control, are slim to none. The only hope is that they might help with her irritability, pain, or even dystonia. Also keep in mind, we have tried the Ketogenic Diet, Modified Atkins Diet, and a host of CAMs (complimentary alternative medications) including herbs, vitamins, amino acids, and minerals.

The lack of efficacy of traditional pharmaceutical medications has forced us to look at alternatives, one of which is cannabis. While some may find this option controversial, I find it very straightforward. Pharmaceuticals do not work for Mira. They have very specific (assumed) targets and questionable mechanisms of action. Most pharmaceuticals admit they have not been tested on children. The literature and documentation discussing the side affects of these medications is frightening and depressing. Let's move on and try something different.

Over the past 18 months, I have been researching and advocating CBD/THC as a viable option for Mira. There are obvious legal issues to contend with, primarily the fact that we live in Kansas, which is a non-legal state for medicinal CBD. We are trying to change that. I am on the board of an advocacy group to facilitate the Compassionate Care Act in Kansas, called Bleeding Kansas. The next few months will be critical for the group, as we need to get the appropriate legislation (SB9 and HB2198) in front of the appropriate committees again this session. Missouri has already legalized cannabis for medicinal use, joining 25+ other states who are understanding the need for a viable treatment option for debilitating diseases and syndromes.

I will avoid going into a lengthy soapbox on cannabis, but I can assure you that there is an astounding amount of history and research on the efficacy of the plant and before forming an opinion on it, I would strongly encourage you to become educated. If you would like links or information, please let me know and I will provide. The bottom line is that we all have an endocannabinoid system within us and CBD/THC compounds, which are natural to the plant, target those receptors and genes in the body, with therapeutic results. You need not have a degree in neuroscience or medicine to comprehend the potential for cannabis. Just look at the USNLM-NIH and read a few of the 400+ articles on CBD and THC therapeutic studies.

Historically speaking, hundreds of companies, including Eli-Lilly and Parke-Davis used to market and distribute cannabis tinctures for the treatment of epilepsy, among other sydnromes. All of this was wiped away in the 1930's by the movement (thanks Harry Anslinger) to demonize cannabis and pave the way for it to be promoted as a dangerous, addictive Schedule I narcotic. This was reinforced through the 1970s-80s with the enforcement of a drug 'war'. We all know where that has gotten us as a society. Fortunately, perceptions have shifted and will continue to do so. End soapbox.

Fast forward to 2015, GW Pharmaceuticals is leading the way with cannabis research, releasing Epidiolex for several clinical trials in the U.S. over the past 18 months. The results that are coming back for the treatment of Lennox-Gastaut Syndrome (LGS) and Davet Syndrome are extremely promising. Companies are jumping on the CBD bandwagon to try and reap the potential profits of this societal shift, which is great, so long as they all keep in mind that the end result will be to stop people from suffering.

I am optimistic that there is something that out there that will help Mira and am always open to reading and listening to whatever anyone has to contribute. Sarah and I thank you for all of your love, support, and help over the years and we are expecting great things to happen in 2015!