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


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 if you need additional information.