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!