Tuesday, May 8, 2018

Medical Cannabis Benefits: Treating Dravet Syndrome



Dravet syndrome is a type of severe epilepsy that develops during the first year of childhood and is characterized by frequent febrile seizures. Studies have shown cannabinoids and their influence on the endocannabinoid system have the capability of reducing, and in some cases eliminating, seizures.
Overview of Dravet Syndrome
Dravet syndrome, which is also referred to as severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy that typically develops during the first year of life. The high frequency of seizures caused by the syndrome commonly causes children to experience difficulty developing language and motor skills. In addition, they often experience hyperactivity and have problems relating to others.

Dravet syndrome typically causes febrile seizures, which are fever-related (associated with a high body temperature but without any attributing health issue). Seizures can be triggered by slight body temperature changes, flashing lights, emotional stress or excitement. In addition, myoclonic seizures, characterized by involuntary muscle spasms, and status epilepticus, a state of continuous seizure that lasts longer than 30 minutes and requires emergency care, can arise.

According to the National Institute of Neurological Disorders and Stroke, 30 to 80% of Dravet syndrome cases are caused by defects in the SCN1A gene, which are essential for the brain cells to properly function.

There is no cure for Dravet syndrome, but in some cases medications can help control seizures. In addition, adjusting the diet so that it’s high in fats and low in carbohydrates may be beneficial. While it’s a lifelong condition, it’s not uncommon for children with Dravet syndrome to experience improvements and better cognitive function with age.
Findings: Effects of Medical Cannabis on Dravet Syndrome

Studies have found that the body’s endocannabinoid system can be modulated to produce anti-seizure effects (4,5). Cannabinoids found in cannabis interact with the endocannabinoid systems cannabinoid receptors (CB1 and CB2), suggesting that they may hold therapeutic potential for managing seizure activity.

The endocannabinoid systems CB1 receptor in particular has shown that it can be used to inhibit the release of a particular neurotransmitter and reduce overall neuronal excitability, thus silencing the triggers of seizures(1,2). Body-synthesized cannabinoids, called endocannabinoids, have shown to play a role in the regulation of seizure threshold and intensity, suggesting that cannabis-derived cannabinoids and their influence on the receptors may also support the endocannabinoid system and its efforts to control seizures caused by Dravet syndrome(4).


Using Medical Cannabis to Treat Dravet Syndrome
Cannabis is a complex medicinal plant that may actually be used to treat a variety of debilitating symptoms caused by a surprisingly large number of ailments. It’s usefulness as a non-lethal medicine (you cannot die from an overdose of cannabis) cannot be overstated and it’s versatility in terms of how it can be consumed and as to how it can be useful for so many illnesses is something to be excited about. However, it is important to remember that consulting with your primary care physician should be your first priority when considering incorporating cannabis into one’s medical regiment and that cannabis is to be used as an adjunct therapy and not a replacement. It is also your responsibility to communicate with your doctor as to how your use of cannabis has affected your health and of your progress with utilizing medical cannabis.

It is both exciting and important to know that medical cannabis is currently being heavily studied for a number of therapeutic effects that may treat this ailment; most noteworthy, the cannabinoid compounds Tetrahydrocannabinol (THC), Cannabidiol (CBD), CBN (Cannabinol) and even THCV (Tetrahydrocannabivarin) for their ability to manage and reduce seizures in those suffering from Dravet syndrome.

Overall, medical and scientific studies throughout the past several years and even decades have shown that cannabis may be quite useful for treating individuals suffering from Dravet syndrome for the following reasons: reducing the frequency of seizures; protecting the brain from further damage and degeneration; assisting with possible psychosis; helping to manage depression; easing possible anxiety; aiding with sleep; and providing significant pain relief.

CNN ‘Weed’ -A CNN Special Report by Dr. Sanjay Gupta (2013 Documentary HD)

Arguably one of the most popular stories that first landed CBD in media headlines is that of Charlotte Figi, a young girl from Colorado, USA, who suffers from Dravet syndrome and depends on a CBD-rich tincture to keep her life-threatening seizures at bay.

Charlotte’s story first made headlines in 2013, after Charlotte’s parents began treating their 5-year-old daughter with a CBD tincture manufactured by The Stanley Brothers in Colorado. The tincture has since been renamed Charlotte’s Web.

Charlotte suffered from severe tonic-clonic seizures every 20-25 minutes and was using up to 7 daily seizure medications, none of which actually controlled her seizures. Her seizures could easily last up to 15 minutes, and her mother had to resuscitate her on 2 occasions using CPR.

Charlotte’s parents reached out to The Stanley Brothers, who then managed to successfully breed a CBD-rich cannabis strain that didn’t contain THC, and quickly got Charlotte involved in the Stanley Brother’s treatment plan, which was based on research conducted in Israel exploring the anticonvulsant effects of CBD.

Instantly after starting her treatment, Charlotte experienced positive effects, and her seizures dropped from 400 per week to 0-1 per week.

But Charlotte’s story isn’t unique. Since 2013, countless other stories have surfaced of children with Dravet syndrome finally finding relief from their condition using CBD. Some of these children have been seizure free for up to 1 year since they began treatment.

The success of CBD in treating seizures in children like Charlotte sparked a variety of clinical studies that analyzed CBD’s ability to reduce seizures in dravet syndrome as well as other forms of epilepsy.

Beneficial Cannabinoids and Terpenoids Useful for Treating Dravet Syndrome
The cannabis plant offers a plethora of therapeutic benefits and contains cannabinoids and terpenoid compounds that are useful for treating the symptoms of Dravet syndrome.

The following chart denotes which cannabinoids and terpenoids work synergistically with each other for potential therapeutic benefit. It may be beneficial to seek out strains that contain these cannabinoids and terpenoids.

Credit: Elemental Wellness

Americans For Safe Access Condition-based Booklets
These booklets summarize the history of medical cannabis and the recent research used to treat a variety of conditions, including Cancer, Multiple Sclerosis, Chronic Pain, Arthritis, GastroIntestinal Disorders, Movement Disorders, HIV/AIDS, and conditions related to Aging. (About Americans For Safe Access)

Movement Disorders



The therapeutic use of cannabis for treating muscle problems and movement disorders has been known to western medicine for nearly two centuries.


Article: "How to Qualify for Medical Cannabis in New Mexico"


The surprising story of medical marijuana and pediatric epilepsy | Josh Stanley | TEDxBoulder


States That Have Approved Medical Cannabis For Dravet Syndrome
Currently, just South Carolina has approved medical cannabis specifically for the treatment of Dravet syndrome. However, several states have approved medical cannabis specifically to treat epilepsy and other seizure disorders. These states include: Alabama (debilitating epileptic conditions), Connecticut, Delaware (intractable epilepsy), Georgia (seizure disorder), Iowa(intractable epilepsy), Louisiana, Maine, Mississippi (intractable epilepsy), Missouri (intractable epilepsy), New Hampshire, New Jersey (seizure disorders), New Mexico, New York, North Carolina(intractable epilepsy), North Dakota, Ohio, Oklahoma (pediatric epilepsy), Pennsylvania, South Carolina (Dravet syndrome, Lennox-Gastaut syndrome, Refractory epilepsy), Texas (intractable epilepsy), Utah (intractable epilepsy), Virginia (intractable epilepsy), West Virginia, Wisconsin(seizure disorders), and Wyoming (intractable epilepsy).

In addition, several states approve medical cannabis to specifically treat seizures. These states include: Alaska, Arizona, Arkansas, California, Colorado, Delaware, Florida, Hawaii, Louisiana, Maryland, Michigan, Minnesota, Montana, Nevada, New Hampshire, North Dakota, Ohio, Oregon, Pennsylvania (intractable seizures), Rhode Island, Tennessee (intractable seizures), Vermont, Washington, and West Virginia.

The state of Massachusetts will consider allowing medical cannabis to be used for the treatment of Dravet syndrome if it’s determined in writing by a qualifying patient’s physician.

In Washington D.C., any condition can be approved for medical cannabis as long as a DC-licensed physician recommends the treatment.

Recent Studies on Medical Cannabis Effects on Dravet Syndrome
The cannabinoid receptor 1 (CB1) of the endocannabinoid system can be used to produce anti-seizure effects.
Activation of the cannabinoid type-1 receptor mediates the anticonvulsant properties of cannabinoids in the hippocampal neuronal culture models of acquired epilepsy and status epilepticus.
http://jpet.aspetjournals.org/content/317/3/1072.long

References:
1. Blair, R.E., Deshpande, L.S., Sombati, S., Falenski, K.W., Martin, B.R., and DeLorenzo, R.J. (2006, June). Activation of the cannabinoid type-1 receptor mediates the anticonvulsant properties of cannabinoids in the hippocampal neuronal culture models of acquired epilepsy and status epilepticus. The Journal of Pharmacology and Experimental Therapeutics, 317(3), 1072-1078. Retrieved from http://jpet.aspetjournals.org/content/317/3/1072.long.

2. Karlócai, M. R., Tóth, K., Watanabe, M., Ledent, C., Juhász, G., Freund, T. F., & Maglóczky, Z. (2011). Redistribution of CB1 Cannabinoid Receptors in the Acute and Chronic Phases of Pilocarpine-Induced Epilepsy. PLoS ONE, 6(11), e27196. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208595/.

3. NINDS Dravet Syndrome Information Page. (2001, September 29). National Institute of Neurological Disorders and Stroke. Retrieved from http://www.ninds.nih.gov/disorders/dravet_syndrome/dravet_syndrome.htm.

4. Wallace, M.J., Martin, B.R., and DeLorenzo, R.J. (2002). Evidence for a physiological role of endocannabinoids in the modulation of seizure threshold and severity. European Journal of Pharmacology, 452(3), 295-301. Retrieved from http://www.sciencedirect.com/science/article/pii/S0014299902023312.

5. Wallace, M.J., Blair, R.E., Falenski, K.W., Martin, B.R., and DeLorenzo, R.J. (2003, October). The endogenous cannabinoid system regulates seizure frequency and duration in a model of temporal lobe epilepsy. The Journal of Pharmacology and Experimental Therapeutics, 307(1), 129-37. Retrieved from http://jpet.aspetjournals.org/content/307/1/129.long.

6. What is Dravet syndrome? (2014, March). Epilepsy Foundation. Retrieved from http://www.epilepsy.com/learn/types-epilepsy-syndromes/dravet-syndrome.



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