Dravet Syndrome

Dravet syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a rare and catastrophic form of epilepsy that begins in infancy. It is typically triggered by hot temperatures and/or fever, and characterized by violent, prolonged seizures lasting over five minutes. The neurodevelopmental implications of this disorder are significant, often resulting in severe cognitive impairment.

Initial seizures are most often prolonged events and in the second year of life other seizure types begin to emerge. Individuals with Dravet syndrome face a higher incidence of SUDEP (sudden unexplained death in epilepsy) and have associated conditions, which also need to be properly treated and managed. These conditions include:

  • behavioral and developmental delays
  • movement and balance issues
  • orthopedic conditions
  • delayed language and speech issues
  • growth and nutrition issues
  • sleeping difficulties
  • chronic infections
  • sensory integration disorders
  • disruptions of the autonomic nervous system (which regulates things such as body temperature and sweating)

This intractable epilepsy is characterized by unilateral (one-sided) clonic or tonic clonic (grand mal) seizures that are prolonged (5 minutes) or progress to status epilepticus (30 minutes) and require emergency management. Myoclonic seizures, often called myoclonic jerks, are common.

Over time seizures present without fever, illness or heat triggers. Seizures are frequent and resistant to treatment. Between one and four years of age, children develop other seizure types including atypical absence, eyelid myoclonia and non-convulsive seizures. All seizure types may be prolonged and uncontrollable.

The Past

Dravet Syndrome is known as an intractable form of childhood epilepsy, meaning it cannot be controlled by pharmaceuticals known to aid in seizure reduction. There is no known pharmaceutical that can successfully treat the disorder.

The Plant

High CBD, low THC cannabis strains–most notably the strain “Charlotte’s Web”–have achieved dramatic success in treatment of Dravet Syndrome.

Charlotte’s Web is a high cannabidiol (CBD), low tetrahydrocannabinol (THC) Cannabis extract oil. The original strain was developed by the Stanley Brothers in Colorado.  The Stanley’s CBD project became known as the Charlotte’s Web™ breeding project after meeting the Figi family in early 2012, and discovering 5 year old Charlotte Figi’s dramatic success in her battle with epilepsy

In this Feb. 7, 2014 photo, Matt Figi hugs and tickles his once severely-ill 7-year-old daughter Charlotte, as they wander around inside a greenhouse for a special strain of medical marijuana known as Charlotte's Web, which was named after the girl early in her treatment, in a remote spot in the mountains west of Colorado Springs, Colo. A few years ago, Charlotte's doctors were out of ideas to help her. Suffering from a rare disorder known as Dravet’s syndrome, Charlotte had as many as 300 grand mal seizures a week, was confined to a wheelchair, went into repeated cardiac arrest and could barely speak. Now Charlotte is largely seizure-free, able to walk, talk and feed herself, with her parents attributing her dramatic improvement to this strain of medical cannabis. (AP Photo/Brennan Linsley)
In this Feb. 7, 2014 photo, Matt Figi hugs and tickles his once severely-ill 7-year-old daughter Charlotte, as they wander around inside a greenhouse for a special strain of medical marijuana known as Charlotte’s Web. (AP Photo/Brennan Linsley)

The Stanley brothers began breeding for high CBD cannabis strains in 2009, shortly after their entrance into the medical marijuana industry. Their purposes for doing so were to find more benefits from the plant’s compounds, specifically for patients who didn’t benefit from psycho-activity as well as for the potential anti-tumor properties suggested by many preclinical studies.

To date, there are several phenotypic strains that have emerged from the Charlotte’s Web (CW) project which have low enough THC to be considered Hemp. Currently, only certain phenotypes are commonly used for the CBD infused into Charlotte’s Web™ Hemp Products, due to the high success rate of these specific cannabinoid profiles in intractable epilepsy.

It does not induce the psychoactive “high” typically associated with recreational marijuana strains that are high in THC.

Difference between CBD and THC in Medical Marijuana

THC, or tetrahydrocannabinol, is the chemical responsible for most of marijuana’s psychological effects. It acts much like the cannabinoid chemicals made naturally by the body, according to the National Institute on Drug Abuse (NIDA).

Cannabinoid receptors are concentrated in certain areas of the brain associated with thinking, memory, pleasure, coordination and time perception. THC attaches to these receptors and activates them and affects a person’s memory, pleasure, movements, thinking, concentration, coordination, and sensory and time perception, according to NIDA.

THC is one of many compounds found in the resin secreted by glands of the marijuana plant. More of these glands are found around the reproductive organs of the plant than on any other area of the plant. Other compounds unique to marijuana, called cannabinoids, are present in this resin. One cannabinoid, CBD is nonpsychoactive, according to the National Center for Biotechnology Information, and actually blocks the high associated with THC.

Cannabidiol or CBD, is the cannabis compound that has significant medical benefits, but does not make people feel “stoned” and can actually counteract the psychoactivity of THC.   CBD does not cause a high, unlike THC. The reason why CBD is non-psychoactive is due to its lack of affinity for CB1 receptors. CB1 receptors are found in high concentrations in the brain, and are the pathways responsible for the psychoactive effects of THC.

CBD and THC levels tend to vary between different strains and varieties of cannabis.  By using selective breeding techniques, we have managed to create varieties with high levels of CBD and THC.

The Process

The oral administration of cannabis extract oil has been the most documented treatment method to date.

The Patient

Nowhere has the case of cannabinoid treatment for Dravet Syndrome been as well documented than in the case of Charlotte Figi. The landmark case brought international headlines and the appropriately named high-CBD strain “Charlottes Web” to the forefront of the medical cannabis conversation.

Charlotte Figi, 6, who was given her life back by marijuana. Charlotte was just 3 months old when she had her first seizure.  By the age of three, Charlotte was having up to 300 grand mal seizures a week. She had lost the ability to walk, talk and eat.

Desperate for a treatment, Charlotte’s father Matt searched the Internet and eventually found a boy with a similar case in which medical marijuana helped his seizures. The Figi’s turned to the Stanley brothers, one of Colorado’s largest marijuana growers and dispensary owners, for help.

 

These six brothers were crossbreeding a strain of marijuana low in THC, the compound in marijuana that’s psychoactive, and high in CBD, which has medicinal properties but no psychoactivity. – Sandra Young – CNN

 

But the Stanley brothers didn’t know what to do with this particular strain, as no one seemed to want to buy it. Then they met Charlotte. The first time Charlotte tried a small dose of cannabis oil, the results were astounding:

 

When she didn’t have those three, four seizures that first hour, that was the first sign,” Paige recalled. “And I thought well, ‘Let’s go another hour, this has got to be a fluke. – Paige Figi

 

Charlotte’s seizures stopped within an hour of her first dose of cannabis oil.

The Stanley brothers started the Realm of Caring Foundation, a nonprofit organization that provides this particular strain of medical marijuana to adults and children suffering from a host of diseases. The strain used by Charlotte and 41 other patients is affectionately called Charlotte’s Web. Charlotte’s seizures stopped for seven days after her first dose and today she is eating, and walking on her own — even riding a bike, her parents say.

Charlotte gets a dose of the cannabis oil twice a day in her food and her seizures are down to 2 to 3 per month.

 

“I literally see Charlotte’s brain making connections that haven’t been made in years,” Matt said. “My thought now is, why were we the ones that had to go out and find this cure? This natural cure? How come a doctor didn’t know about this? How come they didn’t make me aware of this?” – Matt Figi

 

Another significant breakthrough in the Dravet Syndrome arena is found in the case of Katelyn Lambert from Australia. At the age of 6 months she was having as many as 1000 seizures a day–one every 15 seconds–until her parents chose to defy Australian drug policies and order cannabis oil online. They had read of Charlotte’s dramatic success with cannabis oil, hoping they too could save their child with the treatment. Indeed, as Katelyn’s father Michael described,

 

The next day, I’m not kidding you. . .she just woke up. It wasn’t like she was looking through a storm of noise and static, she just looked normal. She stopped jerking. She got so much better right away. – Michael Lambert

 

Following the dramatic success of the cannabis oil treatment, Katelyn’s grandparents donated 37 million dollars to the University of Sydney for cannabis research, ensuring that the conversation between Dravet Syndrome and high-CBD, low-THC cannabis treatment receives steady funding.

 

Comments

Medical Marijuana 411 values providing our readers with information about the latest cannabis related topics for educational purposes and not for the intention of advocacy. This is not an advocacy-based organization. For ailment specific questions, please consult your primary care physician.