Dravet syndrome is a severe kind of epilepsy that affects children in their first year of life. It causes severe seizures, which can in some cases even lead to death.
For a long time there was no reliable treatment for Dravet syndrome, with most patients responding poorly to regular anticonvulsants. However, research shows that CBD, a non-psychoactive compound found in cannabis not only reduces seizures but also leaves some patients completely seizure free.
UNDERSTANDING DRAVET SYNDROME
Dravet syndrome is a very rare form of epilepsy that usually affects children from their first year of life onwards. It is a condition characterized by severe tonic-clonic seizures that can last up to 30 minutes.
Most children with Dravet syndrome will experience their first seizure within their first year of life, which is often accompanied with a fever. During this first year, children will usually experience either tonic clonic (or full-body seizures) or jerking convulsions that affect only half of the body.
After this, children often experience a variety of other seizures, including myoclonic seizures (brief shock-like jerks of a muscle or group of muscles), absence seizures (causing lapses in awareness), and many more. These seizures are usually hard to control and are often intractable to regular treatment.
Children with Dravet syndrome usually experience a large amount of seizures. Some patients, like Charlotte Figi (more information below) experience up to 400 seizures a week.
In many cases, children with Dravet syndrome may enter status epilepticus, a life-threatening state were seizures follow each other continuously leading to a loss of awareness and possibly even death.
WHAT CAUSES DRAVET SYNDROME?
According to the Epilepsy Foundation, roughly 80% of children with Dravet syndrome have a genetic mutation that affects the functioning of ion channels in the brain. In most cases, this mutation isn’t inherited from the parents, but is instead a “new” mutation in the child.
The most common gene mutation found in patients with Dravet syndrome is one that affects a gene called SCN1A. This mutation affects the proper functioning of sodium channels in the brain, which are crucial in process of sending chemical signals around the brain.
WHAT CAUSES SEIZURES IN DRAVET SYNDROME?
Children with Dravet syndrome can experience seizures randomly. However, parents and doctors may notice that certain conditions can trigger a seizure in a patient.
For example, patients with Dravet syndrome are usually more susceptible to fevers or infections and may experience seizures when they are ill. Alternatively, seizures can also be caused by sudden changes in body temperature, which can be caused by a hot bath or even hot weather.
Many children with Dravet syndrome also experience photosensitive seizures caused by flashing lights or patterns. Emotional stress and/or excitement can also trigger seizures in some children.
THE DEVELOPMENTAL IMPACT OF DRAVET SYNDROME
Children with Dravet syndrome usually have no concerning medical histories or examination during their first year of life. Even after the onset of seizures, they generally will not show developmental problems during this first year.
However, developmental challenges may begin to make themselves present in children between ages 2 and 4 and parents may notice that their child isn’t reaching their expected developmental milestones.
The developmental challenges affecting children with Dravet syndrome are very varied and can affect any of the following areas:
- Gross motor skills
- Fine motor skills
- Language skills
- Social skills
Children with Dravet syndrome will typically experience a slower development in any of the above areas. Some patients may also display a plateau, where they show no developmental progress for some time. Finally, some children may also demonstrate developmental regression after a particularly intense seizure.
HOW IS DRAVET SYNDROME TREATED?
In some places (including the US) there is no set treatment protocol for Dravet syndrome. Instead, treatment usually varies from one patient to another.
Dravet syndrome is usually treated and managed using a combination of medications and therapy. According to the Dravet Foundation, the medication with the best supporting literature is Stiripentol.
Other medications used to treat Dravet syndrome include:
- Valproic acid
Apart from medications, a ketogenic diet has also been shown to help improve cognition in some patients. Most children with Dravet syndrome will also partake in a combination of developmental therapies like occupational, physical, and /or speech therapies.
THE SIDE EFFECTS OF MEDICATION
Anticonvulsants like those listed above have a variety of side effects, including:
- Weight gain
It is important to note that the side effects of medication (such as slowed thinking, grogginess, and sleepiness) can also affect a child’s development.
CANNABIS AND DRAVET SYNDROME
There is very strong evidence that cannabis can help treat the seizures caused by Dravet syndrome. In fact, cannabis’ role in treating these seizures is arguably what first got it the media attention it deserves.
The main cannabinoid responsible for giving cannabis it’s anticonvulsant properties is cannabidiol (or CBD), a non-psychoactive compound found in cannabis and hemp plants.
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.
One of the most recent studies looking into the field was published in 2017 in The New England Journal of Medicine. The study involved 120 children and teenagers with Dravet syndrome and tested Epidiolex, a CBD drug, against a placebo or 14 weeks. It found that seizures in the CBD group dropped by over 50%.
It isn’t completely clear how CBD helps reduce seizures. However, it has been shown that the compound has extremely powerful anti inflammatory and neuroprotectant properties (some of the highest found in nature).
A seizure, in its most basic sense, is caused by the synchronized, abnormal firing of neurons in the brain. This then interrupts the flow of information in the brain which ultimately control muscle, nerve, and gland cells in the body.
By interacting with cannabis receptors in the brain, CBD seems to be able to prevent this rapid, non coherent firing of neurons. In fact, some parents and doctors using CBD on their children/patients also note that the treatment not only stops seizures but also helps improve the way patients function.
This may suggest that CBD not only helps prevent seizures but may also help recover some of the damage caused by Dravet syndrome and treatment.
THE FUTURE OF CANNABIS DRAVET SYNDROME
There is no doubt that cannabis can work wonders for children with Dravet syndrome as well as other types of epilepsy. However, one major fall back is that the substance still isn’t widely available due to legal restrictions.
Epilepsy is a qualifying medical condition in various US states under their medical marijuana programs. These include:
- New Hampshire
- New Jersey
- New Mexico
- New York
- North Carolina
- North Dakota
- Rhode Island
- South Carolina
- West Virginia
Outside of the US, medical cannabis is also available in a variety of other countries, including Australia, Chile, Argentina, Canada, and more. To find out more about treating epilepsy with CBD in your local area, check with your health authority.