Two years ago, Natalie Pembleton was having so many epileptic seizures each day that doctors put her in a drug-induced coma while they scrambled to figure out how to help her.
She was hospitalized for months, forcing her parents to pull her out of sixth grade. Her UCLA medical team even considered brain surgery that might’ve left Pembleton partially paralyzed and unable to speak.
Today, the Yorba Linda girl is seizure-free and focused on the start of eighth grade.
For that, both her parents and her doctors credit Epidiolex, the first government-approved drug derived from naturally grown marijuana.
Epidiolex won’t be widely available until this fall, but some patients with seizure disorders have been getting an early chance to try it out. And so far — as Natalie’s case and mounting research shows — the results are promising.
“I’m feeling good,” Natalie 13, said as she sat in her favorite park. “I feel like I can finally live my life like a normal kid.”
Some Epidiolex patients experience minor side effects, such as fatigue and changes in appetite. And doctors say the drug doesn’t seem to work for every patient, and it’s too early to have solid answers about its long-term efficacy.
There also aren’t any head-to-head studies to show whether the pricey pharmaceutical calms seizures better than less expensive cannabis products people can buy now at dispensaries and health food stores, according to Dr. Shaun Hussain, a pediatric neurologist at UCLA who’s treated Natalie for years.
That’s because of the federal illegality of marijuana, and the resulting stigma, continue to stifle independent, scientific research of cannabis-based medicines and development of related treatment options.
So, as promising as Epidiolex and similar drugs in the works seem to be, Hussain described the arrival of an FDA-approved cannabis-based medicine as an early milestone.
“It’s an important step forward,” he said, “but it’s really just the beginning.”
Novel drug from ancient source
Epidiolex is a syrup made by using a proprietary strain of greenhouse-grown marijuana to produce purified cannabidiol, or CBD.
The chemical is one of more than 100 compounds found in cannabis. It doesn’t make consumers high. But, while Hussain said researchers don’t fully understand how CBD works in the brain, it has been shown to ease seizures in patients with certain kinds of epilepsy.
The Food and Drug Administration approved Epidiolex on June 25. Before then, the government had only signed off on three drugs made from synthetic marijuana, all to treat nausea and appetite issues caused by diseases such as cancer and AIDS.
Epidiolex is cleared to treat patients 2 years and older who suffer from Lennox-Gestaut or Dravet syndromes, rare forms of epilepsy that begin in childhood. But GW Pharmaceuticals — the British company behind the drug, with U.S. headquarters in Carlsbad — has said they expect it will also be approved as a treatment for other types of epilepsy.
The concentrated CBD syrup reduced both the severity and frequency of seizures in a majority of adult and child patients who hadn’t been helped by other treatments, according to a study by University of Alabama researchers, published in the journal “Epilepsy & Behavior” earlier this month.
But before Epidiolex can become widely available, the Drug Enforcement Administration must remove the medicine from its Schedule I category on the Controlled Substances list. Marijuana and all of its compounds are currently in that category, which is supposed to be reserved for drugs like heroin, which have a high potential for abuse and no proven medical use. The DEA is expected to carve out a special exception for Epidiolex by late September.
In the meantime, many epilepsy sufferers have been relying either on CBD oil made from hemp, which is widely available in health food stores and online, or on CBD oil made from marijuana, which can be purchased at licensed dispensaries.
Many families using those oils have reported positive, even seemingly miraculous results. The most famous of these cases is Charlotte Figi, a Colorado girl who was profiled on CNN in 2013 after CBD oil helped rescue her from a nearly catatonic state.
Despite their promise, there are drawbacks to store-bought CBD oils.
Not all hemp-based versions are properly labeled or lab tested for safety. There’s no regulatory agency currently overseeing production of these products, and even cannabis attorneys can’t agree on whether CBD made from hemp is fully legal.
Also, marijuana-based CBD oils legally available with a doctor’s recommendation only in states that have approved medical cannabis for epilepsy. In California, Dr. Bonni Goldstein of Lawndale said products that many parents rely on have disappeared from the market overnight as the state’s marijuana industry is forced to comply with recently enacted regulation and licensing requirements.
Doctors often won’t discuss CBD oil as a treatment regimen because cannabis remains federally illegal. It’s also why parents can run the risk of legal or parental custody issues if they medicate their children with standard CBD oil. Also, the annual cost of the products can range from several hundred to tens of thousands of dollars, with no insurance coverage for any cannabis-based treatments.
Right now, one patient can expect to pay about $32,500 for a year’s worth of Epidiolex. That’s significantly less than Hussain feared it might cost, and it’s on par with many other epilepsy medications. And since government-sanctioned Epidiolex will be shipped by pharmacies directly to patients, it’s expected to be covered by many insurance policies — at least for patients with Lennox-Gestaut or Dravet syndromes.
How insurance companies will handle patients with other forms of epilepsy — like the type that plagues Natalie — won’t be clear until the drug hits the market this fall.
Hope for Natalie
Natalie is one of about 1,200 patients who, to date, have been given free access to the drug by the manufacturer, GW Pharmaceuticals, and the federal government’s “compassionate use” program. The program allows physicians to request permission to use a drug that hasn’t yet been FDA approved if their patients face life-threatening conditions where no standard treatment is available.
Natalie was first hospitalized with severe epilepsy when she was 5 years old.
“I just remember laying in bed and shaking,” Natalie said. “And I kind of felt scared to death because I didn’t know what was happening.”
A combination of pharmaceutical drugs helped get things under control, and Natalie had only about one major seizure each year for seven years. But when she turned 12 in fall 2016 and hit puberty, her seizures came back with a vengeance.
Natalie checked into UCLA that October and didn’t leave for 150 days. Nurses made time pass by tacking up posters of her favorite singer, Shawn Mendes, playing his music on repeat, and even getting him to record two video messages that made her swoon.
For two weeks around Christmas, Natalie was in a coma while doctors tried an experimental drug to stop her seizures. She’d previously tried pharmaceuticals that counted blindness as a potential side effect, and others that rendered her depressed to the point of feeling suicidal. But none calmed the electrical charges in her brain.
Natalie’s parents had read about CBD helping other children with epilepsy but, to that point, had stuck with the doctor’s recommendations. When they heard the next option on the table was to remove a portion of Natalie’s left frontal lobe where the seizure activity was triggering, they asked Dr. Hussain about giving CBD a try.
Five years ago, before news of Charlotte Figi broke, Hussain said he would have considered it “lunacy” to use cannabis-based medicine on a child. And even when they were agonizing over Natalie’s treatment, he said there still wasn’t any good data to indicate Epidiolex would help with the particular form of epilepsy she faced.
“We were getting pretty desperate,” Hussain said. “We got to the point where we didn’t have other medical options.”
He and his colleagues plowed through the red tape needed to get approval for the trial drug. They gave Natalie her first round of Epidiolex in January 2017.
“We felt like she was a pioneer when we were giving her that first dose,” her mom, Catherine Pembleton, said.
Natalie recalls thinking: “This is either going to be helpful or I’m going to have to go through surgery.”
By day 12 of taking Epidiolex, a study by Hussain and his colleagues at UCLA says Natalie had gone from as many as 20 seizures each day to no noticeable episodes. On day 21, she got to go home. And on day 64 of taking Epidiolex, in March 2017, she was diagnosed as seizure-free.
Her mom now drives once a month to UCLA to pick up the bottle of Epidiolex, cradling it the entire way for safe-keeping.
“I look at it like liquid gold,” Catherine said.
This summer, Natalie went ziplining at summer camp and on a family cruise to Alaska (where the teen begged her mom to stop quoting the “Titanic” line: “Iceberg, right ahead!”). She’s looking forward to joining her school’s drama club and maybe choir, so she can get even better at singing Shawn Mendes songs.
Going forward, Hussain said Epidiolex and other CBD drugs that might come to market will be near the top of the list of therapies he’ll consider when he encounters patients with stubborn seizures.
“I don’t think it should be viewed as a last resort,” he said.
Because of the lingering stigma, Catherine initially told only her closest friends that they were giving Natalie a drug derived from marijuana. She hopes that sharing Natalie’s story now will encourage more people to consider trying CBD treatments.
Natalie has only one complaint about her medicine. Despite company claims that the syrup is strawberry flavored, she grimaced as she shook her head of brown curls.
“It tastes bitter. Not good.”
Her mom’s only complaint?
“I wish it would have been around sooner.”