PHOTO: Meghan Wilson and her daughter, Vivian, who suffers from Dravet Syndrome.
“A little less panicky, but more tired than ever before. So violent with biting, hitting and scratching, ripping the leads off, and just wanting to crawl out of his skin with not being able to sit still…. No sleep for either Jaxs or I. Every time he fell asleep, then 20 minutes later a seizure. So, he fought sleep and had that panicked ‘I am dying’ fear in his eyes that expressed so much confusion and loss at what was happening. Most helpless I have felt so far in 14 years.”
-From the journal of Jennie Stormes
The words are chilling, heartbreaking, every parent’s worst nightmare. Jaxs has Dravet Syndrome, a rare, incurable form of severe childhood epilepsy.
But the symptoms Jennie describes are not from Dravet Syndrome itself – they are symptoms of withdrawals from one of the dozens of drugs her son Jaxs has been prescribed over the past 14 years to treat Dravet.
Dravet Syndrome is a genetic disorder caused by a mutation of the SCN1A gene, and usually causes seizures before the age of one.
The standard treatment for Dravet and other childhood epilepsies is a barrage of pharmaceutical medications: barbiturates, steroids, benzodiazepines. Most of these drugs have not been tested on young children and are not recommended for pediatric use.
Heather Jackson’s 10-year-old son Zaki has Doose Syndrome, a childhood epilepsy with no known cause. When Zaki began treatment, Heather was extremely concerned about the warnings that came along with his medications.
“Every medication came with a side effect list as long as my arm including some medications that are ‘black box’ labeled,” Heather explains. “It is the strongest warning that the FDA requires, and signifies that the drug carries a significant risk or serious or even life-threatening adverse effects.”
The side effects Zaki has experienced include insomnia, body cramps, anorexia, hair loss, bone loss, weight gain, incontinence, kidney stones, cataracts, hypertension and paradoxical reactions, meaning that certain medications actually increase his seizure activity.
Zaki has also experienced numerous behavioral effects from the seizure medications: anger, aggression, self-harming behavior, and loss of cognitive abilities including speech.
The side effects are devastating to early development, says Meghan Wilson, the mother of a 2-year-old Dravet patient named Vivian. “I don’t even know who Vivian is because she’s been on medication her entire life.”
When Vivian was just four-months-old, she was prescribed phenobarbital, a barbiturate. “With the phenobarbital, she would just bang her own head on the floor. That’s very common with phenobarbital and it’s a very disconcerting thing to watch,” recalls Meghan.
Vivian was prescribed six additional seizure medications before her second birthday, none of which were recommended for pediatric use.
“Her lack of muscle control, poor walking, and physical weakness I think is because of Depakote,” says Meghan. “For the three months she was on Topamax her development was stagnant. She should have been starting to babble, and she was just mute. And all clobazam did was introduce a new seizure type for her.”
When a paradoxical reaction occurs or when a prescription stops working, as often happens, the child must be weaned slowly from the drug to prevent life-threatening withdrawal symptoms.
But even a slow wean can cause devastating symptoms, something that many severely epileptic children experience dozens of times in their young lives.
“People say benzodiazepine withdrawal is worse than heroin withdrawal,” says Meghan. “It’s that dangerous and horrible for the children.”
While being weaned from the drug Keppra, Vivian Wilson experienced severe withdrawal symptoms. As Meghan remembers, “It would feel like there was electricity running through her body, she was just twitching and twitching. When I held her she’d sleep, but I’d think, how can this baby sleep when it looks like there are bugs running through her veins?
Recently, a new childhood epilepsy treatment has been extremely successful in stopping seizures without causing horrific side effects: a specialized form of medical marijuana that is high in Cannabidiol (CBD), a non-psychoactive component of the cannabis plant, and low in Tetrahydrocannabinol (THC), marijuana’s psychoactive component.
“It’s unlike anything that I, in my 30 years of medical practice, have experienced,” Dr. Alan Shackleford explains in an informational video featuring Heather and Zaki Jackson. “To be able to control seizures the way [CBD] evidently is able to do in a condition which results in absolute uncontrolled seizure activity is unprecedented.”
But because of the politics surrounding medical marijuana, only a handful of pediatric patients have been able to access CBD medicine.
Even in states with programs, medical marijuana laws are often more restrictive for children. And even doctors who believe these children could benefit from CBD often do not recommend it due to fear of legal repercussions.
These obstacles are frustrating to Dr. Sunil Aggarwal, an MD and PhD whose career has focused on researching medical cannabis.
“Pediatricians should be doing more for this population,” says Dr. Aggarwal.
“They have no problem routinely prescribing ketamine, morphine and barbiturates (to children) despite the side effects. I wish patients didn’t have to suffer so many seizures before trying (CBD). ”
More awareness and education, says Dr. Aggarwal, is essential in order to increase physicians’ willingness to recommend medical marijuana for children.
And awareness is beginning to grow, with CBD medication for children generating a lot of recent press.
Zaki Jackson’s story was recently featured on NBC’s Today Show and Tonight with Brian Williams.
But while mainstream media has become increasingly sympathetic toward pediatric cannabis use, a disconnect still exists between what is reported and what advocates say would be most beneficial for patients.
“Regardless of where you stand, no one is arguing that medical marijuana or cannabis should be a first line drug of choice,” says Dr. Nancy Snyderman, at the conclusion of her NBC Tonight report. “But when all efforts have been exhausted and parents are really frustrated and have nowhere else to turn, increasingly the medical community and parents are looking to this as a last ditch effort.”
In fact, many parents would prefer to start treating epilepsy with CBD without first having to subject their children to the side effects of prescription epilepsy meds, their addictive properties and withdrawal symptoms.
“Zaki’s life would look very different today had we started with cannabidiol when he was much younger,” says Heather Jackson. “He would not have lost all of his early childhood to a fog of seizures and prescription medications. He spent prime time brain development years lost to hundreds of daily seizures.”
Parents of young patients are acutely aware of the damage that can occur in early stages of development if seizures are not controlled. Every seizure and every debilitating effect of pharmaceutical medication decreases a child’s chance at a normal and healthy life.
“Dravet is a progressive disease, that’s just a fact,” explains Meghan Wilson. “Taking Vivian off prescription meds and treating her with CBD might halt the progression and prevent regression of skills. I don’t want her to have to wait to try it.”
But the path to CBD’s use as a first-line treatment is a rocky road with many obstacles. Dr. Aggarwal believes that this is purely political.
“Restrictive medical programs are a result of prohibition,” Aggarwal explains. “Legalization is so important, because the two are going to feed each other.”
When I ask Dr. Aggarwal what it would take for CBD to become a first line treatment, he sighs deeply.
“We’re stuck until politics allows certain types of science to be done. We shouldn’t need that to get Meghan’s kid treatment,” he explains. “Not to say it’s impossible for this to be a first line treatment, but the pediatric community must be more engaged. Doctors need to be advocates in situations where families are demanding this now.”
In New Jersey, where the Wilson family lives, there is legislation pending that would help CBD medication become available to children. But if these changes take too long to implement, Meghan Wilson says her family will move to Colorado, where CBD medication is most accessible.
“People say you’re putting all your money into (moving) and you don’t even know if CBD will work, but I think Vivian deserves the option to try it,” says Meghan. “It’s out there – it’s 1000 miles away, but it’s out there.”
Zaki Jackson is one of the children in Colorado who has had great success with CBD. After years of suffering dozens of daily seizures, on July 4, 2013, Zaki celebrated 9 months seizure free.
“I thank God for where we are now,” says Heather Jackson. “We focus on moving forward!”
Since starting on CBD, Zaki’s new side effects include pain relief, a better appetite, normal sleep patterns, improved memory, and overall good health.
But Heather remains intimately aware of the struggles other families are experiencing, and continues to be a vocal advocate.
“Parents should have the right to make their own health care decisions,” Heather explains. “We should all have the freedom to try and heal our children. CBD should not be a last resort option and should not only be available to people living in a compassionate state and that have access to a high CBD strain.”
“I hope we move beyond this,” says Dr. Aggarwal. “To get to become a first line treatment takes years. It requires studies. Medicine is a very conservative institution.”
He sighs deeply again. “We’re trying to get it there.”