Raina Tilghman had her first seizure when she was just weeks old, on the day of her two-month check-up with the family pediatrician.
The night before, the little girl had been up for hours with the hiccups, said her mother, Carey, who lives with Raina, her husband, Matt, and their two other children at her parents’ home in Jefferson. Raina’s eyes were wide when she arrived at the doctor’s office, and the physician initially diagnosed her with a case of acid reflux. As Carey was loading the baby into her car seat and taking her out the door, Raina lost consciousness and was suddenly wracked with muscle spasms — a grand mal seizure.
“We went directly from the doctor’s office to the hospital, and a chopper came and took us to Children’s in D.C.,” Carey said. “We spent the week of Thanksgiving in the hospital. She was back in-patient for Christmas, and then she was in the Hopkins Intensive Care Unit for Valentine’s Day.”
In the months afterward, much of the family’s attention and energy was dedicated to controlling Raina’s severe epilepsy. At 5 months old, the baby was put on her fourth anti-seizure drug by doctors at Johns Hopkins. Around the same time, it was recommended that Carey and Matt try Raina on the ketogenic diet — a high-fat nutritional plan that’s believed, in some cases, to alleviate difficult cases of epilepsy.
The diet didn’t work, Carey said. At 7 months, Raina was flown back to Johns Hopkins and spent around six weeks in the ICU with seizures that just didn’t seem to stop. The doctors tried her on a series of different drug combinations. Those didn’t work, either. The staff then told Carey that they wanted to look at Raina as a candidate for neurosurgery.
Across the country, parents of children with rare, difficult-to-treat conditions have helped to drive the discussion surrounding medical cannabis and its legalization. Epilepsy has been especially prominent, not only because of the brutality of the disorder and its devastating effect on children, but because preliminary research has shown promise for treating seizures with the drug.
In Colorado, a child named Charlotte Figi gained national attention when her parents successfully used cannabis oil to significantly reduce her severe epilepsy, caused by a rare genetic condition known as Dravet Syndrome.
In 2016, neurologists at the New York University Langone Medical Center published a small study of 162 patients that showed medical cannabis reduced monthly motor seizures by a median 36.5 percent — roughly the same rate as existing anti-seizure medications. Two percent of patients even became completely seizure-free.
The medicine used in the study, and at home by many hopeful parents, was cannabidiol, or CBD — a compound that’s found in marijuana but lacks the psychoactive properties of THC. Carey also started Raina on a low dose of CBD-heavy oil, the same blend used to treat Charlotte.
“Then we went a year admission-free,” Carey said. “She just did fantastic for that year on a very low dose of CBD oil.”
In Maryland, too, parents became major advocates for the state’s nascent medical cannabis industry. Carey testified in Annapolis about the drug, citing its efficaciousness in treating the now 2-year-old Raina and her oldest daughter, Paisley — a pediatric stroke survivor who was later diagnosed with cerebral palsy.
Shannon Moore, the manager of Sustainability and Environmental Resources for Frederick County, went even further. Her 6-year-old twins, Nicholas and Byron, started having seizures when they were 4 months old and were eventually diagnosed with Miller-Dieker syndrome, another rare genetic disorder that causes abnormal brain development. Most significantly, the twins were both found to have lissencephaly, which causes an atypically smooth, underdeveloped brain.
“We took Nicholas in first and that’s when they determined that,” Moore said. “My husband was with me at the time, and I remember saying, ‘Oh God, he has a twin.’ And we held each other and cried.”
Even on traditional anti-seizure medication, the twins went through dozens — and sometimes hundreds — of episodes every day. Miller-Dieker does cause severe intellectual disabilities, but Moore said she witnessed her children lose what little progress they had made before the seizures started, including the ability to eat. Both Nicholas and Byron now have feeding tubes that lead into their stomach and intestines; seizures robbed both boys of their swallowing reflex and will eventually end their lives prematurely.
In 2014, just before the boys had their feeding tubes put in, Moore found herself watching a CNN special with Sanjay Gupta on medical marijuana and its effectiveness for a variety of conditions, including epilepsy. The documentary stuck with her and she found herself with one nagging thought: why isn’t this available here?
Maryland had technically legalized medical cannabis in 2012, but the industry was essentially defunct, caught in a quagmire of legislative and legal difficulties. The possibility of products becoming available in the state was still years away. Moore hesitated at first at the thought of becoming an advocate, nervous about being seen as a “pot supporter,” but eventually decided the reward was worth the relative risk.
“My kids went into the hospital to get their feeding tubes put in and I remember holding one of them and thinking that he was going to die,” Moore said. “And I thought about how I had nothing left to lose. I thought to myself, OK, if this is working for other people, how come we don’t have access to this? Your access to lifesaving meds shouldn’t depend on your zip code. It just bugged the hell out of me.”
Eventually, Moore became something of a spokeswoman for the medical cannabis industry in Maryland. She joined the board of the state Medical Cannabis Commission and started lobbying in front of the General Assembly, working to guarantee that licensed growers, processors and dispensaries offered a diversity of products that would address specific medical needs.
She also started buying CBD oils from companies in Colorado and South Carolina, administering it to the twins through their feeding tubes. The effects of the drug, she said, were startling. Even a relatively small dose led to a 70 percent reduction in the boys’ seizures, and the oil sometimes worked as a rescue medication, stopping the seizures in their tracks.
That’s not to say that cannabis, or CBD, is a guaranteed cure for epilepsy. Studies on its effects are extremely preliminary, due largely to the lack of federal funding for marijuana research, and the drug has not been shown to reduce all seizures in all cases. In the study published in The Lancet Neurology, 79 percent of patients also reported adverse side effects, ranging from diarrhea to fatigue.
Anecdotally, some parents say the drug can also stop working after a certain period of time. Raina’s seizures were significantly reduced for a year after she started taking cannabis oil, until she experienced another outbreak of seizures in July that couldn’t be treated by the medication. When she was finally released from the hospital in August, her development had essentially reverted to the neonatal phase, Carey said.
“She couldn’t lift her head. She couldn’t roll. She couldn’t swallow,” Carey said. “We went to the rehab hospital for three weeks, and we left the hospital on an [feeding] tube, so we were fully dependent on all of our feeding through the nose.”
Now a wriggly 2-year-old, Raina regained some of her developmental abilities with the help of several ongoing therapies, including chiropractic visits and physical therapy. She’s taking a break from the cannabis oil for now, and Carey hopes that eventually both Raina and Paisley will become registered patients with the state of Maryland and find strains that are more specific to their individual conditions.
Currently, there are fewer than 10 patients in Frederick County under the age of 18 who are registered with the Maryland Medical Cannabis Commission, and only 92 statewide — a tiny fraction of the more than 18,000 people who have already been approved. But both Carey Tilghman and Moore expect that number to rise as the industry matures. Even with the unresolved questions surrounding medical cannabis — from the sometimes prohibitive cost to the current lack of products across the state — most parents who support the drug do so not only for its efficacy, but for its tertiary effects.
Traditional seizure medications are notoriously rough on young bodies. Leah Schust, another cannabis advocate in Frederick, said that her son, Ben, almost died from a regimen of ACTH hormones prescribed to help control his seizures. The 7-year-old’s liver also swelled after months of taking Depakote, another anti-seizure medication that carries the risk of full liver failure.
Raina, too, had difficulties on traditional medications. Behind the physical side effects from drugs such as Dilantin — which Carey blames for Raina losing the ability to swallow — the toddler was put on phenobarbital, a barbiturate with the risk of cognitive effects.
More than anything else, the drug made her flat, Carey said. Raina wasn’t curious, or inquisitive, or interested in the world around her. She wasn’t a normal, happy baby.
“I hate to say she was a boring baby, but it is what it is,” Carey said. “She was boring.”
Ben, Schust said, experienced many of the same effects on his medication. And while cannabis also reduced his seizures, the biggest impact was on his cognitive improvement.
“Ben suddenly seemed more alive,” she said. “His eyes brightened. He was able to engage and be present in this world. It brought him back to us. He hadn’t smiled in years, and now he’s smiling again. It was the first time I could tell he was happy.
“It made dealing with the life-threatening medical problems so much easier. To know he was happy. And that matters. It’s the quality of life that really makes all the difference. It made dealing with the medical stuff so much easier.”
By Kate Masters – Frederick News Post
Photo Credit – Bill Green