In 2012, just before starting kindergarten, Sammy Meyers had the first of what would become thousands of seizures.
“I found him semi-unconscious,” recalls his mother, Becky. “I thought he was choking and checked to see if his airway was open, and then called 911. It didn’t even cross my mind that he was having a seizure.”
But Sammy had recently been falling a lot. The neurologist in the local emergency room in Albany, N.Y. diagnosed epilepsy, but thought it was a relatively benign form that medications could help.
Instead, Sammy got worse. “Within a matter of months, he went from four seizures a day to hundreds,” says Becky.
Sammy was having head-drop seizures, involving a sudden loss of muscle tone; myoclonic seizures, causing muscle jerking; and absence seizures. He would lose consciousness, sometimes so abruptly that his head would slam down on the table. Eventually, he was diagnosed with Doose syndrome (myoclonic astatic epilepsy), a severe form of generalized epilepsy that is known to be medication-resistant.
Through the Epilepsy Foundation of Northeastern New York, Becky knew of another child with Doose syndrome who had been treated with a high-fat, very low-carbohydrate diet called the ketogenic diet and was still seizure-free after four years.
The Albany neurologists were skeptical, so the family contacted the other child’s neurologist, Ann Bergin, MB, ScM, at Boston Children’s Hospital. “By this time, Sammy was having a seizure every 30 seconds to a minute,” says Becky.
“Even in direct face-to-face examination, he was having multiple seizures and short absences,” confirms Bergin, who first saw Sammy in January 2013 and has run the Epilepsy Center’s ketogenic diet clinic for 15 years. “He was the most severe case I’ve ever seen.”
While Sammy’s cognitive skills had clearly declined, he was alert and cooperative enough during the exam that Bergin felt hope. To everyone’s relief, genetic testing ruled out progressive, neurodegenerative epilepsy, and Bergin agreed that Sammy should try the diet.
“It’s known in the epilepsy community that Doose syndrome responds to the diet in about half of cases, though no one knows why,” she says.
An ancient approach
The ketogenic diet’s roots are deep; in ancient times, it was recognized that fasting reduced seizures, and the ketogenic diet causes biochemical changes resembling those caused by fasting. The diet gets more than 90 percent of its calories from fat, inducing a state known as ketosis, in which cells get their energy from ketone bodies that form in the liver, rather than from glucose. Carbohydrates, which are converted to glucose in the body, are severely restricted.
“Something about switching over to ketone metabolism seems to change the seizure threshold in many epilepsies,” explains Bergin.
Interest in the ketogenic diet surged in the early 20th century when few anti-seizure drugs were available. But as new medications like Dilantin became available in the 1930s and 40s, the diet fell out of fashion.
Starting in the 1970s, John Freeman, MD, of Johns Hopkins, revived use of the diet and saw good results. In 1997, the movie First Do No Harm was released, starring Meryl Streep. It was inspired by the director’s son, whose seizures were eliminated after Freeman put him on the diet. As Bergin, who trained with Freeman, reports, “that led to an absolute tidal wave of community and family interest.”
Coconut oil cookies
Sammy was admitted to Boston Children’s for four days. His care team, anchored by ketogenic diet nutritionist Karen Costas, gave the family training about the diet and a binder of ketogenic recipes. Team members checked ketone levels in Sammy’s blood and made sure his parents had a gram scale to precisely weigh food ingredients. After Sammy was discharged, they made many follow-up phone calls to offer guidance.
“It’s not a natural diet: it has significant side effects, such as severe constipation and kidney stones, and should only be done under medical supervision,” Bergin notes.
The diet made Sammy miserable for the first few months. It was hard to get used to. After nine months, he was still having seizures, unable to maintain high ketosis. But there were hopeful signs.
“Three months into the diet, Sammy had a stomach flu and stopped eating, which can make ketones go really high,” says Becky. “He was seizure-free for two weeks. The seizures came back when he started eating, and his ketosis dropped.”
Costas suggested one final tweak to the diet. “We need to add coconut oil at every mealtime,” she told Becky.
Coconut oil contains medium-chain triglycerides, a type of fat that is a fast-acting source of ketones and is often recommended for children who have trouble maintaining ketosis. Becky and her husband included it in every meal and baked Sammy coconut cookies. Bergin’s office phoned to report that Sammy’s ketone level was very high.
The last two seizures
Three weeks later, Sammy’s seizures stopped. The same day, coincidentally, he was scheduled for surgery in Albany to implant a vagus nerve stimulator (VNS), sometimes used for drug-resistant epilepsy. The family went ahead with the VNS surgery, during which Sammy had two seizures. But for the next two weeks, while the device was kept off to allow for healing, Sammy was seizure-free.
This December, Sammy marked a year without seizures. His electroencephalogram (EEG) no longer showed seizure patterns, his memory is noticeably better, and he has caught up with his first-grade peers. Once on five anti-seizure medications, he’s now on just one and is being weaned off it. Recently, the Epilepsy Foundation of Northeast New York named Sammy a Winning Kid for 2015.
“He’s one happy kid,” says Becky. “The milestones are incredible to watch. For a while, he was so sick, he couldn’t take off his shirt. We were undressing him.”
Bergin last saw him in November. “He looked so great. He’s like a regular boy now,” she says. “At some point, we might turn off the VNS too, if he continues to do well.”
While it’s controversial whether the ketogenic diet is a permanent cure, some children can be weaned off the diet if they do well on it for one to two years, says Bergin. Children with Doose syndrome sometimes outgrow the seizures; until then, the diet can hold the seizures at bay.
“I tell Sammy, ‘The doctors can’t explain why it works, but it’s almost like it’s magic,” says Becky. “Let’s just go with that.”
Visit Boston Children’s Epilepsy Center for more information on the ketogenic diet and sample menus.