Jason Simon, now 15 and an 8th grader in Sandwich, was only 9 on the day when, as he recalls, he “woke up one morning with a bunch of men standing around to take me to the hospital.”
He had no idea what was going on, but his family was terrified. During the night, his sister had heard him making “gurgling noises,” the first sign Jason was having seizures.
At first, like many of the other 400,000 children with epilepsy — a condition in which people have recurrent seizures — Jason was treated with drugs. They worked, for a while.
But soon, like 30 percent of children on medications, Jason found that no matter which drugs he tried, he still had seizures — and the brain-numbing side effects of the drugs as well.
In 6th grade, he had so many dizzy spells and seizures that he missed 77 days of school. In 7th grade, he was taking 16 pills a day — but still missed 108 days of school.
Reluctantly, his parents agreed to surgery to remove the right temporal lobe of his brain, where his seizures — caused by abnormal discharges of electrical energy from brain cells — began.
Surgery did have one benefit. The courage it took to get through it left Jason “not scared of much anymore,” he says.
But though surgery helps some kids, it did nothing for his seizures. They soon came back, two or three times a day, each one causing him to stare and be unresponsive for a few seconds to several minutes.
Today, however, Jason is nearly seizure-free, thanks to a special diet that is 80 percent fat and has been sweeping the often-desperate world of epilepsy like a January blizzard.
No one is sure why it works, but the high fat/low protein/low carbohydrate diet seems to push the body into a starvation-like state called ketosis, says Dr. Gregory Holmes, director of the epilepsy program at Children’s Hospital in Boston.
Patients are first starved for several days to deplete the body’s stored sugar, then put on the diet, which contains almost no carbohydrates. Soon,
because the body has no glucose for fuel, cells begin burning fat from the diet instead, which slows the frantic firing of brain cells.
Burning fats in the absence of glucose leads to the production of fatty molecules called ketone bodies. Some scientists think ketone bodies may change the lipid membrane of brain cells to produce a lower firing rate. Others think the diet changes the rate at which charged particles called ions cross the lipid membranes of nerve cells. But the truth is, nobody knows.
So far, the diet is being studied only in kids, but there is “no theoretical reason” why it couldn’t help some of the 2 million adults with epilepsy as well, says Dr. Patricia Crumrine, director of the pediatric epilepsy program at Children’s Hospital in Pittsburgh.
In the approximately 200 children studied to date in a handful of medical centers, initial results suggest that 20-30 percent become “seizure-free, usually in a month to six weeks,” and about half have a 50-percent reduction in seizures, says Dr. John Freeman, director of pediatric neurology at Johns Hopkins Medical Institutions and the leading proponent of the ketogenic diet.
Most children stay on the diet for two years, he says. When it works right, seizures rarely return, even after normal diet is resumed. So far, there is no evidence that the high fat diet raises the risk of atherosclerosis, heart disease or stroke.
“Even if there were a slight increase,” says Freeman, “after two years, the children come off the diet” and any buildup of fatty plaques in arteries should disappear. “And even if you shortened the lives of these children by a year or two or five, you would have dramatically improved their quality of life.”
But there are caveats.
The diet is very stringent, even more so than diets for diabetes, and is appropriate only for children whose seizures are difficult to control by drugs. The slightest error — too much liquid, eating seven nuts instead of three or changing brands of sausage — can bring back the seizures.
Also, the diet “should only be done under medical supervision,” adds Freeman, noting that unconfirmed reports suggest at least three children have died after parents tried it on their own.
And while the diet may sound like a picnic, it isn’t.
Jason has lived on sausages and eggs, choked down heavy cream at every meal and, despite teenage temptation, has had little non-fatty food other than bouillon and lettuce since he started five months ago.
His parents are “always in the kitchen,” says his father, Steve, 42, a certified public accountant. “One of us is always cutting, cleaning, weighing” everything that goes into Jason’s mouth.
“You have to be totally committed — it’s an entire family commitment,” adds Jason’s mother, Lori, 39. “We never go out to eat anymore or have pizza at home. It’s absolutely amazing that Jason has the willpower to do this. I know I never could.”
Jason’s sister Julie, 12, says she has to eat any tempting foods she has out of his sight, be a good sport at holidays like Thanksgiving — when the whole family ate eggs — and put up with the fact that her parents “spend so much time on his meals.”
But the diet is toughest on Jason. Though he consumes 2,000 calories a day, he says, “It’s not enough. I’m always hungry.”
Which is precisely the point.
Ever since 400 B.C., people have observed that under conditions of starvation, seizures decline, says Dr. Ed Dodson, president of the Epilepsy Foundation of American.
Earlier this century, in fact, that knowledge led a few American doctors to deliberately starve their epilepsy patients. It often worked to reduce seizures, but the achievement — and interest in the diet — dropped from sight in the late 1930s when Dilantin, the first anti-epileptic drug, was developed.
But even as new drugs trickled onto the market, interest in the ketogenic diet never completely waned at Johns Hopkins, says researcher Freeman. Until recently, though, he used the diet with only eight to 12 children a year.
All that changed in October 1994, when Dateline NBC aired a program on the ketogenic diet, spurred by California movie-maker Jim (“Airplane” and ”Naked Gun”) Abrahams, whose 18-month-old son Charlie had become seizure- free on the diet.
Like Jason, Charlie had been given numerous drugs and had had brain surgery, his father says, yet still had dozens of seizures a day. Eventually, Abrahams quit work to pore over the medical literature, including Freeman’s book on the diet.
Abrahams, who founded the Charlie Foundation to publicize the diet, is thrilled that his son, now 3 1/2, is well, if a bit skinny. But he’s angry that all the doctors he had consulted for years knew nothing, or told him nothing, about it.
“We would not have drugged Charlie into oblivion if we had known about the diet,” Abrahams says. “And we certainly would never have let them carve into his brain.”
To some, the failure to take the ketogenic diet seriously smacks of the old days, when epilepsy carried so much stigma that patients were housed in ”colonies” for treatment so that other people would not have to see their jerking, twitching and other uncontrollable behaviors.
Some states laws forbade people with epilepsy to marry or have children, and some permitted involuntary sterilization of epileptics, says Dr. Orrin
Devinsky, director of the epilepsy program at New York University Medical Center.
But for kids like Jason Simon, the focus is not on past mistakes, but on the future. Thanks to the diet, he’s down to two seizures a month and four pills a day.
He hates the hunger, but he rarely complains, he says, “because complaining is not going to do anything. I figure why waste my breath. I’m just going to annoy myself and everybody else.”
Besides, he’s got his eye on more important things.
“I really want to drive when I’m older,” he says, “and I won’t be able to drive if I’m having seizures.”
SIDEBAR:
MEDICATIONS WORK FOR MOST
While the ketogenic diet can be useful for children whose epilepsy does not respond to medication, most people with epilepsy — adults and children alike — are helped by the growing array of available drugs, including newer ones like Lamictal and Neurontin.
If you are not sure whether you or your child is having seizures, ask your doctor — some seizures can be subtle and difficult to detect.
Often, doctors cannot tell what triggers seizures, though common causes are head trauma, infections, genetic defects and problems that occur as a child’s brain develops.
In general, seizures fall into two classes: partial seizures, in which the abnormal firing of nerve cells begins in just one part of the brain and then may spread; and generalized seizures, which begin in both sides of the brain at once.
Some partial seizures are so mild the patient merely twitches or feels odd but does not lose consciousness, though patients with complex partial seizures may lose consciousness.
Even in generalized seizures, some patients merely stare into space for 10 to 15 seconds, particularly with a type once called “petit mal” but now called “absence” (prounounced the French way). A child with an “absence” seizure may simply stop talking and stare, blink his eyes, then return to normal.
Other generalized seizures, particularly the tonic-clonic type once called ”grand mal,” are more severe. During these attacks, the body stiffens and jerks rhythmically, sometimes for up to three to five minutes.
Lay people often worry that a person having a seizure may swallow his tongue. This does not literally happen, though the base of the tongue may slip toward the back of the throat.
Contrary to a popular misconception, you should never put anything in the mouth of a person during a seizure. Instead, roll the patient onto his side and make sure the head is turned toward the ground to help clear the airway.
For more information, call:
The Epilepsy Foundation of America, 1-800-EFA-1000 (or 1-800-332-1000)
The Charlie Foundation to Help Cure Pediatric Epilepsy, 1-800-FOR-KETO (or 1-800-367-5386).