Johns Hopkins Magazine - April 1995 Issue

High Fat and Seizure Free

Winter sunlight splashes the cornflower blue walls in Connie Hurst's kitchen as she prepares lunch for her 4-year-old daughter, Korinne. Her husband, Chuck, slouches in a chair and struggles to stay awake. He hasn't slept since coming home from his night welding job in this rural part of southern Pennsylvania. Connie Hurst, another night-shift worker, is groggy too. But Korinne has energy to spare.

"I'm hungry. I'm hungry, Mommy! Daddy, I'm hungry!" she cries, wriggling on a vinyl-covered kitchen chair. She has a sunshiny face and rosy chipmunk cheeks. However much she squirms, she's careful not to drop a precious object she cradles in her hands. It's a small plastic cup of the type hospitals use to dispense medication, and it contains a little blob of sugar-free strawberry Jello. Now Korinne rocks back and forth on her elbows, sending long rivulets of wavy blond hair across her face. She takes a whiff of the cup's perfumey contents with each forward rock. "Smell this, Mommy. Smell this, Daddy," she says.

"Wait, Korinne," says her father. "Wait till Mommy gives the rest to you so you can eat it all at once."

Korinne loves corn, lima beans, sticky buns, candy, and cookies. But she can't eat those foods. For lunch today she's having precisely 14 grams of melted butter sipped through a straw, 50 grams of heavy whipping cream, 14 grams of chicken, 16 grams of apple, and 30 grams of the precious Jello. For dinner, she'll have another strange assortment of foods, mainly fats. Each item will be weighed to the gram.

The Hursts' refrigerator is always stocked with two quarts of heavy whipping cream, a couple pounds of butter, and plenty of cottage cheese, cream cheese, and mayonnaise. These are the staples of a peculiar high-fat/low-carbohydrate and protein diet Korinne has been following since October. It is called the ketogenic diet, and it is curing her of epilepsy.

Before beginning the unusual diet, Korinne suffered as many as 60 epileptic seizures a day. During a seizure, her eyes would dart around and she'd stutter. In the worst episodes, she'd fall to the ground, foam at the mouth, and lose control of her bladder. No medication helped. On the diet she is seizure free and takes no medication. Her parents say it's a miracle.

Korinne's neurologist, John Freeman, MD '58, director of Hopkins's pediatric epilepsy center, says he's seen the ketogenic diet help scores of children like Korinne. The diet, which is generally used for two years and always under a doctor's supervision, can cure epilepsy permanently in many children, he says. It significantly cuts down on the number of seizures for many others. Many patients have been able to stop or cut back on the potent medications that can make them groggy.

Epilepsy, which stems from erratic surges in the brain's electrical rhythms, affects some 2.5 million people in the United States. In cases where medication won't help, seizures can batter the brain so frequently that normal life becomes impossible. So why don't all epileptics try the ketogenic diet? Why haven't more people heard about it?

Developed at Hopkins and the Mayo Clinic in Minnesota 70 years ago, at one time the ketogenic diet was the only alternative to the handful of seizure medications available. It started to fall by the wayside in the 1940s and 1950s, however, when new drugs came on the market.

At Hopkins, though, the ketogenic diet remained an option, although one exercised only occasionally. Freeman, who took over pediatric neurology in 1969, together with dietitian Millicent Kelly, then director of Johns Hopkins Hospital's Nutrition Clinic, administered the diet to only 12 to 18 patients each year.

Then, last fall, NBC's Dateline aired a segment on the diet, and Freeman's phone hasn't stopped ringing since. Neurologists from around the country are seeking him out for advice on the diet, and parents are asking how to get their children into one of the dozen programs that administer it.

At the moment, Freeman stresses, the ketogenic diet is being used "to treat the worst of the worst. Our criteria are that children are having at least three seizures a week and continue to have seizures despite using medication appropriately."

That rules out a lot of children. "Seventy percent of people who have a seizure never have a second," says the neurologist. "That's why you probably don't need to treat someone who's had a first, second, or maybe even a third seizure. And 70 percent of people who have a second seizure can be completely controlled with medication."

Many of the calls Freeman has received are from adults who want to try the diet themselves. Presently, he says, "nobody is doing adults," though that could change in the future. Historically, pediatric neurologists were the ones who prescribed the diet for their patients, so it has remained a therapy for children.

Eating butter and cream to cure a disease sounds almost like snake oil. But it has a scientific basis, says dietitian Kelly, who started teaching families at Hopkins how to use the diet more than 40 years ago.

The diet works by reproducing the metabolic effects of fasting, Kelly explains. "Normally, the body uses glucose, which is a sugar, for fuel," she says. Carbohydrates supply most of the body's glucose, proteins contribute some, and fats yield just a bit. "We can store only a day's supply of sugar. So if we fast for 24 hours, we're going to deplete that store." Once the sugar is gone, as happens during a fast, the body starts burning fat.

On the ketogenic diet, the body has little glucose to burn so it resorts to burning fats supplied by the cream, mayonnaise, and butter, says Kelly. Chemical by-products of fat metabolism called ketone bodies build up in the blood. Ketosis, as the physiological state is called, somehow, in some cases, inhibits seizures. In many cases, the effect lasts even after patients return to a normal diet. Exactly how is not known.

To keep the body in ketosis, patients consume only a minimum amount of protein and carbohydrate, and only enough calories to maintain their weight. (Weight gain would indicate that a patient is storing fat rather than burning it for fuel.) Therefore, food portions for the diet seem skimpy. Korinne's 14-gram serving of chicken, for example, is about the size of a silver dollar. Patients must also restrict the amount of fluids they drink. The diet is always done under a doctor's supervision, Kelly stresses. At Hopkins, patients starting on the diet are admitted to the hospital for several days.

When patients leave the hospital, Kelly provides their parents with plans for about 20 sample meals. She also offers ketogenic recipes, some of which are quite bizarre--like strawberry chocolate chip ice cream with bacon.

The dietitian likes to point out that the ketogenic diet, or at least a primitive version of it, has been around for a long, long time. "I always say the ketogenic diet dates back to biblical times," she says, referring to the New Testament books of Matthew 17: 14-21, and Mark 9: 14-29, in which Jesus refers to fasting when a man suffers from what appear to be epileptic seizures.

Early in this century, a spiritual healer named Bernarr Macfadden claimed that by fasting and praying with an epileptic he could "cure" the person's seizures. His clients' seizures often did stop during the weeks of fasting. However, once they started eating again, their seizures resumed.

About the same time, a team of physicians at Johns Hopkins Hospital who had been exploring child nutrition and disease got word of Macfadden's practices. The Hopkins group, and in separate efforts a doctor at Minnesota's Mayo Clinic, deduced the link between fasting, ketosis, and a halt to seizures, and devised the diet.

By the 1920s, Hopkins, the Mayo Clinic, and Cornell University's medical school were using the ketogenic diet. Later, Freeman's predecessor at the pediatric epilepsy center, Samuel Livingston, developed the version of the diet that is used today at Hopkins.

In 1992, Freeman and his colleagues reported in Epilepsia on results for 58 children who had used the ketogenic diet for two years. The patients in the study were the toughest epilepsy cases because most had been treated with one drug after another unsuccessfully. On the diet, 17 of the patients stopped having seizures. Another 22 had the frequency of their seizures cut in half or more. Furthermore, all 39 children who improved on the diet were able to stop taking anti-epileptic medication or to take fewer drugs than before. This is no snake oil, says Freeman. The ketogenic diet works.

Nevertheless, when he tried to publish a book about the diet, publishers rejected it. "They were concerned that it might get into the hands of parents who would want to try it without medical supervision," he says.

As well, there were some physicians who reported poor success with the diet. Freeman now believes that was because they tried a version that uses medium-chain triglyceride (MCT) oil as the staple, instead of butter and cream. "We've had less success with the MCT diet than with the ketogenic diet," he says. "It has more side effects, like diarrhea, and appears not to work as well."

Some doctors doubted that children could be kept on such a strict diet, one that forbids sugar and starch. What child would not try to sneak a cookie, or even a piece of bread? Still others said there were no well-controlled studies showing the ketogenic diet works. William Theodore, chief of clinical epilepsy at the National Institute of Neurological Disorders and Stroke in Bethesda, Maryland, still has reservations.

"It's never been subjected to a controlled study," he says. The Epilepsia report was "a review of clinical experience, and did not include a case-control group. It's meaningless intellectually to talk about results in a small number of patients." Nevertheless, he adds, "it's worth a try. We put some people on it. We think it works." But no one knows how it works.

Freeman understands such views. "Good physicians should be skeptical," he says. "I remain a skeptic. But I've seen the diet work often enough that my skepticism has been overcome. Until you've tried it and had success, it's just another alternative therapy."

Conducting a case-controlled study of the ketogenic diet is very difficult, he adds. A control group would have to go on the identical diet, plus take glucose daily for two years. Few patients would want to endure such a strict diet while taking a substance--glucose--that would almost certainly negate any benefits from the diet.

For now, anecdotes and testimonials of clinical success are all there is for the ketogenic diet. But it's hard not to be swayed by stories such as that of Christopher Slinker, 13, whose mother, Judy, says the ketogenic diet "has meant a metamorphosis" for her son. Before going on the diet at age 7, Christopher frequently went into status epilepticus, or "status," a series of seizures that seems never to end, and that requires emergency care. He was taking five different seizure medications, says his mother, and "was a totally drunken person. He lived to go to bed."

Now off the diet for four years, Christopher is a solid B student, plays basketball, skis, and swims. He is seizure free and drug free. "We are grateful every day this child gets up and smiles," says his mother.

The ketogenic diet moved out of relative obscurity and into the limelight in 1993, when Hollywood film producer Jim Abrahams came across a four-page description of the diet in Seizures and Epilepsy in Childhood: A Guide for Parents (The Johns Hopkins University Press, 1990), a classic text now in its third printing. It was written by Freeman; Eileen Vining, associate professor of pediatric epilepsy; and Diana Pillas, counselor and coordinator of the pediatric epilepsy center.

Abrahams had been desperately seeking solutions to his 1- year-old son Charlie's intractable seizures. With Freeman's team, he and his wife, Nancy, started Charlie on the diet, and found that it eliminated their son's seizures. Convinced that the diet was a godsend, and amazed that it was not widely known, the Abrahamses launched the Charlie Foundation To Help Cure Pediatric Epilepsy to publicize it.

With funding from the foundation, Freeman finally got the opportunity to publish a book on the subject: The Epilepsy Diet Treatment: An Introduction to the Ketogenic Diet (Demos Press) by Freeman, Millicent Kelly, and Jennifer Freeman came out in 1994. Abrahams also produced a video about Charlie's story--narrated by actress Meryl Streep--that was sent to every child neurologist in the country. The Foundation is also helping to support a seven- center trial to test the diet's effectiveness.

The latest boost came in October, with the Dateline piece that featured Freeman, Kelly, and the Abrahamses. Since then, Freeman's office has received 5,000 calls about the diet, and The Epilepsy Diet Treatment has gone into a third printing. Johns Hopkins Hospital now has a two-month waiting list for children whose parents want to start them on the diet. "We're down to 40 to 50 calls a day," said Freeman in late February.

With funding from the Charlie Foundation, Freeman's team has also trained physicians, nurses, and dietitians from 11 hospitals throughout the United States and Canada.

In a small examining room at the Johns Hopkins Outpatient Center one day recently, Freeman plops down on the floor and flips through the ream that shows Korinne Hurst's EEG, while Connie and Chuck Hurst report on their daughter's progress. Connie Hurst is concerned about "sub-clinical seizures"--ones that show up only on the EEG but don't affect Korinne's behavior.

An imposing figure with fly-away gray hair and elastic facial features, Freeman relishes a bit of pedantry in his exams. "I don't treat EEGs," he tells the mother in a soft voice, raising his eyebrows to catch her reaction. "I treat seizures." As he speaks, coins mysteriously fall from his pockets onto the floor. "Where did those come from?" he asks.

Korinne gives him a dimply smile, then jumps around the room to pick up the coins. A healthy, active child, she has not had any seizure medication since Christmas.

Sticking to the diet has not been without its anxious moments, Connie Hurst says. She tells of a day a few weeks earlier when she came home from work, tossed a package of sticky buns on the kitchen table, and went to take a shower. When she returned, Korinne was sitting at one end of the kitchen looking sheepish, and four buns lay on the table, minus their frosting. The mother knew that sugary frosting is definitely not on the ketogenic diet--it could push Korinne's system out of ketosis and back into seizures! Panicked and in tears, Hurst immediately phoned Millicent Kelly.

Kelly told her not to worry. Korinne might come out of ketosis, she said, but could probably get back into it easily by fasting.

Korinne skipped two meals and ended up squeaking by without a seizure. Aside from the sticky bun episode, she has followed the diet's rules.

"How do you get Korinne to stick to the diet?" Freeman asks her parents.

"We just tell her, if something's not on your magic diet, you can't have it," says Connie Hurst.

Korinne pipes in: "It's my magic diet!"

In a conference room down the hall, another couple, Stephanie and Patrick Larson, is hoping the diet will work as much magic for their 2-year-old daughter, Angela. (The names of this family have been changed.) The Larsons, along with two other couples, have brought their children to Johns Hopkins to begin the diet that evening. Their children will be admitted tomorrow. Over the next five days, while their children progress into ketosis under medical supervision, they will continue to receive instruction from Kelly.

As their children snooze in their strollers, the adults listen to the dietitian describing the rudiments of the ketogenic diet. "This diet--with heavy whipping cream as a basis--is not like anything in your wildest dreams," Kelly tells the parents, her voice still rich with a Carolina drawl. Tonight the children will start fasting to kick their bodies into ketosis, she says. After they've gone into ketosis (which takes anywhere from 24 to 48 hours), they will start the diet, drinking a small serving of "eggnog" (heavy cream, egg, and saccharin) for their first few meals, and then starting on the other foods that they're allowed to have.

"We usually start with a four-to-one ratio," Kelly continues, "four times as much fat as protein and carbohydrates combined." (A normal diet consists of about half carbohydrate, a third fat, and the rest protein.) If all goes well, the child switches to a three-to-one ratio, then moves to a two-to-one ratio. "If all is well after two years, you get to stop the diet. If your child hasn't had a seizure in two years, he probably won't," she says.

When parents first hear about the diet, says Kelly, many wonder whether it will cure their child's epilepsy only to cause heart disease later on. She and Freeman tell them that some children on the diet do have slightly higher cholesterol levels, but that those levels return to normal once they resume eating normally. They appear to be at no greater risk of heart disease or related problems than the average child. Since the diet is low in certain nutrients, she adds, children on the diet need to take a multi-vitamin daily.

Stephanie Larson jots notes while her husband keeps an eye on Angela, whose cherubic face often flops down onto her chest as she sleeps. She appears unable to hold it up. Patrick Larson gently pushes it back up.

Like the Hursts, the Larsons have seen their daughter's seizures resist many different powerful medications. Angela has 10 seizures a day.

But unlike the Hursts, the Larsons are helping their daughter battle several different disabilities as well as her epilepsy. At age 2, Angela cannot speak or walk, and she cannot hold her head up well. Currently she has trouble completing two motions at the same time, like holding her head up while moving her arm. Angela sees a string of therapists--physical, developmental, occupational. So far she has no official diagnosis for her problems. "Severely developmentally delayed" is the medical verdict for now. Like Angela, the majority of children who have used the diet through Hopkins have mental retardation, cerebral palsy, or another neurologic problem that compounds their epilepsy.

Stephanie Larson says that ever since Angela started having seizures at age five weeks, the couple and their two older children have often felt that they were walking on eggshells. Although they try to include Angela in normal family activities, such as church, grocery shopping, or holiday celebrations, she is easily spooked by lights and sounds. "If I were to laugh while holding Angela in my lap, that would set off a seizure," says Stephanie Larson. The organ at church can trigger one, as does anything that rocks her, like swings or a rocking chair.

"We're committed to doing things as a family," she says, so they've looked for ways to include Angela in family activities. For example, each week a different member of the congregation volunteers to hold Angela in a quiet chapel during the church service. "But it's been a constant trade-off," says the mother. "If we take her out, she gets more stimulation, but then she gets more seizures."

She and her husband say they have modest goals for the diet. If she could take Angela to the grocery store, the park, or for long walks without fear of triggering a seizure, she says, "we would open up her world so much more."

The Larsons, who both have backgrounds in public health, say they've weaved through the medical labyrinth for two years searching for explanations and ways to help their daughter. The ketogenic diet is one of their last hopes. So they sop up every piece of information. Kelly has a lot to offer.

"We don't want any weight gain during the first year," says the dietitian. "We don't give snacks, except for three ripe olives or two macadamia nuts, two pecans, or three filberts with half a cup of diet cola or weak tea."

"You have to watch out for dog food or anything spilled on the floor," she continues. "Anytime you turn your back they're going to go for it."

One father asks whether, if a recipe calls for 100 grams of something, he could use 95 or 105 grams instead? No, says Kelly firmly. "This is why we're successful here at Hopkins. We're very exact. If you make a mistake you've thrown your measurement off. You can't make a mistake." All amounts of food are keyed to body weight. That extra two grams of chicken, if given over several days, could upset the metabolic balance and force the child out of ketosis. Although she can come off sounding like a drill sergeant, Kelly says that precision is crucial. Many parents swear by her approach, and her files are full of appreciative thank-you letters.

Now in her second stint at Hopkins, retired once already, Kelly is full of verve for her 67 years. "I'm the type of person who has so much hope," she says. "I want them to do so well. I've always thought, if we do this right, it's going to work."

Her enthusiasm is infectious. But she acknowledges that while more than half of the children on the diet show improvement, roughly a third do not. No one knows why some children do better than others.

Each morning that Angela and the other children are in the hospital, Kelly gives their parents a different lesson in the ketogenic diet.

Become label sleuths, she tells them during one class. Look for hidden sugar. "If you see the 'ose,' it's sugar. Fructose, lactose, galactose, sucrose, dextrose, maltose, xylose--they are all going to turn into glucose in the body." Even some products labeled "sugar free" contain some form of sugar. "A lot of medicines contain manitol or sorbitol. Those turn into sugar."

Holding up a can of "lite" powdered fruit drink, Kelly points out that although the front of the can says the mix is sugar free, the back label reads "1 gram carbohydrate." Kelly suspects the carbohydrate comes from concentrated lemon juice. It's sugar, she says, and children on the diet should not have the beverage.

Angela's mother asks if she can use fresh lemons. No, even a lemon has about half a gram of sugar, says Kelly. "This is the frustrating thing about the ketogenic diet."

But although the diet is restrictive, Kelly says, "it liberates the child. You can take your child to restaurants. A lot of places will microwave a meal you've prepared and bring it out on a plate."

The diet also liberates parents, she says. "You can sleep without getting up at night to give your child medication or worrying that your child is going to choke during a seizure. One woman said her sex life came back after her child started on the ketogenic diet."

Two weeks later, the Larsons are back in their home north of Baltimore. On the couch, Angela sleeps curled up in her mother's arms wearing purple footsie pajamas. Two weeks into the diet, she is having only one or two brief seizures a day, her mother says.

At first, Angela wouldn't drink the cream. "I finally found the magic combination for her," says Stephanie Larson. "I warm it, dilute it with water, add five drops of coconut extract and a little saccharin." Angela's 8-year-old sister, Connie, walks up to the couch and asks to hold the baby. The mother places Angela in her arms. The older girl cuddles her sister and kisses her head.

Stephanie Larson is pleased to report on other improvements Angela has made. For one thing, she's started to babble again. "We last saw that a year ago," says Larson. The other day Larson listened to a rock station on the radio for four hours. "Angela didn't jump once," she exclaims. And now she can rock Angela in a rocking chair. "If that continues, I'll be thrilled. It opens up everyday living for us.

"Both my husband and I feel like we've been falling for two years," she says. "We were in horrible limbo. Now we can only climb up. We're picking up our life again."

The diet is part of their renewed hope, she says. "Whatever this diet does, it's doing it, and it's very good. It's a new beginning for us and for Angela. I can't tell how much it will help. It won't be leaps and bounds, but she will get better."

For further information, call: The Johns Hopkins Pediatric Epilepsy Center, (410) 955-9100; or The Charlie Foundation To Help Cure Pediatric Epilepsy, (800) FOR-KETO.

Melissa Hendricks is the magazine's senior science writer.

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