Recognizing a 'miracle' The high-fat ketogenic diet can ease seizures in epileptic children

USA Today; Arlington; Jan 12, 1999; Kathleen Fackelmann;

Abstract:

In desperation, they turned to the ketogenic diet, a controversial regimen that involves feeding children huge amounts of fat. The diet is thought to help some children by inducing a seizure-calming condition called ketosis. Ketosis occurs when the body is forced to burn fat instead of sugar.

Within 72 hours of starting the diet, Charlie (Abrahams) was seizure-free. "We felt as though we had had a miracle," recalls Abrahams, who in 1994 started the Charlie Foundation to Cure Pediatric Epilepsy.

While Abrahams' other films such as Airplane! gave fans a laugh a minute, First Do No Harm fueled a grass-roots effort for more research on the diet, says John Freeman, the neurologist at the Johns Hopkins Medical Institutions in Baltimore who treated Charlie.

Full Text:

You know you've produced a hit TV movie or series when it develops its own spin-off -- another show that sustains the impact of its original.

For writer/director Jim Abrahams, the most significant spin-off from his 1997 TV movie, First Do No Harm, had nothing to do with entertainment but a lot to do with the health of his epileptic son and thousands more like him.
The movie, starring Meryl Streep, captured a Chicago family's struggle to conquer the seizures racking their son's body.

Abrahams was uniquely qualified to write and direct the movie. He and his wife, Nancy, would never forget their own fight to help son Charlie when standard epileptic drugs and even brain surgery could not halt the brainstorms that seized his small body.

In desperation, they turned to the ketogenic diet, a controversial regimen that involves feeding children huge amounts of fat. The diet is thought to help some children by inducing a seizure-calming condition called ketosis. Ketosis occurs when the body is forced to burn fat instead of sugar.

The diet can be risky. But it can represent the only hope for children who don't respond to standard treatment.
Within 72 hours of starting the diet, Charlie was seizure-free. "We felt as though we had had a miracle," recalls Abrahams, who in 1994 started the Charlie Foundation to Cure Pediatric Epilepsy.

While Abrahams' other films such as Airplane! gave fans a laugh a minute, First Do No Harm fueled a grass-roots effort for more research on the diet, says John Freeman, the neurologist at the Johns Hopkins Medical Institutions in Baltimore who treated Charlie.

That groundswell is paying off. In December, Freeman and colleagues published the largest, most definitive study to date of the ketogenic diet.

The findings, reported in Pediatrics, confirm research that suggested the diet does control seizures in some children. Two additional new studies highlight a naturally produced substance that may underlie the diet's success.
Neurologists, who even a few years ago had viewed the diet with extreme skepticism, are now acknowledging its value. "There's recognition now," says Harvard neurologist Steven Schachter. "This form of therapy does have a role to play" in treating some cases of pediatric epilepsy, he says.

About 350,000 children and teen-agers suffer from epilepsy, a disorder with a variety of causes that leads to a disturbance in the brain's electrical activity. Doctors can harness seizures 80% of the time with drugs. But for the others, drugs simply don't work.

Doctors said Charlie's seizures would ultimately damage his brain, leading to mental retardation. "You feel your child has been given a fate worse than death," Jim Abrahams says. That frustration led the Abrahamses on a search that brought them to Freeman, who used the diet to treat a handful of children each year.

Because no rigorous scientific study of the ketogenic diet had been done, most neurologists discounted it as unproved and potentially unsafe. Indeed, the Abrahamses had taken Charlie to top pediatric neurologists in the USA. Not one mentioned it as an option.
The new research has given the ketogenic diet the credibility it had long been lacking. In the Pediatrics study, Freeman and his colleagues examined 150 children ages 1 to 16 who continued to have seizures despite treatment with at least two anticonvulsant drugs.

Doctors first hospitalized the children for five days, then gradually weaned them off their usual diet and put them on the ketogenic diet.

Kids on the regimen eat a high-fat, low-protein, low-carbohydrate diet.

Meals typically include small amounts of fish, meat, poultry, fruits and vegetables and lots of added fat, usually in the form of butter, oil or heavy cream.

The diet provides a whopping 90% of total calories from fat, says Jane McGrogan, a nutritionist at the Hopkins program. The average American diet gets between 30% and 40% of its calories from fat.

Kids love the cream but generally don't like the limits on sugar and carbohydrates. They must avoid pastries, sugary soda pop, potato chips and pretzels, McGrogan says.

Ten-year-old Timothy Finnigan of Satsuma, Ala., says the diet can be tough to follow. "It's hard when you look at what everyone else is eating," says the fifth-grader, who brings his lunch to school.

The diet also puts demands on parents, who must weigh foods and keep strict tabs on calories. Most parents find they must prepare meals without the help of processed foods, which can contain hidden carbohydrates, McGrogan says.

Still, the Hopkins team found that most parents and their children successfully followed the diet. The researchers found that 55% of children who started the diet stayed on it for a year. Nearly all of those children had fewer seizures on the diet, with 27% getting almost complete relief.

"These are the most desperate cases," Freeman says. "Even in those we get a spectacular success rate."
Children who stopped the diet usually did so because it wasn't working, Freeman says.

Freeman thinks the diet shouldn't just be a last resort but ought to be considered earlier. That may happen as doctors learn of a new study by Charles Niesen of the University of Southern California in Los Angeles. His findings show that a naturally produced substance called beta-hydroxybutyrate (BHB) may explain how the ketogenic diet works.
Niesen and researcher Sara Lustig designed a rat study to test BHB, one of a group of substances called ketones manufactured by the liver during the ketogenic diet. The diet forces the body to burn fat instead of sugar, producing BHB and other ketones during that process.

The researchers found BHB could block seizures in the rats. "We actually prevented seizures in 50% of the animals," Niesen says. The team presented results in December at the American Epilepsy Society's annual meeting in San Diego.
Niesen's research suggests that BHB calms the brain's electrical disturbance by activating certain protein receptors on brain cells. That raises hope that BHB in drug form will stop such brainstorms.

If Niesen gets Food and Drug Administration approval, he could launch a study of BHB in children as early as this spring. "I'm eager to get the kids started," he says.

Meanwhile, another study presented at the December meeting indicates that doctors can fine-tune the ketogenic diet to produce the most beneficial levels of BHB in the blood.

Lawrence Brown, Claire Chee and their colleagues at the Children's Hospital in Philadelphia developed a device to keep track of BHB in the blood of 12 epileptic children who were on the ketogenic diet. When eight of the 12 suddenly started having more seizures, the team found the level of BHB in the blood had dropped. The researchers adjusted each child's diet to boost BHB, bringing seizures under control within 12 hours, Brown says.

Just as a diabetic keeps track of insulin, children with epilepsy one day may be able to check their BHB levels at home to make sure the diet is working properly, Brown says.

Although parents might be tempted to view the diet as a natural approach to the treatment of epilepsy, researchers warn that the diet is harsh.

"You're using the diet as a drug," says Philip Sheridan, an epilepsy expert at the National Institutes of Health in Bethesda, Md. Parents who want to consider the ketogenic diet should carefully weigh the risks, he says.

According to Sheridan, those include kidney stones, immune system abnormalities, low blood sugar and clogged arteries. Sheridan worries that the high-fat diet may set the stage for a heart attack later in life.

Many children can be weaned off the ketogenic diet within a few years, Freeman says. Some go off the diet and remain seizure-free, he says.

Yet some children, like Charlie Abrahams, must stay on the diet or the seizures return. No one really knows the effects of maintaining a child on such a diet for years or even decades, Sheridan says.

He worries that such children may be vulnerable to growth retardation. Freeman, Sheridan and other experts advise parents to go on the diet only with the appropriate medical and nutritional help. Parents who have tried this diet on their own have had tragic results, Freeman says; several children have died as a result of an unsupervised ketogenic diets. The risks of an unsupervised diet include malnutrition, vitamin deficiencies and chemical imbalances.

At Johns Hopkins, the staff monitors children closely to make sure they're well-nourished and growing properly.
For Charlie, and many children like him, the future appears considerably brighter today than it did five years ago. In 1993, the Hopkins program was one of only a few that parents could turn to for the ketogenic diet. Now, many major medical centers offer it.

And research on the diet has exploded. The 1993 Epilepsy Society's meeting had no papers on the ketogenic diet, Freeman says. This year, 23 researchers presented findings.

The new era "provides hope for my family and lots of other families," Abrahams says.