THE DIET THAT CURES
When Niko was 2, he used to have up to 20 seizures a day. Now 5, he's seizure free thanks to the ketogenic diet treatment he completed at Children's Hospital.
Charlie Cleberg gives a "toes-up" for the ketogenic diet that helped him become seizure free. Brain bleeds after Charlie's premature birth left his arms paralyzed; now he does everything with his feet.
When medications can’t control intractable epilepsy,
maybe the ketogenic diet can
Niko Cortessis used to have up to 20 seizures a day, sometimes more than five in a half hour. He was 2 when they began. When it wasn’t a head-drop seizure, leaving him limp as a dishrag, it might be one more violent, making him roll around the floor.
Now 5, Niko and his older brother are busily building toys out of plastic Lincoln Log pieces in their family’s playroom in Lafayette.
Charlie Cleberg used to have a seizure nearly every day — frequently between 11 and 11:30 a.m. Five years later, Charlie, 8, is glued to his Xbox video game console in Pinole, playing Need for Speed. “He never finishes the race because he’s so busy flying off the road getting ‘air,’” said his mom, Kathy.
For these two boys, and others with intractable epilepsy, anti-epileptic medications haven’t helped. But thanks to a remarkable treatment option called the ketogenic diet, both boys—and many other children—are now seizure free.
In Northern California, Children’s Hospital & Research Center Oakland is one of only four Neurology departments offering the treatment.
Diet's four main characteristics
At Children’s, the ketogenic diet begins with a brief hospital stay and continues at home. The diet’s four main characteristics are:
- Extremely high fat content: 85 to 90 percent of food energy comes from fat.
- Calories precisely controlled: Every food item in a meal is measured with a gram scale.
- Very low in carbohydrates.
- Supplements and protein must be adequate to maintain growth and development.
More than 125 children treated in 12 years
In 12 years, neurologist Robin Shanahan, MD, and Clinical Nutrition manager Karen Spalding, MS, RD, CSP, veteran Children’s clinicians, have treated more than 125 children with the diet—including Niko and Charlie. “(The diet’s) been a godsend for us,” said Niko’s mother, Kirstin.
Charlie’s mom, Kathy, is also enthusiastic. “His seizures stopped while he was in the hospital beginning the diet,” she said. “He hasn’t had one since; it was dramatic.”
One in three treated with diet see dramatic improvement
While the diet doesn’t work for every child with epilepsy, about one in three children treated with the diet experience a 90 percent or greater reduction in seizures. Niko and Charlie are among the lucky ones in that third who become seizure free, about 5 to 10 percent of all those treated.
A second third experiences a 50 to 90 percent reduction in seizures, while the others experience up to a 50 percent reduction in seizures.
History of the ketogenic diet
Throughout recorded history it’s been known that fasting can reduce or eliminate seizures. Even the Bible mentions the phenomenon. Yet today, how and why the ketogenic diet works remains a mystery.
This seizure treatment option has had its ups and downs in the United States since the early 1900s, when physicians at several institutions, including ketogenic diet pioneer Johns Hopkins University, began studying it. As more anti-epileptic medications became available, the diet fell out of favor.
Television programs catapulted ketogenic diet out of obscurity
In the 1990s, the ketogenic diet was catapulted out of obscurity—first in 1994, when NBC broadcast a Dateline story about it, and again in 1997, with the broadcast of First Do No Harm, a made-for-TV movie that starred Meryl Streep and Fred Ward.
The movie’s fictional neurologist, like many real ones practicing at the time, didn’t consider the ketogenic diet a legitimate treatment and didn’t offer it as an option.
But after the Dateline program aired, the phone was ringing off the hook at Children’s Neurology department. Within weeks, Karen and Dr. Shanahan were on a plane to Baltimore for training at Johns Hopkins. By February 1995 they had admitted their first patient.
Eligibility for the diet program at Children's
As key members of Children’s ketogenic diet team — which includes other neurologists, a nurse practitioner and social workers — Karen and Dr. Shanahan have strict guidelines for choosing patients. They begin with eligibility.
To be considered for the diet at Children’s, patients must have intractable epilepsy that can’t be controlled with medication. Each patient needs to have tried at least three medications without seizure reduction before being considered.
Once everyone agrees the child is ready—parents, child, treating neurologist and Karen—a date is set to begin. Children’s patients begin their ketogenic diet treatment with a 24- to 72-hour medically supervised inpatient stay.
Diet shifts body's metabolism to ketosis
Starting the diet shifts the body’s principal energy source from carbohydrates to fats. Ketones, a byproduct of fat metabolism, are excreted in urine and signal that the body has shifted into ketosis.
Maintaining ketosis is apparently the key to the diet’s ability to reduce or eliminate seizures. Whether ketones themselves are therapeutic or are simply a marker of some other process is not known.
After their inpatient stays, Charlie and Niko, like other patients, returned home to continue the rigidly controlled diet. Karen calculates menus designed to help parents keep their children healthy, yet burning mostly ketone-producing fats for energy.
Maintaining ketosis is tricky
Maintaining ketosis is tricky. Anything from misreading a food label to eating a single piece of candy, to having a routine childhood illness or drinking extra water can throw it off. There may also be complications, including dehydration, nausea or lethargy. But nearly all families find the challenge worth the effort.
During their first few days on the diet, both boys stopped having seizures.
Once seizure free, some can wean off the diet
In time, perhaps after two years in ketosis, it’s possible for some treated children to stay seizure free without the diet. Clinicians and parents work this out case by case.
Their brain chemistry seemingly altered permanently, many seizure-free children may be able to “wean” off the diet, just as Niko and Charlie are now doing.
During the weaning process, the calorie ratio between fats, and carbohydrates and proteins is gradually shifted. It starts in a range from 3:1 to 4:1—meaning for every gram of carb or protein in the diet, three to four grams will come from fats.
During weaning, fewer calories will come from fats and more from carbs and proteins, shifting slowly to a more conventional diet. The actual process is slow, precisely calculated and carefully monitored.
Spectrum of children with seizures
The boys also illustrate the spectrum of children with seizures. While seizures were Niko’s main serious health concern, for most children with intractable epilepsy, including Charlie, brain dysfunctions, including developmental delay, are associated with seizures.
Charlie has faced a host of serious medical conditions since he was born six weeks prematurely. He spent his first five months on a ventilator in Children’s Hospital’s Neonatal Intensive Care Unit and wasn’t expected to live until his first birthday. Brain bleeds led to brain damage, cerebral palsy and paralysis of his arms.
Because Charlie can’t swallow and required surgery that removed much of his intestines, he gets all his nourishment through a tube straight into his stomach. When he was 3, the seizures started.
But Charlie has been blessed with wonderful adoptive parents, Kathy and Joe. “Charlie’s done great,” said Kathy. “He’s almost 8 and is fully included in a regular second-grade class at Castro School, a full-inclusion elementary school.”
Seizures and medications slow children's development
Seizures, and the medications neurologists use to try to control them, also put a crimp in children’s ability to learn and develop, especially during crucial early years. “A year of his life in development was just gone from the drugs he was taking,” said Niko’s mom, Kirstin.
With the help of 2 1/2 days a week at a local intervention program and another 2 1/2 days at a preschool, Niko is now developing at a more normal pace.
When Charlie became seizure free, his development, also slowed, came back strong. “Once he stopped having seizures, he was able to start thriving even more,” said Kathy. “It seems like his brain was able to be more receptive to learning.”
Parents love Children's ketogenic diet treatment team
All four parents appreciate not only the diet, but Children’s treatment team, including Neurology medical director, Daniel Birnbaum, MD.
“We love Karen and Dr. Birnbaum, and Charlie loves them,” said Kathy. “Dr. Birnbaum is the only doctor at Children’s Hospital who actually gets on the floor to play with and examine Charlie. And I think that says a lot about the man’s character.”
Niko’s father agrees. “Dr. Birnbaum was just great,” said John. “He was always attentive.”
Staying on the diet is the hardest part
For most families, the hardest part of the diet, once they see it working, is staying on it. Building menus that follow prescribed dietary calculations is one of Karen’s areas of expertise. Her goal is controlling calories and carbohydrates, while providing a high-fat diet containing adequate protein for growth and development. “We calculate their diets down to the gram,” said Karen.
Food allergies and preferences, and each child’s oral–motor abilities, must also be considered. The diet can be taken by mouth or by feeding tube, the way Charlie does.
“The most exciting thing about the ketogenic diet is you have someone who has poorly controlled seizures, that potentially, on the diet, can be 100-percent seizure free and off medication,” said Dr. Shanahan. “The diet can be stopped after a couple of years, and the kids stay seizure free. That’s such an exciting prospect.”
Menu planning for the ketogenic diet
The recipe for maintaining a child on the ketogenic diet combines art and science. Karen calculates each child’s customized dietary prescription of calories, protein, fat, carbohydrates and supplements to control seizures while promoting growth and development. The art is in creating a variety of palatable, age-appropriate menus.
Karen understands the visceral connection between parents, children and food. “Since feeding is the most down-to-earth thing you do as a parent, if you can control your child’s horrible disease by modifying their diet and being in charge of it, it really leads to a lot of satisfaction,” she said. “I see that over and over again. If it works, they hold on tight.”
Creative parents come up with food ideas within dietary restrictions, sharing them with each other and with Karen. It’s all about letting go of traditional meal combinations. A meal of popcorn, butter and a small portion of bacon may fit the prescription, provide needed nutrients and keep a kid excited about eating.
To learn more about the diet, or to make a Neurology appointment, call 510-428-3590.
This story, written and photographed by senior writer Tom Levy, was first published in the Summer 2007 issue of Childen's Hospital's HandPrints magazine.
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