DiversityNursing Blog

By JESSICA FIRGER

When Jackson Small began having seizures at 7, his parents hoped and assumed at least one of the many epilepsy drugs on the market would be enough to get things under control. But one seizure quickly spiraled to as many as 30 a day.

"He would stop in his tracks and not be aware of what was going on for 20 or 30 seconds or so," his mother Shana Small told CBS News. Jackson was eventually diagnosed with juvenile myoclonic epilepsy, a type of epilepsy characterized by brief but often frequent muscle jerking or twitching.

But a number of medications typically prescribed to patients with this type of epilepsy were not effective. And so the quest to help Jackson gain control over his seizures led the family from their home in Orlando, Florida, to the office of a registered dietician at the NYU Langone Comprehensive Epilepsy Center in New York City.

They were there to discuss the medical benefits of heavy cream, mayonnaise, eggs, sausage, bacon and butter.

A lot of butter.

The plan was to treat Jackson with a diet that is heavy in fat, low in protein and includes almost no carbohydrates. It's known as the ketogenic diet and has long been in the arsenal of last-resort options for patients with epilepsy who are unresponsive to medication. Doctors may recommend a patient go on this special diet after unsuccessfully trying two or three prescriptions.

The diet works by putting the body in a "fasting" state, known as ketosis. "When we're fasting the body needs to find fuel so our body will break down fat storage and break down their own fat and enter a state of ketosis," Courtney Glick, the registered dietician who coordinated and fine-tuned Jackson's diet plan, told CBS News. "But with this diet, instead of breaking down the body's fat, the body breaks down dietary fat."

The ketogenic diet consists of as much as 90 percent fat. Some patients who feel they can't make such an extreme change adopt a modified Atkins diet, which is between 65 and 70 percent fat. It can be nearly as effective for controlling seizures, though every patient is different.

Though experts don't know everything about why this diet is effective for seizure control, they do know that eating mostly fat causes the body to fuel on ketones rather than glucose, which ultimately lowers insulin levels. This can have an anti-inflammatory effect on the body and may prevent seizures by calming the brain, said Glick.

One study by researchers at Dana-Farber Cancer Institute and Harvard Medical School found that a child's ability to stave off seizures is tied to a protein that affects metabolism in the brain. The protein, called BCL-2-associated Agonist of Cell Death, or BAD, also regulates metabolism of glucose. The researchers discovered that by modifying this, they switched metabolism in brain cells from glucose to ketone bodies, which are fat byproducts.

Glick said the diet plan didn't work for Jackson until he tried the most strict version, which was a 4 to 1 ratio of fat to protein and carbohydrates. Each day, he ate approximately 160 grams of fat, 8 to 10 grams of carbohydrates and 30 grams of protein, all of which amounted to about 1,700 calories a day.

Four months into the program, Jackson was seizure-free. He remained on the strict diet for two years with no return of seizures. His mother prepared foods from special recipes such as "keto" pizza made with a macadamia nut crust or chicken nuggets with coconut flour.

Over the summer -- after receiving a green light from his doctors -- Jackson, now 10 years old, began to wean himself off the diet, and his mother has slowly introduced foods such as breads and ice cream. He has maintained seizure-free and takes very little anti-seizure medication.

Research has found that for pediatric patients the anti-seizure effects of the diet often continue long after the child stops following the food plan, though the reason why remains unclear. This is typically not the case for adults, who may need to stay on the diet for life in order to control seizures.

"We've probably seen more kids go on the diets than adults, and adults are really set on their eating patterns," said Glick, adding that social obligations can make the diet difficult to fit into a grown person's lifestyle.

Jackson's mother said his doctors are hopeful that in the near future he may no longer need medication -- or a keto diet -- to stay seizure-free. "I think it's taught him a very important lesson about how food is as important as medicine, and how food affects the chemistry of your body," she said.

Source: www.cbsnews.com and http://www.dana-farber.org/

For three young siblings, eating is a life or death proposition, thanks to a rare white blood cell disease, reported KSL.

The Frisk children— Jaxen, age 9; Tieler, age 7; Boston, age 4— have spent weeks in the hospital and are allergic to pets, pollens and multiple foods. The siblings all have eosinophilic gastrointestinal disorder (EGID), an abnormal build-up of eosinophil white blood cells in their GI tracts that can cause inflammation and tissue damage in response to foods and allergens. While the disease is relatively rare, it has increased in prevalence over the past decade affecting one in 2,000 people, according to the American Partnership for Eosinophilic Disorders.

"You need food to survive. But it is also what can kill you in our house," their mother, Jenny Frisk, told KSL.

When they’re exposed to their triggers, the children could have an anaphylactic reaction— potentially fatal allergic symptoms throughout the body.

"Tieler had one sip of milk when she was 1-year-old, and instantly started projectile vomiting and got hives all over her body," her father, Gary, told KSL. "It's a life and death situation at birthday parties, or religious events, or anywhere we go, because food is such a big part of our culture."

Between the three children, they’ve endured 11 surgeries and eight extended hospital stays, with more expected in the future.

On top of the children’s health issues, Gary battled cancer two years ago and Jenny had to have several surgeries due to serious adrenal insufficiencies that were unrelated to EGID.

The family has been bankrupted twice by medical bills. While they make too much income to qualify for help, they don’t make enough to pay for their children’s medical needs. Friends and family have started a GoFundMe account to raise money to pay for genetic testing and treatment.

"When we're looking at an illness that is not curable, and the treatment isn't covered (by insurance), the light at the end of the tunnel is really far away," Jenny said.

Source: http://www.foxnews.com