Growing Up with Epilepsy: Evan's Story

During his first year of life, Evan had two seizures triggered by fever. At the age of three and a half, his seizures returned, at first sporadically, then more frequently, progressing to 50 to 60 seizures per day.

Seeking Treatment Options: The Ketogenic Diet

Evan was diagnosed with a mixed seizure disorder called myoclonic-astatic epilepsy (MAE), or Doose syndrome, an idiopathic, generalized epilepsy of early childhood.

About seizure types

The seizures associated with Doose syndrome are often difficult to control. Despite trying several medications, Evan continued to have staring seizures, drop attacks, and generalized tonic-clonic seizures. These seizures, combined with the side effects from his medications, made it difficult for him to participate in school and other activities. Evan's parents, Jeff and Jynell, were concerned that he was not reaching important developmental milestones and decided to seek a second opinion for treatment options.

Getting a Second Opinion

In a second opinion consultation, Jeff and Jynell were told about the alternative therapies available for seizure treatment. In 2001, the options included brain surgery, vagus nerve stimulation (VNS), and the ketogenic diet, a high-fat diet with very limited amounts of carbohydrates and sufficient protein for growth and development. Because of Evan's seizure type, he was not a surgical candidate. His parents and doctors discussed the remaining options and decided to try the dietary therapy.

In preparation for the required hospital stay to start the diet, his parents began limiting Evan's carbohydrate intake and switching him over to foods with higher fat content, such as heavy cream and bacon. Remarkably, within four days of modifying Evan's diet, his seizures stopped. This early success was extremely encouraging.

The ketogenic diet was initiated in the hospital over the course of several days, throughout which Evan was monitored as his body adjusted to receiving 90 percent of his caloric intake from fat. During the hospital stay, Evan's family also received training from the dietitian about the kinds of foods that are allowed and how to weigh those foods to the gram to meet the 4 to 1 ratio of fat to proteins and carbohydrates that Evan would need once he was out of the hospital. They also received a book of ketogenic diet menus specific to Evan's dietary needs and food preferences.

Following the hospital stay, Evan remained seizure free. After a few months, his doctors began the process of weaning him off his medications until the ketogenic diet was his only treatment.

Meal Strategies

For many families, the challenges of the ketogenic diet are substantial. Some children don't like, or can't tolerate, the food. Some families find the dietary limitations and the precise weighing of foods too taxing. Jeff and Jynell themselves were not daunted by the challenges of the diet but decided early on that they needed to be direct with Evan. The whole family, including Evan's sister, talked openly about the restrictions of the diet and the importance of staying on it to prevent seizures. Their work paid off. Evan has never had a problem with compliance, and he even enjoys the food.

Evan's parents have, however, made efforts to help normalize his meal experiences. For instance, when Evan was in kindergarten, he really wanted to have hot lunch like his classmates, so Jeff and Jynell met with the cafeteria staff and came up with a plan. Jynell explains, "Evan would give his lunch to the head of the cafeteria, and she would place it on a tray. And then Evan would come through the line with his classmates and it was like he was getting hot lunch. So they were able to accommodate him, which was really wonderful."

They work out a similar arrangement at restaurants. Jynell continues, "We make it easy. We weigh out the meal and bring it with us to the restaurant. When we sit down at the table we have Evan order water for his drink and then he tells the waitress that he's on a special diet. They always bring us an extra plate. So we made it fit into our world."

Weaning Off the Diet

The ketogenic diet is not a long-term seizure treatment. Children typically stay on the diet from a few months to a couple of years. Evan has now been seizure free on the ketogenic diet for three years. For the last 10 months, his family has been in the process of weaning him off the diet, a process that, for Evan, has been happening very slowly over the course of a full year. This allows his body to gradually adjust to burning more calories from carbohydrates and proteins and less from fats.

As with weaning off a medication, this process can be stressful for a family because of the fear of seizure recurrence, but Evan's prognosis is very good. His doctors feel that once he is off the diet completely, there is little chance that his seizures will return. Evan says he's looking forward to ice-cream bars and pizza.

Catching Up at School

When Evan started the ketogenic diet, he was nearing the end of kindergarten. Because he had so many seizures during class, Evan missed much of what was being taught and fell behind his peers, so his parents decided to have him repeat kindergarten. Once his seizures came under control, there was a dramatic shift in Evan's ability to engage in learning.

Kate Tynan-Hardy, Evan's kindergarten teacher, comments, "His parents came to me and told me they were putting him on the diet. At first we went, 'Oh no, what's going to happen?' It was pretty amazing. Within a week, he came alive and was just talking and talking. When I had him again for the second year, he was just a kindergartener fully participating."

Now seizure free, Evan is still in the process of catching up developmentally. He is in a mainstream classroom, but gets pulled out for occupational, physical, and speech therapies, including work on motor skill development, visual memory, and perception skills. Jeff and Jynell are thrilled with Evan's progress. The family enjoys celebrating milestones that at one time seemed far away.

Jeff comments, "Evan's life is endless now. He can do whatever he wants to do. We know that he's delayed, but we know all those milestones will come. We just keep on looking forward to the future. That's the best thing we have right now."

For more information about Doose syndrome, see doosesyndrome.org, a site built by parents of children affected by Doose syndrome.