Swedish girl with rare condition undergoes specialized treatment in Utah

Carl Molstad said his daughter Edith, who turns 11 on Wednesday, has lived two different lives: one before her seizures, and one after.

She used to love playing soccer, hanging out with friends and going to school. But all of that vanished — like “that,” her father said Monday, snapping his fingers — at age 7, when Edith developed a rare condition called FIRES epilepsy, which stands for febrile-infection related epilepsy syndrome.

A seizure usually lasts a few minutes, triggered by abnormal electrical activity in the brain. They can cause muscle contractions as well as changes in thinking and emotions, depending on the type. But FIRES causes prolonged seizures that don’t respond to medication, according to the Epilepsy Foundation. The condition affects one in 1 million children.

It’s what brought the Molstad family — who lives in Sweden — to Salt Lake City, where Edith in June received a pacemaker-like implant designed to monitor and stimulate her brain, intended to stop her seizures before they start. The device isn’t available outside of the U.S., and doctors hope it will help Edith regain some of her old life.

Carl Molstad, Edith, and the girl’s mother, Mathina, were back in Utah on Monday for the first time since the device was inserted into Edith’s skull this summer at Primary Children’s Hospital. It won’t be the last time they return — the strength of the implant’s stimulation will need to be adjusted by physicians every few months over the next few years, her mother said.

The family members each wore matching black T-shirts Monday that read “I’m a FIRESfighter” — in reference to their blog about Edith — while speaking with reporters over a Zoom call about their experience.

In Edith’s case, the trouble began about four years ago in Sweden, when Carl received a call from his daughter’s teacher saying that she was sick. Tired and feverish, Edith had flu-like symptoms for the next couple of days, her father said. As she seemed to improve, his wife called out to him one morning.

“I can’t wake up Edith,” she said. Their daughter was sweating and shaking, with blood around her mouth.

It would be the start of what Carl described as a “horrible journey.” Edith had to be put into a medically induced coma and placed on a ventilator for about a month. Doctors told her parents that she might die. She eventually came out of that first major seizure, but continued to have seizures almost every day afterward.

Doctors in Sweden “saved our daughter’s life,” the father said Monday, “but they haven’t been able to give her life back.”

Before seeking treatment in Utah, the family turned to special diets, various medications and injections to try and help Edith, only to see her seizures persist. Her mother felt powerless, she said.

So when Mathina Molstad heard about responsive neurostimulation (RNS), and learned that Utah-based Intermountain Healthcare could provide it, she knew they had to go.

The RNS device was implanted by neurosurgeon Dr. Robert Bollo, director of the pediatric epilepsy surgery program at Primary Children’s Hospital. It’s made up of two main parts: a computer with a battery that’s inserted into the skull, and electrodes that sit on the brain and plug into the computer.

This device was released a decade ago and has been available in Utah for about six years. About 30 such devices have been implanted in the state so far, Bollo said during the Zoom call, and that number continues to rise.

The FDA approved the device for use in adults, but it’s used off-label in children “at our discretion,” Bollo said. To date, between 150 and 200 such devices have been implanted in children throughout the country, and the results seem promising, he said. NeuroPace, the company that makes the device, is sponsoring a trial that will study the implant’s use in kids age 12 through 17. Edith was the first child from Europe to receive it.

So far, Edith still suffers the same amount of seizures, her parents said. Dr. Bollo said it could take time for her to start feeling better, as children’s reactions to the treatment can vary.

“The data suggests that things get better and better and better over time,” he said.

“It’s like a marathon,” Edith’s mother said, “so we have to be patient.”

The Molstad family is paying for Edith’s treatment and routine travel to Utah out of pocket. Even with a discount from Intermountain Healthcare, her medical bills have reached at least $100,000, her father estimated, with no cap in sight. Anyone interested in helping the Molstads can visit a GoFundMe page that the family created to help pay for Edith’s treatment.