Brain operation that saved boy’s life will help others, too

GRANITE FALLS — Before Alden Bernate’s second birthday, surgeons separated the right and left halves of his brain to stop seizures that wracked his small body up to 50 times a day.

Now the upbeat 12-year-old with a wild imagination doesn’t remember being sick. He knows he had a surgery to save his life.

He also knows that his brain is being used to help other children.

Surgeons removed part of Alden’s frontal lobe when they performed the hemispherectomy. His family agreed to donate the brain tissue for research. Scientists used it and other samples to identify a previously unknown gene mutation behind the condition that threatened Alden’s life. Those findings were published May 9 in the JAMA Neurology medical journal.

Alden doesn’t understand the science behind the groundbreaking research. What he does understand is that his brain could make a difference in the world.

“I want to help other children get healthy,” Alden said.

Clear eyes

As an infant, Alden was diagnosed with focal cortical dysplasia, one of the most common causes of intractable epilepsy. With intractable epilepsy, seizures can’t be controlled by medication.

One in 100 people in the U.S. have been diagnosed with epilepsy and one in 26 likely will develop some form of it in their lifetime, according to the Epilepsy Foundation. Intractable epilepsy accounts for about a third of cases.

Alden took five medicines a day and ate a special diet when he was a baby. He was hospitalized five times before his surgery.

“On a good day, with all the medicines on board, he’d have 15 (seizures),” said Gabriela Bernate, Alden’s mom. “If he had a cold or something, he’d have about 50 a day.”

The symptoms worsened. Alden stopped eating and slept most of the day. He didn’t focus. His eyes were clouded.

He was 19 months old when he went in for surgery. The anesthesiologist took him in around 8 a.m. His parents didn’t see him again until 4 p.m.

Alden opened his eyes.

They were clear.

The relief was overwhelming, but Gabriela Bernate knew her baby boy wasn’t out of the woods yet. Doctors weren’t sure if he’d be able to speak.

She brought a children’s book to the hospital room. Alden said “Mama” and she was “through the roof” with excitement, she said.

The left side of Alden’s face drooped, like someone who just had a stroke. He had little movement in his left arm and leg.

Even so, he danced all the way home in his car seat.

‘A typical seventh-grader’

On May 14, the Bernates put on their second 5K fundraiser for the Hemispherectomy Foundation. Last year, the 5K drew 23 people. This year, 92 participants raised $4,102. The money goes toward scholarships for students who had hemispherectomies and care packages for families.

Bernate hopes to make it an annual event. Along with raising money, it helps other students understand Alden. More than $1,000 of the total raised was from Granite Falls students who walked with Alden and his family.

Alden is developmentally delayed because of the hemispherectomy but doesn’t let it hold him back. He competes in the Special Olympics, is part of National Junior Honor Society and is a volunteer with Hero Club at Granite Falls Middle School, where he’s a seventh-grader.

His family cheers him on. Gabriela Bernate is a speech-language pathology assistant at Clear Speech Inc. in Everett, which sponsored the 5K fundraiser. His dad, Diego Bernate, works at Boeing. Alden has two older brothers. Nicholas, 30, lives in California and Oliver, 16, is a junior at Granite Falls High School.

Beth Wiess has taught Alden in her Life Skills class for three years. She describes him as happy, affectionate and funny.

“He’s a typical seventh-grader in a lot of aspects,” she said. “He wants friends, and he wants to go to dances, and he wants to make his mom proud.”

Alden waffles between becoming a chef or a farmer when he grows up. As a farmer, he’d want to grow peas, carrots and broccoli. It would be fun to grow chocolate, he said. His mom suggested he grow cacao. He likes the idea of growing the “coo-coo” plant.

He has fun at the Boys &Girls Club in town and likes playing video games, especially racing games. He’ll turn 13 later this month and wants to have a slumber party.

Other students high-five him at lunch or in the hallway. The 5K was an eye-opener for them.

“I think before he was kind of the kid they didn’t get,” Wiess said. “Now they understand.”

Critical first step

Dr. Ghayda Mirzaa, a geneticist at Seattle Children’s Hospital, led the study that found the gene mutation in Alden’s and other kids’ brains.

Her team suspected that the cause of focal cortical dysplasia might be something abnormal in the brain’s genetic makeup. They gathered data from 2012 to 2014, then analyzed it over the past two years. They screened more than 90 current and past patients. Alden was one of 10 they focused on.

Researchers found a genetic flaw in brain DNA that wasn’t in saliva or blood. Without brain tissue to study, they might never have identified the problem. Families like Alden’s made a crucial contribution, Mirzaa said.

“This is probably the first breakthrough to understand this disorder better and opens the door to a lot more research,” she said.

The abnormality is found within what she calls a gene pathway, and this pathway has been of interest for cancer research as well as brain disorders. Other researchers are doing pre-clinical trials of potential treatments involving different genes that are part of the same pathway and have been linked to similar disorders.

It’s an exciting time for brain research because scientists can look for problems at the cellular level, Mirzaa said.

“Understanding the causes is only the first step, but it’s a really critical first step,” she said.

Superhero

The research has buoyed Gabriela Bernate’s hopes.

“I remember thinking that in my lifetime they would not find out what was causing these issues,” she said.

She hopes doctors can find a way to treat intractable epilepsy through gene therapy. Then surgeons wouldn’t need to disconnect the halves of a child’s brain to save their life.

Alden overcomes the challenges from his surgery. His vision is impaired, so he sits at the front of the class. He uses a slant board to help him manage papers and pencils at his desk. His left side is weak, so he’s learning to type one-handed. His computer keyboard is coded with different colors of paint so he can remember which finger to use for which keys.

“In PE, just walk versus run, that’s a lot harder for him than it is for a normal kiddo,” Wiess said.

Nevertheless, PE is Alden’s favorite class and track is his favorite part, he said. He also likes yoga.

Alden gets bored quickly if he doesn’t have anything to do but also laughs and smiles easily when he finds something that interests him.

He has a colorful imagination. The people who participated in the 5K weren’t just runners or walkers to him. They were Alden’s Army, his soldiers marching into battle. In a way, they’re helping him fight the sickness that attacked him as a baby.

Gabriela Bernate explained to her son how his brain helped scientists learn about that sickness. Someday, she told him, it could help other kids get better.

That makes Alden feel happy and confident.

“Like a superhero,” he said. “Now I need to get my costume.”

Kari Bray: 425-339-3439; kbray@heraldnet.com.