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Washington boy faces fading vision, hearing

Family has hope for 6-year-old, trying to give him normal live

John Koch, 6, holds a kitty Dec. 9 on his family’s farm near Washington. He has been diagnosed with Usher syndrome type 2A, which doctors say means his hearing and vision will slowly fade. “We’re hoping to have some gene and stem cell therapy that will work for this condition in this child’s lifetime,” a University of Iowa expert said in an interview. (Rebecca F. Miller/The Gazette)
John Koch, 6, holds a kitty Dec. 9 on his family’s farm near Washington. He has been diagnosed with Usher syndrome type 2A, which doctors say means his hearing and vision will slowly fade. “We’re hoping to have some gene and stem cell therapy that will work for this condition in this child’s lifetime,” a University of Iowa expert said in an interview. (Rebecca F. Miller/The Gazette)
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Meet John.

He’s 6. He lives with his family on a small farm near Washington. He likes to fish — he can bait his own hook and take off his own catch. He likes to climb trees. He’s as solid as any 40-pound wrestler.

And he’s walking down a path that’s just as bright as it may be dark.

John’s losing his vision and his hearing. It’s happening slowly, and could take years.

So for now, John’s practicing single-leg takedowns and sprawls. He’s building elaborate train sets and playing “oranges and lemons” on the piano. He’s sitting on his dad’s lap to help drive the tractor.

“We want him to get as much experience in certain things as he can while he can soak them up,” his mom, Heather Koch, told The Gazette.

Because this isn’t a story about a medical miracle. It isn’t a story about some novel discovery sparking new hope for John and his family — although discovery is happening.

It’s a story about a more remarkable kind of hope. The kind that persists without groundbreaking advancements. The kind that isn’t reliant on good news.

The kind that lives in the stark reality that is theirs.

‘Gut-wrenching’

He was born Feb. 4, 2014, and he was perfect.

His older brother, Gus, had died five years earlier — at just 1 month old — on Jan. 8, 2009, from Zellweger syndrome, a rare congenital disorder. His older sister was born healthy in 2012. And John came after.

“He was totally normal,” Heather said. “Everything was fine. He passed his newborn hearing screen.”

But as John grew, she and her husband, Greg Koch, began wondering if something was off. As their 18-month-old John would go toddling down the hall in the predawn hours, Heather would try whispering him to her.

“I would poke my head out of my room and I would say, quietly because everyone else was still sleeping, ‘John, John, come here, come here,’ and he would keep on walking,” she said. “He just wasn’t hearing me.”

John would press musical toys hard against his ear — desperate to hear their song. He also wasn’t saying much besides “mama, “dada,” and “uh oh.”

So when John was 2, they drove him to Cincinnati Children’s Hospital and learned he had “moderate hearing loss.” Because the cause was unknown, the family had John checked for a catalog of possible culprits.

“Usher syndrome was on that list,” Heather said.

Results would take a while, so the family returned home and eased back into their routine. Until eight weeks later, on June 23, 2016, when Heather’s phone rang. She was by herself, on her way to meet her mom and sister to go shopping.

“It was gut-wrenching,” she said. “I called Greg right away, but there was no hiding it from my mom and sister a few minutes later. So they and the rest of our families all knew that day also.”

John had genetic mutations amounting to Usher syndrome type 2A.

“They said he will slowly lose his hearing and his vision for the rest of his life,” Heather said.

Before getting the news, she hadn’t done a lot of research on all the possible causes of John’s hearing loss.

“We’re not the panic type of people, so we were praying,” she said. “But not panicking.”

They carried that mentality with them into their new reality with John which, while devastating, enabled a deeper understanding of how they could support him.

Like giving him new hearing aids. The moment John put them in, Heather said, he could hear their whispers.

“He looked right at us,” she said, adding, “Those next few days were amazing. He could hear the chickens for the first time. And he could hear the airplanes in the sky for the first time. Big things for a 2-year-old.”

The family addressed his vision troubles with new glasses, which opened — to some degree — a new world for John, who spent the next two years in speech therapy learning to talk like everyone else.

“Once I caught seven fish in a row,” John told The Gazette, showing off his storytelling skills. “First cast, I caught one. Second cast, I caught one. Third cast, caught one. Fourth cast, caught one. Fifth cast, caught one. Sixth cast, caught one. Seventh cast, caught one. Like, I just cast it and reeled it in, and I just kept catching fish.”

‘hand you were dealt’

The Kochs don’t have a precise picture of John’s long-term prognosis, but University of Iowa ophthalmic genetics professor Arlene Drack said patients with Usher’s develop “retinitis pigmentosa,” a progressive type of vision loss, typically in their teens.

She reported a “huge amount of research going on at the University of Iowa and around the world on how to replace or repair the genes that cause (retinitis pigmentosa).”

“We hope there will be a gene therapy that will help John in the future,” Drack said.

World-renowned UI Institute for Vision Research Director Edwin Stone is among those paving the way in groundbreaking research that he believes will work for patients like John.

“We’re hoping to have some gene and stem cell therapy that will work for this condition in this child’s lifetime,” he said. “So I think that there’s reason for optimism.”

For now, Heather and Greg are heeding doctor warnings that John’s hearing and vision will continue to fade.

“They’re telling us it probably will,” Heather said. They’ve been given symptoms to watch out for — like trouble seeing at night.

“They said he will start stumbling over things in the dark, he won’t be able to see the stars in the sky,” Heather said. “And we have just started seeing some of the night vision symptoms.”

The guessing is hard. John might trip over something in the garage — and Greg will find himself wondering if it’s the syndrome.

“How do you know?” he said. “Is he a 6-year-old kid whose mind gets ahead of where his feet are going? Or is it part of it? You just don’t know what is what, and you’re always thinking, ‘Is that a sign?’”

They fight off paranoia with hope, like hope for expanding research and new technology, and by homing in on their vision for a “normal” life.

“He’s going to have some things to overcome,” Heather said. “Our job, I think, is to teach him to be tough enough to overcome those things. Because what’s the alternative?”

They acknowledged some things might be different or difficult for their son — like driving.

“So he drives now,” Greg joked about his co-pilot “in the field.”

But they’re not raising John differently from their four other kids — all of whom have tested negative for Usher’s.

And, being so young, John doesn’t yet know the full weight of his diagnosis.

“I think one thing we probably will say is, this is just the hand you were dealt. And it’s not going to stop you from living your life, or accomplishing anything, or being the best person that you can be,” Heather said. “We will expect the same out of him that we do of all our other kids.”

But they acknowledged, someday John will ask.

“And he deserves to know,” Greg said. “But it’s not fair to make a 6-year-old carry that burden in its full weight — like we have to carry it.”

And they do.

“If there’s a day where he’s displaying a symptom of something, that’s heavy,” Heather said. “So you just carry that on that day.”

But largely they stay optimistic.

“John’s going to have the best life no matter what,” she said, citing his involvement in a sport they believe will help make that possible. “That’s part of wrestling.”

‘You’re tough, bud’

John has been wrestling since age 3. And he’s good.

He goes to Husky Wrestling Club in Riverside and started going to Big Game Wrestling Club in North Liberty, too, around age 5.

“John is a light,” said Big Game Chief Executive Officer and Coach Dylan Carew. “His fight, courage, and personality are inspiring.”

The family is close friends with Mike Zadick, a UI All-American wrestler in the early 2000s who went on to have a successful international wrestling career.

Zadick was Heather’s neighbor when they both lived in Solon and, Heather said, “He and his family have become family to us.”

Having seen John in matches and meets, Zadick told The Gazette, he’s got a “natural feel” for the sport.

“At a young age, 4 or 5 years old, he had real natural movement,” Zadick said. “It was like, ‘Wow, look at this.’ You can tell when a kid has a natural feel.”

Not only is John well-suited for the sport, it’s well-suited for him — in the skills it instills might become particularly relevant as he ages.

“Wrestling, the word itself, it’s constant work and battle and struggle,” Zadick said.

Echoing that sentiment, Carew said, “Adversity is every day with wrestling.”

“So when it comes to someone with a challenge beyond normal, what better sport?”

John’s physical obstacles — which may be seen by some as disabilities — have actually given him an edge on the mat, according to Carew.

“You can tell he focuses a lot more to details,” he said. “He pays more attention because he needs to.”

John has to wrestle without his hearing aids or glasses — leaving him reliant on skills he’ll increasingly lean on as he loses more of his sight and hearing.

Without those supports, Greg before matches warns officials his son can’t hear them.

“They need to communicate with him by touching him and using hand signals,” Heather said. “He usually can’t hear the whistle.”

But largely, when John’s on the mat, he’s like any other kid. He’s fighting for take downs and escapes. He’s learning to accept losses — a skill that could become especially helpful as John matures into a braver understanding of the hand he’s been dealt.

“We’re going to make him as mentally strong as possible, so he can carry it to when he gets big enough to carry that burden,” Heather said.

Take last spring, when a 38-pound John found himself in the third-place bout in the kindergarten bracket at AAU Super Pee Wee State.

“I was so tired I almost fell asleep during the match,” John said.

Exhausted and hurting, he barely made it into his stance before the official signaled the start of the overtime period.

“But I kept wrestling, wrestling, wrestling,” John said.

From the side of the mat, Heather shouted, “John, you’re tough, Bud.”

And he was, holding off his opponent’s shot and getting behind him for two points, before jumping into his mom’s arms with a well-deserved sense of accomplishment — one sense he’s in no danger of losing.