Idaho Family Saving Lives After Son’s Death From A ‘Silent Killer’

Sep 19, 2018, 4:31 PM | Updated: 9:24 pm

FRANKLIN, Idaho – Imagine walking around with a deadly genetic disorder and not knowing it. That may be the case for descendants of a Utah family.

In the quiet, picturesque town of Franklin, a family made a heartbreaking discovery.

Two days before Thanksgiving of last year, 7-year-old Baine Bobka got sick.

Kelsey Bobka of Franklin Idaho cherishes the wall of family photos behind her. Her son Baine died of a genetic disorder last Thanksgiving. Now she hopes to save lives.

“It was a Tuesday night in the middle of the night,” said Kelsey Bobka, Baine’s mother. “And we had all had the flu, and he had been with cousins and the youngest had had the flu when he was with them so it just wasn’t surprising when he came in and said, ‘Mom, I feel sick.'”

But the next morning, Baine’s symptoms were much worse.

“He was lying on the couch and he couldn’t even talk to me,” Bobka said.

In the emergency room, doctors did tests, and then flew Baine to Primary Children’s Hospital in Salt Lake City. That’s where they discovered his ammonia levels were dangerously high, damaging his brain.

Baine Bobka, age 7, died of a genetic disorder no one knew he had. Now his family fights to save lives to honor him. (Photo Courtesy of Kelsey Bobka)

“I remember just sitting there thinking, ‘I know he won’t be the same, but I want him back,'” Bobka said.

Baine had a rare, genetic disorder called OTC deficiency, which causes too much ammonia to accumulate in the blood.

He did not survive.

Since Baine’s death, scientists have identified the genetic mutation for OTC. So far, about 35 family members have been tested. Of those, about half are positive.

Baine’s type of OTC deficiency goes undetected at birth, and lies latent until a trigger sets it off, like fasting or in Baine’s case, an illness.

Baine Bobka was a 2nd grader in Franlkin

“It is a silent killer,” said Dr. Nicola Longo, University of Utah Health.

Longo is following several branches of the family in Utah, and believes the genetic disorder is fairly prevalent here.

“These are very normal people who do just like all of us who do their own thing, and all of the sudden something tragic can happen within a family,” Longo said.

The disorder can be traced back to an immigrant family on the Mayflower who brought it to Utah.

“Jane Wright Earl. Yes,” said Sally Tarbet, Baine’s grandmother.

It’s linked to the X-chromosome. Girls are carriers, but boys can die from it.

The family has started an awareness campaign on social media: Baines Legacy on Facebook, Baines Legacy on Instagram and Cure UCD.

They also held a fundraiser to raise money for research.

“I feel like there’s a reason for everything, and as much as I hate it and don’t like it… and then I can’t imagine another family going through this, you know?” Bobka said.

All to save lives, and honor the boy they loved.

The warning signs of OTC deficiency include a family history of unexplained male deaths, and unexplained vomiting in children.

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Idaho Family Saving Lives After Son’s Death From A ‘Silent Killer’