OPINION — Baine Bobka was a 7- year-old boy who was born in Logan, Utah, and loved to swim, ride anything with a wheel, play with his friends and, most importantly, with his awesome family and dog, Howie. Baine loved to share. He had the cutest little face and always had a hug for you every time you saw him.
A few months after his seventh birthday Baine became sick with something they thought was the flu. After 12 hours, Baine could not walk or talk. He was rushed to Logan Regional Hospital, and after many tests, the doctor’s couldn’t find anything wrong as he slowly slipped into a coma.
From there, Baine was life-flighted to Primary Children’s Hospital. When they got to the hospital, they came to the conclusion that Baine had ornithine transcarbamylase, or OTC, deficiency. But it was too late. Baine’s ammonia levels were too high. Within a day Baine passed away.
OTC deficiency is the most common urea cycle disorder in humans. There are over 400 types of OTC deficiency, with an estimated prevalence of 1 out of 50,000-80,000 worldwide. The specific mutation Baine carried has been traced back seven generations to a Utah woman named Jane Wright Earl who settled in the Salt Lake Valley with her husband in 1852.
Since Baine’s death, they have found four family lines out of nine siblings who had offspring and have contacted everyone that they could within those four family lines. Baine’s family has also found that in Utah there are more mutations than what Baine carried.
Since Baine passed away, Baine’s family has raised awareness so no other family has to go through this. Baine’s family has started a Baine’s Legacy Foundation. At this website, you can find out more information about OTC, as well as search your genealogy or family search, and if you find that you are related to Jane Wright Earl, you would need to be tested for this as soon as possible.
With their mutated gene, only boys can die from it. If a male is a carrier, he will always pass it to his female offspring but never to his male offspring, because it is an X-linked gene. If a female is a carrier, she has a 50 percent chance of passing it to either male or female.
Baine’s family has made an educational video about Baine and OTC in hopes to share more knowledge and save a life. They also have started an event called Bring Light to Baine’s Legacy, which is held every summer in July in the small town of Lewiston, Utah. It is a glow-in-the-dark run with vendors, music and fireworks.
They hope to raise awareness in as many ways as possible and share the legacy of Baine and the awesome kid that he was.
Submitted by CHASE HOUSLEY, Richmond, Utah.
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