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ST. GEORGE — Lester and Noreen Jessop were devastated when they learned their three children were diagnosed with a rare, often fatal genetic disease during the summer of 2017.
“It was pretty emotional,” Lester Jessop said, recalling the moment they learned their son, Daron, who is now 9, and their 4-year-old twin daughters, Angela and Aubrey, had been diagnosed with Pantothenate Kinase-Associated Neurodegeneration (PKAN), a rare, neurological movement disorder.
However, with the help of doctors in Oregon researching to find a cure, there’s hope all three children may be able to live long, healthy lives. Now, the family's community is rallying around them to help the children get the medical help they need.
The issues began a few years before the diagnosis. Daron began having medical issues in 2013, after turning five years old. By the time the diagnosis was made in June 2017, the disease already began ravaging his body. He lost mobility of his muscles and needed a feeding tube to eat.
The disease affects a person’s ability to walk, coordinate, speak, swallow and see, according to the Spoonbill Foundation. The couple was told there was no cure and their children likely wouldn’t live past 10 to 12 years.
“The way they went about (it) was like, ‘OK, so take time and enjoy your kids because they usually don’t stay alive for real long,’” Noreen Jessop said.
PKAN affects about 35-50 percent of people in the world who have a Neurodegeneration with Brain Iron Accumulation disorder (a disorder based on abnormal amounts of iron in the brain), according to the NBIA Disorders Association. The association said an estimated one to three out of 1 million people have an NBIA disorder.
The Jessops wanted to know more about the disease killing their children. They reached out to an online support group and found other families with children across the world suffering from the disease. Instead of accepting the death sentence described by doctors, they began searching for solutions.
Another parent in the group referred the family to Dr. Susan Hayflick and Dr. Penelope Hogarth at the Oregon Health and Science Hospital in Portland, Oregon. Hayflick is one of the leading researchers of the disease and both doctors are a part of the Spoonbill Foundation, which is seeking to cure the disease.
The family traveled to Oregon in January hoping for any help and left with encouraging news. They learned the doctors had potentially found a cure that may not just stop the disease from progressing, but also reverse its effects.
The doctors, along with Ody Sibon, a doctor based in The Netherlands, announced in November that they were working with a pair of companies to create enough of the drug for a clinical trial, according to NBIA Disorders Association.
It was the best news the Jessops could have received, and one they hope can save others with the disease.
“It’s been a plead call for parents all over the world because their children are fading daily,” Noreen Jessop said, referring to parents she’s connected to across the globe whose children have died or remain in hospitals. “There are literally hundreds of children that are barely surviving, barely functioning. They’re going into the hospital for a new procedure every other month. It’s very intense when you get to know these kids.”
However, it comes with a steep cost. The Spoonbill Foundation is currently seeking $2 million to fund the clinical trial and a little more than $250,000 had been raised for the project as of January 2018, according to the foundation’s website.
That cost is where the St. George community decided to find a way to help.
Those who knew the Jessops began communicating trying to find ways to raise money. A GoFundMe page* was set up for the family, and a show by the Kalamity Dance Group planned to donate all proceeds from a benefit concert Wednesday night to the Jessop family.
“We’re just super grateful. I couldn’t be more grateful ... It’s been a miracle.” - Lester Jessop, on community efforts to help his family
Other private donations and events to raise money were also in the works, according to Mike Stewart, a family friend. He said the response is just an example of what the family has meant for to those in the community.
“(They) are absolutely salt-of-the-earth good people,” Stewart said. “I’ve seen (Lester) give a job to more people — put a roof over people’s head. He’d give the shirt off his back to a total stranger, so I feel like it’s our turn to try and help them raise some money for medicine for their kids.”
The couple said they were blown away by the support, including with calls they would get from concerned friends while they were in Oregon in January. While the children's futures remain uncertain, there is at least a hope that once didn’t exist.
“We’re just super grateful. I couldn’t be more grateful,” Lester Jessop said, searching for words to describe what the community help has meant for him and the family. “It’s been a miracle.”
“People have been so supportive and incredible,” Noreen Jessop added. “There’s really no words for it.”
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