ST. GEORGE — When the children of Lester and Noreen Jessop were officially diagnosed with the same terminal illness, Noreen Jessop was told to enjoy the time with her children, because they might not make it past 10 or 11 years old. However, this St. George family has found hope in an Oregon-based foundation that has what they believe is the best possible treatment for the disease.
Lester and Noreen have three children: Daron (10), Aubrey (4 1/2) and Angela (4 1/2). All three were diagnosed with pantothenate kinase-associated neurodegeneration, a rare neurological movement disorder. The disease is characterized by the progressive degeneration of specific regions in the central nervous system caused by brain iron accumulation, according to information from the National Organization of Rare Diseases.
Pantothenate kinase-associated neurodegeneration is marked by abnormal involuntary movements, alterations in muscle tone and postural disturbances. People with the disease lack a chemical necessary to metabolize a vitamin in the brain. Without normal levels of this metabolite, part of the brain degenerates, causing severe problems with walking, coordination, vision, speech and swallowing.
It typically appears in early childhood, though it can also appear in adolescence and early adulthood. In most cases, progression of the disease extends over several years, leading to death in childhood or early adulthood.
But a foundation out of Portland, Oregon, known as the Spoonbill Foundation has identified a compound which they believe may change the course of the disorder.
The foundation has been studying and caring for people with pantothenate kinase-associated neurodegeneration for more than two decades, and they have said laboratory test results have shown the following:
- When a mouse with pantothenate kinase-associated neurodegeneration is fed the compound in question, it fixes all signs (biomarkers) of disease in the brain.
- When the compound is fed to pantothenate kinase-associated neurodegeneration cells from humans, the abnormal biomarker levels change to match the cells from healthy people.
- Results have shown the need for only tiny doses to completely fix the signs of disease in the mouse brain, yet even very high doses of the compound cause no bad side effects in the mouse.
- The same abnormal biomarkers in the pantothenate kinase-associated neurodegeneration mouse brain are also abnormal in the blood of people with the disorder. This is important to the foundation because it gives an objective, biochemical way of measuring the disease and its response to drug treatments.
“We have met with the United States Food & Drug Administration and the agency is supportive of our plan to move the compound quickly into humans,” information from the foundation’s webpage said. “We anticipate that regulatory agencies in Europe and around the world will follow the FDA’s lead.”
The promising findings have given hope to the Jessop family, as well as families across the country who see the effects of the disease on a daily basis.
The Jessop’s oldest son, Daron, started showing signs of regression from the time he was a year old, Noreen Jessop said. He didn’t crawl until he was 13 months old and has never walked. The family spent 9 1/2 years doing all sorts of tests, but it wasn’t until June of 2017 that they got the official diagnosis that it was pantothenate kinase-associated neurodegeneration.
Both of the Jessop girls, twins Angela and Aubrey, showed similar patterns to their brother, and all three showed the same brain images indicating iron accumulation.
In the summer of 2017 when they received the diagnosis, the family was heartbroken, but now Lester and Noreen Jessop are hopeful that the new compound will be beneficial in treating the disease not only for their children but for all the families out there who are affected, they said.
“Our son’s whole attitude has shown signs of improvement,” Lester Jessop said, adding that Daron crawled for his physical therapist for the first time in two years.
“That’s hope,” Lester Jessop said.
But moving the compound into clinical trials for humans comes with a high price tag. In order to have a robust trial with as many participants as possible, the foundation estimates it will need $2 million in funding. For a lot of families, waiting for the drug has become a race against the clock, Noreen Jessop said.
Friends of the Jessop family, along with the Kalamity Dance Krew, have organized a benefit concert to be held Wednesday at Pine View High School at 6 p.m. The benefit will feature a bake sale, Hungry Howie’s dinner and concert featuring Kalamity Dance Krew, dancers from The Vault and other area performers.
Tickets are $10 at the door or by calling 435-216-8974. Proceeds go directly to helping the Jessop family raise money for the foundation.
“These are the most generous people you’ve ever met,” said Mike Stewart, a friend of the Jessop family. “They are real salt of the earth people.”
Stewart said that being around Lester Jessop, a member of the Southern Utah Home Builder’s Association with Prime Excavating, is like being with a celebrity: Everyone knows and loves him. This benefit concert is an opportunity to give back to a family who has done so much for the community, Stewart said.
Both Lester and Noreen Jessop said raising the funds is an opportunity to help all the families affected by the disease, especially those who don’t have the means or time to do it for themselves.
“It goes beyond just our kids,” Lester Jessop said.
A GoFundMe page has also been set up to help raise additional funds. Those who cannot attend the concert can donate to the GoFundMe page.
- What: Jessop Family Fundraiser Benefit Concert.
- When: Wednesday, Feb. 28, 6 p.m.
- Where: Pine View High School ,2850 E. 750 N., St. George.
- Cost: $10; bake sale and other purchases vary.
- Purchase tickets: By calling 435-216-8974 or at the door.
- GoFundMe: Give So They Can Live.