The nonprofit Sebastian Velona Foundation invites the Santa Clarita Valley community to the “Extend the Love – Fighting Blue for Batten’s Disease” fundraiser at Priscilla’s Salon in Valencia on Wednesday, May 9, from 10 a.m. to 8 p.m.
The salon will give a “fighting blue” hair extension to guests for a $10 donation, and raffles of additional donated items will generate funds for the foundation’s fight against Batten Disease CLN8, a very rare genetic disorder that affects children.
A reception from 5 p.m. to 8 p.m. will include a live DJ providing music, plus goodies from Nothing Bundt Cake and the Sweet Beams Ice Cream Truck.
Guests of honor will be Sebastian Velona, a Stevenson Ranch teenager who suffers from Batten Disease CLN8 and is the foundation’s namesake, and his mother, Teri Hughes Fox, a tireless advocate for her son and fundraiser for a cure.
Batten Disease CLN8 attacks and degenerates children’s nervous systems. Children lose the ability to walk, talk and see in a long, debilitating process. The disease is relentless, ruthless and usually fatal before adulthood.
There is no cure, yet.
Sebastian, nicknamed Sebi and now 14 years old, began suffering this debilitating neurological degeneration when he was a toddler. After years of misdiagnoses, specialists finally determined Batten Disease CLN8 was the cause of Sebi’s issue. But they had no cure.
Because the disorder is so rare, funding from large pharmaceutical companies for research into a cure has been slim to none.
That didn’t stop Fox and her husband, noted Valencia attorney Michael E. Fox.
The Fox family, fall 2017: Sebastian Velona, Teri Hughes Fox, Michael E. Fox, Skylar Fox and Gage Fox. | Photo: Stephen K. Peeples.
Searching for answers, they discovered that bio-researchers were experimenting with gene replacement therapy — replacing damaged genes with healthy ones — and achieving promising early results.
“A harmless virus is used to transfer good CLN8 genes to replace the defective gene,” he said. “When the good genes get injected via spinal epidural, the virus does what a virus does and it spreads the good CLN8 genes through the body. The defective gene still stays in the body, but becomes inactive.”
The Foxes determined a clinical trial and eventual approval of the therapy by the U.S. Food and Drug Administration would cost around $3.5 million.
So, the couple founded the Sebastian Velona Foundation (he is Teri’s son from a previous marriage) in early 2017. They arranged a series public fundraisers and secured private donations and grants to add to the foundation’s war chest. The Priscilla’s Salon event is the latest.
“The Velona Foundation has raised almost $2 million so far from fundraisers, grants and other donations, but we need to raise another $1 million,” Teri Fox said. “By us funding this, it will change the world on treating rare genetic disorders with genetic replacement therapy, so Sebi and other children can live.”
Doctors and genetic scientists worldwide are now working to develop gene replacement therapy to fight Batten Disease. Current research has shown promise of a breakthrough soon.
The race is on to find a cure, not only in time to save Sebastian Velona’s life, but also the lives of other children and adults who may benefit from breakthroughs in the treatment of rare genetic disorders the Velona Foundation seeks to fund.
Just in the past year, Sebi has suffered faster degeneration of his ability to speak and walk, seizures like the one he had on May 3 have become more frequent and severe.
Not one to tiptoe around the personal urgency, Teri Fox said, “If we don’t get this done fast, my kid is screwed.”
Once genetic researchers obtain FDA approval of the therapy, she said, “Other kids with CLN8 will be included in a clinical trial, along with Sebi, which could begin as soon as December or January. So, it’s a pretty miraculous thing.”
Supporters unable to attend Wednesday’s Pricilla’s Salon fundraiser but wish to donate toward research may do so at www.CureBattenCLN8.org, Teri Fox said.
“Please help us save the lives of Sebi and children with Batten Disease,” she said. “With your help, we can do this.”
Priscilla’s Salon is located at 27644 Newhall Ranch Road, #B-15, Valencia 91355, off Dickason Drive near Ice Station Valencia, Legacy Christian Academy and Valencia High School.
For more info, call Renee Kaehny at 661-618-5205, or visit the “Extend the Love for Batten Disease” Facebook event page.
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