12-02-2025
8-year-old in Stokes County fights rare brain disease
WALNUT COVE, N.C. (WGHP) — To know Bentley Shelton is to love him.
'He brings light to everybody that meets him,' said Brittany Shelton, Bentley's mom.
'He always smiles.'
At 8 years old, Bentley faces more challenges than most kids his age. But it wasn't always that way.
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'He was born like a normal kid. He started walking at age one,' said Shelton.
'For a year, he climbed on top of things, he'd climb up on tables and everything. And then he just kind of started being clumsy and when I went back and looked at videos, and I knew what to look for I noticed signs; his knees would hyperextend, his ankles were not 90 degrees, and it was causing him to just not walk properly.
Six weeks after those initial signs showed up, Bentley was diagnosed with Krabbe Leukodystrophy, a rare genetic disorder that causes progressive damage to the nervous system. Bentley is one of nearly 30 million Americans who live with a rare disease.
'He has to fight really hard to maintain what he has,' said Shelton. 'Every day is a constant struggle.'
There is no cure for Krabbe, and treatment is limited to a stem cell transfer which Bentley underwent in Pittsburgh right after he was diagnosed.
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'He has essentially lost his ability to walk. He loves to try so we help him, but he can't do it unassisted,' said Brittany. 'It did affect his vision. He was diagnosed with cortical visual impairment which I've kind of understood to be your eyes work fine, but your pathway doesn't. So, he looks at something and he can't process it… everything is just jumbled.'
In late January, the Shelton family received another blow. A promising clinical trial involving gene therapy that Bentley was waitlisted for was abruptly canceled.
Brittany turned to Facebook to share the devastating news.
'I think the more noise you make, the more people become aware,' said Brittany. 'That's really the only thing you can do.'
Her post has been shared hundreds of times, and she's heard from people who work with pharmaceutical companies offering advice. 'I was just praying that someone would see it… that a pharmaceutical company would see it and take interest and see that these are real kids out there that have it and hopefully pick it back up and restart it.'
It feels like a relentless race against time that Shelton is determined to help Bentley win.
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'Science tells us you don't live a full life, but you know God tells us another thing, so we're hoping and praying for other treatments to become available,' said Shelton. 'It's a rare disease but it's one in 100-thousand and when you compare that to the whole population, that's a lot of kids. That's brothers, sisters, sons and daughters.'
The Krabbe Disease clinical program by Forge Biologics was scheduled to run until November 2026. FOX8 reached out to Forge Biologics to find out what led to the program's cancellation, but we haven't heard back.
Krabbe disease is not on the newborn screen in North Carolina. It is, however, part of newborn screenings in several other states including South Carolina and Tennessee.
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