Riding to Rocky steps for rare disease

A father and son from West Chester are trying to raise awareness about an extremely rare disease and help researchers uncover more about it.

Not many doctors have treated a patient with Bardet-Biedl Syndrome, and few people know how to pronounce the disease (bar-day beed-el). BBS is a genetic condition that can cause a wide range of symptoms, including blindness, obesity, polydactyly (being born with extra fingers or toes) and intellectual disabilities.

The syndrome affects about 1 in 150,000 newborns, according to the U.S. National Library of Medicine, and only a handful of families in the greater Philadelphia region have a child with BBS.

Nearly 10 years ago, Tim Ogden and his 15-year-old son, Nathaniel, helped establish the Bardet-Biedl Syndrome Foundation, which they say is the only nonprofit in North America dedicated to the disease.

On Saturday, the pair will mount a tandem bike and ride 30 miles from Chester County’s Exton Park to the steps of the Philadelphia Museum of Art in the fourth Rocky Ride for BBS research.

Nathaniel may have been the first person diagnosed with the disease before birth, Ogden said. A Children’s Hospital of Philadelphia doctor detected BBS while he was in utero.

“I have full retinal degeneration and am almost fully blind,” Nathaniel told Metro. “I had hunger all the time, but I got medicine for that. I also had some extra fingers and toes.”

By sixth grade, he had mastered Braille, which he uses for most of his reading and writing as a sophomore at Henderson High School in West Chester.

Ogden said Nathaniel wasn’t able to take his first steps until he was 4 and, then, only with the help of a walker. Building muscle is difficult for him and others with BBS.

In the weeks leading up to the Rocky Ride, Nathaniel and his dad ramp up from 5 to 25 mile rides.

Five other tandem bike teams and a total of 23 riders have signed up to join them Saturday. Ogden said the foundation is hoping to raise $20,000.

Most of the money will be donated to a clinical registry that documents individuals with BBS and collects their health information to learn more about the disease.

“You still need the same amount of money to do the research on a rare disease as you do on a common disease,” Ogden said. “But it’s a lot harder to do and that’s why we’ve been so focused on making sure that we can do this again this year.”

Last year’s Rocky Ride was canceled due to the coronavirus. The father-son duo, inspired by the “Rocky” films, first completed the 30-mile trek in 2017.

“(Rocky) knew he was going to lose, that he was going into a battle that was much tougher than he could ever overcome by himself,” Ogden said. “But he wanted to do it anyway.”

“I am absolutely a huge fan of that message,” he added. “Certainly for Nathaniel and I, this is a battle that matters, and we can’t do it alone. We’re not going to win all on our own. We may never win.”

It’s not too late to ride along with Ogden and Nathaniel or to cheer them on as they run up the Art Museum steps. They expect to arrive on Parkway between noon and 12:30 p.m.

For more information, go to www.bardetbiedl.org/rockyride.