Visionaries: Hope in Focus marks decade battling inherited retinal diseases

Susette Tibus wasn’t looking for another cause to champion when she met Laura Manfre through a mutual friend, Elisse Rosen, in 2013.

Co-owner of a popular Mystic jewelry store and a force on numerous local boards and commissions, Tibus, now president and chief executive officer of Mystic Aquarium, had earned a reputation as a mover and shaker. Manfre needed help advocating for medical research.

“You have five minutes,” Tibus recalled telling Manfre as she peered at her over the top of her eyeglasses. “I asked, ‘Do you have any money? Are you a 501(c)(3)?’”

Manfre and her husband, Charles Priebe, had neither funding nor the tax-exempt status accorded charitable organizations. What the Ledyard couple did have, however, was a fierce commitment to taking on Leber congenital amaurosis, or LCA, the rare inherited retinal disease that could consign their daughter to blindness.

“When she told me about Sofia, I was not only moved, I asked how, as a community leader, could I help get the word out?” Tibus said. “Then I met Sofia. That put me over the edge.”

More than 10 years after that fateful meeting, the nonprofit the Priebes founded in 2014 has become a leading global advocate for families dealing with LCA and other inherited retinal diseases, or IRDs. Founded as Sofia Sees Hope and renamed Hope in Focus in 2021, the organization has raised more than $1 million and contributed more than $600,000 to research.

The Ledyard-based organization now has two full-time employees, Courtney Coates, director of outreach and development, and Katherine Kraines, communications manager.

“When we started, there was nothing for early-stage research,” Manfre said. “Now, there’s one treatment on the market for LCA (the drug Luxturna), and the disease has been related to 27 different gene mutations. Treatments for six of them are in trials.”

“The science has come so far,” she said. “There’s so much hope now, especially for younger people.”

That hope extends to Sofia, 21, whose vision was impaired at birth. Though legally blind, she’s led an active life, dancing and rowing during her Ledyard High School years. Now a senior at Goucher College in Baltimore, she’s majoring in sociology/anthropology, peace studies and French, and hopes to go into human rights. She’s studying abroad this semester in Strasbourg, France, accompanied by a guide dog.

“She’s wonderful, except when she walks too slow,” Sofia said Friday from Strasbourg.

In 2013, genetic testing confirmed Sofia’s LCA diagnosis and identified the mutated gene that caused her vision impairment. The Priebes are hopeful research will lead to a treatment that can at least arrest the further deterioration of her eyesight.

According to Coates, the Hope in Focus outreach director, the Bespoke Gene Therapy Consortium, a research initiative of the U.S. Foundation for the National Institutes of Health, has selected Sofia’s mutated gene for study. Sofia would be an eager participant in clinical trials of a potential treatment.

“If she could just hold onto the sight she has,” Manfre said. “Just halting the progress of the disease ― knowing it could not get worse ― would be huge. Her sight is changing all the time. Everything she does takes longer.”

Sofia said her eyesight has worsened quicker or more severely in recent years.

Dinner, blindfolded

Hope in Focus’ “Dinner in the Dark,” its biggest fundraising event of the year, is set for Saturday, Nov. 2 at Foxwoods Resort Casino. Tibus, her husband Chuck Sneddon, and the staff at Simply Majestic, their jewelry store, will be honored for their decade of support for the organization’s mission.

“They’ve been pivotal in getting us where we are,” Manfre said. “This year alone, they’ve raised more than $75,000.”

In previous years, “Dinner in the Dark” has raised about $200,000. The goal this year is $250,000.

The event’s namesake feature is the dinner itself, billed as a “a true culinary adventure,” in which a secret gourmet meal is served to blindfolded guests. The purpose, Manfre said, is not to replicate what it’s like to be blind, but to create an experience that encourages people to connect more deeply with Hope in Focus’ mission.

Sofia will appear at the event via a video she recorded before she traveled abroad.

The scheduled guest speaker is Paralympian Anthony S. Ferraro, who was born with LCA, wrestled in high school and college and won a gold medal at the 2022 USA Judo Blind and Visually Impaired National Championships. He’s the subject of a 2017 documentary “A Shot in the Dark,” which chronicled his life as a high school wrestler.

For more information about “Dinner in the Dark,” visit hopeinfocus.org/get-involved/dinner-in-the-dark/.

Prior to the fundraising event, from 1 to 3:30 p.m. Nov. 2 at Foxwoods, the Foundation Fighting Blindness, on whose board of directors Manfre serves, will conduct a seminar on inherited retinal diseases, including a panel session with experts, a conversation about living with IRDs and a discussion of the role of patient advocacy in advancing treatments.

Anyone interested in attending should email Amanda Bement at ABement@FighingBlindness.org.

b.hallenbeck@theday.com