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Austin foundation battles Batten disease

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Christiane Benson adores her Labrador retriever, Queenie. The Austin girl loves her brother, Garland. Christiane’s father, Craig Benson, dotes on her. Sounds like a pretty normal childhood, right? For the most part, yes.

What may not be readily apparent about Christiane is that she’s legally blind because of something called Batten disease. It’s an extremely rare, neurodegenerative genetic disorder whose first symptom is vision loss. Craig said 9-year-old Christiane embraces her blindness – she “gets” to have a seeing-eye dog and she “gets” to learn Braille.

Craig said his daughter “has accepted her vision loss with a grace and a positive attitude that anyone would aspire to.”

Craig and his wife, Charlotte, have come to accept their daughter’s diagnosis. In fact, they’ve done much more than accept it. They’ve taken on Batten disease by forming the Austin-based Beyond Batten Disease Foundation. Its two goals: Raise awareness and money for research toward a treatment and cure, and develop an inexpensive blood test to detect genetic mutations for Batten disease and hundreds of other rare and devastating, often fatal, childhood conditions.

Craig and Charlotte established the foundation in August 2008 after learning that Christiane had Batten disease.

“There was so much pain, fear and devastation we experienced when we received Christiane’s diagnosis that at first we wanted to just curl up and shut out the world,” Craig said.

Far from shutting out the world, the Bensons have invited the world to join them in their mission. On Friday, country musician Keith Urban will headline a show at ACL Live at the Moody Theater in downtown Austin to raise money for the foundation. Co-chairs of the Keith Urban event are Sabrina Brown and Shannon Janek. Earlier country-themed fundraisers featured Jack Ingram (2011) and Kelly Willis and Bruce Robison (2010).

Craig said the Keith Urban concert “is not all about the one night. With the visibility and awareness created by this event, there are already donations and funding opportunities that have been brought to our attention which will be coming through … in the months after the event itself.”

The money will help tackle a perplexing disease that affects an estimated two to four of every 100,000 children born in the United States. The progressive disease starts with vision loss. Over time, total blindness sets in, as well as seizures. Gradually, mental and physical abilities deteriorate. Batten disease is always fatal, typically by a person’s late teens or early 20s. The disease is named after the British pediatrician who first described it in 1903.

“There is currently no treatment or cure,” Craig said. “It literally takes away the childhood, then takes away the child.”

In the 3½ years since the foundation was born, it has directly or indirectly funded more than $3.5 million in research regarding Batten disease, said Craig, who is president of Austin-based biomedical company Myriad RBM Inc. The heart and soul of that fundraising is Craig and Charlotte Benson’s little girl.

Craig said Christiane “has certainly been an inspiration to Charlotte and me, and the work of our foundation will be her legacy – and one that will outlive each of us.”

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