Designing a Plan for Drug Discovery in Rare Pediatric Neurodegnerative Disease
by Danielle Kerkovich, Ph.D. and Amy Drew
published July 21, 2011
Editor’s Note: There are currently no cures for neurodegenerative diseases, including Batten disease, a rare and fatal disorder affecting young children. While researchers have made headway in preventing genetic disorders through preconception carrier screenings and have found potential drug targets, the gap between basic research and clinical treatment development remains. To overcome this gap, write authors Dr. Danielle Kerkovich and Amy Drew, researchers in academia and the pharmaceutical industry, supported by government agencies and nonprofit institutions, must come together to share expertise and promote translational research.
To read the full article, click here.
Father Fighting to End Batten Disease
Craig Benson and the Beyond Batten Disease Foundation were recently featured on My Fox Austin. Click here to view the segment.
published on July 1, 2011
Run to the Sun Relay benefits children with Batten Disease
By KVUE staff on KVUE.com
Published May 10, 2011
Batten Disease is a rare and fatal neurological disease that begins in childhood.
Craig Benson is a co-founder of the Beyond Batten Disease Foundation and Lance Thompson is a chairman of Run to the Sun Relay, which raises money for research in to Batten Disease. They spoke to KVUE.
Family starts foundation, raises money to fight Batten disease
by Andrew Ball in the Austin American Statesman
published May 8, 2011
Craig and Charlotte Benson had never heard of Batten’s disease before their then-5-year-old daughter was diagnosed with it in 2008.
But when the Austin couple learned Christiane was afflicted with the fatal illness, they threw themselves into finding a cure. In today’s column, Craig Benson writes about the nonprofit group he and his family started: the Beyond Batten Disease Foundation.
What is the Beyond Batten Disease Foundation? The foundation’s mission is to eradicate Batten disease by raising awareness of it and generating funding for research to cure and prevent this deadly illness. Batten disease is a rare, degenerative and fatal neurological disorder that children can inherit if both parents carry the genetic mutation for it. It strikes without warning, typically between ages 5 and 10. It first causes vision loss and seizures, then progressive impairment of cognitive and motor capacities, then death during the late teen years or early 20s. There currently are no treatments or a cure.
Why was the foundation started? Because Batten disease is rare (1.2 out of every 100,000 live births worldwide), there is very little federal funding for research on treatment and prevention. So we established the foundation to raise money for researchers to develop a cure and a test to screen for the genetic mutations that cause Batten disease and more than 600 other serious — and often fatal — conditions that kids can inherit. The hope with the test is to eliminate these devastating diseases in future generations. This strategy has proved successful on a small scale with Tay-Sachs disease.
What do you spend your money on? Ninety-three percent of the money we raise — a huge percentage by nonprofit sector standards — goes toward programs (versus operating costs) to accelerate the discovery of a treatment and the test that screens for genetic mutations. The foundation gave a $500,000 research grant to Texas Children’s Hospital and very generous friends of ours donated another $2 million. We’ve also invested $1.5 million with the National Center for Genome Resources to develop the carrier-screening test.
Talk about your Run to the Sun relay. The overnight relay run starts Saturday at 2:30 p.m. atop Austin’s scenic Mount Bonnell and finishes at sunrise May 15 at Enchanted Rock State Natural Area. That morning there will be a 6 to 8:30 a.m. breakfast celebration featuring live music by the Gospel Stars. About 30 teams, each with up to eight members, have registered to run the 95-mile course through the Texas Hill Country. Each team has pledged to raise a minimum of $5,000.
Is there still time to participate? Interest in Run to the Sun has been overwhelming. We’ve already exceeded our projected number of teams and runners. There’s still time to register a team, and you can make a donation or come out to cheer on the runners. Our biggest need at this point is for volunteers, so we encourage anyone who has time next weekend to contact us through the website (http://runtothesunrelay.com).
What do you see for your future? Every day is a blessing and we cherish each moment with our precious Christiane and our son, Garland. At the same time, we know that the years ahead will be challenging for her and our family. Nonetheless, we remain very hopeful as we raise money to accelerate research for developing a treatment for Batten disease and the screening test. We will find a treatment for Christiane and other affected children and ultimately eradicate Batten disease.
Run to the Sun
A year ago, Lance Thompson, our good friend and avid runner, came to us with an idea to organize a 100-mile overnight relay run to raise money for the foundation. Admittedly, I’m not a runner, and my first reaction was, “Who in the world is going to want to do that?!!” But as the idea evolved, and he shared his vision of a race whose course would meander under the starlit sky of the Texas countryside and culminate at a stunning destination at sunrise, I began to understand. Now, less than two weeks away, that vision will become a reality on May 14 when 30 teams of eight people each will compete by running a rugged Hill Country 96-mile course starting at Mount Bonnell and ending in the dawn light at Enchanted Rock. Members of each team will follow the route together in a van to support their runner and cheer him on as he steps onto the course alone to face his own unique challenge in the dark. Lance wanted the participants to experience first-hand the physical darkness and challenges that a child who is blinded by Batten Disease faces. It has been transformative to watch Lance’s ambitious dream become a reality and I am struck by how incredibly this race also mirrors our own life, and so perfectly mimics the mission of the Beyond Batten Disease Foundation. I love the way God inspires us only later to reveal His full intention.
Get Movin’ Monday: Beyond Batten Relay
On KTBC Fox 7
Published on May 3, 2011
Ryon Talbot of Pure Austin Fitness and Lance Thompson, a participant in the event, stopped by Good Day to talk about the Beyond Batten Relay.
Batten disease is a fatal neurodegenerative disorder that takes away childhood, then takes away the child. The Beyond Batten Foundation was established to eradicate Batten disease. They seek to accomplish this by:
- Raising awareness and money
to accelerate research and finda cure - Develop an easy and inexpensive blood test to detect the gene mutations for Batten’s and hundreds of other rare conditions like it.
Event details:
Run to the Sun
90-plus miles from Austin to Enchanted Rock
Relay teams of 8
Goal: to raise $125,000
http://www.myfoxaustin.com/story/18298409/get-movin-monday-beyond-batten-relay
Relay Will Help Fight Batten Disease
On KTBC Fox 7
Published on April 25, 2011
The Run to the Sun Relay is a 90 mile race from Mt. Bonnell in Austin to Enchanted Rock State Park to raise money to fight Batten Disease.
Batten Disease is a neurological degenerative disorder that is hereditary.
Craig Benson, whose daughter has Batten’s Disease, joined Good Day Austin to describe what it is like to live with somebody fighting the disease.
Suzane Kho, the director of Beyond Batten Disease Foundation, also spoke with Good Day’s Keri Bellacosa.
http://www.myfoxaustin.com/clip/7223451/Relay%20Will%20Help%20Fight%20Batten%20Disease
Rare Disease Day
In honor of Rare Disease Day, the following retailers and businesses have signed on to donate a portion of their proceeds on February 26, 2011 to the Beyond Batten Disease Foundation:
In Austin, Texas:
1379 Family Sports Shop
Adelante Boutique
Austin Pilates Barn (Feb. 24 and Feb. 25 only)
Bella-Mi
bettysport
Briley’s Upholstery Shop
Corefit Training
Four Hands Furniture
Girl Next Door
Hayden Avery
Hutson Clothing Co.
Kids Cook
Mellow Johnny’s
The Menagerie
Monogram Lady
Over the Rainbow
Perri Berri
Reform Pilates
Tarrytown Nails
The Tavern (922 W. 12th St.)
Teo’s
Touch of Sass
Tracy Bethel Skincare
Valentines Too
Wendow Fine Living
Wildflower Organics
In Dallas, Texas
Chick-Fil-A at 12120 Inwood Road
Pinkberry at 5959 Royal Lane
Sammy’s BBQ
In Houston and The Woodlands, Texas:
Campioni Restaurant
Sweet Lola Yogurt Bar
Thompson + Hanson
Yvette Williams, Mary Kay Sales (Feb. 20 to Feb. 26)
www.marykay.com/ywilliams2.com or 281-686-1301
In Baton Rouge, Louisiana
By Design Interiors (on 2/25 only)
Fireside Antiques
Paper-N-Things
Red Onion
Samir Oriental Rugs
Stephen Black Ltd.
Taylor Clark Galleries (on 2/25 only)