Keith Urban lends talents to raise money for Austin-based foundation aimed at curing rare genetic disease affecting children

by Meredith Rainey in CultureMap Austin

published February 6, 2012

Our job as parents is to protect our children. So what do you do when you find out you unknowingly passed a rare and incurable genetic disease on to your only daughter? If you are Charlotte and Craig Benson, you catch your breath, lean on your faith and after the initial shock fades some, you choose to tackle the greatest challenge of your lives head on — you form a foundation to find a cure to try to save your daughter’s life, and the lives of hundreds of other children confronting a fate no child should ever have to face.

The Bensons, who founded the Beyond Batten Disease Foundation in 2008 after their then 5-year-old daughter Christiane’s Batten Disease diagnosis, have a lot they’d like to accomplish this year, including launching a screening test for Batten Disease and hundreds of other rare genetic diseases with devastating effects.

Their foundation has enlisted the talents of country music superstar Keith Urban to help raise money for and awareness of the cause they hold so dear to their hearts. Just in time for Valentine’s Day, Urban is headlining the organization’s annual fundraiser on Feb. 10 at ACL Live at The Moody Theater.

“Date Night with Keith Urban” features a VIP party, a live auction and of course a stellar concert crafted by an artist whose long list of awards includes being named CMA Entertainer of the Year.

The money raised from the event will help launch the genetic screening test the foundation helped pioneer. It will also go towards the Benson’s ultimate goal of finding a cure for the fatal neurodegenerative disease that’s taking their daughter’s sight and currently has no known treatment.

“Hopefully over the next generation we’ll see a reduction in incidence of all of these [diseases] and I think that would be a great legacy for us and for Christiane,” Craig Benson says.

Charlotte Benson says Christiane was a beautiful baby, complete with ten fingers and ten toes. The proud parents describe the day she was born as the happiest day of their lives. They never would have imagined this perfect little girl may not live to graduate high school or have a family of her own.

“She had a perfectly normal birth and we had every reason to believe that she would be a perfectly normal child. And then, when she got into kindergarten, when she started to read, she put books really close to her face and so we thought she just needed some glasses. So we took her to the eye doctor, and then one appointment led to another and we ended up in the neurologist’s office. Six weeks later, we had a diagnosis of Batten Disease,” explains Charlotte.

Batten disease is an inherited disorder of the nervous system that is fatal. Early symptoms can appear between age 5 and 10 and may start with vision problems and seizures. Children with Batten Disease eventually go blind and become physically and mentally incapacitated; they don’t live past their teens or early 20s.

That kind of news would make many parents want to crawl into bed and stay there. A few months after the devastating news, the Bensons made a decision: they weren’t going to let this disease define them — they were going to try to define it.

“There were a couple of evolutions of this. Obviously at first, we didn’t want to do anything. You know, the news kind of knocks you down for a bit. I think as we started sharing our story with close friends and family, I think that’s where we got some of the support and strength that we got initially from our faith… we started getting the grasp that there was maybe something we could do to help this,” Craig says.

Charlotte adds, “When we first started the foundation, it’s the absolute horror of a doctor telling you to go home and there’s nothing you can do about it except make your child comfortable and watch the story unfold. And so, as a parent, your instinct is to do something to try to change this and to make a difference not only for her but for so many other kids who are stricken with the same disease.”

The Benson’s say they soon realized they wouldn’t be alone in their mission.

“We found ourselves surrounded with an incredibly golden circle of friends with enormous talent, intelligence, resources and contacts. We’re in a really unique position not only with the friends and community surrounding us. That coupled with Craig’s position as CEO of Rules Based Medicine and all of his contacts to every brilliant medical doctor and scientist, those two things combined made it a natural fit to try to go forward and do something to make a difference,” Charlotte says.

With the support of their family, friends and neighbors and Craig’s connections in the biomedical industry, the Bensons had what they needed to start their mission to find a cure for their Christiane.

They named their foundation the Beyond Batten Disease Foundation, not knowing at the time how appropriate that title would be. Work funded by the foundation has resulted in the creation of a test to screen for not only Batten Disease, but also for approximately 600 other rare genetic diseases. Though the diseases may be rare themselves, collectively they affect thousands of children.

With a simple cheek swab, couples can be screened to find out if they are both carriers for a long list of genetic diseases they may pass on if they have children.

“Part of our mission has certainly resonated with everyone because it does affect everybody,” Craig says. “These rare genetic mutations that obviously affect us — everyone has some. We knew it was very critical early on for us to reach an audience outside of this small group of  500 families in the US that are directly affected [by Batten Disease].”

He says an added benefit is that royalties from the screening test will provide a revenue stream to fund the foundation’s other projects. The Benson’s say while the screening test, which is currently undergoing beta testing before being launched nationally, is a great start, their ultimate goal is to help eradicate Batten Disease — to find a cure for their little girl.

Christiane is now nine years old and in the third grade. Her parents are trying to keep life for her and her younger brother Garland as normal as possible, while managing her symptoms and taking things the only way they can —  as they come.

“[Christiane] knows she has Batten disease. She knows that she’s going to be blind and we told her that that there are some other things that come with it, but we’re sort of crossing that bridge as her symptoms reveal themselves,” Charlotte says.

The Bensons cherish every day and every moment they have with Christiane, while trying to stay hopeful. Charlotte says the horrible diagnosis they got four years ago changed their perspective and their paths forever.

“It’s strengthened our faith. We’ve seen the goodness in people that we’ve never known before, and seen a community of people surround us and empower us on our mission. You never take a single moment for granted with your children – you live for today.”

If you’d like to be part of the biggest fundraiser of the year for the Beyond Batten Disease Foundation and want to see what makes Keith Urban such a sought after entertainer, you’ve got your chance on Feb. 10 at The Moody Theater.

http://austin.culturemap.com/newsdetail/02-06-12-21-38-keith-urban-lends-talents-to-raise-money-for-austin-based-foundation-aimed-at-curing-rare-genetic-disease-affecti/

Date Night with Keith Urban Auction Preview

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LIVE AUCTION PREVIEW

Formula 1 Package

  • 2 premium reserved seats for all three days of the Formula 1 United States Grand Prix™ with on-site parking pass
  • 2 VIP reserved tickets to a concert of choice in the Grand Plaza Amphitheater
  • A “Hot Lap Lesson”: 3 laps on the full Circuit of The Americas Grand Prix course with a professional driving instructor.Instructor will provide driving tips between laps in an effort to improve your performance.Lap times will be recorded so you can compare your times to those of Formula 1 drivers. Includes photo at finish line

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Girls’ Dream Package

  • 4 Lake Austin Spa Resort’s “Full Day Away” Spa Packages. East guest may choose from 2 rejuvenating 50-minute package spa treatments and one indulgent 80-minute package spa treatment. Package includes spa lunch, spa gift and gratuities
  • One Chanel Mademoiselle bag
  • Full makeovers for 4 at Ron King Salon. Package includes cut, color, makeup, and shellac manicure for 4 guests
  • A $2,500 gift certificate to The Menagerie, Austin’s premier fine jewelry, gift, and tabletop salon
  • Private Luncheon for 4 with a Fashion Presentation at Neiman Marcus Austin and 4 $250 gift certificates

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Cabo Luxury Home

  • 6 day and 5 night stay at Casa Malibu in Pedregal, Cabo San Lucas, Mexico. Click HERE for more details on the luxury home
  • Gated, guarded community
  • 5 bedroom, 5 bathrooms plus 2 half baths
  • Sleeps 12-14 and perfect for 2 families or 5 couples
  • Flat Screen TVs, Satellite TV, Wireless Internet
  • Pool, Jacuzzi, Outdoor Bar
  • Dates of stay will be arranged through the property owner. The package excludes Easter (April 2-9, 2012) and Thanksgiving (November 19-26, 2012) dates

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Louisiana Alligator Hunt

  • A thrilling alligator hunt and saltwater fishing adventure in the Louisiana bayou for up to 8 people
  • Package includes a 4 day and 3 night excursion, accommodations, some meals, guide service and alligator hunting tags
  • Great for a weekend with friends or a father and son adventure!
  • Dates of the package must fall within Louisiana’s alligator hunting season and are to be arranged through Beyond Batten Disease Foundation

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Jack Ingram Private Concert and Dinner in Austin

  • Country music star Jack Ingram will perform a private, intimate concert for you and your friends in your Austin area home
  • The package includes a catered gourmet dinner from 34th Street Cafe and flower arrangements from David Kurio Designs

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Meet Keith Urban

  • A once-in-a-lifetime opportunity for you and a friend to go behind the stage, meet, and have your picture taken with Keith before the concert on February 10, 2012
  • What could make this package even better? One autographed guitar with a personal message from Keith!
  • Note: Must be present at the event to participate

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Aspen Mountain Paradise

  • 7 day and 6 night stay at a new, gorgeous home located in Snowmass Village. Click HERE for more details on the home
  • Ski access via a trail out and down the street in (limited to ability)
  • 4 bedrooms and 4 private baths comfortably sleeps up to 10 people
  • Fireplace, hot tub, Jacuzzi and steam showers
  • Large outdoor deck areas for entertaining and enjoying the spectacular views
  • Large flat screen TVs and wireless internet connection
  • Dates of stay to be arranged with the property owner. The package excludes Christmas, Thanksgiving, Spring Break, and Fourth of July dates

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UT Austin President’s Package

  • 2 VIP tickets to the President’s Suite for any home game of your choice! A Longhorn football games is the hottest ticket in town, and the President’s Suite is the best seat in the house. President Bill Powers invites the high bidder and their guest to a Longhorn home football game in the 2012 season
  • Reserved VIP parking
  • The package also includes 2 Southwest Airlines round-trip airfare tickets!

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Texas A&M Chancellor’s Package

  • Be the Chancellor’s guest at the Texas A&M vs. LSU game on October 20, 2012 and receive 2 VIP tickets to sit in the Chancellor’s Box, 10th Floor, Kyle Field
  • Package includes the Presidential Pre-game Buffet and one parking pass
  • Tickets and parking pass will be mailed to recipient after September 1, 2012

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Snowmass Village Getaway

  • 7 day and 6 night stay in a lavish, 6-bedroom mountain home near Aspen
  • Spacious home accommodates up to 10 people
  • 2 cars are available during your stay and shuttle transportation to and from the airport will be provided
  • Gourmet kitchen, a fitness room and a full game room with a bar
  • Natural landscaping with wildflowers and a snowmelt driveway
  • Dates of stay to be arranged with the property owner. The package excludes Christmas, Thanksgiving, Spring Break, and Fourth of July dates

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Duck Hunting Package

  • 4 day and 3 night stay at Wild Wings, a private hunting lodge in Stuttgart, Arkansas, the undisputed Duck Hunting Capital of the World
  • Package includes all hunting arrangements for 4 hunters and accommodations for up to 4 additional family members
  • Lodge accommodations and some meals included
  • Dates to be coordinated with lodge owner and must fall within duck hunting season

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French Chateau Escape

  • A unique opportunity to spend five days/nights in a stunning Gustavian cottage on the property of a fabulous privately owned French chateau located in the spectacular Loire Valley, 2 hours southwest of Paris
  • The cottage has 4 bedrooms and 4 bathrooms and sleeps 8 people
  • Enjoy visiting the other chateaux in this famous wine region, such as Chambord and Cheverny, bike riding, and, of course, the food and wine
  • The package includes weekend dinners with the owners in their exquisite chateau
  • Available between the months of May & July
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Diamond Package

  • 18K White Gold Diamond Loop Earrings, 5.06 Total Diamond Weight (TDW), Round Brilliant Cut (RBC)
  • 14K White Gold Diamond Necklace, 13.72 Total Diamond Weight (TDW), Round Brilliant Cut (RBC)

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Cowboys Luxury Suite

  • Watch the Cowboys play against the Cleveland Browns from the comfort of a luxury “Ring of Honor” suite
  • The suite package includes tickets and parking for 18 people
  • This game will feature Colt McCoy’s return to Texas
  • The auction winner will receive an official certificate to redeem, along with complete instructions and contact info
  • Date of game TBA

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Date Night: Craig & Charlotte Benson

by Lauren Smith Ford in Tribeza

published January 25, 2012

By Day: Founders of the Beyond Batten Disease Foundation

How They Met 
Charlotte says: We met while we were living in Paris. Craig was working for a company based out of Houston, and I had started a business exporting antiques back to the States. We met among a group of friends. Americans have a way of finding each other when they live abroad, and particularly since we are both from the South. Craig likes to say that I was the only other one who understood his French!

Their Perfect Date 
Charlotte says: Craig and I have a standing babysitter every Thursday night. Because of our busy schedules, finding a way to relax and reconnect is our top priority. As a result, our favorite date is often very casual. We love to take a walk around Lady Bird Lake, which gives us a chance to enjoy being outdoors while catching up with each other. Then, Mecca Gym and Spa is our favorite spot for the occasional massage. Afterward, we’ll walk across the street to Whole Foods and pull up a chair at the fish bar.

Off the Clock 
Charlotte says: Our usual standbys are Perla’s, Uchiko and 34 Street Cafe.

Why It Works 
Charlotte says: We have a shared faith, we have a shared life purpose (with our work at Beyond Batten Disease Foundation), we respect each other and we forgive each other.

Keeping Love Alive 
Charlotte says: We spend regular time together — alone. We have a date night every week and take quarterly weekends away.

Beyond Batten Disease Foundation 
Craig and Charlotte Benson started the foundation a few months after receiving the news of their daughter’s diagnosis of Batten disease. They had never heard of the disease before, but they quickly learned that it causes blindness, seizures, mental and physical incapacity and is fatal by late teens or early 20’s. There is no treatment. There is no cure. “Our daughter, Christiane (age nine), is the inspiration for the foundation, and our community has surrounded and supported us with incredible talent and resources,” Charlotte says. “The overwhelming participation from our community has enabled us to make tremendous progress. Austin can definitely take co-ownership in all that’s been accomplished!” Through the Bensons’ goal of eradicating the disease (and hundreds of other rare genetic diseases) with a screening test the foundation has developed, Charlotte says they have “found our life’s purpose.” Beyond Batten’s next fundraising event will be held on February 10 at ACL Live, featuring a performance by country star Keith Urban. For more information and tickets, visit beyondbatten.org/date-night.html.

http://www.tribeza.com/magazine_content/date-night-craig-charlotte-benson

The Battle Against Batten Disease

On KTBC Fox 7

Published February 1, 2012

Country Singer Keith Urban will be in Austin on February 10 to raise moneyand awareness about Batten Disease, a fatal disorder of the nervous system that affects children.

Craig Benson sat down with us on Good Day this morning to talk about the work of the Beyond Batten Disease Foundation, and how it has helped his daughter.

http://www.myfoxaustin.com/clip/7209750/The%20Battle%20Against%20Batten%20Disease

Country singer joins Austin dad’s effort in finding cure for disease

By KVUE staff on KVUE.com

published January 13, 2012

An Austin father is on a desperate search to find a cure for a disease most people know very little about.

It’s called Batten Disease. It begins in childhood and is often fatal. Now his efforts have gotten the attention of country music superstar Keith Urban.

Co-founder of the Beyond Batten Disease Foundation Craig Benson spoke to KVUE about his daughter, who suffers from the disease.

To help out, you can attend a special event at the Austin City Limits Moody Theater. It’s called Date Night with Keith Urban. It’s taking place on Friday,Feb. 10 at 9 p.m.

http://www.kvue.com/story/entertainment/2014/05/20/2296918/

Country Music Superstar Keith Urban Comes to Austin for Feb. 10 Gala Supporting Beyond Batten Disease Foundation

Austin, TX (PRWEB) November 02, 2011

Keith Urban – among country music’s biggest stars and a nominee for CMA’s 2011 Entertainer of the Year and Male Vocalist of the Year awards – will return to Austin for the first time in many years to perform at a Feb. 10 gala benefiting Beyond Batten Disease Foundation. The foundation fights Batten disease and also is working to eradicate hundreds of additional, serious and often fatal, inherited, childhood illnesses.

“Date Night with Keith Urban,” at ACL Live at The Moody Theater, will include a full concert performance by Urban, renowned for blockbuster shows, in Austin’s newest and arguably most spectacular music venue. The evening will begin with a “can’t miss” VIP pre-party attended by a who’s who list of guests from Austin, Dallas and Houston and an interactive auction featuring unique items and experiences.

The foundation is offering a variety of event sponsorship packages including some that provide a meet-and-photo opportunity with the superstar and accommodations at the hip W Hotel next to The Moody Theater (ideal for a memorable Valentine’s Day weekend getaway). Individual tickets, available exclusively through the Beyond Batten Disease Foundation website, go on sale to the public November 21.

Craig and Charlotte Benson of Austin started the foundation in 2008 after their then five-year-old daughter, Christiane, was diagnosed with Batten disease, a rare and fatal neurodegenerative disorder. Children born to parents who unknowingly each carry a gene mutation for Batten disease are at risk to inherit the condition which initially causes vision loss and seizures, then progressively impairs cognitive and motor capacities, and ultimately results in death during the late teen years or early 20s. Because Batten disease and so many other similar inherited, childhood diseases are uncommon, there is not much federal funding for research aimed at treatment and prevention.

In its three short years, the foundation has developed the most comprehensive carrier screening test panel for detecting genetic mutations that cause Batten disease and more than 600 other devastating, rare conditions that strike and kill thousands of kids each year. In addition to being so comprehensive, the test will be easy to administer and more economically priced than the cost of an individual test for any one of the diseases on the panel. The scientific and medical communities have heralded the test as a major breakthrough and it has received extensive media and professional journal coverage around the world.

Funds generated by “Date Night” will help the foundation to complete work to bring the test to market in 2012. The foundation plans to use proceeds from sales of the test to become self-sustaining for continuing Batten disease research.

“Keith Urban is one of the greatest entertainers of our day so we are ecstatic to have him join us at this exciting event,” said Shannon Janek, Event Co-Chair. “We expect his participation to boost awareness of Batten disease and the hundreds of other serious, inherited childhood diseases. He will be a huge draw, generating important financial support for the foundation and spotlighting its work, which is relevant for everyone planning to have children.”

According to foundation-funded research, on average, each person carries three genetic mutations that can cause a severe childhood disease or disorder. Carrier parents can even pass these mutations onto children who do not develop a condition. When these children mature and have kids, their children also are at risk of suffering from one of these conditions. Better-known examples of serious, inherited childhood diseases include Tay-Sachs disease, Cystic Fibrosis and Spinal Muscular Atrophy, or SMA. (One in 40 people are carriers for SMA.) A carrier screening strategy similar to the one that the foundation is developing has been used for Tay-Sachs disease and achieved an impressive 90 percent reduction in the incidence among at-risk populations.

About Keith Urban

Among today’s most celebrated country music artists, Keith Urban has been honored with Grammies and Australia’s coveted Aria Award as well as awards from the Country Music Association and the Academy of Country Music. New Zealand-born and Australia-raised, Urban moved to Nashville in 1992. His first American album came as a member of The Ranch (1997), followed by an increasingly accomplished series of multi Platinum-selling solo albums: Keith Urban (1999), Golden Road (2002), Be Here (2004), and Love, Pain & The Whole Crazy Thing (2006). The compilation Greatest Hits: 19 Kids (2008) included such No. One hits as “But For The Grace Of God,” “Somebody Like You” (named the top country song of the decade), “Who Wouldn’t Wanna Be Me,” “You’ll Think Of Me,” “Days Go By,” “Making Memories Of Us,” “Better Life,” and “You Look Good In My Shirt.” In his sixth and most recent album, Get Closer, Urban dives deeply into the exploration of love and relationships. For more information, please visit http://www.keithurban.net.

About Beyond Batten Disease Foundation

Beyond Batten Disease Foundation works to cure and prevent Batten disease, a rare, inherited neurological disorder that strikes young children, first causing vision loss and seizures, then cognitive and motor impairment, and ultimately death during the late teen years or early 20s. The foundation raises funds for research and is leading development of an easy and inexpensive, groundbreaking blood test to detect the gene mutations that cause Batten disease as well as 600-plus other rare but serious and often fatal childhood ailments. For more information, visit https://beyondbatten.org.

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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.

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