Beyond Batten Disease Foundation and American Brain Foundation Launch First Clinical Research Fellowshup to Treat and Cure Juvenile Batten Disease

June 26, 2012
News, Press Releases

AUSTIN, TEXAS (June 26, 2012) Beyond Batten Disease Foundation today announced that it is partnering with the American Brain Foundation (formerly the American Academy of Neurology Foundation) to create the first clinical research fellowship for developing a treatment and cure for juvenile Batten disease. 

The “Clinical Research Training Fellowship in Juvenile Neuronal Ceroid Lipofuscinosis” is an important milestone in the fight against juvenile Batten disease because it will help translate basic research findings quickly and efficiently into medical practice, benefiting children who have the devastating, fatal illness.

Juvenile Batten disease is a rare, inherited, neurodegenerative condition with symptoms first appearing around the age of six. It initially causes vision loss leading to total blindness and then seizures. The malady progressively impairs cognitive and motor capacities, and typically results in death by the late teens or 20s.  Launched in 2008, Beyond Batten Disease Foundation supports research to develop a treatment and cure for juvenile Batten disease and also to prevent it, along with hundreds of other serious, genetic, childhood sicknesses. 

The new, two-year, $130,000 fellowship comes in the wake of declining government support for Batten disease research. Over the past five years, funding from the National Institutes of Health has decreased by 20 percent. However, there has never been a more promising time for this research, according to Danielle Kerkovich, PhD, Principal Scientist at the Beyond Batten Disease Foundation. 

Kerkovich says new, published findings significantly advance the scientific and medical communities’ understanding of the disorder. She also notes that database resources and bioinformatics (the application of computer science and information technology to the fields of biology and medicine) now provide researchers with unprecedented access to information on Batten disease-relevant topics.  Additionally, automation technology – standard in the pharmaceutical industry – has become available to academia, enabling non-commercial researchers to test thousands of potential drugs during the earliest phases of investigation.

“We’re thrilled to partner with the American Brain Foundation, especially given these developments which open new opportunities to make major headway in research to treat and cure juvenile Batten disease,” said Kerkovich. “Having pharma-grade tools will accelerate the development of lifesaving drugs from the research lab into the clinic to treat young patients.” 

BBDF/ABF Fellowship
June 26, 2012

The fellowship also is a move to cultivate juvenile Batten disease clinician scientists. American Brain Foundation fellowships attract the top research talent and provide a proven starting point for clinical scientists to build competitive careers in translational research. These fellows have gone on to receive more than $23 million in NIH funding to study diseases that affect the brain and spinal cord.  

“We likewise are excited about the fellowship with Beyond Batten Disease Foundation – it helps advance our mission which is to support vital research and education to discover causes, improved treatments and cures for brain and other nervous system diseases,” said John Mazziotta, MD, PhD, Chair of the American Brain Foundation’s Board of Trustees and Professor and Chair of UCLA’s Department of Neurology and Brain Mapping Center in Los Angeles. “It’s particularly important to support research on Batten disease and other rare, neurological disorders – the so-called ‘orphan’ diseases that don’t attract as much funding as more common ailments but that also need to be addressed.”

Financial support for the fellowship comes from The Will Herndon Fund for Juvenile Batten Research, a donor-advised fund within the Beyond Batten Disease Foundation.  In addition to the fellowship with American Brain Foundation, the Beyond Batten Disease Foundation has facilitated or funded many other ground-breaking, research initiatives including: the largest, single grant in juvenile Batten disease research history – a $2.5 million gift to Texas Children’s Hospital in Houston; the first conference focused on drug discovery for juvenile Batten disease (held in partnership with the Alzheimer’s Drug Discovery Foundation); and studies exploring new areas of investigation at leading U.S. and European universities. 

About the American Brain Foundation

The American Brain Foundation, the foundation of the American Academy of Neurology, supports vital research and education to discover causes, improved treatments, and cures for brain and other nervous system diseases. Learn more at http://www.CureBrainDisease.org or find us on Facebook. For more information about the American Academy of Neurology, visit http://www.aan.com or find us on Facebook, Twitter, Google+ and YouTube.

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 by the late teens or 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 www.beyondbatten.org.

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First Clinical Research Fellowship to Treat and Cure Juvenile Batten Disease

June 26, 2012
News, Press Releases

AUSTIN, TEXAS (June 26, 2012) Beyond Batten Disease Foundation today announced that it is partnering with the American Brain Foundation (formerly the American Academy of Neurology Foundation) to create the first clinical research fellowship for developing a treatment and cure for juvenile Batten disease.

The “Clinical Research Training Fellowship in Juvenile Neuronal Ceroid Lipofuscinosis” is an important milestone in the fight against juvenile Batten disease because it will help translate basic research findings quickly and efficiently into medical practice, benefiting children who have the devastating, fatal illness.

Juvenile Batten disease is a rare, inherited, neurodegenerative condition with symptoms first appearing around the age of six. It initially causes vision loss leading to total blindness and then seizures. The malady progressively impairs cognitive and motor capacities, and typically results in death by the late teens or 20s. Launched in 2008, Beyond Batten Disease Foundation supports research to develop a treatment and cure for juvenile Batten disease and also to prevent it, along with hundreds of other serious, genetic, childhood sicknesses.

The new, two-year, $130,000 fellowship comes in the wake of declining government support for Batten disease research. Over the past five years, funding from the National Institutes of Health has decreased by 20 percent. However, there has never been a more promising time for this research, according to Danielle Kerkovich, PhD, Principal Scientist at the Beyond Batten Disease Foundation.

Kerkovich says new, published findings significantly advance the scientific and medical communities’ understanding of the disorder. She also notes that database resources and bioinformatics (the application of computer science and information technology to the fields of biology and medicine) now provide researchers with unprecedented access to information on Batten disease-relevant topics. Additionally, automation technology – standard in the pharmaceutical industry – has become available to academia, enabling non-commercial researchers to test thousands of potential drugs during the earliest phases of investigation.

“We’re thrilled to partner with the American Brain Foundation, especially given these developments which open new opportunities to make major headway in research to treat and cure juvenile Batten disease,” said Kerkovich. “Having pharma-grade tools will accelerate the development of lifesaving drugs from the research lab into the clinic to treat young patients.”

The fellowship also is a move to cultivate juvenile Batten disease clinician scientists. American Brain Foundation fellowships attract the top research talent and provide a proven starting point for clinical scientists to build competitive careers in translational research. These fellows have gone on to receive more than $23 million in NIH funding to study diseases that affect the brain and spinal cord.

“We likewise are excited about the fellowship with Beyond Batten Disease Foundation – it helps advance our mission which is to support vital research and education to discover causes, improved treatments and cures for brain and other nervous system diseases,” said John Mazziotta, MD, PhD, Chair of the American Brain Foundation’s Board of Trustees and Professor and Chair of UCLA’s Department of Neurology and Brain Mapping Center in Los Angeles. “It’s particularly important to support research on Batten disease and other rare, neurological disorders – the so-called ‘orphan’ diseases that don’t attract as much funding as more common ailments but that also need to be addressed.”

Financial support for the fellowship comes from The Will Herndon Fund for Juvenile Batten Research, a donor-advised fund within the Beyond Batten Disease Foundation. In addition to the fellowship with American Brain Foundation, the Beyond Batten Disease Foundation has facilitated or funded many other ground-breaking, research initiatives including: the largest, single grant in juvenile Batten disease research history – a $2.5 million gift to Texas Children’s Hospital in Houston; the first conference focused on drug discovery for juvenile Batten disease (held in partnership with the Alzheimer’s Drug Discovery Foundation); and studies exploring new areas of investigation at leading U.S. and European universities.

About the American Brain Foundation
The American Brain Foundation, the foundation of the American Academy of Neurology, supports vital research and education to discover causes, improved treatments, and cures for brain and other nervous system diseases. Learn more at www.CureBrainDisease.org or find us on Facebook. For more information about the American Academy of Neurology, visit www.aan.com.

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 by the late teens or 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 www.beyondbatten.org.

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I Wish

May 23, 2012
A Mother's Story

On Mother’s Day, I received a poem from Christiane entitled “I Wish.” There was a picture of her at the top blowing on a thistle and written with a black marker in her own handwriting, the last line read, ”I wish that there was no more Batten.” I swallowed hard knowing that this was one of those difficult moments.

Two weeks earlier, Christiane and I had gone for a walk to the hardware store to buy some birdseed. It was the first time we had ventured out so far from home on foot, and she was especially delighted that we had successfully crossed such a busy street to get there.

On our way home we stopped at a friend’s house to visit. While we were there Christiane was introduced to a woman who kindly told Christiane that she had her picture on her refrigerator and that she prayed for her every day. Christiane had a puzzled look on her face and I guessed that she might be wondering why someone she had never met was praying for her every day. So when we left, I asked Christiane if she knew why that lady said that she prayed for her, and she answered “No.”

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Mix It Up

May 13, 2012
News

by Neissa on iGnite

During March I was invited to join a team for a philanthropic relay called Run To The Sun. The event was created to support and raise money for an Austin-based non-profit organization called Beyond Batten Disease Foundation. The founders, Charlotte and Craig Benson, are the parents of their precious nine year old daughter, Christiane, who, in 2008 was diagnosed with Batten’s Disease, an extremely rare, neurodegenerative genetic disorder whose first symptom is vision loss. Knowing the event was for a noble cause, I instantly knew it was something I wanted to participate in, however the specifics sounded down right brutal. Let me explain.

First, the event starts at Enchanted Rock in Fredericksburg on a Saturday afternoon and ends at Mt. Bonnell on Sunday morning. Second, with eight team members, each are responsible for running two legs (as few as three miles and as many as eight). Third, eleven out of the sixteen legs are run in the dark. Forth, the terrain is extremely hilly. Fifth, in theory sleep is possible, but in reality, it doesn’t happen.

Despite the undesirable factors, I enthusiastically agreed to join the team simply because: it was something I had no business doing and second, it would stretch me way outside of my current physical and mental comfort zones, and outside of having a baby and becoming a parent, it had been a while since I had “mixed it up” and challenged myself in such a way. While I once considered myself a runner, I now rarely run the three mile loop at Town Lake. Furthermore, I never run hills, and with a busy toddler, a busy life and teaching 6:00 a.m. iGnite classes, losing a night of sleep was not a good idea. After all, I know that sleep is the fountain of youth, so again, there wasn’t one part of the relay that made logical sense, expect that it benefited a tremendous cause and wonderful family.

As a result of agreeing, two weekends ago I joined my team, “The Batten Butterflies”, along with twenty other teams, to hit the hilly pavement and do something that to most people seems ludicrous.

Without detailing the entire twenty-two hours, I’ll summarize by saying that I’ve never been so thankful to participate in a single event than having participated in the Run To The Sun. While it was extremely challenging and exhausting, it was incredibly exhilarating, hysterical, and fun! Yes, I had to run (and walk) up many hills against a strong wind, one of which was past a never ending sheep farm that smelled so disgusting that my teammates, who often drove alongside me, had to drive past me in an effort to avoid throw up. And yes, I had the last leg that while even in my neighborhood, I missed a turn which temporarily took me off course and increased my overall distance. However, despite the physical and mental fatigue, I would do it over and over again! In addition, I made new friendships with fabulous women and we now have a strong bond that we’ll always share, much like those created within iGnite.

After having experienced the bliss from the Run To The Sun, it reminded me how much my spirit needed to be entirely uncomfortable and set free. And while I need and love my current routine, it was liberating to “mix it up” and do something that was crazy and somewhat scary.

As adults, we are sometimes handcuffed to our routines due to life and our children’s schedules, and sometimes we are even handcuffed as a result of our limited mindsets, self-made boundaries and habits. However, this week I encourage you to “mix it up” and try an iGnite class, time, location or leader that you’ve never experienced before. If not, maybe you take the same classes but walk, run or swim a little faster, lift a little heavier, jump a little higher or stretch a little deeper. In addition, I encourage you to take the advice we often give our children which is, “you never know until you try.” For me, had it not been for participating in the Run To The Sun, I probably wouldn’t have been bitten by the running bug again, met new and endearing friends, and felt the sense of satisfaction that I had been longing for.

One of my favorite quotes is “life begins at the end of your comfort zone” therefore, I hope you’ll be inspired to mix it up and try something new by watching this week’s video, which showcases the Casis Elementary Team running in the Run To The Sun.

Good Tired

May 2, 2012
News

by Kristin Armstrong in Runner’s World

There are all kinds of tired.  There’s sick tired.  Frustrated tired.  Insomnia tired.  Too much wine tired.  Stressed out tired.  Worried tired.  Jet lag tired. Too much on my mind tired.  Too much on my plate tired.  Relationship tired.  Need coffee tired.  Eating poorly tired.  PMS tired.  Mentally tired.

But perhaps the best kind of tired is ‘I ran my ass off tired.’  Especially when you run your ass off with a pack of close friends on an overnight relay road trip.  That’s exactly what we did, starting just after 5 pm last Saturday night and finishing at sunrise on Sunday morning.  The Run the the Sun relay race is a 100 mile relay through the Texas hill country benefitting the Beyond Batten Foundation.  We drove our rented RV out to Fredericksburg and ran (wee, wee, wee) all the way home to Austin.

I’m still coming down from the endorphin-girl power-good cause-sleep deprivation induced high.  I’m waiting for my memories to shift and settle into a nice little diorama so that I can peer inside and tell you all about our amazing adventure.  But I’m afraid that if I wait long enough for my memories to gain clarity, something may be lost in time and translation.

So here are my threaded thoughts.

The wind, whipping at high speed, drying my sweat before it had a chance to soak me, causing chapped lips, a parched throat and chafed thighs.  Running my first leg, my friends yelling encouragement and handing me water out the window of our RV and follow car. Screaming and singing and handing water to my friends.  Driving an RV for the first time, feeling like a road warrior.  Olay face wipes qualifying as a shower.  Hanging my jog bra on the RV kitchen cabinet knob to dry while I wore another one for my second leg, then switching again.  The stench of the RV septic system until Beth found some miracle blue something to pour down there.  Speaking of stench, passing a sheep farm – Lord have mercy.  Wildflowers, fields of yellow and red bloom.  Dead snake.  Paige, faithful leader, jumping out in solidarity with anyone on a terribly hard hill.  Pace charts, elevation charts, driving matrix.  Breakfast tacos – spinach, egg, black bean on corn tortillas.  Slap happy fits of laughter with Dawn.  Seeing my daughter Isabelle and her friend Anna, working at an aid station late at night, swirling her in a giant hug, and getting a bouquet of picked wildflowers.  Telling stories, having enough time to finish conversations.  Scary scary dark, headlamps, clip on red blinky lights, reflector vest, knuckle lights, police escorts, orange cones.  Breathing through my fear and overcoming it.    Paige and Courtney singing the Mama Mia soundtrack in the middle of the night, knowing full well I hated that movie.  Crawling into the upstairs nook above the driver in the RV, hugging my pillow with sweaty arms and face, and passing out.  Seeing my children and their homemade sign at the finish line.  Hugging them and my parents.  Cheering Paige, our final runner, and watching her run in, surrounded by a pack of children.  Coffee, eggs, pancakes, and gospel music at the finish party.  Seeing the family we ran our hearts out for.  Massage tables on the grass under the trees.  My kids making goofy faces at me under the face cradle.  Grace’s sign on the bedroom door, “Mommy Sleeping.  Do Not Enter Until Later.”  My glass of red wine at dinner, which tasted like accomplishment.

That’s the kind of tired I mean.

The kind of tired that means you are depleted, but far from empty.

Watching each of my friends working so hard, churning up hills that made my legs ache just from pushing the gas pedal, made me burst at my emotional seams.  These are the people I rely on, and I got to see them under pressure, exhausted, relentless, and beautiful – giving every last reserve without complaint.

Charlotte, our friend and the mother of the little girl with Batten disease, said at the finish line that she felt “more blessed than burdened.”  If she feels this way,if SHE feels this way, how much more honored and humbled are we who got to run and carry just a tiny little piece of that burden?

It’s a good tired.

Run to the Sun Relay helps children with Batten disease

April 19, 2012
News

By Melissa Gale on KVUE

Published on April 19, 2012

If you are looking for a physical challenge and a way to help out children in need, we have an event that will interest you.

The Beyond Batten Disease Foundation is hosting its Second Annual Run to the Sun Relay.

Batten disease is a fatal neurodegenerative disorder that strikes young children.

Lance Thompson and Paige Alam are co-chairs of this year’s relay. It takes place at the end of the month. They spoke to KVUE about the event.

http://www.kvue.com/story/news/local/2014/05/21/2308922/

Tech fraternity hosts Robert Earl Keen concert to help Austin foundation

April 12, 2012
News

by Laura Waltzer in Lubbock Avalanche Journal

published April 12, 2012

Texas Tech fraternity Sigma Nu will host a charity concert, Rattlesnake Roundup, headlining Texas country artist Robert Earl Keen tonight to raise money and awareness for the Beyond Batten Disease Foundation.

“Every day has to count; every day is a gift,” resounded in Brett Hartig and Kyle McAlonan’s hearts as they learned about the Benson family and their daughter, Christiane’s, struggle with Batten disease, a rare inherited neurodegenerative disorder, while watching the foundation’s promotional YouTube video last Christmas.

Moved by the Benson family’s story, Hartig and McAlonan, president and rush chairman, respectively, of the fraternity, vowed to create the concert.

Keen, with openers The Benton Leachman Band and Haden Burchard, will perform from 6 to 10 p.m. at the Lonestar Amphitheater. Tickets cost $20 in advance or $25 at the door.

Early symptoms of Batten disease show between the ages of 5 and 10, and include seizures and vision problems.

Symptoms evolve from personality or behavioral changes to blindness; physical and mental incapacitation, resulting in 24-hour care; and ultimately lead to death by late teens or early 20s.

The disease, occurring in every two to four children out of 100,000 births, is a common name of a group of conditions known as neuronal ceroid lipofuscinoses (NCLs), and without any known treatments.

Beyond Batten Disease Foundation was created in 2008 by Craig Benson, father of Christaine, to research the treatment and cure, as well as health screening for families who might carry destructive illnesses similar to Batten.

“Since Batten disease is so rare, the government will not fund any type of research,” Hartig said. “When Benson’s daughter was diagnosed, he began the foundation in search of hope for his family and any others who are suffering from this disease. Sigma Nu is proud and honored to help with this organization.”

With a majority of members from the Austin area, where the Benson family lives, Hartig said the cause remains important to the group of young men.

Though a majority of the foundation’s funding comes from private donors, Hartig said the fraternity feels blessed to contribute to the research, referring to their event as a drop of water in a big pond.

“I know in terms of dollars, what we’re contributing is nothing compared to the amount others donate,” Hartig said, “but I think it’s more than that. It’s the fact that a West Texas fraternity is focusing on this amazing foundation that’s going to change so many lives. Our support and the awareness we’re raising means more than the money we’ll donate.”

Besides research for a cure, the foundation focuses on screening tests for family members. Since a majority of NCLs diseases occur through genetic inheritance, the foundation is fighting to promote a service that will help families detect genes which could ultimately cause over 600 life-threatening diseases.

Batten disease, for instance, only occurs if both parents remain carriers for the illness. Though being carriers does not necessarily mean their child would be affected, the foundation hopes screening tests will provide parents knowledge of potential diseases.

Benson, also CEO of Rules-Based Medicine, a biomedical corporation that contributes to the research of treatment and a cure for Batten disease, said he and the foundation are honored for the work of Sigma Nu.

“This event is a beautiful example of an extended community that has enabled tremendous progress to find a treatment or cure, as well as developing tests,” Benson said. “These guys are a real blessing for my family and our foundation. We’ll be forever grateful.”

The Benson family, who has known about their daughter’s disease for 3½ years, rallies around a national and international support system. Describing the news of his daughter’s diagnosis as a blow to the family, Benson said their strong faith and progress through the foundation gives them hope for the future.

Because of the rarity of this disorder, Benson said families rely on each other, giving tips and support in ways doctors cannot provide.

“They understand what it’s like to have a child deteriorating right before your eyes,” he said. “They can provide comfort and ideas in a way no other person can.”

That camaraderie inspires the success of the Beyond Batten Disease Foundation, driving all those involved to make a difference to the small community of families who live with the disease.

For more information regarding the research and foundation, visit www.beyondbatten.org.

http://lubbockonline.com/entertainment/2012-04-12/tech-fraternity-hosts-robert-earl-keen-concert-help-austin-foundation#.UCLC_6NQSFI

2nd Annual “Run to the Sun” Overnight Relay Expanded to Accommodate More Runners

March 29, 2012
News, Press Releases

Austin, TX — March 28, 2012

Beyond Batten Disease Foundation today announced expansion of its “Run to the Sun” overnight relay due to popularity of the oversold 2011 inaugural event and strong registration so far for this year’s April 28-29 run. Event organizers are planning for up to 35 teams with as many as 350 runners in 2012 compared with last year’s total of 25 teams and 250 runners.

Also this year, the foundation is reversing the 95-mile course through the beautiful Texas Hill Country. Last year, the relay started atop Austin’s scenic Mount Bonnell and finished at Enchanted Rock State Natural Area near Fredericksburg. This year, runners will begin at Enchanted Rock on the afternoon of Saturday, April 28, and finish on Sunday, April 29 at the Austin Museum of Art’s Laguna Gloria, with a 6:00-9:00 a.m. breakfast celebration featuring live music by The Gospel Stars.

Based in Austin, Beyond Batten Disease Foundation is developing a treatment and cure for Batten disease as well as a test to screen for the condition along with hundreds of other serious, genetic, childhood illnesses. Batten disease is a rare and fatal inherited, neurodegenerative disorder that strikes young children. The condition 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. The relay run will help raise awareness of Batten disease and funding for the organization’s lifesaving work.

“Run to the Sun is a spectacular event in its own right, sure to be a highlight of the year for participating runners, but it’s also a way to help change the world – by raising funds for research to eradicate Batten disease and hundreds of other devastating disorders that seriously impair and even kill children,” said Lance Thompson, Run to the Sun’s creator and co-chair. “We encourage everyone to get involved – even if you’re not a runner, you can volunteer to help provide support to runners along the route, and we always welcome sponsors.”

A growing number of corporate teams have signed up for the run, including groups from Bulldog Solutions, Motorola, and Rules-Based Medicine.

Run to the Sun’s website has information on how to:

  • Join or start a team
  • Sponsor a runner
  • Volunteer to help at the start or finish or along the route
  • Become an event sponsor

Beyond Batten Disease Foundation was started in 2008 by Craig and Charlotte Benson after their then five-year-old daughter, Christiane, was diagnosed with Batten disease. Children born to parents who unknowingly each carry a gene mutation for Batten disease are at risk to inherit the condition but 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 years, the foundation has developed the most comprehensive rare disease genetic 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.

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