Newsroom Archives: News

AUSTIN, TX — Austin-area golfers are getting ready to take a swing at juvenile Batten disease during the Fourth Annual Brake Specialists Plus “HOPE on the Green” Charity Golf Tournament on Friday, October 19 at Grey Rock Golf Club.

The popular tournament will help raise critically needed funds for Beyond Batten Disease Foundation which works to eradicate juvenile Batten disease, a rare, inherited and fatal neurodegenerative disorder that occurs in young children.

“Hope on the Green is a great way for Central Texas golfers to enjoy a round of golf while also helping to save children’s lives,” said Jamey Whitlock, Executive Vice President, Brake Specialists Plus Total Car Care, title sponsor of the tournament. “Proceeds from the event help the foundation to continue funding live-saving research around the world to treat, cure and prevent this devastating illness.”

Although the tournament is expected to sell out again this year, registration currently remains open for foursomes as well as individual players. Participants will enjoy a fun-packed afternoon and evening at one of the region’s best golf facilities. Activities will include: lunch catered by Benji’s Cantina and dinner provided by Grey Rock Golf Club; 18 holes of golf on a stunning Texas Hill Country course designed by renowned golf architect Jay Moorish; putting, chipping, longest drive and hole-in-one contests for prizes such as TaylorMade and Titleist clubs and bags; a live auction, including memorabilia autographed by well-known sports and music personalities; and live music following tournament play.

Juvenile Batten disease occurs when parents who unknowingly carry the gene mutation for the disorder both pass it onto their offspring. Children are diagnosed when they become symptomatic, usually between the ages of 5 and 9. The illness causes seizures and progressive vision loss leading to blindness. The condition eventually becomes mentally and physically incapacitating and is fatal by the late teens or early 20s.

Because Batten disease is so uncommon, there is very little government support for research. Beyond Batten Disease Foundation funds a growing number of research initiatives in the United States and abroad to develop a treatment and cure. The foundation also has led development of a rare disease genetic test to diagnose and prevent not only Batten disease, but also more than 600 other serious – often fatal – inherited, childhood illnesses.

“All of us fighting Batten disease greatly appreciate everyone who participates in Austin’s Hope on the Green tournament – Jamey, the Brake Specialists’ team and, of course, all the golfers who come out to play,” said Craig Benson, who co-founded the foundation with his wife in 2008 after their young daughter was diagnosed with Batten disease. “The event is an important fundraiser and also helps us raise awareness of and galvanize ongoing support for our cause.”-

In recent years, the foundation has underwritten some of the world’s most promising juvenile Batten disease research. This includes funding a team of researchers at the Jan and Dan Duncan Neurological Research Institute at Houston’s renowned Texas Children’s Hospital. The team has identified drug compounds that may improve brain function in children with Batten disease and currently is analyzing disease models to study long-term effects on progression of the illness. The foundation also has brought in regulatory consultants to partner with the researchers at Texas Children’s Hospital in developing a path to FDA approval for a human clinical trial of the drugs.

Other foundation-funded projects include a partnership with the American Brain Foundation to create the first clinical research fellowship in juvenile Batten disease; research support at Italy’s Telethon Institute of Genetics and Medicine using robotic technology to screen hundreds of drugs for their potential to treat Batten disease; and additional research at London’s King’s College and the University of Iowa to learn more about Batten disease cell function.

THE WOODLANDS, TX — Nearly 700 people are expected to attend one of the Houston area’s most important fundraisers on September 15, the 4th annual HOPE Under the Stars Gala benefiting the Will Herndon Fund for Juvenile Batten Disease Research (WHF).

The evening event at The Woodlands Resort and Conference Center will generate critically needed funds for research to develop a treatment and cure for juvenile Batten disease, a rare, fatal, neurodegenerative disorder that affects children.

Attendees of the “Texas chic”-themed party will enjoy a night of great entertainment including mechanical bull riding, professional artist sketches, dancing and live music by The Buck Town All-Stars, the nine-piece, New Orleans-based band famous for its energizing mix of rock, soul, funk, R&B and Crescent City classics. Additionally, there will be a photo booth, casino tables, a raffle, and both live and silent auctions for fabulous items. Tickets and sponsorship opportunities are still available through the WHF website or by calling 409.454.9330.

Juvenile Batten disease is a rare but devastating illness that typically appears between the ages of five and 10. Early symptoms include vision loss leading to total blindness and also seizures. As the disease progresses, it diminishes cognitive and motor capacities – to the point where affected youngsters require 24-hour care – and ultimately causes death during the late teen years or early 20s.

Because juvenile Batten disease is uncommon – it occurs in two to four of every 100,000 children born in the United States – there is very little government funding for research to find a treatment and cure. So in August 2009, after learning that their then six-year-old son, Will, had Batten disease, Missy and Wayne Herndon of The Woodlands started the Will Herndon Fund. WHF is a directed fund of Beyond Batten Disease Foundation, launched a year earlier to raise awareness of and support research to eradicate the malady. The foundation also has been leading development of a test to detect the gene mutations that cause Batten disease as well as 600-plus other serious, often fatal, childhood conditions.

“As we approach this year’s gala, which is drawing a record number of attendees, we are more heartened than ever by the ongoing support of our friends and neighbors in the Greater Woodlands community,” said Missy Herndon. “Every dollar we raise advances the foundation’s life-saving work and gives new hope to families affected by Batten disease.”

In recent years, WHF has helped fund many of the world’s most promising research initiatives focused on juvenile Batten disease. This includes funding a team of researchers at the Jan and Dan Duncan Neurological Research Institute at Houston’s renowned Texas Children’s Hospital. The team has identified a drug combination that may improve brain function in children with Batten disease and currently is analyzing disease models to study long-term effects on progression of the illness. This includes funding a team of researchers at Houston’s renowned Texas Children’s Hospital which has identified a drug combination that may improve brain function in children with Batten disease. Currently, the team is analyzing disease models to study the long-term effects of this drug on the progression of the disease. Additionally, with funds raised from last year’s HOPE Under the Stars gala, WHF recently brought in regulatory consultants to partner with the researchers in developing a path to FDA approval for a human clinical trial of the drug.

Other WHF-funded projects include a partnership with the American Brain Foundation to create the first clinical research fellowship in juvenile Batten disease; research support at Italy’s Telethon Institute of Genetics and Medicine using robotic technology to screen hundreds of drugs for their potential to treat Batten disease; and additional research at London’s King’s College and the University of Iowa to learn more about Batten disease cell function. Each of these projects is part of WHF’s strategic plan to accelerate research for developing treatments and a cure for juvenile Batten disease.

About The Will Herndon Fund and Beyond Batten Disease Foundation

The Will Herndon Fund is a directed fund of the Beyond Batten Disease Foundation which 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 over 600 other rare but serious and often fatal childhood ailments. For more information, visit www.beyondbatten.org.

Photos: Above–Will Herndon, age 9, was diagnosed with juvenile Batten disease in 2009; Below–The Will Herndon Fund supports important juvenile Batten disease research at the Jan and Dan Duncan Neurological Research Institute at Houston’s renowned Texas Children’s Hospital.

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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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.