BBDF Blog

By Alexa Harris in Austin Fit Magazine

Published April 18, 2014

Later this month, a group of teachers from Casis Elementary will be participating in The Run to the Sun Relay, a race that benefits Batten disease, a fatal disease that hits close to home for this tight-knit community.

In 2008, Casis was shaken by the news that Christiane Benson, a kindergarten student at the time, had been diagnosed with Juvenile Batten disease. The disease is a rare, fatal disorder of the nervous system that begins during the early stages of childhood. After receiving the news of Christiane’s diagnosis, the community of Casis rallied behind her.

Her parents, Craig and Charlotte Benson, established the Beyond Batten Disease Foundation and in 2011, they hosted their first Run to the Sun Relay, an overnight relay race. Teams of six to 10 people participate in this 90-mile venture with great enthusiasm and emotion. The race begins at Enchanted Rock in Fredricksburg and finish’s at Murchison Middle School in Austin.

Larry Chauvin, a PE teacher at Casis, took this relay as an opportunity to not only get himself involved with the foundation, but other teachers as well. The first year of the race there was 15 teams, including Chauvin’s. Within the last three years, Chauvin and his team have raised more than $50,000. In total, $700,000 in donations have been raised by the organization, and this year they hope to break the million-dollar mark.

“The race itself is a great team building event and has brought our campus a lot closer,” Chauvin said. “Not just the parents but the students as well.”

It is also Chauvin’s efforts outside of the classroom that have been extremely fundamental in bringing the community together. He helps put on an after-school fitness program for the students of Casis. The program is two hours and includes different relays and fun fitness games. The parents pay by donation of $25; however many usually give much more.

“That is the biggest fundraiser,” Chauvin said. “All the money supports research and development.”

Currently 21 teams are signed up for this month’s race, with a potential for 30. The fourth annual Run the to the Sun relay will kick off the night of April 26 with more teachers from Casis than ever participating.

Benson’s parents, Charlotte and Craig, are present and visible throughout the race, and wrap up the event with a heartfelt speech.

“The foundation has been a lot of grassroots efforts,” Chauvin said. “It has been amazing to be apart of. It has been amazing to be on board.”

The teachers are excited to participate, and they make sure to remember how and why they are involved. Benson is now in the fifth grade, and her vision is fading quickly. However that does not stop her from living her life to the fullest.

“She never gives up,” Chauvin said. “You would never know anything is wrong. She has a great spirit and energy.”

To learn more about the race, or watch a video from the teams, beyondbatten.org.

To view the article on AFM, click here.

In the Tarrytown Newsletter

Published April 2014

Click here to view the newsletter

published on March 25, 2014 in Woodlands Online

THE WOODLANDS, Texas–Rarity is the greatest challenge orphan diseases face. Affected patients are spread out across the country and around the world. Less than 100 children in the United States suffer from Batten disease: a rare, genetic neurodegenerative disease that affects children and is always fatal. The goal of Beyond Batten Disease Foundation (BBDF) is to consult and collaborate with like-minded groups such as other neurodegenerative disease foundations and Batten families across the globe to pool the resources and funding necessary to drive treatment-based research. There will only be one clear measure of success: providing treatments and a cure for Batten disease.

In April, Batten communities from across the globe will come together to raise funds and awareness for treatments, and a cure for Batten disease at BBDF’s 4th Annual Run to the Sun Relay. Hosted in Austin, Texas, over 25 teams of runners will run more than 90 miles in a spectacular overnight journey through the Texas Hill Country. Run to the Sun serves as an opportunity for all Batten communities from different cities, states and countries to join forces to raise awareness and funds for research. Each leg of the relay will be dedicated to a child suffering from Batten disease. Along the route, participants will get the chance to meet and learn about the children that they are running to save. In the past, teams of runners from other Batten communities made the journey to Austin to participate in the relay in person, while some created virtual teams and ran the 90 miles with us from miles away.

This disease affects Will Herndon here in The Woodlands, Texas. Will will be honored at this year’s relay by having a leg of the race dedicated to him. Runners will be able to view pictures and a biography of Will, giving them the opportunity to get to know who they are running for.

The Herndons started the Will Herndon Fun of the Beyond Batten Disease Foundation in 2009, and in the past four years, have done so much to increase funding and awareness for Batten disease. To watch their story, click on the link below.

Finding treatment and a cure for juvenile Batten disease is a cause that is present and necessary in our community. Please help raise the much-needed awareness about this devastating disease by sharing Will’s story.

To learn more about Run to the Sun, contact Mary Beth Kiser at mbkiser@beyondbatten.org or 512-275-2600.

For more information about juvenile Batten disease and Beyond Batten Disease Foundation, please visit the website below.

www.beyondbatten.org

http://www.woodlandsonline.com/npps/story.cfm?nppage=52475

by Stephanie Buckner in Cleveland Advocate

published March 14, 2014

Juvenile Batten Disease is an extremely rare, fatal and inherited disorder that affects the nervous system and begins in childhood.

For Chuck Meyer, who was most recently a congressional candidate for District 36, the rare disease is something that has become a big part of his everyday life.

Meyer’s 18-year-old daughter, Emily, was initially diagnosed as an explosive inflexible child, which Meyer describes is best understood by imagining a child that never outgrew tantrums that typically die down at an early age. Other effects of the disease became apparent as time goes by.

“Early on, we noticed her inability to differentiate between two objects that she would be looking at — like a plane in the sky — or we would be looking at a fish in the pond and she wouldn’t be able to see the fish at all, so we thought that was really strange,” said Meyer.

The Meyer family began taking Emily to get prepped for eyeglasses; however, optometrists were never able to get an accurate reading for her to do so and informed the Meyers’ that Emily’s optic nerve seemed to have never fully developed. With her condition worsening and with no explanation as to what exactly was happening to little Emily, the family began the pursuit of a diagnosis.

Eventually, a doctor noticed something concerning on Emily’s retina. The discovery led to a series of tests in an effort to rule out a variety of diseases.

“The doctor told us that her optic nerve was deteriorating and that it could be one of a number of things,” said Meyer. “He mentioned Juvenile Batten Disease but he told us not to go and look it up because it would probably scare us and it was highly unlikely that that’s what it was since it is so rare.”

Meyer admits that even though they had been advised not to do so, he and his wife did so regardless. They discovered many facts about the disease, which is essentially the gradual deterioration of the nervous system. One of the first things to be affected is vision, explaining Emily’s sight troubles.

“Many people see personality changes in their children and we never really saw that with Emily,” said Meyer. “Looking back there were some behavioral problems but we just never really suspected that that was what it was. When we did get the diagnosis, I literally fell out of my chair.”

He continues by saying that he “never in a million years thought that Emily would have this disease,” which he describes as incredibly complex.

Meyer credits the diagnosis with launching his potential political career, saying that in 2010, he believed that if he ran against Sheila Jackson Lee, he could use the press to garner some attention for rare childhood diseases. In 2014, he ran for Congressional District 36, but was defeated in the primary. Though he lost the race, his pursuit of awareness has not subsided.

He also advocates research for the disease and other rare childhood diseases. Baylor College of Medicine is one of the few research facilities that is currently striving to find a cure for the rare disease. The Beyond Batten Foundation is the one of which the Meyer family is very supportive, as it raises funding specifically for the research of Juvenile Batten Disease. The foundation has also worked to develop tests that parents can now use to determine the likelihood of children being born with diseases before conception.

“This is my passion; to try to get the word out to anyone who will listen about childhood diseases and about why we need more awareness throughout society,” said Meyer.

Currently, Emily processes things as a 6-year-old would, despite being a young adult. While she does utilize a wheelchair, she is not completely dependent upon it yet, though in time, she will be required to use it full time. She can no longer speak in complete sentences, though she is at a point where she still understands how to communicate her basic needs.

Emily, who once dreamed of being an Irish dancer and at one point, according to her father, was the only blind Irish dancer in the United States competitively, still uses her legs to mimic the movements of dances of which she is familiar. This May, Emily will be rolled across the stage at College Park High School, where she will receive her diploma.

“It is only through prayer that my wife and I have been able to get through this,” said Meyer. “Speaking about this is so much harder for me than any campaign speech because it is so personal. We have hopes that maybe she will live into her 30’s instead of her 20’s since her affliction is slower progressing than other forms.”

For more information about Juvenile Batten Disease, visit www.beyondbatten.org.

http://www.yourhoustonnews.com/cleveland/living/aspiring-politician-aims-to-spread-word-on-rare-childhood-diseases/article_2f7974ad-ab09-5cd6-87af-cbd01f52c8f5.html

CLICK HERE

“A Mother’s Story” by Charlotte Benson

republished by The Global Genes Project on December 21, 2013

With the holidays approaching, the children and I have decided to decorate our front yard with characters from our favorite Christmas story: Dr. Seuss’ How the Grinch Stole Christmas.

Twelve years ago, my brother, John, made a Grinch who sat on the front porch at Christmas, and I remember all of the children being fixated on every detail, fascinated with his yellow eyes, bright green skin, and long fingernails. Every wrinkle of his face was animated with a grin that held the awful thought of his plot to end Christmas by stealing it away from the Whos. Every child imagined what it would be like to wake up with no presents or toys, and the Grinch would finally be satisfied that he had put an end to the noise and commotion of Christmas.

So with inspiration from my brother, I struck out to set up tables in the backyard, pulled out the paint and brushes, and began creating our very own Grinch with a jigsaw. One cut led to another, and it seems now we are creating the whole scene where the Grinch is crouched at the top of Mt. Crumpit with one finger on the sleigh full of stolen presents teetering on the edge of a cliff. Overcome with shock and disbelief, he cups one hand to his ear because he hears music and singing coming from Who-Ville beneath. He realizes that his horrific act didn’t stop Christmas from coming……it came anyway! The Whos are standing hand in hand joyfully singing, in spite of the fact that there are no toys and presents.

I must admit, the child comes out in me at Christmas, and I absolutely love the creative process and fantasy of what it will all look like when it is finished. I enjoy the buzz from waking up in the middle of the night with a new idea and the simple mindlessness of painting and cutting. So with the jigsaw humming, and sawdust flying, the whole scene animated itself in a completely new way for me. It occurred to me that not only was I creating a scene from a popular Christmas story, but I was animating a belief that I hold deep at my core.

Life is difficult. People get sick, loved ones die, parents get divorced and at one time or another, we are all faced with pain and suffering. But in spite of how challenging life can be, God promises us what is constant and unchanging and provides us with what will sustain us through any storm.

Batten Disease is a horrible disease that may cripple my child and leave her blinded and perhaps take her away from us too soon. But I remind myself that Batten Disease CANNOT cripple our love, or blind our faith or take away courage or rob our peace or destroy love, friendship, and community. And it will never take away hope.

So my Christmas wish to you is never forget: Nothing….Nobody……Not even the Grinch can steal away your faith, hope, love, and joy!

http://globalgenes.org/dont-let-disease-be-your-grinch-this-christmas-says-mother-of-patient-with-batten-disease/