BBDF Blog

By Darla Hernandez on WSBT 22

Published May 8, 2014

New challenge for teen stricken by deadly disease

There’s a new challenge for a local family we have been following since 2009.That’s when 13-year-old Tyler Allman was diagnosed with a very rare terminal disease called Batten’s Disease. The toxins eat away good brain cells, causing children to lose their vision and ability to walk and speak.For the Allman family, those days are tough.
“Tyler was fine until he was in 1st grade,” said Tyler’s mother, Heather. “He played t-ball, and then he started to lose his sight and have seizures.”Tyler is now blind and wheelchair bound. Weighing 140 pounds, lifting him in and out of their minivan has taken such a huge physical toll on his mother, she now has extensive nerve damage in her arms and needs surgery.

There have even been times when Heather and Tyler fell trying to get him into the vehicle.

“I sat there on the pavement and shed a few tears until I could physically get him back up,” Heather said.

The Allman family recently started an online campaign-to raise money for a wheelchair lift for their van.

Tyler’s father, Royer, said, “I worry about my wife and my son’s safety and her transporting him and safely, doing that when I’m not around.”

The Allmans say a lot of their medical expenses are not covered by insurance, including the van lift.

“To have a simple wheelchair lift put into our van, it was $28,000. We have so many other expenses and medical equipment we need to get,” added Heather.

Time is crucial for Tyler. The disease is rapidly taking away all of his mobility.

“Tyler’s disease has a life expectancy of late teens, early 20’s, and he’s 13 now, so we don’t know how much time we have,” Royer noted.

Anyone can make a donation at any Notre Dame Federal Credit Union Branch under the name “Team Tyler.”

To donate and learn more about Tyler’s daily fight: https://www.facebook.com/beyondbatten.org

For more information on Batten Disease and to see a special Allman family video:  https://beyondbatten.org/family-stories/the-allman-family-story/

To read the article on WSBT 22, click here.

By Crystal Simmons in The Potpourri – Magnolia Edition

Published April 21, 2014

In 1998, Luke Watson was a normal kindergartner who loved Aggie sports and watching ballgames with his dad. His younger sister Rachel loved animals, jewelry and art. She could talk at nine months old and got in her first fight at 3 after a bully threatened her brother.

Almost two decades later, Luke still loves sports and Rachel still loves animals.

However, everything else has changed. At 20, Rachel can’t walk, see or talk and suffers from seizures and dementia. Her brother Luke, 21, is also blind and his speech is starting to slur. Both have nearly exceeded their life expectancy.

Every day, they get a little worse, said their mother, Melinda Watson.

Both have Batten disease, which can mentally and physically incapacitate a normal child in a few years and leads to the deterioration of the nervous system. So far, there is no cure or treatment.

“The thing about Batten disease is, it’s a horrible disease. It’s slow. It takes away a piece of them all the time,” she said.

The disease strikes about one out of 100,000 people, qualifying it as an orphan disease that is often overlooked for research and funding. Children can develop Juvenile Batten disease around 6 and suffer progressive vision loss, intellectual and motor disability, speech difficulties, and seizures. Afflicted children usually only live to be in their 20s or early 30s.

Time is of the essence for these children, said Watson. But for Rachel and Luke, it may be too late.

From the day of their diagnosis in 1999, Watson has watched as her bright active children turned into mentally and physically handicapped adults. When she taught them something, she knew they could never progress.

“You cannot regrow the cells in the nervous system,” she said. “The best we can hope for is a treatment that can stop cell death or slow its progression.”

Now, hospital beds, shower chairs, wheel chairs, adaptive clothing and adult diapers are the tools that help her get through the day.

“I cannot take care of my daughter by myself. I have to pay someone to help me. She can stand, but that’s it. She’s too heavy for me to stand up by myself. Her care is total at this point,” she said.

According to the National Batten Disease Foundation, medical care for children afflicted with the disease can cost $3-4 million over their lifetime.

Luke and Rachel Batten Foundation

In many ways, Melinda Watson and her husband Sam have been pioneers in fighting to eradicate Batten disease.

Following her children’s diagnosis, Watson established the Luke and Rachel Foundation to fund Batten research.

A former special education teacher at Lowery Elementary in Cy-Fair ISD, Watson had no idea how to start a non-profit. Instead, she found she had a talent for public speaking that opened up new avenues for her.

In the non-profit’s infancy, it averaged $150,000 a year for research and medical care for children with the disease. But these days, Watson said the non-profit has been making less as she has had to transition into a full time nurse for her children.

Now advances in research are what keeps her going. For the past several years, Watson has been combining efforts with the Beyond Batten Foundation, a Texas based non-profit funding promising research for those in the beginning stages of the disease.

“When we came on the scene in 2008, they were still trying to determine what was causing that disease. We’re still trying to determine some of that. You can’t really find a cure until you understand what’s going on in the body,” said Mary Beth Kiser, president and CEO of Beyond Batten.

Since forming in 2008, the foundation has raised over 13 million through fundraisers and donations. It takes an average of $1.3 billion and 12 years to develop an effective treatment for most diseases, she said.

With foundation funding, Dr. Marco Sardiello has been able to move from Italy to Houston, where he is conducting research at the Jan and Dan Duncan Neurological Research Institute at Texas Children’s Hospital.

“One of the first grants we had brought them over to Texas Children’s Hospital in Houston,” said Kiser. “We had them set up lab their so they can do a lot more research and receive funding from the United States.”

Sardiello hopes to begin clinical trials on a treatment for a group of neurological diseases including Batten disease that result from the accumulation of toxic substances in the cell, which leads to cell death and disease symptoms.

The foundation has also funded a pre-conception genetic screening test that identifies carriers of Batten disease and 700 other rare diseases. It’s also being used as a diagnostic tool at Children’s Mercy Hospital in Kansas City.

The test is still not commercially available but is available upon request from the foundation.

“The problem with genetic diseases especially rare ones is that people don’t have any idea they have it. We all carry about four genetic defects and if you partner with someone with the same one you have a higher likelihood of having a child with that particular disease,” said Kiser.

Run to the Sun Relay

To raise funds for research, the foundation is holding its fourth annual Run to the Sun Relay April 26-27 in Austin.

More than 25 teams of runners from different cities and states will run more than 90 miles in an overnight journey through the Texas Hill Country.

The Watson’s will also be participating in the run with their group the LRBF Warriors. Each leg of the relay will be dedicated to a child suffering from Batten disease. A section of the relay will honor Luke and Rachel by featuring their photos and biographies.

Each year, the race raises a little over $700,000, but the foundation is striving to hit the million dollar mark this year.

For more information about juvenile Batten disease and Beyond Batten Disease Foundation, please visit www.beyondbatten.org.

To view the article, click here.

By Chris O’Connell in Alcalde

Published April 22, 2014

Christiane Benson is like any other 11-year-old. She likes cooking scrambled eggs, playing the piano, and running around outside with her younger brother Garland and their dogs. But when it comes time to read, Garland has to narrate the books .

In 2008, when she was five-years-old, Christiane was diagnosed with Batten disease and has since lost much of her vision. The rare neurodegenerative disease is also known to cause mental decline, loss of speech, seizures, and eventually, death, with no known cure. Soon after her diagnosis, her parents Craig and Charlotte founded the Beyond Batten Disease Foundation on the tenets of diagnosis, fundraising, treatment, and eventually finding a cure. The Foundation’s website refers to families’ struggles with Batten disease as, “quite literally a race against time.”

“I get to see Christiane every third day,” Larry Chauvin says, his grin audible through the phone. “It’s cool.”

Chauvin, BS ’03, is a physical education teacher at Casis Elementary, where Christiane Benson is in 5th grade. He is also team captain of a running group participating in a relay this weekend benefitting Beyond Batten, called Run to the Sun, a 90-mile race starting at Enchanted Rock around dusk, and ending at dawn at Murchison Middle School in Austin. That group has grown from eight members to 25 in less than four years, and includes numerous Texas Exes in its ranks.

His co-captain, Sheri Bryant, BS ’03, ME ’10, also has a close personal connection to Christiane. She met the Bensons at a parent-teacher meeting at Casis, where Bryant taught kindergarten at the time. She learned of Christiane’s story and thought immediately, “She has to be in my class; I have a connection with the family.” She still tutors Christiane twice a week, and she and Chauvin will be running in their fourth Run to the Sun relay, having participated in the annual race every year.

While many Texas Exes have joined Bryant and Chauvin—as many as 12, on her last count—current students and student organizations are also joining the cause this weekend, like the Texas Cowboys, who will be volunteering at a checkpoint from 1 to 3 a.m. Sunday morning.

For Zach Solomon, VP of the Texas Cowboys, it was an easy decision to become involved with Run to the Sun. He has a family friend afflicted with Batten, and his father’s company has run in the race since its inception. It was only natural that the Texas Cowboys would volunteer, though until we spoke, he didn’t know how many UT alumni and students would be participating.

“I honestly wasn’t aware of all the Longhorn presence until just now,” Solomon says. “A lot of people on campus feel the need to reach out and give back to the community, so when something unique like this run comes up, it’s hard to not get interested.”

“We engage the students,” agrees Beyond Batten CEO and President Mary Beth Kiser, BA ’90. “Last year we had a girl who put a team together with her sorority, they were Zetas. This year we went to the volunteer fair for this specific event and we found people who are coming out this year.”

Whether it is intentional or not, Longhorns past and present are coming together for this cause, despite it being one of the lesser-known neurological diseases. At the finish line, they all hope, is an end to the illness.

“I think it gives them hope,” Bryant says. “The symbol behind the race is, running through night, it’s dark, like it would be to a child with Batten as they start to lose their vision. It’s a dark diagnosis, and nobody knows of a cure, but the Bensons and Foundation are paving the way to combat this disease.”

It’s not all darkness however.

“The upside of it is you finish as the sun is coming up,” Bryant continues. “We’re getting fundraising awareness out there, and hopefully there will be a cure some day.”

To view the article in Alcalde, click here.

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

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