About Batten Disease & BDSRA

Every year, hundreds of children and their families cope with the diagnosis of
 Batten disease with strength, courage and commitment. As a rare, fatal, inherited disorder of the nervous system, Batten disease has no known treatment or cure. Columbus is home to Batten Disease Support and Research Association (BDSRA), the only organization that serves families with all forms of Batten.

 
 
Maya James (#FightingforMaya), a 14-year old girl from San Diego, CA, is one of the children impacted by Batten.  BDSRA forges strong relationships with families like Maya's, working tirelessly to assure they don't have to face the disease alone, while also pursuing treatments and cures.

Maya James (#FightingforMaya), a 14-year old girl from San Diego, CA, is one of the children impacted by Batten.  BDSRA forges strong relationships with families like Maya's, working tirelessly to assure they don't have to face the disease alone, while also pursuing treatments and cures.

what is batten disease?

  • Lysosomal storage disorder interfering with cells’ ability to break down wastes.  The build-up of waste or, lipofuscin, causes cell death and leads to the early death of children and some adults.
  • According the Centers for Disease Control, we can expect to see 1 to 2 cases for every 100,000 births per year in the US.  These cases will primarily be CLN1, CLN2 and CLN3. 
  • Batten is considered an ultra-rare autosomal recessive disorder.
  • So far, there are 14 known mutations that make up the Batten family of disease with varying onset of symptoms, such as seizures, blindness, loss of walking, talking and until recently, the same life-limiting end.
  • Batten disease is one of 7,000 rare diseases affecting 30 million people in the United States and is considered the leading cause of childhood dementia.
 

the batten disease support and research association (BDSRA): celebrating 30 years of science & service

BDSRA's 30th year marks the advent of life-saving treatments. Because of committed donors and a vibrant research community, they offer hope to families. As the largest North American support and research association focused on Batten disease, they are committed to funding and driving research to discover treatments and cures while fostering a better quality of life for those living with it, and their loved ones. 

BDSRA firmly believe that to effectively unravel the mysteries of Batten disease, the worlds of medical science, research families and advocates must band together toward these common goals.

  • Families experiencing ultra-rare, life-limiting childhood diseases should not have to face them alone
  • Early consultation
  • Peer-to-peer mentors & sibling support
  • Global family support and research networks—has served families in EU, South America, Pacific regions

The BDSRA provides a collaborative support system of medical, educational and ancillary services for Batten families to help them manage the disease and its impact. They work diligently to educate caregivers, medical professionals, teachers and the public bout the special healthcare needs Batten victims face, and fund research to find appropriate treatments to alleviate the challenges associated with the disease.

  • BDSRA supports scientists worldwide through research grants, nurturing partnerships and facilitating innovative discoveries.
  • BDSRA builds alliances and networks across state and federal legislative channels to raise awareness about this rare disease and the needs of Batten sufferers and their families.
  • BDSRA connects families to appropriate resources to help them meet a wide variety of needs related to special education accommodations and specialized care with their home communities.
 
Year after year, families tell BDSRA that the annual conference they host builds their hope, sustains their courage and makes memories that last a lifetime. And because there is no time limit on grief, BDSRA provides life-long grief support for those who missed their loved ones every day.

Year after year, families tell BDSRA that the annual conference they host builds their hope, sustains their courage and makes memories that last a lifetime. And because there is no time limit on grief, BDSRA provides life-long grief support for those who missed their loved ones every day.

BDSRA Annual Family Conference: A destination for families—to learn from researchers, clinicians and each other

  • Each year 450 people attend the conference (80-90 families)
  • During these three days of magic, families and siblings never have to explain a thing, because they are surrounded by their peers who get it (July 6-9, 2017 in Pittsburgh, PA)
  • Sessions for parents to connect with researchers about the state of science, grief support, symptom management, education and schools, newly diagnosed families and many other resources and connection points with other families, including on-site nurses/childcare.
  • Special sessions and outings for siblings where lifelong friendships and support are created.
  • Opportunities for families to participate in research by donating blood and tissue samples.
     

BDSRA Research Initiatives

  • Scientific merit review is catalyst for research leading to trials
  • Global funding partnerships
  • Families key to research discovery
  • Research is key to hope for families