What is Batten Disease?
Batten Disease is named after the British pediatrician who first described it in 1903. Also known as Spielmeyer-Vogt-Sjogren-Batten Disease, it is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs).
Although Batten Disease is usually regarded as the juvenile form of NCL, it has now become the term to encompass all forms of NCL.
The forms of NCL are classified by age of onset have the same basic cause, progression and outcome but are all genetically different. Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with Batten Disease/NCL become blind, bedridden, and unable to communicate and it is presently always fatal. Batten Disease is not contagious or, at this time, preventable.
For more information on Batten Disease, please visit www.bdsra.org/whatis.html.
What is BDSRA?
In order to effectively combat the devastation of the disease and to fully support research efforts to unravel the mysteries on Batten, the worlds of medical science and the children and their families must meet and work closely together to reach understanding and common goals.
In an effort to bring those common goals to fruition, the Batten Disease Support and Research Association - (BDSRA) was formed in July 1986, by Gary and Judith Grant of Spanaway, Washington, who are parents of three Batten children. Seeing the tremendous need for a unified entity that would work toward important goals and recognizing, as well, the need for support among children and their families, the founders designed the association to:
- Maximize the opportunities of children with Batten to lead as normal lives as possible.
- Provide a parent communication network, information and emotional support to families of persons with Batten Disease.
- Educate lay persons and professionals about the special needs of Batten Disease children and their families.
- Act as a national registry for NCL researchers throughout the world.
For more information on BDSRA, please visit www.bdsra.org/history.html.