Batten Disease Support & Research Association - Midwest Chapter 

 About BDSRA

 Mission Statement ...
. . . be an international support and research networking organization for families of children and young adults with an inherited neurological degenerative disorder known as Batten Disease.

 BDSRA Board of Directors (As of July 31, 2005) 

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 victims 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 victims. Seeing the tremendous need for a unified entity that would work toward important goals and recognizing, as well, the need for support among victims and their families, the founders designed the association to:

  maximize the opportunities of victims of 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 victims and their families

  act as a national registry for NCL researchers throughout the world.

All of the services of BDSRA are available across the nation, for a minimal membership fee, and are available at all levels of participation. BDSRA is a not for profit 501(c)3 organization. Some of the individuals who turn to the association have need of the full range of services - contact, newsletter, research, and registry information - and are willing to assist the Association in compiling information and providing professionals with facts and observations. Others want simply to receive the newsletter and remain anonymous to all other aspects of BDSRA Whatever the needs, BDSRA can meet them.

First described in 1826 - more than 170 years ago - Batten Disease (Neuronal Ceroid Lipofuscinoses), thought to be one of the most common neurodegenerative diseases, remains an unsolved mystery today...a puzzling disease that assures its victims of only one consistent manifestation...early death.

An inherited, degenerative neurological disease, - Batten may affect persons of any age, but primarily affects infants, toddlers and school age children, beginning unexpectedly and leading to a progressive loss of brain function that later destroys bodily functions, eventually leaving the victim totally helpless.

Whether in the case of infantile (Santavnori), late infantile (Jansky, Bielschowsky), juvenile (Batten, Spielmeyer, Sjogren), or adult type (Kuf, Parry), the early symptoms of Batten Disease are confusing ones. It strikes without warning, affecting vision and causing seizures or convulsions.

Possibly most frustrating of all is the fact that Batten Disease is rarely diagnosed immediately, often being mistaken for epilepsy or mental retardation, even schizophrenia. And once diagnosed, there is no satisfactory treatment and no cure. The clinical course of the disease includes a marked decline in cognitive function; personality and behavior changes; loss of communication and motor skills; poor circulation; decrease in muscle mass; hyperventilation; hallucinations, and, finally, deterioration to a vegetative state that ends in death.