Welcome to Action For Asher, a website devoted to raising awareness about Batten Disease (Neuronal Ceroid Lipofuscinosis), fundraising for a cure, and most importantly, to support our son, Asher Nikolajevs, who suffers from this devastating disease.

This is Asher Nikolajevs.  He is 11 years old and is entering the sixth  grade at Berlin-Milan Middle School.   Asher is very social and enjoys his friends and classmates. You can usually find him making others laugh with his jokes and silliness!

Asher has a brother, Sam, who also attends Berlin Milan Middle school and is in the eigth grade.  Asher and Sam enjoy many of the same activities such as reading, movies, Nintendo (especially Mario and Luigi!), Toy Story 2, Cedar Point, swimming and, most definitely, TRAINS.  Because of their love of trains, many of their family outings and trips have centered around train exhibits and train rides.  Asher's favorite train is a Union Pacific steam engine from the 1940's and 50's named "The Big Boy".  He also loves Thomas the Tank Engine and has collected them since he was 2 years old. 

Asher has faced many challenges in his young life.  He has been diagnosed with Batten Disease (Neuronal Ceroid Lipofuscinosis) which has caused him to lose his vision very rapidly.  Over the last few years, it has been necessary for Asher to learn to read Braille and explore the world through his sense of touch and hearing as he is now completely blind.  Asher has done this with a positive attitude and maintains a cheery disposition.  Presently, Asher is also dealing with cognitive decline due to the progression of the disease. 

Batten Disease is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs). 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 presently is always fatal. Batten Disease is not contagious or, at this time, preventable.

Batten Disease/NCL is relatively rare, occurring in an estimated 2 to 4 of every 100,000 births in the United States. The diseases have been identified worldwide. Although NCLs are classified as rare diseases, they often strike more than one person in families that carry the defective gene

There are four main types of NCL, Infantile, Late Infantile, Juvenile and Adult, including two forms that begin earlier in childhood and a very rare form that strikes adults. The symptoms are similar but they become apparent at different ages and progress at different rates.

Hope for Asher lies in future research.  The Batten Disease Support and Research Association (bdsra.org) provides funding to keep research moving forward toward a cure.

Within the Federal Government, the focal point for research on Batten Disease and other neurogenetic disorders is the National Institute of Neurological Disorders and Stroke (NINDS). The NINDS, a part of the National Institutes of Health (NIH), is responsible for supporting and conducting research on the brain and central nervous system. The Batten Disease Support and Research Association and the Children's Brain Diseases Foundation also provide financial assistance for research.

Questions or Comments please contact:

Leisa or Vic Niklajevs

lnikolajevs@mac.com

Hosted by: Thin Solutions

Published by: Berlin-Milan Middle School Technology Club