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July 19 5p.m.-11p.m.
Questions or Comments please contact:
Leisa or Vic Niklajevs
lnikolajevs@mac.com
CLICK HERE
BDSRA
Batten Disease Support
& Research Association
Action for Asher Day
Berlin Elementary 2006 |
 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 Elementary and
is in the fifth 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
enjoys taking swimming lessons and participating in Tae Kwon
Do.
 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 almost
completely blind. Asher has done this with a positive
attitude and maintains a cheery disposition.
 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.
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