Infantile Batten Disease

Batten Disease is an autosomal recessive disorder. Which means

the child receives two copies of the defective gene. One from

Mom and one from Dad.Each child has a one in four chance (25%)

of having Batten Disease. If the child only receives one copy

of the defective gene the child will be a carrier and not

develop the disease.

There are three other main types of Batten Disease. These are:

Late-Infantile, Juvenile and Adult. There are variants off the

four types of Batten Disease.

Generally the onset of Infantile Batten Disease occurs between

the ages of 6 months and 2 years. Symptoms include but are not

limited to loss of acquired skills,muscle weakness,seizures,spasms,

lack of stimuli (sounds,pain etc.) and visual impairment.

Some examples are the sudden inability to use their leg(s) and/or

arm(s), inability to chew and swallow solid foods, excessive crying

fits. Basically overnight losing the ability to walk, eat, talk,

hold things in their hands etc. The symptoms may occur slowly over

many months or occur one after another very rapidly.

Eventually the child will reach what some call a plateau. By

doctors and other professionals the child will be labeled as

being in a vegatative state.

From the various sites and literature I have read a child with

Infantile Batten Disease has the average life expectancy of

5 to 6 years of age. There are and has been children that have

exceeded the documented average life span. How each child passes

varies.

At this time there is no known treatment to stop or reverse

the symptoms of Infantile Batten Disease. Seizures can be

reduced or controlled with the use of medication.

Research is being done to find effective treatments to slow

down and halt Batten Disease. There is a strong need for more

public awareness of this terrible disease.

If you would like more information about Batten Disease,

interested in helping or know someone who needs help

please contact The Batten Disease Research and Support

Association at http://www.bdsra.org.

I hope I have been successful in giving you a general

description of Batten Disease. If you have any questions

please feel free to email me and I will do my best to

answer them.

Font used on this page is Whimsical.