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A-T is a rare
progressive multi-system genetic disorder that attacks the neurological and
immune systems of children who carry two copies of a defective A-T gene - one
copy from each parent. There are about 500 children in the U.S. with A-T. An
estimated 1% of the general population are carriers of the defective A-T gene.
A-T affects
energy, immune resistance, balance, handwriting, clear speech and coordination
of eye movements. Children with A-T are of normal intelligence.
Toddlers with A-T
are usually "wobbly" walkers. In their preschool years, children with
A-T begin to stumble and fall. By age 8, most children with A-T use a walker; by
age 10 a wheelchair. Over 38% of children with A-T develop cancer. Mothers of
children with A-T have a 9-fold increased risk of breast cancer - what is
learned about A-T will also contribute to the prevention and treatment of breast
cancer.
As A-T progresses,
a child with A-T also tends to experience the following:
- wheelchair use by age 10
- hand tremors and extreme
fatigue
- dyscoordination of eye
muscles making reading functionally inadequate
- high sensitivity to
radiation including X-rays
- increased risk of
respiratory infections
- need for a full time aide
in school for handwriting and note taking
- a 100-fold increased risk
of cancer
Cognition is not affected.
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