Action for A-T

A-T is a rare, progressive and neurodegenerative disease that starts in early childhood. Babies appear ‘normal’ when they are born and the first signs of the condition usually start when the child begins to walk. At this stage they often appear wobbly with occasionally a lack of balance. These signs represent the first indication of the progressive degeneration of the cerebellum which leads to the onset of the inability to control muscle movements known as “ataxia”.

In about 70 percent of cases of children with A-T another clinical hallmark is immunodeficiency that leads to recurrent, life threatening, respiratory infections. Ultimately this often leads to pneumonia as a cause of death.

Other hallmarks of the disease are the appearance of telangiectasia, or tiny red ‘spider’ veins in the corners of the eyes or on the surface of the ears and cheeks.

Children with A-T have a tendency to develop certain types of cancer, specifically malignancies of the blood system. They are almost 1000 times more susceptible to these types of cancer than the general population. Unfortunately A-T sufferers also have hypersensitivity to x-rays and so they are not able to tolerate the therapeutic radiation that cancer patients would usually receive.

Children with A-T have and maintain normal or even above-normal intelligence which means they are fully aware of the progression of their disabilities.

The prognosis…

Currently there is no cure for A-T. Most children who suffer from A-T are dependent on wheelchairs by the age of ten and generally they do not live past their teens or early twenties. A few live beyond this but this is unusual. Most A-T patients die from either respiratory failure or cancer.

For more information click on the following link...

www.atsociety.org.uk