Stargardt’s disease in young children

Sunday, November 29th, 2009 at 2:43 pm Post in Eye Diseases

In contrast to macular degeneration, Stargardt’s disease most occurs in children and young adults. Symptoms of Stargardt’s include small and yellowish spots of deteriorating tissue in the retina. The disease makes light-sensitive cells in the retina to deteriorate, which leads to central vision loss and eventually blindness. In early stages, peripheral vision remains unaffected.

Using an eye chart, Stargardt’s patients are measured to have vision between 20/50 and 20/200. In this case, children with low vision caused by Stargardt’s disease should be given special care, in order to get proper classroom learning.

Stargardt’s is a form of inherited retinal disease, which explains why the disease usually appears in young children under 20 years old. Studies found out that about 5% of the whole population in the world carries gene mutations that bring inherited retinal diseases. Parents with these gene mutations will pass the disease to their children. As a result, people with Stargardt’s should receive generic counseling before they decide to have a baby. Those having a Stargardt’s disease family history should also be careful before starting their own families.

Stargardt’s disease has signs such as blurry or distorted vision, vision inability in dim conditions, difficulty in recognizing familiar faces, and color vision loss in final. As mentioned before, Stargardt’s disease appears in young people under 20, but the exact period and the speed of progression are not clear so far. Cases have been found that Stargardt’s can occur in one sibling in early childhood and in another sibling at age 19. And some Stargardt’s patients even realized obvious symptoms at a middle age.

People with Stargardt’s disease should avoid vitamin A, since retinal damage can be caused by abnormal vitamin synthesis. Exposure to bright light is also suspected to trigger retinal damage so that experts recommend UV protective eyeglasses or glasses with special filters during outdoor activities. Defective genes in Stargardt’s can bring pigmented waste accumulation, which gradually damage the retina. A study of mice eye drug injection reported that the acne drug Accutane was effective in preventing the formation of these wastes.