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Thursday, June 15, 2006

Bush Gaffe Puts Spotlight on Serious Eye Disorder
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Make the best of a awkward situation, right?

Something about lemons and lemonade?

Wednesday, President Bush, a well-known tease, was poking fun at a reporter wearing sunglasses at a press conference despite the gloomy, overcast skies. It turns out the reporter suffers from a progressive, hereditary eye disorder that requires the dark shades to limit painful light rays. Ooops!

The President personally apologized for the ribbing and the reporter graciously brushed the matter aside. At the same time, however, the world quickly learned about Stargardt's Disease.

Stargardt's is also called "Fundus Flavimaculatus". This is an inherited condition that affects the cells underneath the retina -- the retinal pigment epithelium (RPE). Visual symptoms can precede clinical changes to the inside of the eye. People experience decreased visual acuity, especially in environments where the lighting changes (light-to-dark or dark-to-light). Most folks with Stargardt's do not experience complete vision loss.

The diagnosis is established by performing a test called fluorescein angiogram. Affected patients exhibit a highly characteristic pattern with this study. Supplemental electro-physiological tests (ERG, EOG) are confirmatory.

The core problem is the abnormal accumulation of a cell product (lipofuscin) in the RPE. There is no current treatment, however optimistic researchers are working on ways to correct for the abnormal responsible gene that has been localized to chromosome 1.

Once confined to the secretive domain of medical arcania, Stargardt's Disease is being catapulted to global recognition because of an inappropriate statement made at 1600 Pennsylvania Avenue. The Foundation Fighting Blindness could only pray for such wonderful free publicity!

Related Topics:
The Amazing Human Eye, Eyesight Need a Fix? You're not alone.

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Posted by: Dr. Lloyd at 4:38 PM

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