What is Stargardt's Disease?
Stargardt's disease is most often diagnosed in childhood or adolescence.
It is a genetically inherited form of macular degeneration. Stargardt's disease causes central vision loss. It is often referred to as early-onset macular degeneration, Stargardt's macular dystrophy, juvenile macular degeneration, or fundus flavimaculatus.
Estimates claim that over 30,000 people in the United States alone suffer from this devastating disorder.
Vision loss occurs from the degeneration of photoreceptor cells located in the macula, the retina's center region. The retina is located at the back wall of the eye. The retina tissue is very sensitive. It is responsible for receiving light, then converting it into an electrical signal that travels down the optic nerve to the brain to be processed into images.
The macula is the dense location of cone photoreceptors. They are responsible for seeing sharp details in objects, central vision, reading, seeing faces, daytime vision in light, and color vision. With a decrease in photoreceptors' function, central vision loss becomes the main characteristic of Stargardt's disease. Usually, some central vision may remain intact, but rates of progression can vary.
The retinal pigment epithelium (RPE) tissue is a thin layer of cells supporting the photoreceptors. In Stargardt's disease, damaged protein in the photoreceptor cells may result in lipofuscin buildup in the retinal pigment epithelial tissue. Photoreceptors shed themselves daily, creating layers of toxic necrotic tissue. If this waste material is not cleared away, it can destroy the retinal pigment epithelial tissue. When RPE is lost, so are the photoreceptors cells that lie above them. This leads to loss of vision.