Stargardt’s disease is a strain of juvenile macular degeneration that affects about one in 10,000 children. It is often diagnosed before the age of 20, though the earliest symptoms start appearing between the ages of 6 and 12. The progressive disease has been linked to a specific gene mutation which is responsible for the nourishment of the photoreceptor cells in the eye. While the disease never results in total blindness, many of those with Stargardt’s disease do eventually end up legally blind. There is currently no cure for Stargardt’s disease, and most treatment is aimed at preventing further loss of vision or injury to the eye, and visual aids to help those affected by the disease remain active and live a normal life.

Symptoms and Complications

While the symptoms of Stargardt disease often begin in childhood, an affected person may not notice vision loss until his 30s or 40s. The early symptoms may include gray or black spots in the central vision, blurriness of vision and difficulty reading or doing close work. Vision loss is slow until the vision reaches 20/40, then progresses rapidly until the vision reaches 20/200, which is legal blindness. In later stages, people with Stargardt disease lose color vision and the ability to recognize faces. It does not cause a loss of peripheral vision.

Risk Factors and Causes

Stargardt’s disease is an inherited disease that is passed on through a particular mutated disease. It is a recessive trait, so a parent may pass it on without having Stargardt’s disease himself. In the disease, the cells responsible for nourishing the photoreceptors in the retinal layer of the eye appear to malfunction, causing a buildup of fatty deposits at the back of the eye. As these deposits accumulate, the fatty deposits cause rapid vision loss.

Treatments

Current treatments for Stargardt’s disease focus on slowing the progression of the disease and supporting remaining vision. Glasses with UV filters can reduce light damage to the eyes, and most doctors recommend that patients with Stargardts disease wear polycarbonate lenses to protect their eyes from injury. Scientists are experimenting and exploring a number of promising avenues for treating people with Stargardt’s disease, including gene therapy, stem cell therapy and a promising new drug that may boost mitochondrial function.