Stargardt’s disease

Stargardt disease (also referred to as fundus flavimaculatus or heredomacular degeneration) is an inherited condition that usually causes gradual bilateral decrease in vision in early childhood or the teenage years, which may remain stable for the rest of their lives. This progressive vision loss usually reaches to the point of legal blindness. The progression usually starts between the ages of six and twelve years old and plateaus shortly after rapid reduction in visual acuity.

Patients with Stargardt disease are usually sensitive to glare. Vision is affected when macula (center of retina and focus of vision) is damaged. Symptoms at presentation usually include wavy vision, blind spots, blurriness, impaired color vision, and difficulty adapting to dim lighting.

For treatment, it is recommended to use coloured lenses that filter out wavelengths shorter than 600 nm, at which rods get stimulated. Many patients use magnifiers to help them see, and wear sunglasses to slow the development.

In 2010, Advanced Cell Technology announced the start of a Phase I/II multicenter clinical trial using retinal cells derived from human embryonic stem cells (hESCs) to treat patients with Stargardt’s Macular Dystrophy. Results are awaited.