Stargardt’s Disease

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Stargardt’s disease, similar to Macular Degeneration, but is diagnosed in young children and adults as an inherited disease.

Stargardt’s disease scientifically referred to as fundus flavimaculatus or Stargardt’s Macular dystrophy (SMD). This affects approximately one in 10,000 individuals, defined by the loss of central vision early life not caused by external factors like the environment or nutrition.

The retina contains light sensitive cells rods and cones. Cones cells are sensitive to colour and are more concentrated in the center of the retina. This area of the retina also called the yellow spot. The rods, which are cells sensitive to black and white colour are in the peripheral region of the yellow spot and is responsible for black and white vision.

The retina is protected by a thin yellow covering of epithelial cells, made up of vitamin A. The outer skin of the retina, acts as natural sunglasses protecting the inner eye from harsh UV damage. Stargardt’s disease is when these epithelial cells degenerate and clump together forming dark yellow spots preventing light from reaching the retina. Usually the disease begins in the center of the retina, which is why it affects the central vision first. Gradually, the disease spread to the rest of the retina causing semi blindness and eventually complete blindness.

Studies suggest aggregation or “clumping” of vitamin A in the retina may be associated both with Stargardt’s disease and age-related macular degeneration (AMD). These clumpy deposits are known as “vitamin A dimmers”.

Causes of Stargardt’s Disease

Stargardt’s disease is a hereditary disease that does not have a medical cure as yet The disease is transferred to children when both parents suffer from Stargardt’s Disease. Studies have revealed that the disease is caused by a mutation of a gene that is responsible for processing vitamin A in the eye. However, parents who are only carriers of the recessive gene also transfer this disease to the children.

How fast does Stargardt’s disease progress?

The progression of Stargardt’s disease differs from person to person and vision problems can begin in early childhood to late teens. However, in some cases the onset of the disease does not begin until mid adulthood. As Stargardt’s disease is the degeneration of light sensitive cells, some studies show that by protecting your eyes with proper prescription glasses or wearing medically prescribed sunglasses reduces the progress of this disease.

What are the treatments for Stargardt’s disease?

There are no known treatments so far for Stargardt’s disease. However, initial stem cell research has shown 100% improvement in rats that have a similar type disease. Phase 1 and 2 of the clinical trials began in 2011 and scientists are hopeful that a cure will be found to counteract the onset of Stargardt’s Disease.