Stargardt Disease

Cause

In a healthy eye, Vitamin A is used in the production of photo-pigment cells located in the retina. This process results in a fatty lipid by-product which is cleaned up by a protein encoded by the ABCA4 gene.  In Stargardt disease this protein is absent, which results in a build-up of the fatty by-product which is seen on the retina as ‘Drusen’. This results in damage to the light-sensitive cells in the macula, which in turn affects your central vision.

Symptoms

The most common symptom is a gradual loss of central vision in both eyes. People with Stargardt disease can be sensitive to light, have dark or hazy spots in their central vision, and require more time for their vision to adjust between light and dark places. Eventually people with Stargardt disease may also experience problems with colour vision.

Diagnosis

An Ophthalmologist can examine your eyes and review scans taken of the back of the eye. Genetic tests can be completed to confirm if the ABCA4 gene is involved.

Treatment

There is no treatment for Stargardt disease, however research is continuing for gene therapy and therapeutics.  People with Stargardt disease are recommended to protect their eyes from sunlight when out and about, and refrain from smoking. There are vision aids and training programs that can help people with Stargardt Disease to manage their low vision.

This content has been medically reviewed by Associate Professor Abhishek Sharma and is for information purposes only. Consult a medical professional for advice.