• What Is Stargardt Disease?

    May. 27, 2021

    Stargardt disease is an eye disease that causes vision loss in children and young adults. It is an inherited disease, meaning it is passed on to children from their parents.

    Stargardt disease is often called juvenile macular dystrophy. In people with Stargardt disease, special light-sensing cells in the macula, called photoreceptors, die off. Central, or detailed, vision becomes blurry or has dark areas. It may also be difficult to see colors well.

    Symptoms

    Stargardt disease usually develops in children, teenagers, and young adults. Someone may first notice a problem with their central vision. It can be blurry, distorted or have dark areas. Side (peripheral) vision is usually not affected. Some people may have trouble seeing colors.

    It may take longer than usual for vision to adjust when going between bright and dark areas.

    For some people, Stargardt disease progresses slowly, then speeds up and levels off. At about 20/40 vision (meaning someone sees at 20 feet what a normal-seeing person sees at 40 feet), vision loss can speed up. Vision may rapidly get worse until it reaches about 20/200. After this point, vision generally stays about the same.

    While central vision will be lost, many people with Stargardt disease may keep good side vision for the rest of their lives.

    Who is at risk for Stargardt disease?

    Usually Stargardt disease is passed down from parents. With the classic Stargardt disease, faulty genes (the ABCA4 gene) must be passed down from both parents to have symptoms. Someone who has the gene from only one parent will be a carrier for Stargardt disease, but will not have symptoms. Other forms of Stargardt disease need a gene from only one parent to cause symptoms, but those are very rare.

    Diagnosis

    Your ophthalmologist will dilate (widen) your eye’s pupils to look at your retina. People with Stargardt disease have yellowish flecks called lipofuscin in and under the macula. Sometimes these flecks extend outward in a ring. Lipofuscin are fat deposits from normal cell activity. These deposits build up more in people with Stargardt disease than in other people.

    A test called fluorescein angiography may be used. In this test, a dye is injected into your arm. The dye is photographed as it circulates through the retina’s blood vessels. In people with Stargardt disease, the photos show a dark area within tissue of the retina. This helps the ophthalmologist diagnose Stargardt disease.

    Genetic testing is now available to precisely diagnose what type of macular degeneration a patient has. This is the surest way to know the genetic basis for your condition.

    Treatment

    Unfortunately, there is no available treatment for Stargardt disease. But there are several gene therapy and drug therapy trials going on. With genetic testing, you will know your faulty gene, and can look for and decide to take part in a current trial.

    Wearing sunglasses may help with the bright light sensitivity of Stargardt disease. Wearing sunglasses can also prevent further retina damage from the sun’s harmful ultraviolet (UV) rays.

    People with Stargardt disease should not smoke cigarettes or be around cigarette smoke. Also, some studies suggest that taking a lot of vitamin A could may make the disease worse. While the vitamin A in foods is fine, avoid taking large doses of it as a supplement.

    There are genetic treatments already for similar diseases. Doctors are hopeful that Stargardt disease can be treated soon as well. There are helpful resources for people adjusting to vision loss. These can include special devices, useful tips for daily living and training to help you get around. Ask your ophthalmologist to help you find low vision resources.

    If you have any questions, be sure to ask. Your ophthalmologist is committed to protecting your sight.