Stargardt Disease
What is Stargardt disease?
Stargardt disease is a rare inherited eye condition that happens when fatty substances build up on the macula, the small part of the retina that's crucial for clear, central vision.
Usually, people start to notice vision problems in childhood, but some might not see any changes until they’re adults. While there’s no cure for Stargardt disease right now, vision rehabilitation can help folks make the most of their remaining eyesight.
What are the symptoms of Stargardt disease?
The main symptom of Stargardt disease is a slow decline in central vision in both eyes. The speed of this vision loss can really differ from person to person.
Other symptoms might include:
Gray, black, or blurry spots in the center of your vision
Increased sensitivity to light
A longer time for your eyes to adjust when moving between bright and dim places
Color blindness
Some people with Stargardt disease might also notice a decrease in their peripheral (side) vision.
What causes Stargardt disease?
Stargardt disease usually comes from mutations in a gene called ABCA4. This gene is important for how our bodies handle vitamin A.
Vitamin A helps produce cells in the retina, which is the light-sensitive layer at the back of the eye. The ABCA4 gene makes a protein that helps get rid of the fatty substances that can build up. When this gene isn’t working right, it leads to a buildup of fatty material in yellowish clumps on the macula. Over time, this can damage the light-sensitive cells and affect central vision.
Stargardt disease is an inherited genetic disorder, so it gets passed down from parents to their kids.
How will my doctor check for Stargardt disease?
An eye care professional can check for Stargardt disease during a dilated eye exam. This process is pretty simple and painless; your doctor will use eye drops to widen your pupils and then look for signs of Stargardt disease, like yellowish spots on your macula.
Your doctor might do some extra tests to diagnose Stargardt disease or keep an eye on your symptoms:
Color vision assessment. Since Stargardt disease can cause color blindness, your eye doctor may also check your color vision.
Fundus photography. Your eye doctor might take a picture of your retina to look for yellowish spots on your macula.
Electroretinography (ERG). This test helps your eye doctor see how well your retina responds to light.
Optical coherence tomography (OCT). This exam uses light waves to create a detailed image of your retina.
Genetic testing. Your doctor might suggest a genetic test to confirm the diagnosis of Stargardt disease.
What's the treatment for Stargardt disease?
Right now, there’s no specific treatment for Stargardt disease. But there are some steps you can take to help slow down vision loss:
Wear a hat and sunglasses to protect your eyes from sunlight when you’re outside.
Avoid dietary supplements that have more than the recommended daily amount of vitamin A.
Don’t smoke and try to stay away from secondhand smoke. Some studies suggest this might help slow down vision loss in people with Stargardt disease.
Dealing with vision loss and low vision from Stargardt disease can be tough. The good news is that there are resources available to help, like low vision aids and rehabilitation programs.