Stargardt Disease

Stargardt disease is an eye condition that affects the macula and side parts of the retinas in your eyes. The macula is the part of your retina that lets you see objects directly in front of you (your central vision). Stargardt disease makes you lose your central vision over time. It can also affect your peripheral vision.

Stargardt disease is a type of macular degeneration. Most people with macular degeneration develop it later in life (after age 60). This is called age-related macular degeneration. Stargardt disease usually affects children and adults younger than 20. That’s why healthcare providers sometimes call it juvenile macular degeneration or juvenile macular dystrophy. It’s also known as fundus flavimaculatus when it affects the edges of your retina.

Stargardt disease happens when your body can’t get rid of a fatty yellow pigment called lipofuscin. If you have Stargardt disease, your body makes too much of it. The extra lipofuscin collects in your retina. It damages special cells called photoreceptors. Eventually, the damage permanently affects your vision.

Symptoms of Stargardt disease

Stargardt disease will cause noticeable changes in your eyes and vision, including:

• Blind spots or cloudy areas in your vision
• Blurry vision
• Light sensitivity
• New color blindness
• Trouble adjusting to changes in lighting
• Worsened night vision

Stargardt disease symptoms are usually progressive. This means they get worse over time. Eventually, you’ll lose some or all of your central vision. You might still be able to see out of the sides of your eyes. But you won’t be able to clearly see objects directly in front of you.

Stargardt disease causes

Stargardt disease is a genetic condition. It happens when there’s a genetic change in the ABCA4 gene. This gene helps your retinas work correctly.

Stargardt disease is hereditary. Biological parents pass it to their children. Stargardt disease is passed through families in an autosomal recessive pattern. That means both biological parents need to have a changed ABCA4 gene for their biological children to develop Stargardt disease.

How doctors diagnose Stargardt disease

An eye doctor will diagnose Stargardt disease. They’ll give you an eye exam to check your vision and eye health. Tell your provider when you first noticed changes in your vision or other symptoms.

You might need a few tests, including:

• Electroretinography (ERG)
• Fluorescein angiography
• Fundus photography and fundus autofluorescent photography
• Genetic testing
• Optical coherence tomography (OCT)

Stargardt disease stages

Your eye care specialist may classify Stargardt disease with stages as it progresses, including:

• Stage 1. Flecks of excess lipofuscin form in your macula. You might have mild symptoms.
• Stage 2. The flecks have built up enough to spread beyond your macula to other areas of your retina around it. Symptoms will be more noticeable.
• Stage 3. The flecks have absorbed back into your macula and damaged it (they cause atrophy). This will cause worsening symptoms.
• Stage 4. The atrophy in your macula is severe enough to erase some or all of your central vision.

How is Stargardt disease treated?

There’s no treatment to reverse Stargardt disease or the damage it causes. Your eye care specialist will suggest ways to manage symptoms and slow down the vision loss, including:

• Avoiding dietary supplements that contain vitamin A (getting too much vitamin A may speed up vision loss if you have Stargardt disease)
• Glasses, contacts and/or specific low vision aids
• Quitting smoking (and other forms of nicotine, like vaping)
• Wearing a hat or sunglasses to protect your eyes when you go outside

Is Stargardt disease curable?

Your eye care specialist can help you manage Stargardt disease, but you can’t cure it.

Researchers are conducting clinical trials to find ways to treat all types of macular degeneration (including Stargardt disease). Ask your eye care specialist if you’d be a good fit for a clinical trial. This can help you get access to new, experimental treatments.

When should I seek care?

Visit a healthcare provider or eye care specialist as soon as you notice any changes in your eyes or vision. Stargardt disease can share symptoms with other, more common conditions. It’s important to get any eye issue diagnosed as soon as possible.

What can I expect if I have Stargardt disease?

You should expect to lose your central vision. Most people experience vision loss slowly over several years. It sometimes takes decades. But it can happen faster. How your ABCA4 gene is changed can affect how quickly you lose your vision. This can also control how much of your vision you lose. Your eye doctor will help you understand what to expect.

You’ll need regular eye exams to monitor changes in your eyes. Ask your eye care specialist how often you should schedule routine exams and any follow-up tests.

How do I take care of myself with Stargardt disease?

You can take care of yourself in many ways by eating a nutritious diet and getting enough exercise. Don’t smoke. Deal with stress as it comes. Keep your regular schedule of appointments with your healthcare providers.

When should I see my healthcare provider if I have Stargardt disease?

Your eye care specialists will want to see you on a regular basis. Make sure you keep those appointments. However, if you have any changes in vision or any type of pain, you should contact your provider right away.

What questions should I ask my healthcare provider if I have Stargardt disease?

Here are some questions you may want to ask your provider if you have Stargardt disease:

• Can you suggest a support group for me?
• Am I eligible to take part in a clinical trial?
• Should I speak to a genetic counselor?
• Can you suggest things that will help me cope with low vision?