Stargardt disease is a genetic condition that causes vision loss. There is currently no cure for the condition, but rehabilitation can help people manage their symptoms.

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Stargardt disease affects the central part of the retina — the macula — which is important for central vision. The area is responsible for central vision sharpness and most of the eye’s ability to see colors.

Individuals with Stargardt disease rarely lose all of their central vision or any of their peripheral vision. Therapy can help people manage their symptoms and continue their daily activities.

This article explores Stargardt disease, its symptoms, causes, and possible risk factors. It also discusses diagnosis and treatment and answers some frequently asked questions.

Stargardt disease is a rare genetic condition that causes vision loss. The condition usually develops in children, teenagers, or young adults. Another name for the condition is juvenile macular dystrophy.

Stargardt disease damages the macula resulting in a ring of white or yellow spots around the retina. Damage to the macula can occur with age, causing vision loss in older adults. However, Stargardt disease typically emerges early.

The disease results from irregularities in a single gene that causes the buildup of fatty material around the retina. This buildup prevents the retina from properly functioning and causes a loss of central vision in both eyes.

Stargardt disease primarily causes central vision loss. Central vision can become blurry, distorted, or include areas of darkness. These symptoms can cause difficulties seeing straight ahead but typically have little effect on peripheral vision.

People with the disease experience symptoms at different levels of severity. Some people may experience a slow decline in their vision, while others will rapidly lose their central vision.

The disease can also cause other symptoms, such as:

This disease results from changes in the ABCA4 gene, which affects how the body uses vitamin A. The ABCA4 gene produces a protein that cleans up excess fatty material from when the body uses vitamin A to make retinal cells.

Stargardt disease prevents the ABCA4 gene from producing this protein, which leads to fatty materials accumulating on the retina.

Stargardt disease is a rare condition. The Macular Society in the United Kingdom states that only 1 in 10,000 people get it.

It is also a genetic disease, meaning someone must inherit the condition from their biological parents. Both parents must typically pass down the faulty ABCA4 gene to produce symptoms. However, rare forms of the disease only require one parent to pass down the gene for symptoms to occur.

Genetic tests are available to identify the genes that can cause Stargardt disease. These tests may provide information about whether someone carries the gene but are sometimes inconclusive.

A doctor or eye care professional will diagnose the disease by examining the fatty materials around the retina. They may also assess the extent of the vision loss through tests that include:

  • Visual field testing: This assesses the range and sensitivity of a person’s vision. It can also detect blind spots.
  • Electroretinography: This measures the electrical signals in the retina in response to light. Abnormal responses to light are a sign of disease, including Stargardt disease.
  • Optical coherence tomography: This produces images of the retina by using light waves.
  • Autofluorescence imaging: This involves a special camera that measures retinal cells to check for unusual patterns.

There is no cure for Stargardt disease that prevents the macula from degenerating. However, the National Eye Institute suggests some tips for slowing vision loss that include:

  • wearing a hat and sunglasses while outside
  • avoiding dietary supplements that contain more than the recommended daily amount of vitamin A
  • avoiding smoking

They also highlight the possibility of future treatments that use gene therapy to treat the condition. Genetic treatments are available for other eye diseases, and the American Academy of Ophthalmology (AAO) is hopeful similar treatments will soon become available for Stargardt disease.

Metformin is a medication that could also potentially slow vision loss in people with Stargardt disease.

People who have Stargardt disease may find the experience stressful and could benefit from emotional support.

Below are some frequently asked questions (FAQs).

What is the progression of symptoms with the disease?

According to the AAO, the progression of symptoms varies from person to person, and the ability to distinguish details and shapes — visual acuity — may deteriorate slowly.

Experts class typical vision as 20/20, but once someone reaches a visual acuity of 20/40, it may rapidly decline to 20/200.

Are there any natural treatments?

There are currently no natural treatments that work for Stargardt disease. However, the National Eye Institute states the importance of avoiding supplements with more than the recommended daily amount of vitamin A.

What age does it usually start?

Stargardt disease typically starts in childhood but can also emerge in teenagers and young adults.

Stargardt disease is a rare genetic condition that causes central vision loss. It occurs in children, teenagers, and young adults. The condition worsens central vision over time, but peripheral vision is typically unaffected. Symptoms can develop slowly in some people and more rapidly in others.

There is currently no cure for the disease, but research is ongoing into how gene therapy can treat the symptoms. People with Stargardt disease can slow the deterioration of their vision by wearing a hat and sunglasses while outside and avoiding smoking.