Stargardt disease affects the macula, an area of the retina with the highest concentration of light-sensing cells. Although there is no cure, there are ways to slow its progress and preserve vision.

Stargardt disease is caused by gene mutations that lead to lipofuscin build-up in retinal pigment epithelial cells and gradually destroy central vision.

1. Wear Sunglasses

Stargardt’s disease is a hereditary condition that causes the loss of central vision in the macula, a small part of the retina (light processing tissue lining the inside of the eye). This condition is caused by changes in a gene called ABCA4. The function of this gene is to produce a protein that disposes of waste from the processing of Vitamin A in the eye. Changes in this gene result in the accumulation of fatty deposits on the retina that cause the degeneration of photoreceptors and lead to vision loss. It is estimated that the average person with this condition experiences gradual visual loss beginning in early adulthood. Useful vision may remain for some time, but eventually enough photoreceptors are lost to lead to legal blindness.

There is no cure for this disease, but there are steps that can be taken to slow down the progression of the disease and help patients manage their condition better. The first step is to protect your eyes from ultraviolet rays by wearing sunglasses when going outdoors. This can be especially important for people who have this condition because ultraviolet rays can damage the retina and increase the risk of vision loss.

Other ways to reduce the symptoms of this condition include taking vitamin A supplements and eating a diet rich in fruits and vegetables. These supplements can provide the nutrients needed to support healthy eyes and a stronger immune system. It is also recommended that patients who have this disease seek out a low-vision specialist to discuss the best strategies for managing their condition.

Recently, there has been a lot of talk about a potential treatment for this disease called MCO-010. This drug has shown promise in preserving central vision in a clinical trial of six Stargardt’s disease patients. The results were presented at the 2023 Retina Society conference and published in the journal Ophthalmology. In this study, the subjects were administered MCO-010 and underwent a 24-week trial period. During this time, they underwent an ocular and systemic safety evaluation. They were also tested on various vision functions using ETDRS charts, an octogenarian-specific macular perimetry, Octopus visual field perimetry and the multi-luminance shape discrimination test.

2. Manage Your Diet

Stargardt disease is an inherited condition that affects the retina (light processing tissue lining the inside of the eye). The disease affects the macula, which is a special part of the retina responsible for sharp central vision. Over time, the disease causes the light-sensing cells (rods and cones) in the macula to degenerate. This causes central vision to gradually fade away. Useful vision can remain for some years, but eventually the loss of photoreceptors becomes too severe to be useful.

Research shows that a diet rich in fruits and vegetables, especially those containing antioxidants and zinc, may slow the progression of the disease. In addition, foods containing omega-3 fatty acids have also been shown to improve the visual quality of those with the disease.

The disease is caused by a variety of sequence variants in the ABCA4 gene, which provides instructions for making a protein that plays a crucial role in visual function. Mutations in the gene cause a buildup of a toxic substance called lipofuscin in the retinal cells, causing gradual macular degeneration. These yellowish clumps of fatty material kill the retinal pigment epithelial cells, which in turn leads to a progressive loss of central vision.

3. Consider Low-Vision Aids

Fortunately, Stargardt disease can be treated and managed. Patients can reduce their risk of vision loss by avoiding UV radiation, consuming a healthy diet high in vitamins and minerals (including zinc, lutein, and zeaxanthin), and managing stress. Other measures include wearing protective eyewear and seeing an ophthalmologist who specializes in inherited retinal diseases.

Patients with Stargardt disease experience a gradual decline of central vision and color vision due to damage to the retina’s cone receptor cells. These cells are concentrated in the macula and are responsible for allowing us to see detail and perceive colors. As the disease progresses, these cells become blackened and lose their ability to function. Eventually, the disease causes a “hole” or blind spot in a patient’s central vision, limiting their ability to drive, read, and perform other visual tasks.

While there is no treatment to restore lost vision, low-vision aids can help patients maintain their independence and quality of life. These aids can include magnifying glasses, telescopes, and special software for computers and mobile devices that can enhance vision. These devices can make it easier to read, cook, and navigate. They can also help patients enjoy hobbies like gardening and socializing with family and friends.

The cholinergic medications, such as echothiophate iodide and pilocarpine, have been used for years to treat glaucoma and strabismus. However, recent studies show that they can also improve the vision of patients with Stargardt disease and other inherited retinal disorders.

In one case study, a patient with Stargardt disease experienced significant BCVA and color vision improvement by using a cholinergic medication. This was accomplished by applying a low dose of the drug to the affected eye. This resulted in a substantial improvement in the patient’s quality of life.

A comprehensive eye exam by an ophthalmologist who specializes inherited retinal diseases can identify the signs and symptoms of the condition and determine the best treatment option for each patient. A blood test can also confirm a diagnosis and help identify carriers who may not develop the disease but can pass it on to their children.

4. Schedule an Eye Exam

To slow the progression of Stargardt disease, regular eye exams with an ophthalmologist specializing in retinal diseases is crucial. Your eye specialist can detect early symptoms and suggest specific treatments to slow vision loss.

Stargardt macular degeneration is a hereditary condition characterized by progressive loss of central vision in both eyes, due to fatty deposits accumulating on the macula–the part of retina responsible for sharp, central vision–becoming increasingly dense over time. Most cases first emerge during childhood years, though symptoms can appear later on in adulthood as well.

Gene mutation in the ABCA4 gene produces proteins to clear waste materials from the retina; when this gene mutates incorrectly, these proteins no longer function correctly and fatty waste material accumulates in yellowish clumps in the macula, killing light-sensitive cells and thus impairing central vision.

Ophthalmologists will perform several tests to assess your vision. A fundus photograph will allow them to observe the retina’s condition and detect evidence of eye diseases or conditions; other tests include electroretinography (ERG) which measures function in your retina and optical coherence tomography (OCT), which creates detailed images of it.

An ophthalmologist can also perform blood tests to check your vitamin A levels and identify any issues affecting your vision, while genetic tests can also be administered in order to look for genetic mutations that might cause these conditions.

After two years of transition from military service, she received a letter report from an ophthalmologist indicating her visual acuity had significantly declined since her last exam and diagnosed with Stargardt macular degeneration and legally blindness. To combat her vision loss further, her ophthalmologist prescribed dilute echothiophate iodide daily and recommended taking measures to improve the quality of what vision remains.