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Stargardt’s disease

Stargardt disease is the most common form of juvenile macular degeneration. While there is no cure for it, there are options that can improve visual function.

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What is Stargardt disease?

Stargardt disease affects children and young adults over time by impairing vision and causing retinal degeneration. More than 30,000 people in the United States are affected by Stargardt Disease, the most common form of inherited juvenile macular degeneration. With Stargardt disease, progressive vision loss is caused by the degeneration of photoreceptor cells in the central portion of the retina called the macula.

The onset of Stargardt disease typically occurs during childhood or adolescence, however it can also start much later in life. Juvenile onset is defined as starting before age 21, adult onset is defined as beginning between 21 and 40 years, while late onset is defined as starting at age 41 or older.

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Symptoms of Stargardt disease

One of the most common symptoms of Stargardt disease is a progressive loss of central vision in both eyes. Central vision loss occurs more quickly in some people than in others. Peripheral vision is typically preserved to some extent.

There may also be other symptoms, such as:

  • You can see gray, black, or hazy spots in the middle of your vision
  • Light sensitivity
  • Adjusting your eyes to bright and dark environments takes more time
  • Color blindness

Risk factors for stargardt disease

A child is usually at risk of developing Stargardt disease if their parents have it. Stargardt disease is caused by faulty genes (the ABCA4 gene) that must be passed down from both parents. A person who inherits the gene from only one parent will carry Stargardt disease, but won't exhibit symptoms. Some forms of Stargardt disease require a gene from just one parent to manifest symptoms, but they are extremely rare.

Inheritance of stargardt disease

Inheritance of stargardt disease

It is believed that genetic conditions are inherited either dominantly or recessively. 

Dominant form

A dominant form of a disease occurs when only one faulty gene comes from one parent. If a parent has a faulty gene, there is a 50% chance that he or she will pass it on to their children. The chance for each child is the same regardless of his or her gender and order of birth.

Recessive form

The other form is recessive. In this case, both parents are responsible for passing on the defective gene. In the recessive form, neither parent has the condition, although both carry the faulty gene. Recessive inheritance has a 25% chance of passing on the condition to each child. The chance for each child is the same regardless of his or her gender and order of birth.

Usually, Stargardt disease is inherited as a recessive trait. A gene called ABCA4 on chromosome 1 is associated with this form of Stargardt disease. There is a less common dominant form of the disease caused by alterations to another gene called ELOVL4.

Inheritance of stargardt disease

Inheritance of stargardt disease

As part of the clinical diagnosis, eye doctors perform visual acuity tests, visual field tests, electroretinography (ERG), fundus autofluorescence (FAF), and optical coherence tomography (OCT), which reveal macular anomalies and yellow-white fishtail flecks that are usually restricted to the central macula.

Fluorescein angiography can also be used to diagnose stargardt disease. It involves injecting dye into your arm. As the dye circulates through the retina's blood vessels, it is photographed. The photos of people suffering from Stargardt disease reveal a dark area within the retinal tissue. In this way, the eye doctor can diagnose Stargardt disease.

There is now genetic testing available to determine exactly what type of macular degeneration a patient has. It is the most accurate way to determine your condition's genetic basis.

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Treatment of stargardt disease

Treatment of stargardt disease

Currently, there is no treatment available for Stargardt disease. There are multiple clinical trials evaluating the efficacy of different treatments for stargardt disease including trials looking at gene therapy, cell therapy, and pharmacology.

How can a low vision optometrist help?

There are steps you can take to maximize your remaining visual function and enjoy your life to the fullest after vision loss. 

Driving with stargardt’s disease

A large number of Americans are at risk of losing their ability to drive due to eye conditions, like macular degeneration and stargardt's disease, which can cause them to lose their license. Having such conditions can have a profound impact on mobility, employment, and independence.

While each state has its own laws, in most states, someone with vision loss can drive with the aid of bioptic telescope glasses or other devices that improve their vision.

A bioptic telescope consists of a pair of glasses with a telescope attached above your normal line of sight. These can be attached to your regular eyeglasses and can be used either for one or both of your eyes. Bioptic lenses magnify images just like binoculars and help you see things better. Using these lenses allows you to see things that are much further away, which is one of their main benefits.

Enhancing contrast and reducing glare

One of the ways that a low vision optometrist will help a patient with Stargardt’s is through the reduction of glare and the improvement of contrast. This can be done through the use of colored tints which filter out certain wavelengths of light or through the use of digital devices such as handheld or tabletop CCTV’s.

The following tints are effective at reducing glare and increasing contrast for low vision patients:

  • Amber tints in light, medium, and dark shades are effective at reducing glare and increasing contrast.
  • Light, medium and dark Infrared-blocking Amber colored tints significantly increase contrast and reduce glare.
  • An orange tint significantly increases contrast and reduces glare on overcast days, making it a good overcast filter

Sunglasses and UV protection

The bright light sensitivity associated with Stargardt disease can be reduced by wearing sunglasses. Sunglasses can also protect your retina from UV rays that cause retina damage. It is not recommended to take more vitamin A than the daily recommended amount.

Smoking

Don't smoke and stay away from passive smoking. There is some evidence that this may slow down the progression of Stargardt disease.

Inheritance of stargardt disease
Inheritance of stargardt disease

Why you or your child needs an optometrist who specializes in low vision

When someone loses their vision, especially at a younger age, it can lead to severe impairment in their quality of life. A low vision optometrist will help guide the patient in the process of improving their remaining vision with the help of low vision devices such as magnification, telescopes, high-tech devices, CCTV and lighting.

In many cases, people who use low vision devices can read, drive, sew, play cards, work, go to school, watch TV, use a computer or cell phone, and more, which they could not do without them. Since every patient has unique needs, it is imperative that you speak to a low vision optometrist, because they are capable of helping you find the best low vision aid tailored to your needs.

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Common Questions

In some cases, students with stargardt's disease can be considered to have a vision disability. Visually impaired people and students need to know what their rights and responsibilities are, as well as what schools and universities are responsible for.
People with stargardt disease can experience slow degeneration and progressive vision loss, but it is rare for them to go completely blind. Visual loss can progress at different rates and in different degrees from person to person and even from family to family. A study in 2018 showed that although many patients with Stargardt disease lose visual acuity to the 20/200 to 20/400 range, and some lose visual acuity beyond 20/400, none of these patients reached either light perception or no light perception.
Stargardt’s disease
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Summary

There are more than 30,000 people in the United States who suffer from Stargardt Disease. Stargardt disease is the most common form of juvenile macular degeneration that affects children and adults. This condition affects the macula, an area of the retina responsible for sharp, central vision. Stargardt disease is characterized by progressive loss of central vision in both eyes. As of now, there is no cure for Stargardt disease, although there are many clinical trials exploring different treatment options. A low vision optometrist will help a patient with Stargardt to maximize their remaining vision by guiding them on the many different devices such as magnification, electronic devices, lightning, tints, and prisms that enable each patient to regain their independence.

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