Early Onset Macular Degeneration

Macular degeneration is an eye condition that causes damage to the macula, which is part of the retina. The macular plays a role in central vision. Central vision is essential for all types of activities, from driving to reading. 
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The most frequently occurring form of macular degeneration is age-related. According to the American Academy of Ophthalmology, age-related macular degeneration (AMD) is the number one cause of vision loss in people over the age of 50. 

Although macular degeneration is more commonly a disease of middle-aged or older adults, it can also develop in younger people. Early-onset macular degeneration is a disease that affects children, adolescents, and young adults. 

​What is early-onset macular degeneration?

Stargardt disease is a form of macular degeneration that has an early onset. The onset of the disease can occur between birth and young adulthood, but symptoms most often start in childhood or during the teen years. Stargardt disease is also called juvenile macular degeneration.

The condition is considered a genetic form of macular degeneration. To develop symptoms, a person must have inherited a gene called ABCA4 from both parents. A person that inherits the gene from only one parent is considered a carrier of the disease but will not have symptoms. Similar to AMD, Stargardt disease causes damage to the macula and a loss of vision. 
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How does age-related macular degeneration differ from Stargardt disease? 

AMD occurs more often in people over the age of 50. Stargardt disease affects much younger people and most often starts in childhood. Also, Stargardt disease is an inherited condition, while AMD can develop in people that do not have a family history of the condition. 

Symptoms

Symptoms of early-onset macular degeneration usually start in childhood or the early teen years. According to the National Eye Institute, people that have an earlier onset of the disease tend to experience more rapid vision loss.

Although it is uncommon for people with the disease to become completely blind, central vision loss does occur. According to the Bright Focus Foundation, by age 50, about half of people with Stargardt disease will have acuity of 20/200 or worse.

People with Stargardt disease may experience the following symptoms:

• Blurry vision
• Sensitivity to light
• Color blindness
• Gray or hazy spots in the central vision

Eventually, central vision can be lost. Usually, side vision remains intact. 
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Treatment 

According to the American Academy of Ophthalmology, currently, there is no cure for Stargardt disease. There is also no treatment to slow the progression of the disease.

Eye doctors may recommend a few things to help manage the condition. For example, wearing sunglasses can help people with the disease cope with light sensitivity. It can also prevent further damage to the retina.

It is also essential that people with the condition do not smoke and avoid secondhand smoke, as smoking can make the disease progress faster. According to the AAO, some studies indicate that taking large doses of vitamin A may make the disease worse. Although it is OK to eat foods containing vitamin A, it may be best to avoid supplements that contain it.

Low vision rehabilitation may be helpful for people with Stargardt disease. Since vision loss starts young, it can interfere with education. Learning how to use low vision devices may help. Low vision rehab can help teens and young adults with the disease in the form of vocational training and tips for daily living.

Research and various clinical trials are ongoing to find a way to treat Stargardt disease, so vision loss does not occur. These include trials to test treatments, such as stem cell therapies and lipofuscin blocking drugs, to determine their effectiveness.

Treatment for similar genetic diseases is available. Researchers hope that Stargardt disease will also be treatable in the near future.

If you have any questions about any form of macular degeneration, including Stargardt disease, please give us a call. Also, remember it is essential to get regular eye exams to help diagnose eye diseases early. If you would like to schedule an exam with one of our eye doctors, please phone us at 508-746-8600.