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.
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.
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:
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.
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.
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.
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.
There are steps you can take to maximize your remaining visual function and enjoy your life to the fullest after vision loss.
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.
See our page on the driving laws in Tennessee with low vision.
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:
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.
Don't smoke and stay away from passive smoking. There is some evidence that this may slow down the progression of Stargardt disease.
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.
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. Call at (423) 710 3965 to schedule a low vision evaluation with our low vision optometrist.
Vision therapy is well worth the response, time and effort. Our Son had a hard time focusing and writing neatly. After vision therapy Seth could complete a task in half the time if previously took. His abilities to focus improved greatly and so did his handwriting.
Also, he was better at listening. As a parent, we wanted learning to be fun for our Son, and vision therapy made this possible.
Vision Therapy is well worth the expense, time and effort. Our son had a hard time focusing handwriting neatly. After vision therapy Seth could complete a task in half the time it previously took his abilities to focus and improved greatly and so did his handwriting.
Also, he was better at listening. As a parent you wanted learning to be fun for our son and vision therapy made this possible.
Vision Therapy has given or son the tools he needs to be able to scan and read the written word more effectively and efficiently.
He love working with John and these working sessions give him the motivation to gladly work on his homework assignments.
It amazed us to see the difference in the tracking of his eyes and along a line of it's from the beginning to the end of the treatment.
Dr. McBryar , Kristen and John are all marvelous and we would recommend them to anyone I only wish that we would have found them sooner!
Prior to coming to the institute for vision development my son complained of daily headaches. Therapy has eliminated his headaches completely. I love knowing my son is able to learn pain-free for the rest of his life because of the work that has been done over just a few weeks in this office. He he absolutely loved coming that didn't even feel like going to a doctor or therapy. We are grateful for the relief he was able to find by coming here.
Thank you!
Seems much less frustrated with life
Reads non-stop and fast
Spelling abilities have been hugely improved
Seems much more confident
Thank You!!!
When we first came to the practice, my son did not have huge issues, but lots of small ones- trouble with reading, sports (hitting the baseball consistently), coordination, etc- that were not easily detectable,but when put together presented issues. The staff here were all very professional and loving towards him. While I was concerned in the reading, my son was concerned with the athletic side. Seeing improvement night off the bat gave him confidence that i have nor seen in him and made him want to not only Come to therapy, but also made him want to work hard. He has surpassed his grade level and above in reading and hit his first home run this year! Thank you!!!
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