Eye Health Central

Stargardt Disease

Stargardt Disease

Stargardt disease is acquired genetically and responsible for deteriorating vision in young people who have inherited it. Usually detected before the individual turns 20, Stargardt disease impacts about 1 in 10,000 people. The disease works by slowly destroying the macula, which is the thin film on the center of the retina that makes sharp, clear central vision possible. Much like macular degeneration in the elderly, Stargardt disease is progressive and usually results in legal blindness. On the positive side, Stargardt does not affect any other body system, giving patients with this diagnosis otherwise normal health and typical life expectancies.

Symptoms of Stargardt Disease

Stargardt Disease

One of the first hallmarks usually noted in children and teens with this disease is progressively worse visual acuity. The individual may also complain of wavy or blurry vision, impaired color vision, an inability to adapt to low light and, as the disease progresses, blind spots in their central vision. Glare is especially bothersome to those with Stargardt, making cloudy days and soft lighting indoors helpful. As central vision fades, patients will need to rely more on their peripheral vision which is usually retained to some degree.

Inheritance of Stargardt Disease

Most people with Stargardt received the genes from both of their parents being carriers. This means neither parent actually has Stargardt, but they both carry the potential for it in their genes. When two such people have a child, there is a 25 percent chance of the child having Stargardt and a 50 percent chance the child will also be a carrier. In pairings where one parent does have Stargardt disease, the chance of the child inheriting it rises to 50 percent. For this reason, coupled with the young age of onset, patients diagnosed with Stargardt will likely need psychological counseling and, possibly, to speak to a genetic counselor about their future family planning.

Treating Stargardt Disease

Unfortunately, there are no treatments available for this disease. While there is promising research involving stem cells, gene therapy and a new form of vitamin A, none have come to fruition yet. This doesn’t mean this disease is hopeless, however. There are steps patients can take to slow the progress of their disease and adapt to lowered vision, as well as therapy for the emotional and psychological issues that can arise from receiving their diagnosis.

To slow disease advancement, there are several actions patients can take. First, complete avoidance of cigarette smoking and second-hand smoke is important. Second, avoiding large doses of vitamin A is recommended. While there’s no risk from vitamin A found in food, vitamin supplements need to be carefully monitored to ensure no more than the daily recommended dose of vitamin A is taken. Lastly, the patient can wear dark sunglasses while outdoors to avoid UV exposure and even more damage to the macula.

As this disease is usually found during childhood and the teen years, counseling and therapies are integral. The patient will need to learn to adapt to assistive devices as soon as possible, as legal blindness is a common outcome. The patient may want to talk about the emotional issues involved with going blind with a qualified therapist. It’s not uncommon for some patients to request to speak to a genetic counselor to learn the details regarding future family planning. All of these discussions are integral to patient wellness and should be offered at the first sign of emotional distress or as soon as a diagnosis is reached.

Author: John Dreyer Optometrist Bsc(Hons), MCOPTOM, DipCLP
Created: 26 Jun 2017, Last modified: 4 Mar 2020