What is Stargardt disease?
Stargardt diseases, also known as fundus flavimaculatus or Stargardt's macular dystrophy (SMD), is a form of juvenile macular degeneration that affects children and young adults. It is a disease that affects 1 in every 10,000 people. Patients have difficulty with central vision and may even have difficulty in focusing on objects in dim lighting. While these two are the most common and significant symptoms of this genetic condition, many patients have had issues where the vision is blurred or distorted. In order to help the patients who suffer from Stargardt disease, it is important to understand the cause of this disease.
The primary cause of Stargardt disease is gene altercation. The macula, which is the crucial part of the retina, where light-sensing cells are developed, gets wasted away due to the condition. As time passes by, the central area of the retina becomes surrounded with various yellow and white color spots. The retinal pigment epithelium (RPE), which is a very important part of the retina, is also adversely affected because of Stargardt disease as lipofuscin (which is basically the waste material) gathers in the eye. Photoreceptor cells that send information to the brain to help you see properly are damaged as a result.
Stargardt disease can be inherited in two forms - dominant or recessive. In the majority of cases, this disease is inherited in a recessive form, meaning the condition is inherited from both parents. This can be true even if both parents don't have the disease but are able to pass down the faulty gene to the child. In this form of inheritance, the chances of the child suffering from the disease are about 25 percent, and these chances are regardless of the gender and birth order.
What are the symptoms of Stargardt Disease?
The symptoms start to show up before the age of 20 (somewhere between 6 to 12 in most cases).
- Central vision is first effected and deteriorates over time. Peripheral vision remains intact.
- Blurry vision
- Difficulty recognizes familiar faces
- Issues in seeing when the lights are dimmed
- In later stages, color vision can be lost
What are ways to manage Stargardt Disease?
The research has shown that bright light and UV rays from the sun can further damage the retina if suffering from Stargardt disease. It's important to avoid ultraviolet light. Those with the disease are recommended to wear sunglasses with 100% UV ray blocking whenever going outside.
Possible cures and treatments for Stargardt Disease
At the moment, there is, unfortunately, no treatment for Stargardt disease. However, at our Low Vision Clinic in Burlington, Vermont, we offer a number of aids and tools that can help make the life of the patient much more manageable.
The most common support tool is electronic eyeglasses which can help tremendously, the patient can not only read but also drive with the help of these glasses. While Stargardt might be more severe in some cases than others, these glasses are custom made for each patient. The use of these glasses for driving is legal in most states. If properly made and used, these glasses can help a patient live a normal, independent life.
Doctors are working on a treatment for this disease. Stem cell treatment that recreates the damaged phoropters is being researched. However, because Stargardt's is a genetic condition, it can take a couple of years until patients can actually undergo stem cell or some genetic modification therapy.