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Stargardt's disease

Know About Stargardt’s Disease

Stargardt’s disease, a common form of Macular Degeneration, is an inherited disease that causes loss of central vision and affects color vision and night vision. This condition is usually diagnosed in patients under 20 years of age. Signs and symptoms of the disease occur in late childhood to early adulthood, and over time they become worse. The prevalence of Stargardt’s Disease is 1 in 8,000 to 10,000 individuals.

This disease is inherited when both parents have one gene for the disease and one normal gene (autosomal recessive disorder); children then have 25% chance of inheriting the two recessive copies of the Stargardt gene (one from each parent). To inherit the disease, therefore, one needs to acquire both of the genes from both parents, otherwise one is only a carrier of the disease without it actually affecting them. Visual loss progression, after being diagnosed with the disease, varies from minimal to complete loss of the central vision.

The condition affects the retina which is located at the back of the eye. The retina is responsible for converting light into electrical signals which are then transmitted to the brain for further processing. More specifically, Stargardt’s affects a smaller portion of the retina, near its center, called the macula. The macula is responsible for the acuity of vision needed for detailed tasks (i.e. recognizing faces, reading, driving, distinguishing colors, etc). Stargardt’s Disease causes the development of a fatty yellow pigment which builds up in cells underlying the macula. Accumulation of the yellow pigment may result in damage to the cells that are essential for sharp vision.

Another form of this condition is autosomal dominant Stargardt disease. This type of the condition occurs in patients who inherit one dominant copy of the mutated gene; here it is enough for one copy of the gene to activate the disease (hence “dominant”). People who carry the autosomal dominant Stargardt will always be affected, unlike the autosomal recessive Stargardt carriers. Patients who carry this dominant gene are usually diagnosed later on in life.

Currently, there is no specific cure against this disease, however, with the help of low vision aids (such as binocular lenses and magnifying screens) and some training, the problems patients face may be minimized.

If you know anyone who has severe vision problems such as Stargardt Disease or other eye conditions such as Age Related Macular Degeneration, Diabetic Retinopathy, Glaucoma, Retinitis Pigmentosa, Nystagmus, and Albinism causing low vision, SB Optical CAN HELP!

SB Optical, which also specializes in low vision cases, carries special devices that may be suitable for you.

References:

Genetics Home Reference. (2013). Stargardt macular degeneration. Genetics Home Reference. Retrieved January 27, 2013 From: http://ghr.nlm.nih.gov/condition/stargardt-macular-degeneration

MacDonald, I. (n.d.). What is Stargardt macular dystrophy? The Foundation Fighting Blindness. Retrieved January 27, 2013, From http://www.ffb.ca/eye_conditions/stargardts.html?PHPSESSID=a445809548a15d20a621a5bf5df0f0c7

Retina International. (n.d.). Stargardt’s Disease. Retina International. Retrieved January 27, 2013, From http://www.retina-international.org/eye-conditions/retinal-degenerative-conditions/stargardt/

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