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STARGARDT’S DISEASE WHAT IS STARGARDT’S DISEASE? Stargardt’s disease, also called juvenile macular degeneration or fundus flavimaculatus), affects approximately one in 10,000 people and is characterized by central vision loss early in life. This condition affects the retina, which forms a thin membrane lining at the back of the eye. The retina is light sensitive nerve tissue that converts images from the environment received through the eye into impulses sent to the brain. In people with Stargardt’s disease, one layer of the retina collects a substance called lipofuscin. This causes discrete yellowish round or fish-shaped flecks around the macula. As a result, vision loss in Stargardt’s is most intense in the macula, which is in the center of the retina and the concentration of the most sensitive vision. Additionally, the best color vision resides in the macula. Therefore, damage to the macula results in loss of visual acuity (definition and sharpness), decreased color vision and blind spots. SIGNS AND SYMPTOMS Stargardt’s is a progressive disease which usually shows up in children and young people within the first 20 years of life. A first sign of the disease may be trouble reading or blind spots. Initially, the blind spot or blurriness may be small but may gradually increase in size. A person with the condition may have reduced visual acuity, causing blurry vision. Most Stargardt’s patients have visual acuities from 20/100 to 20/400 (In the U.S., 20/200 or worse is considered legal blindness). Central vision in those with the disease is typically decreased but peripheral (side) vision usually remains stable. In late stages, color may also be affected. PROMINENT YELLOW FLECKS AND MACULAR CHANGES ( courtesy of Common Macular Cases) CAUSES AND TREATMENT Stargardt’s is most often an autosomal recessive Delete text and place here. inherited condition requiring the photo person to receive a gene from each parent to cause the disease. Recently, though, small number of families were found to have an autosomal dominant pattern of inheritance, requiring only one gene from either parent. Researchers have identified the recessive Stargardt disease is caused by mutations in a gene called ABCA4. A second gene called ELOVL4 has been found to be the cause of the dominant form of the disease. Unfortunately, there is no current treatment that has been proven to improve the visual loss or to slow the progressing of the disease. The Foundation for Fighting Blindness and Oxford Biomedical’s gene therapy treatment has been given orphan drug status and clinical trials began in late 2010. Additionally, Advanced Cell Technology received a patent for producing RPE cells from human embryonic stems cells and was granted orphan drug status. ACT announced FDA approval to begin clinical trials in November 2010. Developed by Molly Beasley STARGARDT’S DISEASE (CONT). IMPLICATIONS FOR STUDENTS In students with Stargardt’s disease, it is important to have adequate low vision care. Students may have light and glare control problems in their classrooms. Adaptations including sitting away from the window or providing window coverings may be necessary. Also, these students should be allowed to wear “sunglasses” when needed to decrease glare and light sensitivity. Large print materials may be indicated for these children. This may include textbooks, worksheets and tests. Due to their vision loss and difficulty reading, some students may require extended time on tests and quizzes. OVERALL PROGNOSIS Stargardt’s never causes total vision loss. Peripheral vision is left intact while central vision is usually in the range of 20 /100 to 20/400 with younger patients usually showing less loss. Low vision care can help Stargart’s patients lead very normal lives. NORMAL VISION Delete text and place photo here. REFERENCES American Macular Degeneration Foundation (n.d.). Stargardt Disease. Retrieved June 19, 2011 from http://www. macular.org/stargardts.html Branham, K,, Heckenlively, J., and Openshaw, A. (2008, February). Understanding Stargardt Disease. Retrieved June 19, 2011 from http://www.kellogg.umich.edu/ patientcare / downloads/Understand-Stargardt.pdf Common Macular Cases (n.d.). Stargardt’s Disease/Fundus Flavimaculatus. Retrieved June 19, 2011 from http://www.mrcophth.com/macula/stargardtdisease.html Deutman, A.F., (2003, January). Orphanet Encylcopedia. Stargardt’s Disease. Retrieved June 19, 2011 from http://www.orpha.net/data/patho/GB/uk-Stargardt.pdf Lighthouse International. (n.d.). Stargardt’s Disease. Retrieved June 19, 2011 from http://www.lighthouse.org/about-lowvision-blindness/childrens-vision/pediatric-eye-disorders/ stargardts-disease/ LATE STAGES OF STARGARDT’S (courtesy of National Eye Institute) Delete text and place photo here. Developed by Molly Beasley