Stargardt’s Disease



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Stargardt’s Disease
Stargardt’s Disease is a form of juvenile macular dystrophy that begins early in life.

It includes a group of inherited diseases causing light-sensitive cells in the inner back of the eye (retina) to deteriorate, particularly in the area of the macula where fine focusing occurs. Central vision loss also occurs, while peripheral vision is usually retained.


Diagnosing and Causes

Stargardt disease is the most common cause of macular degeneration and central vision loss in young people. Vision loss generally begins to show up before the age of twenty, usually in early childhood.

Stargardt’s is usually a recessive inherited condition requiring the person to receive a gene from each parent to cause the disease. It is not likely they will pass it on since a gene from both parents is required. With the disease, the macula and surrounding retina are affected. The macula is the very center of our retina. The retina concentrates the most sensitive vision in the center. It is also where color vision resides. So, damage to the macula results in loss of visual acuity or sharpness of vision, decreased color vision and small blind spots.
Symptoms

Many patients have yellow flecks in their retina.

The macula, often has a shiny appearance, called a “beaten bronze appearance”. A dye injected into the arm follows a course through the blood vessel system beneath the retina.

When the dye cannot be seen, it takes on an unusual appearance called a dark choroid which means there is a blind spot. There is also noted sensitivity to light.

Symptoms can include blurry or distorted vision, inability to see in low lighting and difficulty recognizing familiar faces. In late stages of Stargardt’s color vision also may be lost. As Stargardt’s progresses, patients may experience small areas of blind spots. As images fall upon the damaged areas of the retina, objects may disappear and reappear causing a come and go effect.
Adaptations and School Issues

Stargardt’s creates central blindspots that increase in size as the disease progresses.

Patients must learn to turn their eyes in a specific direction to see around the blindspots. They learn how to move the eye so that the object is imaged onto a seeing part of the retina. They need to look slightly above what they want to see. However, this increases the distance between the retina and the center of the macula, so larger letters are needed in order to see. Make sure large print materials are available in textbooks, worksheets and tests. Extended time should also be offered as reading may be difficult and slow going due to their vision loss.

A variety of low vision devices can be helpful. Stargardt’s patients respond well to magnification. Simple bifocals can be helpful, as well as CCTV systems.

There is also great difficulty adjusting to dark after being in the sunlight. Most of this can be lessened by wearing hats and using sun filters when outside.

Adaptions should include sitting away from the window or closing shades if light and glare becomes a problem. Some children or adults may need to wear sunglasses inside to decrease glare and light sensitivity.

Parents and teachers should be aware of safety issues for children playing sports. Sports with fast moving projectiles are dangerous as the child may not be able to see them in time. With very young children, glasses should be worn for protection from unexpected injury.

Depth perception is also affected. Depth perception is dependent on two good eyes and when even one eye is affected it can cause depth perception problems.

Far peripheral vision remains intact, and while patients may not be able to see faces, they can see clearly around this and because of this, family members or teachers may think they can see better than they claim. Be aware of central vision loss and accept what child says they can see.

Good News

Stargardt’s never causes total vision loss. Peripheral vision is left intact. Low vision care can help patients lead normal lives. Support groups and counseling are beneficial.

Patients can adapt and have busy, active lifestyles. Some patients are even able to drive, becoming bioptic drivers, depending upon the rules in each state regarding the minimum visual acuity required to get a restricted license.
Treatments

There is no known treatment for Stargardt’s disease at this time. People with this condition are often advised to wear sunglasses with UV protection to reduce the possibility of additional eye damage caused by the sun. Research is ongoing.


References:

Drs.Richard and Laura Windsor (2004, April 9) stargardt’s. Retreived July 3, 2010 from



http://www.visionww.org/drswindsor-stargardt.htm.

Stargardt’s Disease. (n.d.) Retrieved July 3, 2010 from

http://www.allaboutvision.com/conditions/stargardts.htm.

Hoover Services for Low Vision and Blindness (2006, March) Stargardt disease



Retrieved July 3, 2010, from http://www.mdsupport.org/library/stargardt2.html.

Stargardt Disease (n.d.) Retrieved July 3, 2010, from .

http://www.macular.org/stargardts.html.

Stargardt Disease (n.d.) Retrieved July 3, 2010, from

http://improvevision.in/improve-vision/gotopage?page=stagardt_macular_dystrophy.
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