ENCYCLOPEDIA DETAIL

DISEASE

Retinitis Pigmentosa

Rods and Cones Retinitis Pigmentosa
Retinitis pigmentosa is one in a group of diseases classified as hereditary chorioretinal dystrophies. There are multiple forms of retinitis pigmentosa, each occurring as a result of a specific abnormal gene. The disease is inherited in a number of different ways. Genetic testing and counseling are very helpful in determining the form of the disease, how it has been inherited, and what treatments are available or are being researched. Retinitis pigmentosa is usually diagnosed during young adulthood and can be difficult to accept. Knowledge is power, and information about retinitis pigmentosa can empower a young adult to learn to live with this condition.
In contrast to other chorioretinal dystrophies, retinitis pigmentosa results in the loss of night vision and peripheral vision first and will eventually affect central vision as well. During the progression of the disease, cellular malfunctions cause the destruction of rods, which are the photoreceptors responsible for vision in low light conditions. Rods are interspersed throughout the retina, which is why peripheral, or side, vision loss occurs. Vision loss will sweep inward, until only a small, central area of vision remains. Most people with retinitis pigmentosa are legally blind by 40 years of age.
There is no cure for retinitis pigmentosa, but it is an area of promising research. Technology continues to improve upon low-vision aids. Gene therapies, delivery methods of agents to rods and cones, and the effects of vitamin A and DHA on the progression of the disease are currently being studied.
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