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Retinitis Pigmentosa

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What is Retinitis Pigmentosa?

Retinitis Pigmentosa is the name given to a hereditary disease of the retina in the eye. The retina is at the back of the eye and it acts like the film in a camera, receiving and processing everything you see. The retina is a delicate layer of cells which picks up the pictures and transmits them to the brain.

What causes Retinitis Pigmentosa?

Retinitis Pigmentosa could be caused by a breakdown in the function of the rods or the cones in some part of the retina. The retina is so large and complex that breakdowns may occur in a variety of ways. Retinitis Pigmentosa is not a single disorder but a large number of disorders. The breakdown of cone function is also sometimes called Macular Degeneration.

What are the symptoms of Retinitis Pigmentosa?

Different types of Retinitis Pigmentosa have different signs and symptoms. One early sign of Retinitis Pigmentosa is difficulty seeing at night or in dark rooms. Retinitis Pigmentosa also starts by causing tunnel vision. Other symptoms includes low light to dark adaptation times and poor contrast sensitivity.

Does Retinal Degeneration lead to blindness?

Some people with retinitis pigmentosa may become blind. However, most retain some small amount of vision and they are termed "blind" for legal purposes.

How quickly does sight diminish?

Each person has a different rate of sight loss. If the retinitis pigmentosa runs in the family, the pattern could be the same within that family. Usually the loss is gradual so you are able to adjust.

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Is Retinal Degeneration inherited?

Unfortunately in most cases the answer is yes. The most common form of inheritance is called recessive. In this type both the parents carry the genes which causes Retinitis Pigmentosa. They themselves are unaffected but there is a 25% chance that their children will have Retinitis Pigmentosa.

Another form of Retinitis Pigmentosa inheritance is X-linked. In X-linked Retinitis Pigmentosa, females carry the gene causing Retinitis Pigmentosa. When a female carrier marries an unaffected male, then there is a 50% chance that their sons will be affected, and a 50% chance that their daughters will be carriers.

X-linked Retinitis Pigmentosais the one form for which carriers can often be detected. In X-linked RP when an affected male marries a non-carrier female, their sons will not be affected but all their daughters will be carriers.

There is also a dominant form of Retinitis Pigmentosa which can strike in every generation of a family. Since Retinitis Pigmentosa runs in families, all members in an affected family are urged to have a thorough eye examination. When planning a family, expert genetic counseling should be sought.

Retinitis Pigmentosa