Retinitis pigmentosa produces a gradual deterioration of the retinal light sensing cells with clumping of the pigment cells in the retina. The most characteristic symptom is difficulty with night vision, and loss of side vision. Certain patients may have more problems with center vision and difficulty with bright lights. Difficulty with night vision is termed night blindness, often severely limiting function in the dark.
RP tends to run in families and a pattern of inheritance is often identified. The overall incidence in the United States has been calculated to be approximately 1:3700 with about 10% autosomal dominant, 84% autosomal recessive, and 6% X-Linked recessive inheritance. About 40% of RP patients have no known previous family history. Knowledge about the heredity is helpful in making decisions about marriage, occupation, and whether to have children. Considerable research is currently in progress towards finding the hereditary cause of RP. As these hereditary defects are discovered it may be possible to develop treatment to prevent progression.
Usher's syndrome is the name given to the association of RP with partial or complete congenital deafness. The incidence of Usher's syndrome is difficult to determine but surveys of patients suggest up to 10% of RP patients are deaf. The incidence of Usher’s syndrome is about three cases per 100,000; it is the most frequent cause of combined deaf-blindness in adults.
It has been reported that of vitamin A can slow of the progression of RP. Large doses of vitamin A may be harmful to the body and supplements of vitamin E alone may make RP worse. Vitamin E will not be harmful if taken with Vitamin A or in the presence of a normal diet. It is important to seek the advice of your ophthalmologist regarding safe vitamin use.
Low vision devices can frequently help affected individuals continue with many of the normal activities of daily life.
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