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Retinitis Pigmentosa

Description of Retinitis Pigmentosa

The disorder can be caused by defects in any of several different genes which have recently been identified. Retinitis pigmentosa commonly runs in families. The cells controlling night vision, called rods, are primarily affected, but other types of the disease with mainly damaged retinal cone cells are also known. Dark pigmentation may be present on the retina. As the disease progresses, peripheral vision is lost as well and may lead to blindness. Signs and symptoms often first appear in childhood, but visual incapacity usually does not develop until early adulthood. The main risk factor is a family history of retinitis pigmentosa. It is an uncommon condition affecting about one in 4,000 in the U.S.

Symptoms of Retinitis Pigmentosa

The symptoms of this group of disorders usually become apparent between the ages of 10 and 30, although some changes may become apparent in childhood. In one type of RP, Leber's Amaurosis, children may become blind or almost so, within the first six months of life. By contrast, some types of RP may only show symptoms late in life. In the more common types of RP, a person will have a history of visual problems at dusk or in poor light, so-called night blindness and a gradual reduction in the field of vision, loss of the outer edges, resulting in a tendency to trip over things. In some cases RP is first diagnosed following a road accident. The first reaction amongst many people is to go for an eye test believing that they need glasses. Although glasses do a marvellous job correcting problems with the eye's lens, they cannot help a deteriorating Retina. As the outer fringes of the field of vision gradually disappear, the RP sufferer is left with the condition commonly referred to as tunnel vision. Many people retain this tunnel of useful vision until quite late in life. The speed of loss of vision varies considerably from person to person, due to the many forms of RP. In one category of RP, the loss of vision begins in the central area. This is referred to as macular dystrophy (because the central macular area is affected). It causes an inability to read or carry out other work involving detail, although the person may have few mobility problems for several years.

Treatment of Retinitis Pigmentosa

There is no effective treatment for this condition. The use of sunglasses to protect the retina from ultraviolet light may have a vision-preserving effect. Though controversial, recent studies have indicated that treatment with antioxidant agents such as vitamin A palmitate may delay the progression of this disease. Referral to a low vision specialist is very helpful in maintaining patient independence. Regular visits to your eye care specialist are important to monitor for the development of cataracts or retinal swelling, both of which can be treated.

What the world looks like for a sufferer of Retinitis Pigmentosa

Retinitis Pigmentosa