What is retinitis pigmentosa?

The eye is often compared to a camera. The front of the eye contains a lens that focuses images on the inside of the back of the eye. This area, called the retina, is covered with special nerve cells that react to light.

These nerve cells include the rods and cones. The rods and cones react to light because they contain pigments that change color when light strikes them. In some people, however, there is a problem with these pigments. The rods (and sometimes the cones) gradually stop working, and the retina begins to deteriorate.

There are several kinds inherited medical conditions that can cause this problem, which is called retinitis pigmentosa. According to The Foundation Fighting Blindness, there are about 100,000 people with retinitis pigmentosa in the United States.

Why do people get retinitis pigmentosa?

Today, doctors know a lot about how the condition is inherited, but researchers are still trying to find out exactly why certain combinations of genes cause retinitis pigmentosa.

How does the doctor know whether someone has retinitis pigmentosa?

Someone with retinitis pigmentosa will notice gradual changes in vision, including:

Retinitis pigmentosa usually affects both eyes. In some forms of the condition, vision continues to get worse. In other types of retinitis pigmentosa, only a small area is affected and vision might not change at all for several years.

Because the symptoms usually develop very slowly, someone with retinitis pigmentosa can lose quite a bit of vision before noticing anything is wrong. In fact, the eye doctor sometimes notices the problem first when he or she sees changes in the pigments when examining a patient's retina with a special instrument. These changes in the retina can start to occur as early as childhood or as late as middle age, depending on what type of retinitis pigmentosa the patient has.

What can be done about retinitis pigmentosa?

Unfortunately, there is no effective treatment to stop or cure retinitis pigmentosa. Research is being conducted, but there is currently not even an experimental medication or surgical treatment.

There is some research showing that certain vitamin supplements may help slow the loss of vision in some types of retinitis pigmentosa. Your doctor will be able to tell you more about this.

One thing that patients with retinitis pigmentosa can do is to keep all scheduled appointments with the eye doctor.

The doctor will check for changes in vision and recommend ways to use as much of the remaining vision as possible. Most importantly, the doctor will be able to treat any other conditions that could reduce vision.

Because retinitis pigmentosa is a genetic condition, patients who have children or who are planning on having a family should also get advice from a specialist in genetic counseling. There are also organizations that offer support and advice on living with retinitis pigmentosa, including The Foundation Fighting Blindness. Tel: 888.394.3937.

Does everyone with retinitis pigmentosa go blind?

There are many different types of retinitis pigmentosa, and not all of them cause a total loss of vision. The best way to keep and use as much vision as possible is to have regular eye examinations and follow the doctor's advice.

Reviewed by a Cleveland Clinic medical professional.

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