RETINITIS PIGMENTOSA

What is retinitis pigmentosa?

Retinitis pigmentosa is a degenerative eye disease, which affects the light receptors of the retina, known as the rods and cones. As the disease progresses, these light receptors are destroyed, reducing the eye’s ability to detect light. It usually does this from the outside inward, but in some cases receptors may be affected from the centre outward, or in sporadic patches. The most challenging aspect of retinitis pigmentosa is that it is not stable. Sufferers must continually adapt to less and less sight, and the impact this has on their lives and relationships.

Normal View NORMAL VIEW
Ritinitis View VIEW WITH RETINITIS PIGMENTOSA

What causes retinitis pigmentosa?

Retinitis pigmentosa is an inherited condition which involves both eyes. If it starts in one eye, the other eye usually develops the same condition later. A thorough genetic history is advisable, often with the aid of a genetic counselor, to determine the risk of future generations acquiring the disease.

Retinitis pigmentosa is usually diagnosed during the teenage years but may be present at birth. Cases that are diagnosed later in life are often milder and may progress more slowly. The disease is sometimes associated with other genetic conditions.

What are the signs and symptoms of retinitis pigmentosa?

The patient first notices increasing difficulty with night vision, followed by difficulty with peripheral vision, often years later. Slowly progressive constriction of the visual field eventually leads to 'tunnel vision'. A small area of central vision in both eyes usually persists for years but in the late phases central reading vision may also be lost.

How is retinitis pigmentosa diagnosed?

Retinitis pigmentosa is primarily diagnosed by examination. The two tests essential in the diagnosis are visual field testing, which detects loss in peripheral vision, and electrophysiological testing, which is usually performed by an ophthalmologist.

What is the treatment for retinitis pigmentosa?

As of now there is no specific cure for retinitis pigmentosa. Vitamin A therapy has been recommended for many RP patients, based on research dating back to the early 1990s. Other studies have shown that a diet rich omega-3 can slow the progression of the disease. Such a diet includes oily fish such as salmon, tuna, herring, mackerel, or sardines.
Stem cell research is being conducted, with some success being reported in rats. In addition, researchers have started testing defects in genes to clarify the basic cause for retinitis pigmentosa and assist in ultimately finding a treatment. As the visual field constricts, efforts are being explored for the use of visual field expanding glasses.

Living with retinitis pigmentosa

Lorie H was diagnosed with retinitis pigmentosa at the age of 18. Now at 41, this mother of two young boys lives in San Francisco and runs a business from her home. Immediately after being diagnosed, Lorie was embarrassed to share the news of her condition. Today, she is involved in raising funds and creating awareness of the disease, her latest initiative being to swim from Alcatraz to San Francisco.
Although she tries to remain as positive and independent as possible, there are daily reminders of her condition, such as tripping over and walking into things out of her line of vision. In spite of this, Lorie is optimistic about the progress being made in research.

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