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Retinitis pigmentosa is a rare disease in which the light-sensitive retina of the eye slowly and progressively degenerates, eventually causing blindness. Retinitis pigmentosa is an inherited condition of the retina in which specific photoreceptor cells, called rods, degenerate. The loss of function of these rod cells diminishes a patient's ability to see in dim light and with time can also diminish their peripheral vision. Retinitis pigmentosa is probably caused by mutations in at least ten different genes.
Not much is known about the cause of retinitis pigmentosa, except that it is inherited. Sometimes the disease is dominant, meaning only one gene needs to come from a parent for the child to develop the disease. Other times the disease is X-linked, meaning that only one gene passed from the mother will cause the child to develop retinitis pigmentosa. On occasion, males will develop a related hearing loss. Rods, the light-sensing cells of the retina that are responsible for vision in low amounts of light, gradually deteriorate so seeing at night and in low levels of light becomes more difficult.
Early signs of retinitis pigmentosa are worsening night vision and a shrinking field of vision. This disease often begins in early childhood and worsens over time. During later stages of retinitis pigmentosa, a small area of central vision remains, along with slight peripheral vision. Patients with retinitis pigmentosa (RP) may present with varying symptoms. The onset is often gradual and insidious, and many patients fail to recognize the manifestations of this condition until it has progressed significantly. When patients do report symptoms, they commonly include difficulty with night vision (nyctalopia) as well as loss of peripheral vision. Many patients with RP also experience photopsiae as the disorder progresses; typically they report small flashes of light or a twinkling, shimmering sensation in the midperipheral or peripheral field. These are believed to represent aberrant electrical impulses from the degenerating retina.
Retinitis pigmentosa has no treatment or cure, although some practitioners believe that vitamin A may slightly delay vision loss. Occupational therapy may be wise before much of vision is lost. It may be easier to learn how to work around vision loss while vision is still available. Two treatments for the disease are currently under investigation. The first is an intraocular lens retinal prosthesis, in which a computer chip is implanted in the retina to determine if electrical stimulation of the retina aids the vision of people diagnosed with retinitis pigmentosa and age-related macular degeneration. To date, about 15 procedures have been performed on an experimental basis, and research is expected to take at least five more years. The second is a new use for the drug diltiazem, commonly known by the trade names Cardizem and Tiazac, which is prescribed for high blood pressure and angina. Diltiazem has delayed blindness in mice with a condition resembling retinitis pigmentosa. Researchers caution retinitis pigmentosa patients not to begin taking the drug on their own in hopes of preventing vision loss, and note that the drug levels given to the mice were much higher than what is prescribed for heart conditions. Side effects of the drug include headache, dizziness and a slowed heartbeat. Researchers are unsure if the diltiazem will be as effective in slowing human blindness as it has in mice.
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Source(s): All above information & images are based on an article written by Gretchyn Bailey allaboutvision.com and from other sources. All rights reserved by respective owners. For our full credit and copyright information please view our Credit List.
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As many as 90 per cent of people visually impaired, live in developing countries, and approximately 80 per cent are blind from diseases which are either curable or preventable. These statistics portray a needless tragedy of millions of human beings trapped in their lonely world of darkness. [submit fact]
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