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Definition of Retinitis Pigmentosa

Retinitis Pigmentosa (RP) is defined as a hereditary, progressivedegeneration of the neuroepithelium of the retina characterized by nightblindness and progressive contraction of the visual field. RP refers to agroup of hereditary disorders that affect the retina's ability to respondto light. It primarily affects rod cells, or the photoreceptorsresponsible for night vision and seeing in dim light. Rod cells alsoaccount for peripheral vision. Cone cells can also be affected as thedisease progresses. Cone cells are responsible for color vision and seeing RP is a relatively rare condition. It affects an estimated fiftythousand to one hundred thousand individuals in the United States.Worldwide, RP is estimated to affect 1.5 million people. The incidence of Ocular signs start with the breakdown of rod cells. Rods are presentwithin the outside macula, or center of the retina. The peripheral retinais predominately composed of rod cells. Symptoms of RP usually manifestbetween the ages of 10 and 30. First, night vision decreases along withthe ability to adjust to dim lighting. When the rod cells and outer cone


org/retinitis[v] Rivolta et al (2002) "Retinitis Pigmentosa and allied diseases" HumanMolecular Genetics 22/10 (p. Insome cases, the retinal pigment epithelium (RPE) of the retina is affectedas well. The progressive nature of the disease presents several milestones inthe life of the patient. While actions canbe taken to compensate for the loss of sight, the more serious consequenceis the patient's self-image. Schooling and Retinitis Pigmentosa The difficulties in vocational choices for individuals affected byRetinitis Pigmentosa are mirrored in the challenges they face in acquiringan education. It creates obstacles to healthy, lasting relationships. Asthe condition worsens, other important moments will be the loss ofmobility, such as driving privileges, or the loss of the ability to read. However, even patients withextremely poor vision can compensate for this by increasing the font sizeon their monitor, or using a special magnifying glass. Etiology of Retinitis Pigmentosa Retinitis Pigmentosa is caused by a genetic defect composed of threedistinct patterns. By and large, public schools lack the resources and trainingnecessary to fit the needs of these students. Especially in families where otherchildren are attending public school, the RP child sometimes expresses adesire to attend public school to be like the "normal" kids. Since RP has such severe consequences in both areas,those who are diagnosed with it must take the necessary time to pondertheir vocational choices.

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