Retinitis pigmentosa type 26 (RP26) is one type of inherited eye disorders known as retinitis pigmentosa, causing a gradual loss of vision.
The problem lies in the cells that sense light in the eyes. People with RP26 often start having trouble with their vision in their early adult years. Night blindness is usually the first sign, and it can progress to losing side vision, creating “tunnel vision,” and eventually, central vision may also be lost. Many individuals may legally become blind in adulthood, although complete blindness is not very common. RP26 is a condition that only affects the eyes, and there are no other symptoms in the rest of the body. Currently, there is no cure for retinitis pigmentosa. However, it’s recommended to use sunglasses that block UV-A and UV-B rays, and certain optical aids can make eyes more comfortable. Some individuals might slow down the degeneration of the retina with vitamin A palmitate therapy. Counseling and lifestyle adjustments can also help individuals manage the progressive loss of vision.
RP26 is caused by pathogenic (disease-causing) variants in the CERKL gene and exhibits autosomal recessive inheritance. This means that both parents must be carriers to have a 25% chance to have a child with the condition. The risk of being a carrier is based on a person’s ancestry or ethnic background. For example, individuals of Sephardic Jewish descent have a 1 in 24 chance to be a carrier.
Other names for this condition are pigmentary retinopathy, rod-cone dystrophy, and tapetoretinal degeneration.
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Revised November 2023