Retinitis pigmentosa is a hereditary eye disorder that can already develop in children and young people. It involves the loss of light-sensitive photoreceptor cells (light sensing cells) and progresses from the peripheral areas towards the centre of the eye.
It is caused by the accumulation of metabolic waste products that can no longer be removed from the lower retinal layers. This ‘metabolic waste’, which is normally removed through the retinal blood vessels, builds up and first causes the death of rod cells found in the peripheral retina before advancing to the centre of the retina. Since rods are not present in the very centre of the retina, this area of sharpest vision is not affected. Patients experience “tunnel vision”, making it difficult for them to find their way around. To counter this adverse effect, special telescopic systems are used that provide a larger field of vision and help patients regain their independence and mobility.
One in five people carries a defective retinitis pigmentosa gene.