Overview
Retinitis pigmentosa (RP) is the name given to a group of hereditary eye disorders. These disorders affect the retina, which is the light-sensitive tissue lining the back of the eye, in which the first stages of seeing take place. In RP, sight loss is gradual but progressive. It is unusual for people with RP to become totally blind as most retain some useful vision well into old age.
About the Retina
The retina in your eye serves a similar purpose to a film inside a camera. Light is focused by a lens at the front of the camera onto a light-sensitive film at the back, to form a picture. In a similar way, light entering your eye is focused onto a light-sensitive tissue which lines the inside of the eye at the back. This tissue is the retina.
The retina consists of two main layers, a thin one called the pigment epithelium and a thicker one, made up of many layers of cells, called the neural retina. One particular layer in this neural retina contains many millions of cells called photoreceptors, which are able to respond to light. People with RP usually first notice difficulty seeing in dim lighting and gradually lose peripheral vision. The effect this disease has on vision can range from mild problems to blindness.
RP affects males more than females. Though it is frequently diagnosed during childhood, some people do not have noticeable symptoms until later in life.
Causes of RP
It is now known that there are many different inherited problems causing RP. In all RP-related conditions however, the ability of the retina to respond to light is affected. The problem can be in many parts of the retina such as the rod or cone cells, or in the connections between the cells of the retina.
Signs and Symptoms
The most common first symptom is difficulty in seeing in poor light, for example outdoors at dusk, or in a dimly lit room. A second symptom is reduction of the visual field, in which sight is lost from the sides, or from above and below. This is often referred to as tunnel vision and means that the rod cells, and some of the outer cone cells, have been affected first.- Difficulty seeing in dim lighting
- Tendency to trip easily or bump into objects when in poor lighting
- Gradual loss of peripheral vision
- Glare
- Loss of contrast sensitivity
- Eye fatigue (from straining to see)
Retinitis Pigmentosa Treatment
Currently, there is no standard retinitis pigmentosa treatment or therapy available to patients at our Florida practice. However, researchers are studying possible treatment options such as retinal implants and drug therapy. Work is also being done to isolate the genes that are responsible for the disease.However, co-ordinated research, in many centres around the world, has been expanding for around 20 years. Many of the genes responsible for the numerous types of RP have been located and their defects identified. Scientists are following many lines of research, among which is the development of a safe system for introducing corrective genetic material to the appropriate cells of an eye. There is some evidence that this will take hold and assist cell regrowth.
There have also been some very positive developments in stem cell and retinal cell transplantation. However, research in these areas is still in the very early stages. It could take a number of years before these new developments might become available as treatments.
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