What is Retinitis Pigmentosa?

Retinitis Pigmentosa (RP) is the name given to a group of diseases, which affect the retina of the eye. The retina, located in the back of the eye, is the part of the eye that acts like the film in a camera.  It is a delicate layer of cells which picks up the picture and transmits it to the brain - where "seeing" actually occurs. In RP, the retina begins to degenerate, which causes vision to diminish. 

One of the earliest symptoms of RP is difficulty seeing at night or in dimly lit places (night blindness). Later there is a reduction in peripheral vision.  As yet there is no effective treatment or cure for RP.  RP is the leading cause of youth blindness in Australia. It is second only to diabetes as a cause of adult blindness in working - age people in Australia.  About one in every 3,500 people are affected by RP.

Living with Retinitis Pigmentosa

Slow loss of sight is a very difficult thing to live with, especially as you may not receive the immediate understanding offered to the totally blind.  Indeed, many people will not believe that you have a problem because you have no apparent signs of visual impairment.  The first and hardest step towards living positively with a visual impairment is accepting it.  For the RP sufferer, that means knowing the extent and limits of your vision and using intelligently the visual clues you still receive.  A normally sighted person must do this too in certain circumstances.

The driver who plunges suddenly into a fog has two alternatives – he can decide that he can’t see a thing, panic and stop, which might cause an accident, or he can see (even if it is only the nearside kerb) and move cautiously down the road.  The RP sufferer too can panic or move on down the road.  Accepting that you have a visual impairment is never easy.  You may go through times of despair and of feeling resentful and bewildered.  All these reactions are quite understandable, especially as the very nature of RP makes adjustment difficult, but the way in which you deal with RP will determine the type of life you and your family will share from day to day.

Try to ignore it and you will suffer constant reminders that it is there.  Write yourself off as totally incapable and you will be missing out on many of life’s enriching experiences.  Tackle each new problem steadily as it arises, using clues from all your senses and you will find that you can live more positively with RP.

Everyone is Different

RP can manifest itself in different ways.  For some, visual loss is slow and there will be only slight loss, over perhaps ten years.  Others have periods of rapid loss, often with years in between of no apparent decline.  Still others have been aware of impaired vision from childhood or teens, when they had difficulty with ball games, especially at dusk.  Some will lose peripheral sight but will remain near-perfect central vision, while others will remain side vision but will have very blurred or otherwise defective central vision.

With so much variation in the symptoms and effects, it is not surprising that the public find RP difficult to understand.  If the RP sufferer can still see to read and can gaze into the distance and pick out landmarks and can respond to a smile, it is hard to appreciate that he or she may be visually impaired.  It is most important for mutual understanding, especially within the family, that the normal sighted person should recognize and comprehend the difficulties involved.  For example, a person suffering the early stages of RP may have almost perfect day vision but at night or in brilliant sunshine, or in rapidly changing light conditions, that same person may react as if almost totally blind.

Equally difficult to understand is the loss of part of the visual field.  As people with normal sight stare ahead, they can recognize objects on either side, above and below the point on which their eyes are fixed.  The facility is very useful, enabling them to be aware of more than that which is directly in front, thus keeping them alert and free from danger.  How often do we use the expression “I saw it out of the corner of my eye?”  It can be appreciated, therefore, that losing the boundaries of one’s vision is very alarming.

Genetic Inheritance

Find out how genetics play an important role for inherited eye diseases by clicking here