Retinitis pigmentosa (My Experience of Retinitis Pigmentosa)

Member Name: jo1l

Product:	My Experience of Retinitis Pigmentosa
Date:	08/07/01 (155 review reads)
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Retinitis pigmentosa ( RP) is a heridetary condtion that effects the retina in the eye.In RP the lost of vision is gradual but progressive ie it can be a very slow process. Most people will not go totally blind with this condition, but will obviously have visual problems.


The retina

If the eye was to be described as a camera, then the retina is the equivelant of a film for the eye. It is made up of several different layers which pick up the diffrent images a person sees and they are then transported along the visual pathway in the brain and turned into images. Sorry thats a bit basic but I could spend hours on explaining the visual pathway to you.

The retina is a structure that has several different layers to it. One layer is made up of rods and cones. Rods enable us to see in dim or dark conditions the eye has around 120 million of these. They are located away from the centre of the retina. Cones enable us to see fine details and colours and are located in the centre of the retina. together they pick up varying amounts of light and these images are transported to the brain via the optic nerve.

What are the symptons of RP?

The commonest first symptons of RP is a difficulty in seeing in poor light eg at dusk or in poor lite situations and a gradual reduction in a persons prepheral vision or visual field ie what you can see to the side of you without turning your head.
This indicates that the rods in the retina are affected.

For some suffers it is a lost of central vision that is effected ie they have problems with detailed work, reading etc. Ie the cones are effected. But the commonest first symptons are the problems with dim light and the lose of parts of the prepheral vision.

With all suffers the condition is a progressive condition, the lose of vision varies from individual to individual and at what speed the vision is lost varies.

When does it develope?

The majority of RP
suffers will notice early symptons between the age of 10 and 30. others may develope symptons a little earlier or later on in life.

What causes RP?

Rp is an inherited disorder. With both a dominant and recessive inherited pattern and and an X chromosone inherited pattern.

Dominant inheritance

This pattern of inheritance effects both males and females and the suffer inherits it directly from a parent. The risk of it being picked up by a child who's parent has RP is 50%

Recessive inheritance

With this pattern of inheritance, there is usually no none suffers of the condition in either sides of the family. both parents will be carriers of RP but will not be suffers. There is therefore a 25% risk of any child being born going onto devolpe RP

X linked inheritance

This pattern means that any male children are at risk developing RP and female children are at risk of becoming carriers. A female who is a carrier has a 50% of any male children developing RP and a 50% risk of any female children becoimng carriers.

How is it detected?

The sufferer may complain of visual problems as detailed earlier or a routine check of the back of the eye - the fundus may show obvious signs. When a person has RP the fundus is not the normal orangey colour it is in a healthy eye instead patterns of dull black or brown pigmented areas can be seen. A visual field test would also show loss of prepherall vision.

Is there any treatment for RP?

Unfortunately there is not any known treatment for RP at present. The genes that cause RP have been identified but no none treatment to prevent or arrest the progress of RP has been found as yet.

Associated other disorders:

It has been found that some RP suffers can go onto develope Ushers syndrome. Ushers syndrome causes hearing loss in the affected individual.

Further information

Further information on this condi
tion can be gained from the British Retinitis pigmentosa Society (BRPS). They do a range of booklets which include a guide to RP, Genetics and RP and young people with RP.

BRPS P.O box 350, Buckingham MK 18 5EL
Tel 01280 860195 Helpline 01280 860363.

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