My Aniridia

I was born 1980 with Aniridia. Though aniridia is a genetic condition, I did not inherit it from my parents, my family does not have a history of eye conditions let alone aniridia. So when my parent inquired about my unusually dark eyes they were told that I was just a dark baby and I would have dark brown eyes and they had no reason to assume otherwise, though they were never happy with that conclusion. I developed just like any normal baby, though as a small baby I seemed very sleepy but nothing too out of the ordinary. Then one day When I was about a 1 year old, my mum took me and my older brother the optometrist and when he looked into my eyes he saw what was really wrong, he sent us to the hospital where i was diagnosed with Aniridia.

Aniridia is a greek word meaning "no iris" I had unusally dark eyes because I only have a pupil, I don't have the colour ring around it, the part of the eye we refer to when we say "I have blue eyes" and so on. This is often the first sign that people have aniridia, and it makes people with aniridia sensative to light, glare distorts vision, and some people, like me find it very uncomfortable and it sometimes leads to migraines.

But aniridia is more than just missing an iris, one of the pair of PAX6 genes that control the development of the eye does not work so the eye cannot develop as well as most peoples eyes. Because of this people with aniridia are born with and a prone to developing an number of other eye conditions. I have been quite fortunate in that for most of my life the conditions That have affected my vision I have had since birth, I therefore, do not really miss the vision that I have never had.

I have macula hypoplasia, which means that a tiny part of the retina (back of the eye) that sees fine detail is undeveloped, it has less cells than it should and some blood vessels growing over it, which they shouldn't, as a consequence my vision for detail is low, I find it difficult to look at specific objects in detail. My vision is described as 6/36 (20/120) This means that for what the average person can see at 36 metres I have to be 6 metres away to see in the same detail it can be also described as 17% of the average persons vision. I find it hard to read normal sized print and I cannot recognise people that I know unless they are very close to me or I hear their voice. However, My peripheral vision is good so I am very aware of my general surroundings and do not need the help of a cane to get around. Though sometimes you may see me bump into objects because I do not have depth perception, so I need practice in knowing how far away object are from me and each other, some times I don't always get it right and that is when I bump into things or trip over. Also I'm not very good a catching on the first attempt but with practice i can catch a ball every time!

I have no depth perception because the vision in each of my eyes is not equal, the vision in my left eye is better than my right eye, so my brain tends to favour the pictures it receives from my left eye, rather than the pictures from my right eye. Because of this I have strabismus more commonly known as a "squint" or "cross eyed" for me my right eye tends to turn in and up. I had operations on both my eyes to try and correct this when I was 2 and 3 years old and I was also had my left eye patched for a while. The treatment wasn't 100% successful however, it mostly corrected when I wear glasses, or contact lenses. Also my left eye is dominant but I have tendency towards being right handed (though I'm also do a lot of things left handed). because the cross in dominance, my hand to eye co-ordination is not great, and my handwriting is readable but not particularly neat,

I also have Some Corneal pannus Which is when the white of the eye takes over the clear cornea at the front of the eye. Fortunely the Pannus only covers 2mm around the edge of my eye and is not growing, so it doesn't affect my vision.

While a number of common condition are associated with aniridia, as underdeveloped eyes are fragile and tend to age early they are also proune to other minor eye conditions. In 2002 I had a vitreous detachment. This is when the gel like substance in the middle of the eye becomes brittle and breaks away from the back of the eye, the retina. Some times the vitreous breaking away from the retina can cause a tear in the retina and this leads to a retinal detachment. Fortunely this has not happened to me. The Doctors said that my vitreous was too small for my eyes and then tension between the retina and the vitreous was too much and it eventually broke away from each other. I now have lots of debris floating around in my vision. I can see them and they often get in the way when I'm looking at something.

I am also very longsighted (+5.5, +6.0) and I have an Astigmatism (an uneven cornea) but neither of these are realated to my aniridia.

Having no family history of aniridia, as a child I was at a high risk of getting Wilms' tumour and having learning difficulties, which are part of the WARG syndrome, However, I managed to have a very healthy normal childhood, I have always been to mainstream schools and I Graduated University with a 2:1 in international relations and I am now studying for a law masters in International Human Rights Law. I have never used my aniridia as an excuse to underachieve and never let it get in the away of doing the things I enjoy.

To help cut down glare and to correct my longsightedness I have contact lenses with iris shape on them to help reduce glare. I use a monocular to read road signs, various magnifine glasses to help me read as well as a computer with a scanner and screen magnifyer/ reader programme called Zoom Text. I also have a magnifying mirror to help me see myself a little better when putting make up on and that sort of thing.