Wednesday, 3 February 2010

How I would describe Usher Syndrome.

Firstly, I'd just like to say, I'm totally and utterly gobsmacked at the response to this blog so far! To have total strangers say that it's good implies that it might well be worth carrying on! (People I know have said it too, but you always wonder if they're just being kind!)

Secondly, big thanks to my two spelling nazi's! You know who you are, and although there were just TWO mistakes I'm so glad you pointed them out!

So, I've told you the tale of how I got diagnosed with Usher Syndrome, and thought before I carry on with the next part of the story I should tell people what it actually is.

I'm going to focus on Usher Syndrome Type 2, because thats what it is that I have, and I am talking about me..... Me, me, me, me, me, me, ME!

There are two ways of explaining what Ushers is, there's the medical way, and then there's my way. I'll start off with the medical way... Well, I'll cut and paste the description from the Sense website!

Usher syndrome Type 2
People with Usher 2 are born with a partial hearing loss and then develop a visual impairment called Retinitis Pigmentosa (RP) in their teens. This may not be diagnosed until their early twenties or even much later.

What does this mean?

People are born with a moderate to severe hearing loss which often remains stable throughout their life and is generally helped by hearing aids. Problems with vision are first noticed at night followed by increasing difficulty with side or peripheral vision. At times this may lead to bumping into or falling over people and objects. People may notice difficulty moving into bright light from a darker place or vice versa. However, they may have adapted to these changes already, particularly in familiar situations.

What does the future hold?

In most cases people retain good reading vision well into middle age. There is no treatment available for Usher at the moment. Research is taking place in a number of universities in Europe and the USA, which is looking into the types of Usher, the genes that are involved and what messages the genes are passing to the cells. However, much can be done to help people make the best use of their vision and hearing.


Most people with Usher type 2 will be born with a partial hearing loss and so will probably use speech and lipreading in addition to a hearing aid to communicate. In many cases, attending mainstream school means that they are likely to feel part of the hearing rather than the deaf world.

So there you go, the rather glib, and unemotional description of Usher Syndrome, and to be honest, the kind of reply I usually give to people when they ask me about it and what it is. It's easier than getting all deep and sometimes a little teary in the middle of a pub!

Now here's my way of explaining what it is.....

It's a pain in the arse and I hate it...........!

Hate it, hate it, hate it!!!

There are, as far as I can see (that was an intentional pun by the way!), no sodding benefits to it at all! Not like there are with the deafness, if I fancy some time out from the world I take my hearing aids out and can have a whole day with no interruptions. I wont hear the front door, I wont hear the phone, I wont hear the sound of police cars screaming past my house, I wont hear the kids in the garden down below. It's just peace and quiet! And as soon as I want back in the normal world, in go the aids and I'm back!

I HATE being partially sighted.

I HATE not seeing in the dark.

I HATE not seeing things just outside my field of vision, when my field of vision is so bloody narrow.

And I HATE the fact it's an 'invisible' disability, so that people who don't know me or just don't know about the Ushers think I'm thick as two short planks! People are in general far more sympathetic to things they can see..... A wheelchair, a long white stick, walking sticks.....

But when they can't see why someone walks slap bang into a low wall, or when they ask 'How are you?' the reply they get is 'No thanks I just had a cuppa' the assumption, more often than not, is 'Hmmm..... Bit simple that one!'

It does make me an interesting and slightly eccentric person to know apparently, as conversations can go off on random tangents, walks can suddenly become far more dramatic as I fall down rabbit holes and twist an ankle!

I've found since the diagnosis, that I'm actually far more aware of the things I don't see. You learn, fast, to know your way around places, your home, your road, your families home, your friends homes, the places you go to often. You even learn to remember when there's a mess, so that when you wake up in the middle of the night to go to the loo you can get there without turning on lights, and without falling over the shoes you just threw off because you couldn't be bothered to put them away properly.

It irritates me no end when things aren't as they should be, or rather how I remembered them to be. Coming home late the night before bin day is really really really annoying! I know where every branch sticks out, I know where every paving slab is a little uneven..... Tuesday evenings when it's got dark and walking down my road its CRASH, walked into a wheelie bin, two seconds later WALLOP, walked into another wheelie bin, two seconds later SMACK, another fecking wheelie bin, and so on and so on and so on!

My legs are constantly bruised, my knuckles usually have some kind of graze or mark, and my pride and dignity is often really dented!

It's a totally and utterly pointless and dare I say it cruel thing to have, the sight thing far more than the hearing thing. I think, in fact I'm pretty certain I would do ANYTHING not to have it.

That's all for now folks,I feel I've rambled enough for one day and next week I shall carry on with the 'story'!


  1. Great post. And as a fellow deaf-blindie, I can really relate to it. Although, being blind, I'm fortunate in that it is visible.
    but I do think the visual impairment sucks far more than the hearing loss, too.

  2. Yes, Usher sucks, but we just do what we can day to day. I have US2, too. Not happy with it, but what do you do? I am at the stage where I walk with the white cane or my guide dog. I have just about no peripheral vision left and the central isn't as clear as it used to be. I can barely read hard copy, but large bold print can help. I read on the computer with a black background and white font (inverted or reverse colors or high contrast). I can barely tell color differences (black or navy? olive green or gray? orange or fushia/hot pink?, even light yellow or light pink? Brown or purple?) It does suck. We just have to carry on and do what we can. I also have bilateral cochlear implants now. It just seems that I feel "blinder" when I can't hear, though there are days when it's good to just "stay off the air" for a few hours to just relax and not have to listen to the noisy world. I wish you well. I blogged for a time and then stopped for a while. I might go back to it, but not yet. I know when I was in my late 20s and early 30s I was in denial for a long time, then I kept thinking, oh, okay, I can handle this, but as it kept getting worse, you just face the fact that you do have RP and it's not going to go away. It's a long process, but there is life after Usher.