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Living with Usher Syndrome

My name is Lynne and this is about living with Usher Syndrome.

What is Usher Syndrome?

Usher syndrome is a condition characterised by hearing loss or deafness and progressive vision loss. The loss of vision is caused by an eye disease called retinitis pigmentosa (RP), which affects the layer of light-sensitive tissue at the back of the eye (the retina).

Vision loss occurs as the light-sensing cells of the retina gradually deteriorate. Night vision loss begins first, followed by blind spots that develop in the side (peripheral) vision. Over time, these blind spots enlarge and merge to produce tunnel vision. In some cases of Usher syndrome, vision is further impaired by clouding of the lens of the eye (cataracts). Many people with retinitis pigmentosa retain some central vision throughout their lives, however.

Researchers have identified three major types of Usher syndrome, designated as types I, II, and III. These types are distinguished by their severity and the age when signs and symptoms appear. Type I is further divided into seven distinct subtypes, designated as types IA through IG. Usher syndrome type II has at least three described subtypes, designated as types IIA, IIB, and IIC.

I have Usher type II. I was born hard of hearing, this was diagnosed at pre-school, I attended mainstream schools and went on to start work in a training placement. I was diagnosed with Usher at the age of 22 in 2004, i was asked to giving up driving the same day as i was deemed unsafe. My whole life changed from then. I had to adapt fast and learn about what was going to happen.

I had always had difficulty seeing at night but other than being classed as clumsy and jumpy i had no idea i was slowly losing my eyesight. It does cause a lot of frustration as i try to get on without being a burden and try to be 'normal' a wife / mum and i work part time. There are lots of ups, downs and barriers i have come across along the way because i have a dual sensory loss impairment. This is because i don't fit into one particular category.

I have had to accept that i am unable to do this alone and have had to seek guidance and help from the RNID, RNIB, Deaf-blind UK and I've joined a few groups on Facebook where I have made friends with people with the same condition, this has helped me the most as we all can vent out our frustrations and we all completely understand each other.

In the last year I have recently been registered as severely sight impaired and I've trained using the long cane to help me out and about but also to help other people be aware that I have a problem. You'd be amazed at how many people don't actually know my reason for having a long white stick with red bands on it! I am also in the process of applying for a guide dog as my confidence being out on my own is very low.

Usher syndrome is very deceptive as to look at me I don't appear to have any problems and I can come across as rude and ignorant to people around me, I bump into people and objects, I've knocked children over by walking straight through them, the grief I've had off parents, my own children and dogs get knocked about on a daily basis by me not seeing them. I am covered in bruises from my mishaps.

It is very exhausting having Usher as you have to plan your day, from getting dressed and learn where things are, when out and about to have to plan your route ahead of time and think as well as scan the area as you're walking along to plan for any hurdles or obstacles that could be in your way. You have to try and plan what other people might do. Just because you have a white stick, it doesn't mean people will automatically get out of your way or make allowances for you. I do the best I can with what I have.

Thanks to Lynne for writing this guest article.

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