So, whats “wrong” with me?

A lot some would say and I would jokingly agree. However, on a more serious note I do suffer from a very serious and disabling medical condition. A condition that dominates my existence, even if I try hard not to let it.  It is crucial to understanding my life, photography and general motivation. Photography is about pushing the limits of my physical capability and a part of the fight to have every experience I can have while my world slowly fades into permanent darkness. 

While I would be happy if I could inspire anyone to go out there and try to accomplish things, I make images for my own sake more than anything else. 

My retina

A photo of my retina. Notice the black spots and streaks, the tell-tale sign of Retinitis Pigmentosa. Also the shadow near the center, early cataracts.

My nemesis: Usher Syndrome 
The Usher syndrome is the combination of the eye-disease, Retinitis Pigmentosa, and a hearing loss. Also, in some types of the syndrome, inner-ear functions do not work as supposed to, meaning that those suffering from this have balance issues. Usher Syndrome is a hereditary and progressive genetic disease that is one of the major causes of deaf-blindness in the world. Still, it is an extremely rare disease that only affect much less than a percent of the worlds population.

Wikipedia has a fairly decent article on the subject.

I suffer from Usher Syndrome, type 3. There is no family history of this condition. My sibling, parents and other relatives are healthy.  These days I have a peripheral vision of about 5-6 degrees (of the normal ~180 degrees) and a hearing loss averaging minus ~75db in the frequency range where human speech normally reside. I do not have malfunctioning balance organs in the inner ear. By international standards I’m considered deaf-blind. In practical terms my functional ability is highly dependent on whether its a “good” or “bad” day, if it’s winter and dark, or summer and sun. I’m still relatively mobile when there is light. I can also point the camera and frame a scene. Lots of experience helps to overcome the narrow vision field when I make images. I do bump into things frequently and carry many scars. My greatest fear is small children running amok in crowded streets and shopping malls. 

I would say that in many ways the amount of extra energy you burn trying to function in a seeing and hearing world is the most noticeably annoying side-effect of being severely hearing and vision impaired. At times it’s costly to do things, so very costly. And scary.  

This image below is an illustration I made to show what my world might look like. I literally do stumble through the light! 

My world one December afternoon

My visual experience one December afternoon. Blurry, shady and glaring lights that overwhelm.