Moving Forward

Own the Equinox Day 10 | Journey Through Hearing and Vision Loss: Living with Usher Syndrome

By Martha Steele

Life can be quite humbling when it deals you a blow that comes out of nowhere. But the very definition of resilience means that you can take those blows, learn from the experience, and see beyond the short-term setback and believe in the long-term promise and hope of your future. The humbling experience of learning about RP perhaps gave me perspective about my own fragility that also strengthened me with confidence in my abilities to cope and made me more empathetic for challenges that we all confront at various times in our lives

I was fortunate in that my RP was destined to result in gradual and relatively slow vision loss. In truth, because my central vision, in particular, was still normal at the time of the diagnosis and because the prognosis was a prolonged and gradual decline, I adjusted quickly to this new reality. There really were not that many adjustments to make at the time. The difference was that I now had an explanation for my peripheral field and night vision losses. Still, the RP hardly slowed me down.

In January 1978, I entered the University of Vermont to complete my undergraduate work that had begun at Hampshire College in 1970. I needed 2.5 years to get a degree in zoology with a minor in the history and philosophy of science. During the summers, I held temporary jobs, sometimes commuting to and from work on my bicycle. I also did the journey to my parents home in Westmore (about 90 miles) several times on my bicycle, which I loved riding. I played softball in a local woman’s league and did wilderness hikes and canoe camping with friends and family.

Unless I said something, no one who met me knew that I had any vision issue. And most of the time, no one knew I wore hearing aids either. I was still driving a car, although I clearly remember that by the time I graduated from UVM in 1980, I was not as confident driving at night as I had been in previous years. I remember one night when I had come to a four-way stop sign. I looked one way, saw nothing, then looked the other way, and saw nothing. I proceeded to move forward only to slam on the brakes when I realized there was a car directly in front of me crossing from my left to my right. My peripheral and night vision combined to rob me of knowing that any car was directly in front of me while I was moving my head from one side to the other.

A similar situation happened when I crossed a street in Burlington, where I looked to my left, saw nothing, and started to step off the curb. I was stunned to step right into the side of a car that was turning right. I don’t know who was more surprised, me or the driver. Again, I had missed that car in the process of turning my head in one direction or another.

It is hard to simulate for someone with normal vision the experience of narrow peripheral field. Although it is described as tunnel vision, functionally, I never feel like I am looking at a pin point field with everything black except for whatever is in that pin point field. I think this is because I am constantly moving my eyes in all directions to maximize what I see. So, I can’t say that, even today, I have a pinpoint of sight in front of me. My eyes are always moving so the view just seems wider and more open to me.

After graduating from UVM, I went on to graduate school at the University of Michigan and earned a Master’s in Public Health, specializing in environmental health. I did not have a car in Ann Arbor, and the few times that I borrowed someone’s car, I began to realize that driving was becoming a little less comfortable for me. You really do need good peripheral vision in order to safely drive a car, and I was realizing that I might not be able to drive much longer. Still, my central vision continued to be fine, and I charged on with high expectations for my career and myself as a person. The summer in between the two years in graduate school was spent interning with Vermont Senator Robert Stafford and living with my cousin, Mark Friedrichs. It was a fabulous summer, as Mark took me under his wings and showed me as much as he could of the Washington, D.C. area.

Somewhere during this time of undergraduate and graduate education, I figured out that I probably had Usher syndrome. I don’t actually remember
how or when, but I do know that it was not a physician who told me. I somehow learned about Usher syndrome and put two and two together. Usher syndrome combines hearing loss with retinitis pigmentosa, and I obviously Had both. At the time, I recall that we knew of two subtypes, Usher 1 and Usher 2. Usher 1 was much more severe, in that children were often born deaf and they lost their vision at a much younger age, Usher 2 resulted in varying degrees of hearing loss from moderate to severe and a much slower progression of vision loss. I remember attending with my mother a national conference sponsored by the Foundation Fighting Blindness in Las Vegas in the early 1980s and reviewing materials at a display table devoted to Usher
syndrome. By this time, then, I had a pretty clear picture of where I stood and what was likely to happen with time. But how much time? That was a
question no one could answer.

Tomorrow: Moving to Boston

Walking on Lewis Avenue, our home street in Arlington, MA

September 1, 2016: 17 Days Until Usher Syndrome Awareness Day
Miles Walked Today: 7 miles, Arlington and Winchester, MA; I have to brag about Alvin today, as he guided me without any direction from me from my home in Arlington to my brother’s house in Winchester, a distance of about five miles. Although we have done this route several times, we had not done it for about two months; he is one terrific guide dog!
Miles Walked To Date: 55 miles

Martha Steele is a member of the Board of Directors of the Usher Syndrome Coalition, as well as the Board of Directors of the Carroll Center for the Blind in Newton, MA. She is also a National Trustee and Boston Chapter President of the Foundation Fighting Blindness.

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