The Diagnosis

Own The Equinox Day 8 | Journey Through Hearing and Vision Loss: Living with Usher Syndrome

By Martha Steele
I returned to Vermont in late August 1977 to prepare to enter the University of Vermont for the Spring semester and complete my undergraduate degree. I would live in our Burlington home with my parents, Erland and Ruth Gjessing, who were splitting their year between Burlington in the winter and the Northeast Kingdom, in the summer.

One of my first tasks after returning home was to make an appointment with a Burlington ophthalmologist. My mother and I were staying at a family camp on the shores of Lake Champlain when I borrowed her car to drive into the appointment.

The day was an absolutely gorgeous September day, the sort of day that lifts spirits with its brightness and crisp, refreshing air. Looking from the porch at our summer camp, the water was sparkling and the view of the Adirondacks across the lake crystal clear.

Going to the appointment, I still was not worried, just expecting some sort of diagnosis that would result in a benign treatment to correct any problems that I might have. At the appointment, I went through the usual battery of tests with technicians, such as testing of the visual field, description of symptoms, and examination after dilation of the eyes. The final stop of the appointment was with the ophthalmologist, an older and experienced man well respected in the area.

What happened next left me crushed in my chair. He peered into my eyes for several minutes, asked to look left, right, up, and down, and then rolled his chair back to talk to me. The conversation went something like this.

“Well, I recently went to a conference and I think you might have retinitis pigmentosa. You will be blind in 10 years. So, I would not advise that you become a truck driver.”

I stared at him in stunned disbelief and silence.

The doctor continued, “I think you should see someone in Boston to confirm this diagnosis. My secretary can provide you with the name of the doctor and give you a referral. Have a nice day.”

The doctor then offered his hand to shake mine and left the room. I must have said something during this exchange, but all I remember was sitting there in shock. Blind in 10 years? Blind? That’s impossible. If he said anything more about RP, I don’t remember it. There was really only one word that shook me to my core: blind. I was going to be blind.

I managed to stand and in a short of shocked daze, walked out to the reception area, picked up the name and contact information for the Boston doctor, and walked to the car. I got in and simply started sobbing. I just could not believe what I had just heard.

It was a long time sitting in that car, trying to pull myself together to drive the 15 or so minutes back out to camp to tell my mother. I could not stop crying but managed to get back to camp.

My mother knew the moment she saw me that this appointment did not go well. I started sobbing again and for a few long moments, I could not say anything to her. I was finally able to tell her the news with barely intelligible words. She hugged me and we stood there together, crying and staring out the window to the beautiful view we were privileged to share. I kept saying that I would not be able to see the lake, the mountains, the sunsets, the brilliant blue sky, or anything at all in what seemed to be just a few short years. I could not look anywhere without thinking, my god, I won’t be able to see that. I wasn’t even sure that I could have any sort of career or what I would do after I completed my undergraduate degree. I was devastated and scared, very scared about how I could possibly cope with being blind. Being told by the doctor that I was going to be blind meant only one thing to me at the time: I would see a world go completely dark with no light perception at all sometime in about 10 years. I was only 25 years old and my future was suddenly shattered.

I was reeling. All I could think of was what I would not be able to see or do. I would not be able to ride my bicycle, would not be able to ski, would not be able to drive a car, would not be able to travel, would not be able to see sunsets, would not be able to read, would not be able to see my family or friends, would not be able to do all the things that I had planned to do. My world had been totally turned upside down. My mother must have been reeling herself seeing her daughter so distraught and herself coming to grips with the enormity of the diagnosis. But she had the presence of mind to take the contact information for the Boston specialist and call that afternoon for an appointment. We were off to Boston within a few weeks. That appointment would prove to be crucial in stabilizing my emotions, better understanding RP and blindness in general, and giving me greater optimism for my future.

But on that brilliant September day in 1977, optimism for the future disappeared. Anyone who experiences an unwelcome and unexpected turn in their lives knows full well the profound turmoil that can result. But many of us are nothing if not resilient and resolute in working through these life-changing events. Of course, coming to grips with a diagnosis of blindness takes time, and it would indeed take time to go through a mourning process and lift myself up again.

Tomorrow: Going to Boston

Taking a Break Along Massachusetts Avenue in Arlington, MA.

August 30, 2016: 19 Days Until Usher Syndrome Awareness Day
Miles Walked Today: 8 miles in Arlington, Somerville, and Cambridge,
Miles Walked To Date: 42.5 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 also serves as a National Trustee and Boston Chapter president of the Foundation Fighting Blindness.