Retinitis Pigmentosa

Discovery Eye Foundation is pleased to present the following excerpt from a just-released inspirational book called Walk in My Shoes. It is the result of two years of collaborative effort and is a unique collection of 27 powerful stories by individuals who are experiencing or witnessing the challenges of losing not one, but two senses: hearing and sight. The writers of Walk in My Shoes offer a glimpse into living with Usher syndrome, a progressive disease leading to blindness and deafness. Walk in My Shoes speaks to the more than 400,000 people worldwide dealing with Usher syndrome, to their families, to the professionals working with them, and to the rest of the world.

All proceeds from book sales will be donated to the Usher Syndrome Coalition to help fund scholarships to its annual conferences and to support research for a cure. The writers inspire hope for anyone dealing with difficult life challenges.

MY USHER’S LIFE LESSONS
By Mary Dignan

I remember how I wanted to die, or at least for the Earth to open up and swallow me forever, when I read that memo telling me how I’d been asking questions that had already been asked and answered, and how I’d said things that were irrelevant and inappropriate at our meeting earlier that day. I had always known that the hearing problem was more of an issue than the visual field loss associated with Usher syndrome. Now, this memo was the proof that I never should have tried being a lawyer, that I had no business in this profession, and should just go home.

Instead of going home, I got up to close the door to my office, sat back down at my desk, and picked up the memo from Tom to read it again. Tom was my supervising attorney, and we had both been looking forward to that meeting with a potential new client. The work would be on an issue that no one at the firm knew better than I and we were sure we’d close the meeting with the retainer agreement in hand.

But the meeting just didn’t go well. It started off well enough, but there were some odd pauses in the conversation, moments of uncertainty and careful courtesy, and I didn’t feel good about it when it was over even though we did, in fact, end up with the retainer agreement. I was still thinking about the meeting a couple hours later when Tom’s secretary came into my office and handed me an envelope, sealed and marked “confidential.”

It was a memo from Tom. “Mary, I need to make you aware of some things I observed during our meeting today.” He listed specific things I’d said and described how I’d asked questions that had just been discussed, and how I’d said things that were irrelevant to the actual conversation. He said he and the client both knew I wore hearing aids and assumed I simply hadn’t heard things accurately. He said that because I had an excellent reputation as a truly competent professional, and because he and the client knew how well I knew the issue, they made allowances for me, and we got the account. Still, Tom was concerned. “Mary, I’m wondering if you’re not hearing as well as you used to, and if there is anything we can do to help.”

My hands were trembling as I put the memo back down on my desk, and I felt a hot flush rise up from my toes to my face. God, what an incompetent idiot I must have sounded like. I was even more embarrassed by the courteous smiles and patient repetitions, the polite allowances they had made for the incompetent idiot. It would have been better if someone had just growled at me to go put in some fresh hearing aid batteries.

But, when I read the memo yet again, I began to appreciate the inherent respect and sincere consideration Tom was showing me. Instead of confronting me with the painful truth, Tom could have just stopped working with me. He could have started whispering behind my back: ”Uh, best not give that assignment to Mary, she can’t handle it.” Or, “No, Mary’s not the best one to attend that meeting, she can’t handle it well.” And I would have slipped into miserable mediocrity.

He didn’t and, instead, he came to me and told me exactly how I wasn’t cutting it, and gave me the chance to find a way to measure up. I decided that before I handed in my resignation letter, I’d at least find out if there was a better hearing aid out there. I called my audiologist and told him what had happened. He told me I was already using the best and most powerful hearing aids available, but there might be one other thing I could do, “It’s time for you to get an FM system. Come on down this afternoon and we’ll get you set up.” This was an assistive hearing device that would enhance the use of hearing aids and therefore allow me to hear better.

I was in my early 40s then, down to less than 5 degrees of tunnel vision and wearing two high-power hearing aids. I still had good precision vision within my little tunnel. For the last 20 years of slow but steady vision and hearing losses, I had always figured out ways to work harder and smarter. It was the hearing losses that troubled me most. I’d been wearing the best high-power hearing aids for years, and was so good at reading lips and body language that it was easy to forget I had a hearing problem. But as my vision started to go and I could no longer read lips and body language well, we all began to comprehend just how deaf I really was. It didn’t matter so much that I was mowing down my colleagues in our office hallways, but responding inappropriately to clients and judges was a huge problem.

It took practice and patience to develop the skill to use my new FM system effectively, but the effort paid off. My FM system worked well because I made it work, and I made it comfortable for everyone around me to work with me.

There were several lessons, or rather reminders, for me out of that whole incident, including the fact that it’s just about impossible to die of humiliation, no matter how much you may want to. More importantly, I was reminded that everyone around me took their cues from me—if I was uncomfortable with the fact that I had to use an FM system to hear, everyone else would be uncomfortable with it too. So I not only learned to use the FM system well, I also learned to introduce myself and my tools with candor and humor.

”Hi, I’m Mary Dignan from Kronick, Moskovitz, Tiedemann and Girard, representing the State Water Contractors, and that’s my FM system in the middle of the table there. It helps me hear, and I would appreciate it if you would not touch the mike or the wires because it sends a lot of irritating static directly to my hearing aids.” I’d pull my aids slightly out from behind my ears to make them obvious and then I’d put them back, and go on. “The other thing you need to know about me is that I only see through a little keyhole,“ and here I would make a keyhole tunnel of my fist and peek through it, “which means I can see you.” Then I would point at someone else, “But I don’t see Tom sitting next to you,” and pointing at Tom I would continue, “This is a good thing because I don’t like looking at Tom anyway.”

That would generate a few chuckles. “This is my cane,” I would add, picking up my telescoping white cane from the table and opening it. “I use this to find the stuff that doesn’t show up in my keyhole when I’m walking around and it’s also highly useful for whacking people in the ankles and patooties.” More chuckles, and then we’d move on and get down to business.

I learned not to waste energy on trying to cover up my vision and hearing challenges. That meant I had more energy to focus on doing my job, and doing it well. I didn’t worry about attending conferences or night meetings, because I learned how to use my white cane to get around on my own safely and gracefully. I also learned to ask for and accept a helping arm with grace. I learned that if you are good at your work, and especially if you are a good team player, your colleagues will be willing to make any reasonable accommodations you need. Even before the Americans with Disabilities Act (ADA) and the term “reasonable accommodation” joined our vocabulary, I had no problem getting hearing-aid-compatible phones, lighting and other low-vision aids, adaptive computer technologies, and even cab rides when I had to stop driving.

I learned that when my colleagues knew I was putting my best effort into making things work, most of them were willing to put in a little extra effort themselves to help me out. Sometimes it was as simple as giving me an extra few seconds to get my FM system set up before they started the meeting, or steering me around pillars, potted plants and people that seem to always get in one’s way at a crowded conference, or giving me a ride home. Sometimes it was sending me a memo telling me honestly about some problems I needed to be aware of in order to figure out ways to solve them.

The FM system was a good solution for a few years. It turned out that, apart from the Usher Syndrome, one of the reasons I kept losing more of my hearing was an acoustic neuroma brain tumor that grew out of the cochlear nerve to my right ear. The surgery to remove the tumor saved my life but exacerbated my hearing and vision challenges so much that I had to give up my legal career.

Ten years after the brain tumor surgery I received a cochlear implant, which greatly improved my hearing. I still have trouble with background noise, and I can only hear out of one ear, but I hear better than I ever could before. It is the one thing in my life that has gotten easier. I am 60 now, with almost no vision left except some high-contrast shapes in a world of murky shadows spiked with glare.

Those lessons I learned years ago still apply today and I have come to realize that this is because they are Life Lessons, not merely issues particular to Usher Syndrome or any other disability. I have also come to realize that part of learning and developing one’s abilities is as much a lesson of disability as it is ability. One such lesson came from my friend Brian, an extraordinarily competent computer wizard who was born blind.

Brian can listen the way I used to be able to read a thousand words a minute, and his orientation and mobility skills are awesome. We often talked about the differences between being totally blind from birth, and going blind later in life. Apart from the life adaptation and grieving issues, the main differences we noted involved the ways we perceived and comprehended the world around us. I told him about a totally blind guy who had a hard time wrapping his mind around the concept of transparency. He just couldn’t make out how a hard cold pane of glass that he could rap his knuckles on was something you could “see” through.

“Oh, I don’t have a problem with that concept,” Brian said. “My problem is pictures.”

“Pictures?” I questioned.

“Yeah, pictures,” Brian said. “How can you put a three-dimensional world onto a flat piece of paper?”

I remember my jaw dropping as I stared at him and began to comprehend how rich and real the world is to Brian. He can’t see any of it, but he knows it intimately. He moves in a world that he perceives physically and kinesthetically through all his other senses. He is always aware of how his surroundings feel, smell and sound. In a lot of ways, he is much more aware of and intimate with his world than most sighted people who superficially see their worlds from a distance and from a flat piece of paper.

Brian taught me that even though I am losing all my sight, I still have a rich world to perceive through touch, taste, sound, feeling, smell and experience. This, too, is a Life Lesson, not just an Usher thing, but it is a lesson sweeter because of Usher syndrome. It does not lessen the pain of losing my vision, and it doesn’t even necessarily make my life easier. But it does give me hope and joy. It is the reason I’m still doing my mosaics (with a little sighted help here and there), the reason I’m still in my kitchen baking from scratch and making up new recipes, the reason I can tell when I’m on the beach or in the redwood forest, or even just out on my patio enjoying the evening breeze and garden scents. And it is the reason I know I still have a good life to live.
 
 

ABOUT THE AUTHOR

Mary Dignan was diagnosed as mentally retarded before the moderate-severe deafness was diagnosed close to age five. She was then fitted with hearing aids and attended public schools. At the age of 20 she was diagnosed with Retinitis Pigmentosa and, years later, was told she had Usher syndrome, type 2.

Ms Dignan earned a B.A. in English/written communication from Santa Clara University in 1976. Her 21 year career in agriculture and water resources management issues includes work as a news reporter, legislative aide to the U.S. House of Representatives in Washington, D.C., and the California State Assembly Committee on Agriculture in Sacramento. She earned her juris doctorate with distinction from the University of Pacific-McGeorge School of Law in 1994 and practiced law until 1997. She now creates and teaches mosaic art and her work has been shown in several public venues including: Sacramento Society for the Blind and the Canadian Helen Keller Centre in Toronto, Canada. Her community service includes serving on the Sacramento Board of Supervisors’ Disability Advisory Committee, and on the Board of Directors of the FFB, Sacramento Chapter. She is a present member of the Sacramento Embarcadero Lions Club. Ms Dignan lives in Sacramento with her husband, Andy Rosten.