…but Steven Frank has found happiness despite a formidable genetic disease called Usher syndrome.
When I arrived at Steven Frank’s townhouse for his public radio interview, I wondered who would answer the door. I pressed the buzzer and waited. Surprisingly, he came to the door himself. The doorbell had activated a vibrating device on his belt to let him know he had a visitor. His partner, Ed Knight, then greeted me and spelled what I presumed was “B-E-N” in Steven’s palm to let him know whom he just let in.
Steven Frank is deaf and blind from a genetic condition called Usher syndrome. He has no hearing and only a tad of light perception in the corner of his left eye. That’s it.
Steven communicates through tactile interpretation — hand-to-hand communication. And while I couldn’t understand his speech, I did pick up on the feeling and emotion it conveyed. During the interview, an interpreter voiced Steven’s American Sign Language (ASL) for us and interpreted my questions for Steven by signing into his hands, a communications mode known as Tactile ASL.
I was there representing my employer, the Foundation Fighting Blindness, and had set up the interview to help raise awareness about Usher syndrome. I had met Steven a few months earlier at a conference, and his upbeat attitude and independent lifestyle made a lasting impression on me. I wanted the world to know about how he takes on the challenge of being deaf and blind, so I asked my friend Aaron Henkin, a co-producer of The Signal, an eclectic human interest show on WYPR, National Public Radio in Baltimore, to tell Steven’s story.
Previously, I had interviewed dozens of people who are blind or have severe vision loss. It is a tough road for them — a world of canes, guide dogs, technological accommodations and continual help from friends and loved ones. But as I learned so well during that conference, people with Usher syndrome — those who have been dealt the one-two punch of hearing and vision loss — face extraordinary circumstances that are hard to fathom.
Usher syndrome is a retinal and cochlear condition affecting nearly 50,000 people in the U.S. Because it is recessively inherited — parents are completely unaware that they are carriers — the onset of the disease to their children comes as a complete shock to the family.
The symptoms of Usher syndrome usually begin early when a child is diagnosed with significant hearing loss or complete deafness. There are many decisions and accommodations to be made early in life for children with hearing impairment. They might get hearing aids or cochlear implants. They might learn sign language or lip reading. Maybe they’ll enroll in educational programs for the deaf. They may also have social difficulties, especially at a young age, because other children aren’t always sensitive to those with disabilities. Later, finding work and forming relationships in the hearing world are likely to pose challenges.
Let’s say that you are born deaf and learn to manage your deafness relatively well, as many deaf people do, perhaps by assimilating into the tightly knit deaf community. But somewhere in your teens, you begin to notice problems with your vision. You have trouble seeing in the dark. You walk into edges of doors and trip over things more frequently. You can’t see a friend wave to you from across a room. You go to your eye doctor, who sends you to a retinal specialist, who refers you on to an academic research center. After a grueling day of testing and exams, you are told that you are going blind and there is no treatment or cure. You’re told, “There’s nothing we can do.” That’s often the devastating sequence of events for people with Usher syndrome.
When I first met Steven at the conference, he was taking part in an evening networking session for people with Usher syndrome. I was just the room monitor, making sure the air conditioning was working and the water pitchers were filled. Even though I’d occasionally written about individuals and families with Usher syndrome, never had I been with such a large group, about 15, who were affected with the condition. Some still had enough vision to see sign language interpreters. Others like Steven relied on tactile interpretation. I was drawn to the expressions and motions of the interpreters, and the people they were helping to communicate. There was a seductive quality to the flurries of hand and body movements. They spoke powerfully and passionately about their experiences, always commanding attention. Even though I didn’t understand the sign or tactile language, some people verbalized well, so I often knew what was being said.
The attendees were quick to talk about the complexities of day-to-day life. Many participants spoke about how they had been laid off, or, like Steven, forced into early retirement. Virtually everyone had contacted the Equal Employment Opportunity office or some other advocacy organization, because of workplace discrimination. One couple talked about their deaf daughter, who was on the verge of beginning a promising professional career, but had been recently diagnosed with Usher syndrome. The heart-broken parents struggled with how and when to break the news to her that she was going blind.
Moira Shea, who served as the networking-session moderator, told the group how she paved the way for allowing guide dogs on the Senate floor. At the time, in 1997, Shea was a nuclear policy aide to Sen. Ron Wyden (D-Ore.) and the Senate didn’t allow service animals into its chamber. She earned a master of public administration from Harvard and lives in Washington, D.C. Moira is also on the Foundation’s board of directors, helping lead the drive for vision-saving treatments and cures.
Many at the networking session, including Moira, said they were in a constant state of grieving, because of gradual, progressive loss of vision, and along with it, the diminished ability to function independently. Just when someone learned to function at the level of vision they had, further degeneration of the retina brought a whole new set of challenges. Some people in the session had only a few degrees of visual field remaining. It is as if they look at the world through a straw or a pinhole. Others like Steven could only perceive traces of light and relied on touch to communicate.
Total blindness, I learned, is a deaf person’s biggest fear.
After the evening session with the Usher syndrome group was over, I was exhausted from the emotional intensity. Every person in that room spoke openly and honestly about the about the myriad obstacles they encountered at home, in the workplace, and out in the hearing-sighted world. A lot of fear and anger were expressed. Never before had I been so thankful that I could navigate my way to the hotel elevator and see the buttons to get to my floor.
To learn more about their culture and challenges, I made it a point to hang out with the group for much of the remaining days of the conference. I couldn’t help but be awed by the courage and tenacity they brought to their daily lives. But it was difficult and draining to communicate with them. We usually needed an interpreter to have even a simple conversation. In many cases, the best I could do was just listen, which was no small feat for me. I must confess that after a whole weekend of watching sign language, I only learned the sign for “wow,” which is to shake your open hands near your mouth while you have a look of surprise on your face. (Try it. It’s very liberating.)
In a sense, people with Usher syndrome are in a race against their diminishing vision, trying to see and accomplish as much as possible before they lose their eyesight entirely.
For Steven, who is in his 60s, the race is essentially over. But he maintains a remarkably positive outlook, thanks to the preparations he made while his vision was fading. Steven learned Braille, so he can use a computer with a Braille keyboard and output device to stay connected to the outside world. He even has a Braille note-taker and printer to make paper versions of his communications. He continues to explore new technologies, and is currently learning how to use an iPhone.
Steven retired in 1997 after a long career working in the library at Gallaudet University — perhaps the United States’ most highly regarded college for the deaf. Even there, he had difficulty getting the visual accommodations — a document scanner — he needed to do his job. Steven was born deaf, and by the late 1990s, had very little useful vision left. While he spoke of frustration with the lack of support he got from Gallaudet, he expresses very little anger or bitterness about his situation there. In fact, he is a strong supporter of the university, is very active in its alumni association, and recently enjoyed participating in its 150th anniversary celebration.
What keeps him going is his insatiable appetite to socialize. When he’s home, he’s always in his upstairs computer command center talking to people, many of whom are deaf-blind, through the Internet or TTY. But he also has no fear of going outside the comfort of his home to participate in the vibrant social community of the deaf-blind. He travels the country to attend all sorts of events for the deaf-blind. He even goes camping. Can you imagine going in the woods unable to see or hear all the trees and animals around you?
Steven also travels solo on occasion. For one plane trip, a little preparation helped him navigate the airline system successfully. He prepared index cards with messages in English and in Braille, such as: PLEASE LEAD ME TO THE RESTROOM; I WOULD LIKE CRANBERRY JUICE; PLEASE LEAD ME TO THE BAGGAGE CLAIM WHERE MY FRIEND IS WAITING. He showed a photo of his luggage so whoever was meeting him could find it at the baggage claim carousel.
Could you imagine being deaf-blind and going solo on a “flight from hell” where you encounter a lot of turbulence, or worse yet, get stranded on the tarmac for a few hours? Steven says that he spells out words in flight attendants’ palms if he has questions, and he teaches them to do the same so they can keep him informed. I wonder how quickly an attendant can finger spell, “WE ARE ABOUT TO CRASH. THANKS FOR FLYING AMERICAN.”
Actually, Ed told me there is an emergency tactile sign — a big X traced on the back. It basically means: “There is an emergency and we have to evacuate right now, follow me, I’ll explain later.” Now, do flight attendants know it? That is another matter.
“Whenever I drop off Steven at the gate, I take a moment to try to teach the flight attendants about Print On Palm, which is tracing block letters (capital letters) on the palm of the deaf-blind person,” Ed explained to me. “Some of them get it without any problem, but others get quite flustered. So I think flight attendants need training in that area. Really, I feel like each time Steven boards a flight is a teachable moment for the flight crew.”
Admittedly, I expected a little more drama from Steven during his radio interview. That’s part of the reason I thought his story would make such a great piece for public radio. I knew he was a relatively happy guy, but certainly there must have been some things he needed to get off his chest, and this would be a great opportunity for him to vent. He did tell us he’d been made fun of when he was young — even by other deaf kids who didn’t understand his vision loss. And there was one girlfriend who broke up with him, because her mother was afraid they’d have deaf-blind children if they got married. But mostly, Steven was excited to demonstrate all his high-tech communications gadgets in his upstairs command center. He was like a kid showing us all his new toys, though for him, the devices provide a vital link to the outside world and his huge circle of friends, many of whom are also deaf-blind.
Steven said he would like to have his sight restored some day — perhaps through an artificial device or retinal cell transplantation. Watching movies and television would be fun for him. Most of all, he would enjoy seeing how his family and friends have changed over the years. He misses perusing family photo albums to recall memories of his earlier days.
As far as his hearing is concerned, he said, “I am used to being deaf. I don’t think I’d like being in a noisy world.”
More than anything, Steven wants people to understand his needs and accommodations. He’d like people to take the time to sign in his hands. At a party or a gathering, he needs his sighted friends to come up to him and say hello, because he doesn’t know they’re there. He’s not asking for miracles — just an opportunity to have a place in the world. Like all of us, he just wants to belong.
Photo courtesy of the author