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A Rare Genetic Disorder Stole Her Sight and Hearing, But Not Her Courage
Monday, January 31, 2000
By CECELIA GOODNOW
SEATTLE POST-INTELLIGENCER REPORTER

Being with Janie Smith is like looking through a one-way mirror.

It's almost voyeuristic, openly studying her intelligent, expressive face, knowing that a discreet cough or shuffle of the feet won't give you away -- knowing she doesn't realize you're just across the room.

In her aqua scrubs, surgical cap and white oxfords, Smith looks like any other worker in Swedish Medical Center's sterile-processing workroom.

What sets her apart is her blue lapel pin, the size of a demitasse saucer. Its bold print announces, "I am deaf and blind."

Here, in the bland atmosphere of the workaday world, Janie Smith is taking back her life.

"I don't want to stay at home all the time," says Smith, who donates her labor three days a week. "I'd just get bored."

Besides, she adds, "I need to know how to take care of myself."

Her co-workers display quiet admiration for the woman battling darkness and silence in their midst. Some call her "our next Helen Keller."

"You gotta meet this woman to realize she's not an ordinary person," says Smith's supervisor, Pennie Clemmer.

Smith, 48, has Usher syndrome, a rare genetic disorder that has robbed her of hearing and sight. There is no cure.

Like others with the disorder, she was born profoundly deaf and slowly lost her vision in adulthood to retinitis pigmentosa, a degenerative disease of the retina.

At 19 Smith started to experience night-blindness, the first step in her inexorable visual decline.

"It was like I was wearing sunglasses," she says, signing into an interpreter's hand. "I couldn't see very well."

Later she developed tunnel vision and could only watch helplessly as her visual field narrowed like a door slowly swinging shut.

Two years ago her world went dark.

Smith, who lives across the Sound in Port Orchard, began volunteering at Swedish last July.

In the hushed chambers of the sterile-processing department she assembles kits of medical supplies. Her main job is to restock the bedside drawers used by intensive-care nurses.

Clemmer has told her which supplies each drawer must have. There are 30 different items in all, including sterile gauze packs, alcohol preps, four types of syringes, lotion bottles and different types of IV tubing.

She also prepares postpartum packs of diapers, soap, comb, bulb syringe and other supplies for the nearly 400 babies born at the hospital each month. Last month she spent her spare time at home sewing and hand-decorating 100 infant buntings shaped like Christmas stockings to swaddle babies born at Swedish over the holidays.

Although she isn't paid, she works with the focused intensity of a new recruit bucking for promotion.

Soon she hopes to turn her volunteer skills into a paying job at Swedish. Although the idea is still in the pipeline, her acting department manager, Thomas Fields, says he's enthusiastic about trying to make it work.

It's an exciting prospect and a rare chance for someone with her disabilities.

"Job opportunities for deaf-blind people are severely limited," says Jennifer White, Smith's interpreter and job coach. But, she added, "the limitations are only set by the environments deaf-blind people are put in."

White, who runs Able Opportunities Vocational Consulting, has spent a year grooming Smith to beat the formidable employment odds. The state Division of Vocational Rehabilitation is paying for White's services.

Smith is an easy person to like. Dark-haired and solidly built, she has the pleasant, open face of a good-natured farm wife who has weathered a few crop failures and come out smiling.

She has made friends at Swedish but says some people are "a little bit scared because they've never met somebody who's deaf-blind."

Even so, she says, "This is the best place to work. It's so cool."

She recently told her colleagues so, in a hand-printed note of appreciation. Although her syntax is unusual, she has a strong grasp of English for someone with her disabilities.

"Hi, there," she wrote, "I want to say thank you so much for letting me to work in sterile supply lab room ... I'm anxious can communicate you all if you can learn to ASL (American Sign Language) or finger spell!"

They're working on it. Smith and White have held several well-attended training sessions. Some hospital employees now wear finger-alphabet cheat sheets on chains around their necks so they can spell brief messages.

Clemmer has grown especially adept at signing into Smith's hand, allowing her to feel messages she can no longer see.

Although they have formed a strong bond, they rely on after-hours e-mail to add the fine brushstrokes of nuance and detail.

"Did you get what I was saying this afternoon?" Clemmer might message Smith from home that evening.

Smith's Braille computer translates incoming e-mail, then relays her lengthy and exuberant responses.

This lady likes to talk, and the computer is her tool of liberation, giving her direct mind-to-mind access to the larger world.

"I've gotten to know who Janie is more from the e-mail than the bits and pieces we get out (at work)," Clemmer says.

The oldest of five children, Janie Smith is the only one in her family who carries the double-recessive gene that triggers Usher Syndrome. Researchers estimate the disorder affects anywhere from 1 in 25,000 to 1 in 40,000 people. Some of the milder cases are never diagnosed.

Smith has the rarer, more severe form. Others are born with mild to moderate hearing loss and lose their eyesight at a slower rate.

No one suspected Smith was deaf until she was 9 months old, when she failed to look up at her uncle's loud, playful greeting.

Smith tells about it in a lengthy, autobiographical e-mail, using syntax that straddles standard English and the distinctive grammar of American Sign Language.

She writes, "My grandmother said to my parents that Janie's ears were not work right. They took me to see a doctor for exam and he answered to my parents that I am profound deaf by birth to make my parents felt sad and real love for me."

On their doctor's recommendation they sent Janie to the John Tracy Clinic in Los Angeles, where she enrolled in a free, therapeutic preschool for children up to age 5.

The program, which stresses parent involvement and education, was founded in 1942 by Louise Tracy, wife of actor Spencer Tracy, 17 years after their infant son was diagnosed with profound hearing loss.

There Smith learned to lip-read and speak but not to sign, based on a belief -- prevalent then -- that learning to sign would prevent deaf children from assimilating into the hearing world. That philosophy is now controversial.

Although Smith's speech is hard for most people to understand, it's an important link to her mother, Patricia McDermott, who isn't fluent in ASL.

"When I'm with her she talks all the time," said McDermott, 79, who lives in Issaquah with her husband, Frank. "I can understand her."

Smith, who was born in Santa Monica, Calif., spent her earliest school years in Los Angeles, first at the Tracy Clinic, then at an elementary school for deaf children. After the family moved to Orange County, she attended regular public schools where much of her instruction -- including math and reading -- was in mainstreamed classes.

"There were no sign language interpreter ugh," she writes. "I just read teacher's lips sometimes it was difficult to read some people's lip was so thin ha ha."

In 11th grade, concerned that she wasn't being adequately prepared for college, Smith transferred to the California School for the Deaf in Riverside, where she learned to sign and was reunited with many of her friends.

At Golden West College at Huntington Beach, Calif., she earned an associate degree as an office technician, then landed a clerical job at an insurance agency. Her employers liked her work so much they spoke of hiring more deaf employees, to be placed under Smith's supervision.

Her career stalled after she married and moved to Pomona. Unable to find office work there, she took a job as a power sewing-machine operator.

She and her husband had a daughter, Molly, now 22. Three years later she gave birth again ... and again.

"We thought I was pregnant with one," Smith says, "then out came another."

Twins Cathy and Carrie weighed 4 pounds each and soon made their presence felt.

"It was very overwhelming," Smith says. "I was busy all the time. But my mother helped me."

Smith's eyes had begun troubling her in college, but no one identified the problem.

"I was fighting with the doctors," she says. "They kept telling me I was fine. I didn't think I was. People would wave to me and I would totally miss it. My family was concerned."

Smith was nearly 30, with three young children, when a doctor finally diagnosed Usher Syndrome. She and her parents were devastated.

"I was so angry and saying why me and why me," Smith says.

Her marriage ended a short time later and she collapsed into depression.

Her mother says, "I was just frantic, trying to get her help."

Smith and her kids moved in with her parents, who were still in Orange County. They lived together for five years.

Before Smith's vision failed completely, the state of California and community organizations paid to send her to the Helen Keller National Center for Deaf-Blind Youths and Adults, on Long Island, N.Y. There she learned the skills that would prepare her for a life without sight. She was there 10 months.

"She learned to live as a blind person," says her mom, Patricia McDermott. "As a matter of fact, she had too much eyesight. They had to blindfold her to teach her."

It was an emotional time. For six months Smith rebelled at using a white cane because it made her feel "fully blind."

"That means I did not accept my (declining) vision at all," she writes.

Through counseling and support groups, which Smith calls "solve problems groups," she began the long process of reconciling herself to her genetic blueprint. The struggle continued through 16 years of gathering darkness.

"Finally I accepted (it) two years ago," she says, "imagine that."

Although her psychological journey had just begun, Smith left the Helen Keller Center with the practical skills to take the first steps. She writes:

"When I returned home from Helen Keller I felt a big change in my life not Janie anymore ha ha. I felt so good and happy to be able my own independently to travel by cane to ride bus and walk in malls ..."

Smith's mother offered her a different kind of opportunity by posing a question: What is the one thing in the world you really want to see before your vision slips away?

"I'd like to go to Washington, D.C.," her daughter said. "I'd like to go to the White House and see President Reagan -- all the things I've studied at school."

McDermott wrote to Nancy Reagan, asking if there was any way her daughter could meet the president.

Six days later they got a call inviting them to a celebrity-studded tennis match and dinner at the White House on May 24, 1981. The event was to thank companies that had supported Nancy Reagan's "Just Say No" antidrug campaign.

"Do you think you can come?" asked the first lady's secretary.

They went, and were treated like royalty, starting with the spit-and-polish Navy guard who greeted them at the White House gate.

"Janie could see him," her mother says, "and she absolutely died."

They sat front-row center at the tennis match, directly in front of the Reagans, then queued up to go through the receiving line to shake the First Hands. The Navy band burst into tune as the guests sipped cocktails in the East Room and grazed at a buffet in the state dining room.

At 7 that evening, when the party disbanded, Nancy Reagan's secretary approached the McDermotts with another invitation.

"The President and Mrs. Reagan are going upstairs," she said, "and they would like to meet you and Janie personally."

Just Say Yes.

"They were so wonderful and warm," Patricia McDermott says, "and they hugged me. I said, `This has been the most wonderful day for Janie."'

That was the day Janie Smith turned 30.

One night in 1988 Smith accompanied a friend to a Halloween party in the San Fernando Valley. There she encountered Dan Smith, a man she'd known throughout her school years, since they were preschoolers at the John Tracy Clinic.

They started dating and were married 10 months later at Fullerton, Calif. Suddenly Smith's world expanded to include Dan and the three children from his first marriage, two of whom came to live with them. With her own three kids, it was a full house.

Dan proposed, the story goes, after learning that Smith and her parents planned to move to Seattle, where they had relatives. McDermott says Dan left a good job in California to be with the childhood friend he had grown to love. Three days after the wedding, they all moved to Seattle.

Smith got a job at the Lighthouse for the Blind and worked there for eight years, mainly operating a power sewing-machine.

Last year, with their kids out of the nest, Dan and Janie moved from a four-bedroom house in Bellevue

to a townhome at Port Orchard's McCormick Woods, where Dan can indulge his taste for golf.

"They're just like honeymooners," says McDermott.

Last month the Smiths rendezvoused with some of their kids in Las Vegas, where Janie enjoyed the "very beautiful new hotels" and won $135 at one of the casinos.

At Christmas the entire family -- including an infant grandson, Jared -- gathered at their new home. Smith baby-sat one day while Jared's mom was in Seattle. "I had fun to have him smile," she writes. "He is happy baby."

McDermott, who used to drive her daughter to doctor appointments and other errands, says, "Janie has gotten so much more independent since she moved (across the Sound). It was hard to let her go, but I wouldn't have stopped her for anything in the world."

The most challenging part of Smith's day is the arduous commute from Port Orchard to Swedish. Wearied by six months of full-time commuting, she and Dan recently took a tiny apartment near downtown Seattle as a part-time layover, but they aren't sure they'll keep it. Port Orchard remains home base.

On commute days they leave home by 5:30 a.m. to catch either the Southworth or Bremerton ferry to Seattle. Dan Smith then heads off to south Seattle, where he works as an accountant.

Janie Smith takes the bus -- sometimes two buses -- up First Hill with her guide dog, Maggie, a golden lab.

Sometimes Smith, like the fictional Blanche Dubois, is forced to rely on the kindness of strangers. She carries printed cards, also labeled in Braille, that tell passers-by what kind of assistance she needs -- crossing a busy street, perhaps.

Metro drivers are required to stop for people hold-ing up bus-route numbers on the distinctive black-and-yellow cards that identify the deaf-blind.

"They stop and open the doors for me," Smith says. 'I feel the warm air. That's how I know the door is open."

She hands the driver a printed card that explains where she needs to go and asks for a warning tap when her stop comes up.

"Usually," Smith said, "the driver will shake my hand as I leave."

From there, Maggie guides her down the street and up the hospital steps. Smith holds Maggie's tail out of harm's way as they circle through the revolving door.

She leaves her backpack and coat in a room near the volunteer office, then pulls sterile scrubs over her black jeans and knit top.

This is where Maggie gets off. The golden lab retires to a large kennel in the corner and patiently awaits Smith's return. She used to bark mournfully, but volunteer coordinator Karen Stay says, "Now that she knows Janie will come back, she's OK."

Smith unholsters her collapsible white cane. It guides her to the sterile-processing room, located around the corner and one flight down. Inside the lab, she follows a trail of abrasive floor tape that was applied as a tactile guide to her work area.

Smith works intensely throughout the morning, plucking new supplies from carefully arranged bins and sorting through each drawer's leftovers for salvagable supplies. Her sensitive hands skim the piles searchingly, then strike like a hungry hawk.

When Smith approached Swedish about volunteer opportunities, no one was more surprised than her father, Frank McDermott, a hospital volunteer for nine years. At 83, a year after open-heart surgery, he still assists one day a week in the pharmacy at Swedish.

"What possibly is there for her to do?" he asked Stay.

Stay wasn't sure, but her background in occupational therapy -- finding adaptive ways to master everyday tasks -- made her a trained optimist. "There's got to be some way," she said.

She hit pay dirt right away, when the sterile-processing department found a slot for Smith and made her one of their own.

"The staff love her," Stay says. "She's got a very good sense of humor. Just her body language -- you can tell when she's tickled."

Frank McDermott is proud of his daughter's gumption.

"When she wants to do something," he says, "you just better accept it. I just hear nothing but good things (about her) around the hospital."

Lunchtime, always welcome, is especially exciting this day as the department gathers for a holiday potluck.

Cheek-by-jowl in a small meeting room, Smith and her co-workers heap their plates with pasta and green salads, sliced ham and dips. Smith savors a bite of holiday pie. "Pun-i-kin," she murmurs, recognizing the taste of pumpkin and spice.

A relaxed warmth fills the room as the gift exchange begins and wrapping paper goes flying. To oohs and aahs, someone holds aloft a gravy boat in the shape of a sleigh. Envious laughter greets someone else's trophy, a bottle of premier whiskey. Smith joins in the joke, placing hand on cheek in an expression of mock scandal.

Her own gift, courtesy of Clemmer, is a fluffy white snowman with a "carrot" nose and red and green muffler. Smith gives it an exuberant hug.

"I love him!" she signs, reaching over to pat Clemmer's shoulder.

Everyone clusters for a Kodak moment. Smith, towering in the front row, jokingly bends her knees. At this gesture of humorous self-awareness, you wonder anew -- is this amazing woman really blind?

The answer comes a few minutes later, when she walks smack into a half-open door that you've failed to guide her around. A red welt appears on her cheek.

Yes, she's really blind. And, boy, did you screw up.

As 4 o'clock approaches, Smith checks her Braille watch more frequently. Dan is coming soon to drive her to the ferry. Working her way through an endless stack of drawers, she finally throws up her hands with a harried look that says, Enough already; the rest can wait.

Back at the office, she greets Maggie and gives her some kibble before changing into her civvies. Breathing heavily, she fights to rethread a shoe lace that has slipped from a grommet of her gray hiking boots.

With Maggie in harness, they head for the elevator, where Smith presses both the up and down buttons. When a car arrives, she doesn't know which way it's going, but either way she'll eventually reach her destination.

Outside, in the gathering darkness and bitter wind, Smith carries out one of the most amazing feats of the day:

When Maggie leaves her calling card on a patch of grass, Smith fits a plastic bag over her hand, bends down and deftly removes the offending deposit. She rolls the bag back over her hand, turning it inside-out, then ties a neat knot with an efficiency that borders on sleight-of-hand. She deposits the whole affair inside a paper bag.

Maggie whimpers from the chill wind, so they retreat to the vestibule. Dan Smith pulls up in a dark green Honda SUV. Tall and gray-haired, with a friendly manner, he leads his wife to the car and installs Maggie in the back. They wave and smile, then they're gone.

Smith's parents, who worked so hard to smooth their daughter's path, have made peace with a diagnosis that once seemed so hopeless.

"Have I accepted it?" Patricia McDermott asks rhetorically. "I had to, to help her."

Frank McDermott proudly calls his daughter's accomplishments "just amazing."

Life goes on, as Janie Smith assured her mother recently when they met for lunch.

"Mom," she said, "I can't see you anymore but don't be sad. I think this is the way God wanted me to be."

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