Machine stores speech for patient's future use
Thanks to technology, those with ALS can continue to 'talk'
Published 9:16 am, Wednesday, August 14, 2013
Carole Shearn isn't quite sure when she will lose the ability to speak, but she is sure of one thing: Her voice will still be heard, even when the words can no longer be spoken from her lips.
The 70-year-old West Palm Beach, Fla., resident was diagnosed with ALS, also known as Lou Gehrig's disease, last October. According to the National Institute of Neurological Disorders and Stroke, 20,000 to 30,000 people are living with ALS in the United States. The Connecticut chapter of the ALS Association reports that roughly 300 people in the state are living with ALS right now.
Shearn's form of ALS weakens the throat muscles first. The progressive disease has no cure, but at the University of Miami Miller School of Medicine's Department of Otolaryngology, Shearn is the first patient to take part in voice banking -- a program where patients who will eventually lose their voice due to diseases such as Parkinson's, ALS and cancer -- can record key words, phrases and personalized messages to communicate when speaking is no longer an option.
Shearn uses Tobii Assistive Technology, a speech-generating device, which stores her recordings into categories. She can then use a mouse, touch the screen or even use her eyes to retrieve the sound files.
In case of an emergency, Shearn has even programmed a message saying "get help immediately" and "call 911." Tobii, which is compatible with Bluetooth technology, will call for her.
"Truthfully, I was so amazed when I found out about the Tobii," she said. "It makes me feel good that I can personalize my messages to whomever. It is my lifeline."
ALS is a neurodegenerative disease that attacks the motor neurons. As the disease progresses, these neurons begin to degenerate and stop sending messages to muscles. Eventually, individuals diagnosed with the disease lose the ability to move their legs, arms and body. The average lifespan for a person with ALS is three to five years.
Because ALS patients lose strength and movement in their limbs, Tobii has a built-in eye tracker. Shearn can sit in front of the device -- without moving any part of her body but her eyes -- and still communicate effectively.
Over past few decades, assistive technology for people with ALS has been on the rise, said Juliana Gregory, a speech and language pathologist at Bridgeport Hospital. She said she knows of several different companies that make these kind of products, including DynaVox Mayer-Johnson, a Pittsburgh-based company that is a leading provider of speech generating devices used to help people with ALS and other conditions that affect speech, language and learning.
"It seems that every six months something new and wonderful comes along," she said. "The technology has improved so much, just over the past eight years (she's been working in this field)."
Voice banking is still a relatively new concept and it isn't yet widely used in Connecticut, said Dr. Peter McAllister, director of clinical research at Associated Neurologists of Southern Connecticut in Fairfield. But it's idea he expects to catch on in the near future. Though the current technologies are helpful for people losing speech to ALS, "the problem with them is that they use an artificial voice or someone else's voice," McAllister said.
Nevertheless, the current technologies are a lifeline for ALS sufferers, he said. "ALS is a devastating disease and lose your voice is one of the most devastating things for patients, because they lose the ability to communicate their needs," McAllister said.
Shearn, meanwhile, has been committed to retaining her voice in spite of her condition. Jocelyn Odlum, a speech pathologist at the University of Miami, met Shearn at an ALS support group in West Palm Beach and then began seeing her at the clinic. After evaluating Shearn, she got her started on voice banking. Shearn has been recording her voice every day for the past two months.
"Carole is an inspiration," Odlum said. "She took this disease and is doing everything she possibly can to be prepared."
Odlum said that once individuals are diagnosed with ALS, they should see a speech pathologist immediately so they can be introduced to these devices and helped. "Unfortunately by the time people come to see me they have no voice," she said.
Gregory said she's often helped educate patients about assistive devices.
Shearn has recorded basic phrases such as "Hello. My name is Carole," and "How was work?" She also has recorded some of her other favorite phrases: "What's up buttercup?," "What's new, super glue?," and "You snooze. You lose."
When Shearn was initially diagnosed, she had no idea what ALS was. She also didn't know that the disease had no cure. "That was very hard to hear," she said.
"I had tears in my eyes, of course, and I asked how long I would have to live and my doctor said he didn't know, three to five years, so we left the office on that," she said.
Shearn's daughter, Jennifer Wagner, had been suspicious of early symptoms she had been noticing -- slurred speech and choking spells, even when she wasn't eating. She researched her mother's symptoms and learned about ALS.
"It was very daunting and difficult to read," she said. "I had a pretty good idea of what the disease was and what it was going to entail, and I didn't want my mom to go through that."
Although Shearn can still drive and walk without any assistance, Wagner drives her mother from West Palm Beach to the university. She also started an open Facebook page called Carole's Crusade, where people can become more aware about the disease and follow her mother's progression.
"I know that I cannot cure the disease, so my main goal is to bring attention to it," she said.
For Shearn, keeping a positive mindset has helped her cope with the disease, but she acknowledges she is not as hopeful and optimistic as she was eight months ago because she notices herself "getting a little worse."
"I was certain I was going to live past 90," she said. "I would still like to." But if she doesn't, Shearn said she feels she has done everything she has wanted to do -- traveled to Rome, Alaska, Paris, England, Italy, Spain and the tip of Africa, been surrounded by good people, and had a successful 36-year teaching career.
"My life has always been about people so I don't crave a lot of outside influence," she said. "Our family is small, but we are tight. That has always been everything to me. I feel my life has been blessed."
Staff writer Amanda Cuda contributed to this report.