Alisa Brownlee, ATP, CAPS blog offers recent articles and web information on ALS, assistive technology--augmentative alternative communication (AAC), computer access, and other electronic devices that can impact and improve the quality of life for people with ALS.
Email--abrownlee@alsa-national.org.
Any views or opinions presented on this blog are solely those of the author and do not necessarily represent those of the ALS Association.
Scientists at Rice University, the University of Houston
(UH) and TIRR Memorial Hermann have received a $1.17 million grant from the
National Institutes of Health (NIH) and the President's National Robotics
Initiative (NRI).The combined device
will be validated by UTHealth physicians with as many as 40 volunteer patients
in the final two years of the four-year R01 award, the oldest research grant
offered by the National Institutes of Health (NIH).
The multidisciplinary team hopes to develop and validate
a noninvasive brain-machine interface (BMI) to a robotic orthotic device that
is expected to innovate upper-limb rehabilitation. The new neurotechnology will
interpret brainwaves that let a stroke patient willingly operate an exoskeleton
that wraps around the arm from the fingertips to the elbow.
Purchase this photo at dailystatesman.com NOREEN HYSLOP - nyslop@dailystatesman.com Farel Robins of rural Advance, who suffers from ALS, is shown sharing a lighter moment with his wife, Eileen, as he utilizes a specially equipped computer that voices concerns or commands. ALS strikes about five in every 100,000 people worldwide. [Order this photo]
By NOREEN HYSLOPManaging Editor ADVANCE, Mo. -- At just over six feet, five inches tall, Farel Robins was always a towering figure. A retired postal clerk, Robins has always had a passion for flying and has been an accomplished pilot for most of his 68 years. But Robins no longer walks tall, and the veteran pilot has taken his final flight above the skies of Stoddard County. Robins was diagnosed with ALS nearly three years ago. The first symptoms appeard in December 2008. "We were out shopping one day," recalls his wife, Eileen, from their spacious rural Advance home, "and suddenly he was dragging his right foot behind him. We had no idea why. I was afraid he had suffered a stroke." The initial visit to the doctor resulted in a prescription for therapy for a condition called "drop foot." Therapy was not at all beneficial at the time. It was a prescription, the couple would learn later, that did more harm than good. But the couple was months away from a confirmed diagnosis of ALS, and were following doctor's orders. Finally in September 2009, the Robins drove to St. Louis for an appointment at Barnes Hospital. Experts there confirmed that Farel had amyotrophic lateral sclerosis -- more commonly referred to as ALS or Lou Gehrig's disease. ALS is a disease of the nerve cells in the brain and spinal cord that control voluntary muscle movement. The disease strikes about five out of every 100,000 people worldwide. In about 10 percent of the cases, ALS is caused by a genetic defect. The cause is unknown in the other 90 percent of patients. The nerve cells of an ALS patient wither away and die. As the disease takes its toll, the nerves are no longer capable of sending messages to the muscles. The process leads to first weakening of the muscles, twitching, and then an inability to move the arms and legs, eventually affecting the entire body. There is no cure for ALS. It is an ugly, unforgiving disease that, in the end, leaves its victims virtually motionless. The brain, however, is unaffected by the disease, and so the patient is ever aware of his or her diminishing capacity as the illness progresses over a period of three to five years. In the end, the disease robs the patient of the ability to swallow. When the muscles in the chest stop functioning, it eventually becomes impossible to breathe on one's own. Farel Robins is in the late stages of ALS. The man who once stood so tall is now confined to a wheelchair, specially designed for his height. And he seldom takes a breath without the aid of oxygen. Until recently, he could sip from a straw and relieve a dry mouth with ice chips, but those days are now behind him. He is equipped with a feeding tube. For a four-hour period each day, a smooth liquid meal of nutrition makes its way into his stomach through a line that is fed into a port on his left side. He is lifted with the aid of a swing-type brace that is placed under and around his long frame. Eileen helps Farel as the belted contraption "hoists" him from one setting to another. Eileen is never far from her husband's side. Since a specially equipped recliner now offers more comfort than a bed, he spends his nights there. "I just pull up a twin mattress and lay it down on the floor next to him in case he needs me through the night," Eileen says, with the same smile that gets her through the everyday continual challenges of the disease. Eileen has witnessed the steady decline of her husband for nearly four years. Every doctor's visit entails a breathing capacity test. "He still had 58 percent lung capacity last year," she notes. "But that dropped down into the 40s, and in June this year, it declined to 19 percent. When we visited the doctor in August, he didn't want to tell me what the number was." Farel's voice is now reduced to a raspy whisper, and his breathing is shallow. And yet, he smiles and converses with what little speaking ability remains. His wife acts as an interpreter when the words don't come or when they are too difficult to comprehend. Despite the toll the disease has taken and the fear of the unknown, Robins projects a positive attitude, never questioning why he has been stricken with such a devastating diagnosis. Rather, he still enjoys a little time each morning at his computer, catching up with friends with Eileen's assistance, and reading some online news. He follows a daily routine that changes only as more limitations surface. Frustration seems to be a thing of the past. For Farel Robins, there is no point in dwelling on the 'whys' -- he'd rather wake up spending what quality time remains for him in the company of a devoted wife who approaches each task seemingly with the ease and poise of an expert in the field. "I've learned to do what I have to do," Eileen explains. "We've been very fortunate to be equipped with this equipment to help Farel. I have no doubt that without them, he would not still be here."The machines to which Eileen refers are within her reach at all times. One is a cumbersome "cough assist" unit. When phlegm makes its way to Farel's chest or throat and causes choking, Eileen places a mask from the cough assist over her husband's face. Once in place, the mechanism forces air into the chest and then pulls it out, usually releasing any obstruction. If that doesn't suffice, she uses a suction tube that she places down Farel's throat in a second effort to clear the airway. "That's a very scary procedure," Eileen says, "but when he can't get relief from the cough assist, that's what we have to do." Another device is utilized twice daily for 20-minute intervals. Again a bulky device, it wraps around the chest, and once put into operation, it literally shakes the chest wall, vigorously vibrating to loosen any chest congestion that has developed as a result of Farel remaining in a stationary position. At night, Robins is equipped with a bipap machine that works by maintaining a certain amount of air pressure within a mask that he wears while sleeping. The air pressure keeps the wearer's airway open, allowing him or her to breathe easily while at rest. A former waitress, Eileen's full attention these days is on her husband. Other than bi-weekly, one-hour long visits from a visiting nurse organization and occasional visits from a friend and neighbor who is an RN, Eileen is her husband's caregiver. Farel is limited to the use of only his left hand these days. A uniquely programmed computer is available to serve as his voice. His functioning hand wraps around an extended wooden dowel that gently taps an icon on the computer screen. An audible command says, "I need a pillow," or "Please get me a drink of water," among a number of other requests. "Most of them are outdated now," his wife says, indicating that he can no longer drink or eat. In spite of the outdated data, though, Farel smiles at his wife and teasingly taps out a command. The couple deals with the inevitable with grace and an appreciation for every day that dawns. Eileen seems to judge her husband's needs by only the look in his eyes and is quick to respond to any request. They still anticipate sunny days and special television broadcasts to watch together. Farel fights tears as he tries to find the words to describe how one prepares for life's end. When words don't come, his eyes tell the tale and his wife, who is now his voice, interpets. "We just take one day at a time and consider it the gift that it is," she says, with a squeeze of Farel's hand.
The next time you consider taking a sick day, think about this guy. Avichai Kremer was 29-years-old and on top of the world. A native of Israel, he’d been accepted to Harvard Business School and was just beginning his studies within those ivy walls when the devastating news arrived.
After feeling numbness in his fingers, which made holding a coffee mug difficult, he was diagnosed with ALS – Amyotrophic Lateral Sclerosis — commonly known as Lou Gehrig’s disease, a degenerative condition in which muscles gradually atrophy, ultimately leading to complete paralysis. After nine years with the disease, Kremer is unable to move his arms, legs, or to speak. But his brain functions perfectly as does his brilliant mind, which he decided to put to very good use.
When he received the terrible news, Kremer retreated to bed depressed for several days, but upon arising, he decided he had to take action, both for himself and for the hundreds of thousands with his condition. He recruited friends from Israel, his Harvard classmates and professors who together founded the American non-profit Prize4Life.
Of course, as business school students, they had to put a capitalist twist on the project (which is ironic considering Chinese communist revolutionary leader Mao Zedong is believed to have had ALS!). Kremer and crew constructed a business plan, bringing their financial acumen and innovative spirit to the project focused on how to find a cure that’s evaded scientist for 140 years.
Kremer tells TheBlaze:
I was a young student at Harvard Business School then and the world was my oyster. To receive the diagnosis of a disease which is 100% fatal within 3-5 years on average, was incomprehensible. For a few days I simply couldn’t get out of bed. But I knew it was also an opportunity; an opportunity to use my business skills – and the skills of my HBS classmates – to make a difference. An opportunity to change ALS. An opportunity to show that hope is stronger than fear.
Early on, Kremer and his colleagues realized they needed to redefine the game. They knew that great breakthroughs come from unexpected places. But how to lure the top minds to research ALS? While other medical charities offer grants for ongoing research that may or may not succeed, Prize4Life believed a radically new approach was needed. As students of history, they realized offering inducement prizes that reward only successful results was the way to go. Kremer explains:
Inducement prizes worked for centuries to incentivize people — they attract attention to a problem and define what is needed to solve it. Furthermore, looking from the donors’ perspective, prizes are very appealing — you only pay for results!
Last year, they granted their first million-dollar prize that rewarded the scientist who developed a biomarker that tracks the progression of the disease cheaply and accurately. This means clinical trials will be less expensive and more efficient. This year, Prize4Life launched its second offer: one million dollars to whoever can develop a treatment to prolong survival in mice by 25%.
Prizes have driven innovation for a century. Most famously, the Orteig Prize encouraged Charles Lindbergh to be the first aviator to cross the Atlantic. Kremer says [emphasis added]:
I view Prize4Life as a start–up and myself as an entrepreneur. Entrepreneurship is about seeing an opportunity and seizing it. And we use our prize model to create markets and action, where there was none or little before. We are using our prizes strategically to lower the barriers to entry. We are helping find drug leads, making clinical trials more efficient. We want people to do good – we want this disease cured – but we also understand it’s a business and want people and companies to win, financially, for doing so. We don’t ask for any rights in any inventions that are made — we want the inventors to keep their full motivation to get to a real, viable product and make money.
While the goal of entrepreneurship is to capture value, usually monetary. For a man facing death, money is insignificant. Shakespeare knew it when his Richard the 3rd said: “my kingdom for a horse!” Hope is my currency of value. A purpose is another. Purpose to still make a difference even if there’s a chance you won’t be around to enjoy it. I get both from Prize4Life. Would I have done it if I wasn’t a patient myself? Probably not, unless someone close to me was a patient. Money is a powerful incentive, and a very convenient one being able to trade it for almost anything, but it can’t be traded for everything (like an ALS cure) and in those cases, a higher from of value capturing kicks in.
Kremer operates a censor between his eyes to type on virtual keyboard. A screen for guests communicates his words.
TheBlaze visited Kremer in his Haifa home, where he painstakingly communicates using his facial muscles to move a sensor between his eyes to type each letter on a virtual keyboard on the screen in front of him. What he lacks in motor abilities, he makes up for in mental acuity, creativity and optimism. He told Israel’s Channel 2 News: “Today is wonderful because I woke up alive. All the rest is a bonus.” [Hebrew link]
Kremer and his team have successfully raised $10 million over the past six years. We asked him to describe the challenge of running the organization across the globe headquartered in Boston in light of his physical limitations. Kremer says:
Doing everything is harder with my physical limitations. As you can see I can’t speak or move my hands anymore and I communicate by slowly typing with a sensor attached to my forehead and I need to move it for every letter. I cannot walk and am being fed through a tube to my stomach. Soon, as my breathing muscles weaken, I’ll need the help of a breathing machine. Still, my brain remains intact and with the assistance of modern technology I manage to run the organization. For every motor function that I lack I compensate with extra dedication, extra persistence, extra creativity, because I am working to save my life and the lives of the people who are being diagnosed today … who are going to get it next year. The disease is relentless and will keep coming, and will keep killing us, and we must be relentless too if we want to beat it. I am not going to leave any stone unturned.
That dedication is being recognized in high places. Kremer won the Dean’s Award at his Harvard graduation. His main goal then was to walk with his own feet to accept his diploma, which he did, to a standing ovation from his classmates.
Avi Kremer accepts M.B.A. and Dean’s Award from Harvard Business School, June 2006 (Screen shot from: Israel Channel 2)
Others are taking notice. Israeli President Shimon Peres said of Kremer: “Although people sometimes receive an extremely harsh sentence, the only option that we have is not to surrender.” And last year, Kremer received the prime minister’s prize for innovation from Benjamin Netanyahu.
Avi Kremer receiving award from Israeli Prime Minister Benjamin Netanyahu last year (Screen shot from: Israel Channel 2)
At the ceremony, his mother Hedva read the speech Kremer wrote, in which he said:
Regrettably, I can no longer speak. But I will not let my illness take away my voice. Leonard Bernstein once said, “In order to achieve great things, two things are required – a good plan and not enough time.”
“It’s about creating change,” colleague Neta Zach, who is the scientific director for Prize4Life Israel says. “To be around Avi is extremely inspirational.”
Kremer says the most important thing he wants people to know is, “We are racing against time to save hundreds of thousands of lives, and everyone can help.” For more information on Prize4Life and ALS, visit their website.
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