ALS Advocacy
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Susan Cohen / Rapport for Newsweek
Benched: Medical science has made virtually no
progress
MY TURN
Diagnosed with a deadly but uncommon illness, I call upon the game of my youth to take action.
By Michael Goldsmith | NEWSWEEK
Published Nov 1, 2008
From the magazine issue dated Nov 10, 2008
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I received my death sentence in September 2006 when doctors told me I had amyotrophic lateral sclerosis (ALS), a progressively paralyzing neuromuscular disorder. There is no cure. Commonly known as Lou Gehrig’s disease after the Yankee Hall of Famer who died of it, ALS is so uncommon that medical researchers consider it an “orphan” illness—so few people have it that pharmaceutical companies lack financial incentive to invest in finding a cure.
The public also pays scant attention to ALS. (May 2008 was ALS Awareness Month. Who knew?) Public attention and contributions understandably go to more widespread killers like cancer, heart disease and diabetes.
All this means that ALS patients must seize the initiative for funding research. Of course, the vast majority of ALS patients are too sick and incapacitated to take such steps. I am one of the lucky ones. My neuromuscular decline has been steady, but slow enough to let me lead a reasonably normal life. After holding endless pity parties for myself, I decided—not entirely successfully—to transform myself from victim to ALS funding advocate.
Lacking any fundraising experience (I’ve rarely even asked for a pay raise), I took some time off and returned to my childhood roots: the baseball field. While I still had the strength to hold a bat, I attended a Baltimore Orioles fantasy baseball camp. Some might call it Old Man’s Little League, but I reveled in what would likely be my last chance to play the game of my youth. And as a lifetime Orioles fan, this particular camp held special appeal to me.
I expected to have a good time. I did not expect to find the potential solution to my ALS fundraising problem. But I did.
If Little League makes men out of boys, Orioles camp makes boys out of men. The games were highly competitive, but they were also marked by youthful enthusiasm, pure joy and moments of compassion. When my teammates saw me struggling to swing a standard bat, they bought me a lighter one that could still generate power (this helped, but often I just missed the pitch faster).
We hung out with former Orioles, most of who were blue-collar guys thrilled to have made it to the majors. They didn’t just give us cursory face time; they coached us intensively and did their best to improve our game. Everyone played, talked and laughed baseball. Orioles manager Dave Trembley told us how he tried to get thrown out of a game without using cuss words; it wasn’t easy, and he succeeded only after calling the umpire a “den mother.” There was much more. We also shared life stories, and I learned that I was not the only one battling a terminal disease.
At some point, we talked about what Major League Baseball could do to fight ALS, and I realized that next July 4 will mark the 70th anniversary of Lou Gehrig’s famous farewell speech at Yankee Stadium. Since his retirement, more than 600,000 Americans have shared Gehrig’s fate, as medical science has made virtually no progress toward finding a cure. Through the years some players and a few teams have occasionally helped raise funds, but Major League Baseball has never taken comprehensive action against ALS. Defeating ALS will require the same type of determination, dedication and drive that Gehrig and Cal Ripken demonstrated when they set superhuman records for consecutive games played. With this in mind, why not make July 4, 2009, ALS-Lou Gehrig Day? Dedicate this grim anniversary to funding research for a cure; every major- and minor-league stadium might project the video of Gehrig’s farewell, and teams, players and fans could contribute to this cause. An event of this magnitude has the potential to raise millions, dwarfing the relatively scant sums that ALS walks, rides and similar small-scale efforts have produced.
To this day, Lou Gehrig is still named in some polls as the greatest player in baseball history; by all accounts, he also had a reputation for uncommon decency. His legacy for greatness will live forever, but it’s time to end the heartbreaking legacy of the disease that bears his name. Major League Baseball can help make that happen.
Of course, this is just a distant dream of a single ALS patient who played baseball every day of every summer growing up. I now look to the game of my youth to help give me and others like me a chance for life.
Goldsmith lives in Heber City, Utah.
© 2008
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The ‘EYES’ Have It |
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by Alyssa Quintero
Eye-controlled devices, commonly referred to as eye-tracking or eyegaze systems, enable users to access speech-generating devices and computers. Eye control isn’t without its challenges, but many with ALS say the technology has changed their lives, providing greater independence and allowing them to live better. In recent years, eye-control technology has evolved, resulting in systems that are faster, more accurate, smaller and more portable, highly customizable, more cost-effective, and typically able to be calibrated in 30 seconds or less. The June ALSN article “A Complete Communication Solution” discussed dynamic display (large touch screen) alternative augmentative communication (AAC) devices with multiple access methods, which also serve as fully functioning Windows XP computers. Eye-controlled systems open these speech-generating computers to users with little or no movement. Most systems can be mounted to wheelchairs, floors, desks, tables, beds, etc. Here’s a look at what users are saying, all with their eyes. ‘I love you’ Terry Sickels of Akron, Ohio, received an ALS diagnosis in late 2005. When he started experiencing speech problems last year, Sickels, 58, tried several AAC devices, but none worked very well for him. Sickels’ wife, Tammy, researched other options and discovered eyecontroltechnology and the MyTobii P10, manufactured by Tobii Technology Inc. in Sweden, and distributed in the United States by Tobii Assistive Technology Inc. (Tobii ATI). From the start, it worked well for him. Sickels uses the dwell selection method in which he focuses steadily on a certain part of the screen for an adjustable period of time before the device makes the selection. “The most consistent movement I have is my eyes, and this is the only unit that accommodates that without recalibrating all the time,” Sickels wrote via e-mail. He uses the device for text-to-speech, as well as Internet, e-mail, text messaging and environmental controls such as the television remote. Sickels also uses buttons programmed with greetings, questions, yes/no answers, and other generic sayings. Customizing the system with phrases, names and topics has made it more personal and easier to have conversations.
Sickels has difficulty using the system’s onscreen keyboard but is improving with practice. Currently he can type about 10 to 15 words per minute. The premade text boxes work well in the meantime. “We’re not speculating what he’s trying to say anymore,” Tammy says. “He’s more relaxed, and he’s got that little spark in his eyes again. He’s laughing more, and it seems like he’s having fun because he has some freedom, and he’s more in control.” Sickels now can tell doctors and therapists how he’s feeling or if he’s experiencing pain. Prior to appointments, Sickels creates text buttons that ask questions for him. Because he tends to lock on message buttons when he doesn’t mean to say anything, Sickels and his caregiver created an onscreen “rest” button that he can click with his eyes to go to a “blank” page. A “return” button allows him to go back to the previous page when he’s ready to resume speaking. After using the device less than a month, Sickels surprised Tammy on her birthday by programming buttons that said: “Happy 50th Birthday,” “I have a present for you,” and “I love you.” “It was more personal for the words to come from me as opposed to having someone else say it for me,” Sickels writes. “I’m tired of people treating me like I’m not in the room. This device allows me to voice my opinion, offer suggestions and show people that I’m the same person I was before. People have started to talk to me and ask me questions.” ERICA speaks Don Taylor of Collierville, Tenn., has used Eye Response Technologies’ ERICA system since June 2007, and writes via e-mail that the learning curve wasn’t very steep because he’d been using onscreen keyboards with word prediction through the EZ Keys software prior to ERICA. “I have over 22 years of computer experience, and I demand my computer system to be well integrated so I can do all the things that I could do before I became disabled,” Taylor, 51, wrote via e-mail. “I don’t want to be slowed down by a system that offers scanning or Morse code. And, ERICA runs on a Windows platform, so I can run the same applications that I ran on my desktop computer.” Taylor, who spends most of his time on the Internet, began typing with his eyes immediately after the initial setup. He maintains a blog and serves as the Webmaster for his son’s Boy Scout troop. He credits ERICA’s tech support and software upgrades with helping him stay on track. He adds, however, that every time he turns off or moves away from the system, he has to recalibrate, which he can do without assistance. “What I like best is the way the makers of ERICA designed the software to allow me to do everything with my eyes that I would do if I could type with a keyboard,” Taylor says. While he recommends the system to others with ALS, he wouldn’t suggest it for people who don’t have any experience with a Windows-based computer because “it’s not stable enough and requires maintenance, including virus protection, Windows updates, data backups, etc.” One drawback for Taylor is that the camera is fragile; he says the system would be more versatile if the camera were built into the system (it sits under the screen). And, he cautions that moving ERICA onto the wheelchair mount from the floor mount is difficult. “No computer system is without flaws,” Taylor notes, urging potential buyers to “talk with a user in addition to the salesmen.” To learn more about Taylor’s experiences with ERICA, visit his blog (http://alsdon.blogspot.com). It’s going to cost …
While eye-controlled communication devices sound like a great solution, many speech-language pathologists and AAC specialists caution that what works for one person may not be the ideal solution for another. To learn more, contact the manufacturers, as they may be able to put you in touch with users who have ALS. Of course, each manufacturer asserts that its system and camera is faster, more accurate, easy to calibrate, has better IR sensors and mounting solutions, etc. But since eye-controlled systems are pricey, in the end it’s advisable to “try before you buy.” Start by working with the speech therapist at the local MDA clinic, and check with the local MDA loan closet to try loaner devices. In addition, most manufacturers have rental or loan programs; in some cases, rental costs can be applied to the purchase price. These programs also are helpful if someone needs a loaner device for an extended period such as while waiting for insurance approvals or lengthy delivery times. Dedicated AAC systems with eye-control options typically range in price from approximately $6,800 to $15,000. The cost increases with additional hardware and software options. (See“Eye Technology Manufacturers.”) Medicare will cover up to 80 percent of the cost for an AAC device, and MDA offers a one-time $2,000 grant for devices prescribed through its clinics. MDA also will provide $500 annually for repairs and modifications.
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Congress Appropriates Additional Dear James, We are excited to let you know that The ALS Association achieved another victory in Washington this week when the President signed into law the FY 2009 Department of Defense Appropriations bill. For the first time, Congress included $5 million in the bill to fund the ALS Research Program (ALSRP) at DOD! We now have a congressionally established program at the DOD that is dedicated solely to ALS research. It is the only ALS specific program at the DOD and is the only program focused on translational research with the goal of finding new treatments for ALS. This is an especially significant victory not only because very few funding bills even passed Congress this year, but also because we overcame a significant amount of competition for scarce federal dollars. The $5 million we secured will be available to ALS researchers across the country. Moreover, the collaboration facilitated by the ALSRP and the additional dollars will provide the ALS community with greater opportunities to develop meaningful treatments. Funding Continues Critical Partnership with DOD We look forward to continuing our strong partnership with DOD in 2009 to advance ALS research and find a treatment. Thank You! For the third time in the past two weeks, we have succeeded in accomplishing one of our top priorities. Passage of the ALS Registry Act, implementation of historic new regulations for veterans with ALS, and now funding for ALS research could not have been accomplished without the active involvement of the entire ALS community. THANK YOU!! Join the Roll Call of Veterans! If you have any questions, please contact us at advocacy@alsa-national.org or 1-877-444-ALSA. Thank You! |
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**************************************************************************************************************ALS Registry Act on its way to President’s Desk |
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A few minutes ago, the United States House of Representatives passed the ALS Registry Act!! The legislation now heads to President Bush, who is expected to sign it into law. Thank you to everyone for your tireless efforts to reach out to Congress. We are just one step away from realizing one of the most significant accomplishments for people with ALS and their families. A national ALS patient registry at the Centers for Disease Control and Prevention will collect information leading to the cause, treatment and cure of ALS. You should be proud of your efforts. We have made a difference! The building blocks for a national registry are underway and the ALS Registry Act will enable us to take the next steps in building the registry and advancing the fight for a treatment and cure. Again, thank you!! We will let you know once the President signs the bill into law. In the meantime, please make sure to thank your Senators and U.S. Representative for their support. Let them know that their efforts are appreciated and have not gone unnoticed. Sample letters you can send are available in the Advocacy Action Center of our website, here. Thank you! |
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It’s Official!!!
VA to Grant Benefits to All Veterans with ALSWe did it! Tomorrow, September 23, the Veterans Administration will publish regulations officially establishing ALS as a service connected disease! Effective immediately, all veterans with ALS will now have access to the highest level of VA benefits without having to prove that their disease was caused by service in the military.
This is a tremendous victory for all veterans and is the culmination of years of work by The Association, our VA Issue Team and veterans across the country. It is clear that our outreach - testifying before Congress, partnering with the VA, advancing research to identify the connections between ALS and military service and educating the public about this issue - have paid off. The Association has championed legislation (H.R. 5454) that would have established ALS as a service connected disease. However, thanks to our efforts at Advocacy Day and throughout the year, this legislation no longer is needed!
ALL Veterans with ALS Eligible for Benefits
This new policy means that ALL veterans with ALS will receive the benefits they need, deserve and have earned. Importantly, it is broad in scope and applies to all veterans diagnosed with ALS regardless of when or where they served and regardless of when they were diagnosed with the disease following service in the military. A summary of the regulation can be found here. The text of the regulation is available here.
Tomorrow and in the coming days, The ALS Association will post additional information about the regulation, including answers to frequently asked questions, an overview of benefits available to veterans with ALS, as well as guidance veterans can use to obtain service connected veterans benefits.
The ALS Association would like to thank VA Secretary James Peake, Congressman Henry Brown (R-SC) and Senator Lindsey Graham (R-SC) for their efforts to work with us and the ALS community in support of veterans across the country. We also would like to thank all veterans with ALS whose outreach to Congress has helped make this important benefit possible.
Additional information will be available on our website on September 23. If you have any questions, please do not hesitate to contact the Advocacy Department at advocacy@alsa-national.org.
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WHAT IS ALS?
Amyotrophic Lateral Sclerosis (ALS, sometimes called Lou Gehrig’s Disease, or Maladie de Charcot) is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. As one of the motor neuron diseases, the disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, develop fasciculations (twitches) because of denervation, and eventually atrophy due to that denervation. The patient may ultimately lose the ability to initiate and control all voluntary movement except of the eyes.
Cognitive function is generally spared except in certain situations such as when ALS is associated with frontotemporal dementia[1]. However there are reports of more subtle cognitive changes of the frontotemporal type in many patients when detailed neuropsychological testing is employed. Sensory nerves and the autonomic nervous system, which controls functions like sweating, generally remain functional.
Cause and risk factors
Scientists have not found a definitive cause for ALS and the onset of the disease has been linked to a several factors, including: a virus; exposure to neurotoxins or heavy metals; DNA defects; immune system abnormalities; and enzyme abnormalities. There is a known hereditary factor in familial ALS (FALS); however, there is no known hereditary component in the 90-95% cases diagnosed as sporadic ALS. An inherited genetic defect linked to a defect on chromosome 21 is believed to cause approximately 40% of familial cases of ALS. This mutation is believed to be autosomal dominant. The children of those diagnosed with familial ALS have a higher risk factor for developing the disease; however, those who have close family members diagnosed with sporadic ALS have no greater a risk factor than the general population [2].
According to The ALS Association, military veterans are at an increased risk of contracting ALS. In its report ALS in the Military, the group pointed to an almost 60% greater chance of the disease in military veterans than the general population. For Gulf War vets, the chance is seen as twice that of the general population in a joint study by the Veteran’s Affairs Administration and the DOD.
Symptoms
Initial Symptoms
The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS.
The parts of the body affected by early symptoms of ALS depend on which motor neurons in the body are damaged first. About 75% of people experience “limb onset” ALS. In some of these cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. Other limb onset patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock.
About 25% of cases are “bulbar onset” ALS. These patients first notice difficulty speaking clearly. Speech becomes garbled and slurred. Nasality and loss of volume are frequently the first symptoms. Difficulty swallowing, and loss of tongue mobility follow. Eventually total loss of speech and the inability to protect the airway when swallowing are experienced.
Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski’s sign (the large toe extends upward as the sole of the foot is stimulated) also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations). Around 15–45% of patients experience pseudobulbar affect, also known as “emotional lability”, which consists of uncontrollable laughter, crying or smiling.
To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
Emerging Symptoms
Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Difficulty swallowing and chewing impair the patient’s ability to eat normally and increase the risk of choking. Maintaining weight will then become a problem. Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed. A small percentage of patients go on to develop frontotemporal dementia characterized by profound personality changes; this is more common amongst those with a family history of dementia. A larger proportion of patients experience mild problems with word-generation, attention, or decision-making. Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation). Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance.
As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset ALS, this may occur before significant limb weakness is apparent. Bilevel positive pressure ventilation (frequently referred to by the tradename BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well. It is recommended that long before BiPAP becomes insufficient, patients (with the eventual help of their families) must decide whether to have a tracheostomy and long term mechanical ventilation. Most patients do not elect this route, and instead choose palliative hospice care at this point. Most people with ALS die of respiratory failure or pneumonia, not the disease itself.
ALS predominantly affects the motor neurons, and in the majority of cases the disease does not impair a patient’s mind, personality, intelligence, or memory. Nor does it affect a person’s ability to see, smell, taste, hear, or feel touch. Control of eye muscles is the most preserved function, although some patients with an extremely long duration of disease (20+ years) may lose eye control too. Unlike multiple sclerosis, bladder and bowel control are usually preserved in ALS, although as a result of immobility and diet changes, intestinal problems such as constipation can require intensive management.]
Diagnosis
No test can provide a definite diagnosis of ALS, although the presence of upper and lower motor neuron signs in a single limb is strongly suggestive. Instead, the diagnosis of ALS is primarily based on the symptoms and signs the physician observes in the patient and a series of tests to rule out other diseases. Physicians obtain the patient’s full medical history and usually conduct a neurologic examination at regular intervals to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are getting progressively worse.
Prognosis
Regardless of the part of the body first affected by the disease, it is usual for muscle weakness and atrophy to spread to other parts of the body as the disease progresses. It is important to remember that some patients with ALS have an arrested course with no progression beyond a certain point despite extensive follow-up. Such a pattern is particularly true for young males with predominant upper limb weakness especially on one side (so called “monomelic or Hirayama type” motor neuron disease). Eventually people with ALS will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with ALS survive for 10 or more years.
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ALS Related Web Sites
ALS Association: The ALS Association seeks to promote awareness and understanding of ALS and the work of The ALS Association by providing up-to-date information and education materials to the ALS community
ALS Network: The mission of this site is “to serve as a resource and support for PALS, their families and caregivers and to unite with other PALS to educate the public about ALS.”
ALS Links.com: Portal of links to several ALS related web sites.
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VA EXPANDS FULL CARE BENEFITS TO ALL VETS WITH ALS
TUCSON, Ariz., July 16, 2008 – Great news for many of America’s military veterans who have ALS.
On July 14, the Secretary of the Department of Veteran’s Affairs announced his decision to broaden VA compensation for all ALS-affected vets.
Prior to this time, only veterans with ALS who had served in the Gulf War (August 1990-February 1991) were accorded “service-connected disability” status by the VA. It’s the agency’s highest disability rating, and it makes the greatest degree of no- or low-cost VA medical care available.
Now VA secretary Dr. James Peake says all vets with ALS will be accorded the service-connected designation, effective Aug. 1.
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