Ceftriaxone trial enters third stage

Ceftriaxone, administered intravenously, may help clear away potentially toxic glutamate.

A trial of intravenous ceftriaxone in ALS has now entered its third stage and is enrolling participants at approximately 50 U.S. and four Canadian medical centers. The study is supported by the National Institutes of Health.

Ceftriaxone is marketed as an antibiotic to treat infections. Recently, laboratory screening showed it might also increase the amount of a protein called a glutamate transporter, which helps clear glutamate from the area around nerve cells. Glutamate, though necessary to transmit signals in the nervous system, can be toxic in large amounts.

The first stage of the study, which has completed enrollment, is determining whether ceftriaxone enters the fluid surrounding the spinal cord in high enough amounts to be of possible benefit. The second stage, which also has completed enrollment, is looking at safety and side effects of ceftriaxone administered for at least 20 weeks.

The third stage, which began enrolling participants this spring, will determine whether the drug prolongs survival or slows functional decline in ALS.

The study has a “nonstop, adaptive design,” meaning interim analyses of data regarding safety, tolerability and distribution of the drug in the body are reviewed by a monitoring board and used to determine whether the investigators can proceed to the next stage. However, the data themselves aren’t released to the investigators or anyone else until the entire study has been completed.

For more information, contact Fran Murphy at Massachusetts General Hospital in Boston at (617) 643-3980 or fmurphy@partners.org. To see a complete list of study sites, go to www.mda.org/research/ctrials.aspx  and search under “ceftriaxone.”

Antimalarial drug under study in FALS

Dale Lange, who directs the MDA clinic at Joan and Sanford I. Weill Medical College of Cornell University, New York, is set to begin an MDA-supported phase 1 trial to study the effects of pyrimethamine in people with FALS, or familial (inherited) ALS due to a mutation in the SOD1 gene. (SOD1 ALS accounts for about 20 percent of familial ALS, or about 2 percent of all cases of ALS.)

Enrolling in clinical trials and studies doesn't necessarily benefit the participant, but it's crucial for moving research in ALS from the laboratory to the community. To view active and completed studies in ALS, see Clinical Trials & Studies at www.mda.org/research/ctrials.aspx.

Marketed under the brand name Daraprim, pyrimethamine is approved by the U.S. Food and Drug Administration for the treatment of malaria and toxoplasmosis, both parasitic infections.

Evidence from animal studies indicates the medication may reduce the amount of toxic mutant SOD1 molecules. A number of patients with ALS in an earlier pilot study also experienced a reduction in their SOD1 levels while taking pyrimethamine. It’s thought that reducing levels of the mutant SOD1 protein may alleviate the disease.

The researchers will study the drug in 40 people with mild to moderate familial ALS and SOD1 mutations. The goals are to determine whether pyrimethamine is safe and tolerated, and whether it can lower the amount of SOD1 in immune system blood cells known as lymphocytes.

Contact Mona Shahbazi at (212) 774-2361, or shahbazim@hss.edu for more information.

Tamoxifen may have benefit

The drug tamoxifen (Nolvadex), approved for the treatment of breast cancer, has shown possible beneficial effects on survival and respiratory function in ALS. There was, however, no increase in strength or change in the rate of decline on the ALS Functional Rating Scale. The results are considered preliminary.

Tamoxifen is a “selective estrogen receptor modulator,” a drug that mimics the effects of the female hormone estrogen in some tissues and blocks them in others.

Neurologist Benjamin Brooks was the principal investigator on this study, which was funded in part by MDA. Brooks said the tamoxifen trial results are now being prepared for publication. He also noted that further studies of tamoxifen or a similar drug called raloxifene (Evista) are in the planning stage.

Brain-computer interface research continues

Scientists are exploring how best to enable a person to control a computer by thoughts. The BrainGate device utilizes electrodes that are implanted in the motor cortex, a part of the brain that controls movement. Other experimental devices sit on the brain’s surface or the scalp.

BrainGate, an investigational “brain-computer interface” technology being developed to detect brain signals generated by thoughts and allow people with paralysis to use those signals to control assistive devices, is now undergoing further testing in a second clinical trial.

The BrainGate 2 pilot clinical is funded by the National Institutes of Health, the U.S. Department of Veterans Affairs and philanthropic sources. It’s taking place at Massachusetts General Hospital (MGH) in Boston, in close collaboration with an interdisciplinary team of researchers from MGH and Brown University in Providence, R.I.

For more about BrainGate, including a video and information about participating in the research, see BrainGate at www.braingate2.org; or contact investigator Leigh Hochberg at (617) 726-4218 or clinicaltrials@braingate2.org.

KIFAP3 gene variant prolongs survival

A variant version of the gene for a protein known as KIFAP3 has been found to increase survival time in people with sporadic (nonfamilial) ALS by an average of 14 months.

Researchers found a variant in the gene for the KIFAP3 protein that correlated with longer survival time.

The findings of John Landers at the University of Massachusetts Medical School in Worcester, with colleagues from institutions around the world, were published online May 18, 2009, in Proceedings of the National Academy of Sciences. MDA supported Orla Hardiman and Simon Cronin at the Royal College of Surgeons in Dublin, Ireland, for this work.

The researchers conducted a “genome-wide association” study, in which they scanned the entire genome (set of genes) of study participants, looking for any association of gene variants with ALS risk, survival time or other ALS-related factors.

They analyzed the genomes of 1,821 people with sporadic ALS and 2,258 without the disease from the United States and Europe. Included in the analysis was the survival time of 1,014 people who had died of ALS.

The researchers found a single variant, in the gene for KIFAP3 (kinesin family-associated protein 3), that significantly correlated with ALS survival time.

Human chromosomes are found in pairs, and people with ALS who had the survival-enhancing KIFAP3 gene variant on both copies of chromosome 1 survived an average of 14 months longer than people with this variant on only one chromosome 1 or on neither chromosome 1.

None of the variant forms of any genes in this study were significantly associated with ALS risk, site of onset or age of onset.

The variant in the KIFAP3 gene is one that reduces output from the gene, so that less KIFAP3 protein is produced. The investigators say they don’t know why having less KIFAP3 protein would prolong survival in ALS, but they believe, based on the normal function of this protein, that its reduction may reduce transport of toxic molecules inside nerve fibers. They note that KIFAP3 levels are increased early in the disease process in mice with an ALS-like disease due to mutated SOD1 genes.

“Few genetic factors that modify ALS survival are reported,” the authors write, noting that none have been identified in previous genome-wide association studies in ALS. “The identification of KIFAP3 as a determinant of progression rate of sporadic ALS is therefore promising.”

They note that lowering KIFAP3 production or changing the interactions of this protein with other proteins might be worth investigating as an ALS therapy.

If ALS hasn't developed in four years, full disease is less likely

Full-blown ALS usually makes itself known within four years of the first symptoms, found an MDA-supported study published June 2, 2009, in Neurology.

A diagnosis of ALS requires signs that both upper (brain) and lower (brainstem and spinal cord) motor neurons are degenerating. When only the upper motor neurons are lost, the disease is often called “primary lateral sclerosis,” or PLS. This disease, which is rarer than ALS, allows for a higher level of functioning and longer survival time than ALS. (See “When It’s Almost ALS, Will the Disease Progress?” June 2007)

Upper motor neuron loss is associated with muscle spasticity, while lower motor neuron loss causes weakness or paralysis.

Upper motor neurons are nerve cells that send signals to lower motor neurons, which in turn send signals to muscles.

When lower motor neurons degenerate, the main effects are weakness or paralysis in the muscles they control. These can include limb and trunk muscles, respiratory muscles (such as the diaphragm), and muscles associated with chewing, swallowing and speaking. Muscle atrophy (shrinkage) and weight loss are also effects of lower motor neuron degeneration.

When upper motor neurons are lost, the main effects are spasticity (tightness) in muscles that continue to receive input from lower motor neurons alone.

Neurologist Paul Gordon and colleagues at the Eleanor and Lou Gehrig MDA/ALS Center at Columbia University in New York reviewed the records of 34 patients with signs of dysfunction of upper motor neurons, lower motor neurons, or both, who visited the center between 1984 and 2007.

They found a diagnosis of PLS with a very slow rate of progression and prolonged high-level functioning applied only to patients who did not develop any signs of lower motor neuron dysfunction.

They found patients whose disease was mostly confined to the upper motor neurons but who developed even subtle lower motor neuron abnormalities had a shorter survival time than those whose disease remained confined to the upper motor neurons. Even if lower motor neuron signs were initially subtle, patients who developed them eventually showed functional deterioration similar to that of typical ALS.

Signs of lower motor neuron abnormalities include weakness, diminished respiratory capacity, weight loss, and abnormalities of nerve-signal transmission detected on an electromyogram (EMG).

Most (77 percent) of patients who had only upper motor neuron degeneration when first seen at the center but who later developed lower motor neuron signs did so by the fourth year after the first symptoms began.

The Columbia researchers suggest that, if after four years, signs of lower motor neuron degeneration have not yet been seen, a diagnosis of PLS can be made, although they caution that a minority of these patients still develop ALS later on.

Barriers in spinal cord appear leaky in ALS

Proteins that keep large molecules from moving freely across blood-vessel walls in the spinal cord appear to be deficient in people with ALS, MDA-supported researchers say. They don’t yet know, however, whether a lower-than-normal level of some of these so-called “tight junction” proteins, is helpful or harmful in the disease process.

Neurologist and MDA grantee Stanley Appel, who directs the MDA/ALS Center at Methodist Neurological Institute in Houston, and colleagues, published their findings in the May 5, 2009, issue of the journal Neurology.

The researchers measured levels of messenger RNA, the final genetic instructions from which a cell makes a protein, in spinal cord samples from 30 people with sporadic (nonfamilial) ALS, four with familial ALS and a control group of 16 people with no neurodegenerative disease.

They found the messenger RNA for the tight junction protein called zona occludens 1 (ZO1) was lower in lumbar spinal cord tissue in people with sporadic ALS compared to tissue from the control group. There was no difference in the RNA for ZO1 in the four familial ALS samples compared to the control group.

Messenger RNA levels for another tight junction protein, occludin, were lower in lumbar spinal cord samples from people with familial ALS than they were in samples from the control group, but there was no difference between the sporadic ALS samples and control group samples.

RNA levels for a third tight junction protein were the same in the ALS samples as a whole and the control samples.

The researchers note that previous studies have suggested that the vascular barrier between the bloodstream and the spinal cord, known as the “blood-spinal cord barrier,” is leakier than normal in ALS, and they say this study adds further support to that evidence.

They say the leakiness could lead to the entry of toxic molecules into the spinal cord and contribute to the nerve-cell damage that characterizes ALS.

Conversely, they note, the enhanced permeability could allow the entry of immune-system cells that have been associated with protection of nerve cells in this disease.

by Margaret Wahl

Recently, media outlets reported a possible link between developing ALS and living near Lake Mascoma in Western New Hampshire.

The Union Leader in New Hampshire and other news outlets  indicated that the risk of developing ALS is “25 times” higher than average for people living around Lake Mascoma, located in Enfield and Lebanon, N.H. The source of this statistic was not explained.

As often happens, there’s an element of truth in these media reports, and an element of hype.

Researchers at Dartmouth-Hitchcock Medical Center in Lebanon in fact are considering whether there is a true “cluster” of ALS cases in the lake area, and whether it is the result of exposure to cyanobacteria produced by blue-green algae in the water, combined with a genetic predisposition to develop ALS.

But scientists knowledgeable in this area say that, at present, there’s no clear evidence that the ALS “cluster” is more than coincidence, or that there’s a link between blue-green algae — pond scum — and developing ALS.

Evidence circumstantial

Elijah Stommel, a neurologist at Dartmouth-Hitchcock who’s involved with the study, said there is evidence of a link between living near Lake Mascoma and an increased risk of developing ALS, but this evidence is “circumstantial to some degree at this point.”

Stommel said results are preliminary, and his group is working on “better establishing the link.” A scientific publication is in preparation.

Tracie Caller, in her second year of postgraduate training in neurology and preventive medicine at Dartmouth-Hitchcock, also is involved in the study.

“The media attention caught us unprepared,” Caller said, noting that it occurred following a workshop at Lake Mascoma where she presented information on cyanobacteria, which have health implications in general. (Different cyanobacterial toxins can cause skin and eye irritation and damage to the liver or nervous system.)

In an e-mail message to MDA, she emphasized, “Our research is very preliminary, and we have not published any of our work in the scientific literature yet because of this. The publicity was not something we had anticipated.”

BMAA toxin is suspected

Caller explained that cyanobacteria, which can contaminate lakes, can produce a toxin called BMAA, which some evidence suggests may be linked to a higher-than-average incidence of ALS on the island of Guam in the Western Pacific Ocean. (See “Toxic bats.”)

“BMAA has been identified in other water bodies but not yet in New England,” Caller said. “The [cyanobacteria] species that we’re finding in our lakes are capable of making BMAA, but we don’t know if they are making the toxin. At this time there is no correlation between any cyanobacterial toxin in lake water and ALS.”

She said the investigation was prompted because cyanobacteria had been found in lakes near where preliminary data suggested a higher-than-average ALS rate.

She cautioned, however, that “there are a number of other environmental or other exposures these communities can share, and the fact that ALS takes many, many years to progress makes it even more difficult to prove a link.”

Controversial hypothesis

MDA also contacted Lorene Nelson, an epidemiologist at Stanford (Calif.) University who has MDA support to study gene-environment interactions in ALS.

“There is one research team that strongly advocates the hypothesis that BMAA can cause ALS,” Nelson said, citing studies by ethnobiologist Paul Cox and colleagues. They propose that bats on Guam accumulate BMAA in their bodies and that people eating these bats develop ALS at a higher-than-average rate because of this practice.

“For the most part, research findings are inconsistent, and there is controversy surrounding this hypothesis, although this remains an active area of investigation,” Nelson said. “But we know so little about the environmental causes of ALS, and therefore, any biologically plausible hypothesis merits investigation.”

Real and apparent clusters

“We do see what appear to be clusters of ALS in New England around certain lakes,” Tracie Caller noted. “Our preliminary data suggest that these are statistically significant, but further analysis is certainly needed.

“The ‘25 times higher risk’ reported by the media was a crude estimate and may not accurately reflect the true risk. More sophisticated analysis will give us a better estimate. Proving the presence of a true cluster statistically is very challenging, and there are many, many limitations.”

Lorene Nelson also commented on whether or not a true concentration, or “cluster,” of ALS cases exists around Lake Mascoma or in other areas or groups.

She said news reports about Lake Mascoma lack details needed to judge the validity of the suspected ALS cluster.

“One important factor to consider is the expected number of ALS patients in the region, given the age distribution of the population,” she said. “For example, if it is a retirement community, it could have proportionally more elderly and therefore have a higher than expected number of persons with ALS.”

Notably, she said, Enfield has six elderly housing projects, while most other towns in New Hampshire have only one or two.

Nelson noted that many apparent clusters of ALS are reported each year, but many do not bear up under the scrutiny of further investigation.

A certain number are due to “chance alone,” she said. “For example, in the United States, there are 5,200 counties, and so on a probability basis, you would expect approximately 130 of these to have an apparent statistical excess of ALS cases.”

So far, she said, true ALS clusters have been found on Guam and among veterans of the first Gulf War. While BMAA has been implicated as a possible factor in the Guam cluster, chemical weapons toxic to the nervous system have been cited as a possible contributor to ALS in Gulf War veterans. (See “Study suggests Gulf War-associated increase in ALS is time-limited and correlated with location,” September 2008.)

The consumption of fruit bats on Guam may have contributed to the relatively high rate of ALS on the island.

Toxic bats

In 2002, researchers proposed that the unusually high incidence of ALS found on the island of Guam between 1940 and 1965 probably had to do with the islanders’ tradition of eating fruit bats. These bats eat cycad nuts that contain a known toxin, BMAA, and then concentrate the toxin in their tissues.

A subsequent investigation reported in 2005 that people with ALS were eight times more likely to have lived on Guam, even for a few months, than were those who didn’t have ALS. The study included 140 people with ALS and 140 without the disease.

Investigators on the 2005 study said their findings “could indicate that one of the key characteristics of the exposure occurring during the period of endemic ALS on Guam is the long delay in clinical expression [outward signs of disease].” They said their data “support the vision that exposure to slow-acting toxic agents is important in the pathogenesis of ALS, most likely in combination with a genetic predisposition.”

by Gerald C. Weinberg

MDA was founded as, and remains today, a grassroots fundraising organization.  The overwhelming majority of dollars raised for MDA are through community-driven events. 

It’s the amazing additive effect of these many local events that make it possible for MDA’s ALS Division to fund quality research and services programs. 

Among the many community-organized special events around the country in the past year were:

•    The Affair in Dallas
•    All in for ALS Tournament in Houston
•    Augie’s Bash in Southern California
•    Augie’s Golf, Food and Wine Extravaganza in Newport Beach, Calif.
•    Champions of Hope in Los Angeles
•    Evening of Hope in Westboro, Mass.
•    Night of Hope Gala in Atlanta
•    Phil Villapiano Field of Hope Gala in Long Branch, N.J.
•    A Gift of Time St. Patrick’s Day Bash in Denver
•    Tradition of Hope in Los Angeles
•    Wings Over Wall Street in New York City

Events such as these and many others across the country, large and small, combine to continue MDA’s quest for a cure.  They are vital to advancing the fight against ALS.
MDA is deeply grateful to committee members everywhere who lead these events for their hard work and dedication to raising funds for our ALS research and services programs. It’s thanks to such community fundraising that one day this battle will be won.

Around MDA, we have a saying, born of experience: Working together, we can make it happen.  Fundraising committee members are living proof that this is so.

Gerald C. Weinberg is MDA President & CEO. For more information on the committees of the events listed here, please visit www.als-mda.org/help/2009events.html.  To learn about launching an MDA event in your area, please contact your local MDA office at (800) 572-1717.

It’s a dance, it’s a dip, it’s an … ALS research network?

“SALSA,” MDA’s Southeast ALS Alliance, brought together a piquant blend of nearly 30 professionals from 11 hospitals in the Southeastern United States this May to share information and discuss topics such as ALS clinic management and practices, patient resources, current ALS research and patient care approaches.

Jonathan Glass, director of the MDA/ALS Center at Emory University in Atlanta, organized and hosted the event. Conferees included neurologists, clinician-scientists, social workers, dieticians, respiratory therapists, nurses, and physical and occupational therapists.

MDA’s Emory clinic is one of five centers that comprise MDA’s ALS-focused Clinical Research Network, which was launched in fall 2008 to develop studies into standardized clinical care; develop outcome measures for clinical studies and trials; and develop and test new treatments. (For more on the network, see “ALS to be a major focus in new MDA Clinical Research Network,” October 2008.)

Each of MDA’s four other ALS Clinical Research Network centers plans to host regional meetings like SALSA, with the goals of sharing the latest research, recruiting institutions for future multicenter clinical trials, and enhancing care.

Peter Donofrio, director of the MDA/ALS Center at Vanderbilt University Medical Center in Nashville, Tenn., says one of the most important issues discussed at the SALSA meeting was “working together as one group in the Southeastern United States to further research and development in the area of ALS.”

The exciting part for individuals affected by ALS, he says, is “the potential research in the disease that may affect them and their families.”

Notes Donofrio, “This integrates very well with MDA’s goals to coordinate and encourage research in ALS that affects patients directly.”

by Kathy Wechsler

The future is bright at the Georgia Institute of Technology’s Healthcare Robotics Lab in Atlanta.

Georgia Tech professor and health care robotics researcher Charles Kemp and his team of researchers are working on a project that one day will change the lives of people with disabilities by allowing them greater independence —- an assistive robot.

The first version of their robot assistant, EL-E, received its name because its single arm resembles the trunk of an elephant. Measuring 5 feet 7 inches tall, the skinny robot is mainly metal and gets its power from motorcycle batteries.

Robotics progress

By casting a green spot on the table with a laser pointer, Charles Dyer tells the robot, EL-E, where to set the cordless phone it has retrieved.

Researchers worked with 38 patients from the MDA/ALS Center at the Emory School of Medicine in Atlanta, to learn the types of robot assistance that would be most valuable to people with progressive paralysis. Georgia Tech also conducted several studies at the Healthcare Robotics Lab, where they had the opportunity to watch people interact with EL-E and better understand the challenges.

Charles H. Dyer, Jr., of Jonesboro, Ga., who received an ALS diagnosis in 2003, was involved in two of the hands-on studies. Dyer, 66, wanted to participate because he frequently drops things and has to rely on his wife, Saralyn, to pick them up.

During both studies, Dyer spent several hours working with EL-E, whose built-in system of cameras and sensors enable it to retrieve items and place them in desired locations by following a laser pointer. Dyer also had EL-E open doors, drawers and cabinets by tugging on a red cloth with its arm.

Dyer discussed challenges and needed improvements with the researchers, but overall was impressed by EL-E. “It’s truly amazing what the robot can do to help with daily problems,” he says. “You have to see it perform to believe what it can do.”

EL-E is very accurate when selecting an object sitting all by itself, but still has trouble picking out a particular object from a cluttered area, says Kemp. Since there’s only one version, it only has been tested in a laboratory setting, but Kemp’s goal for 2009 is to build a version that can be tested in a home setting.

The future

Kemp and the Georgia Tech team are developing a robot with two arms that they expect will be able to do some things that the one-armed robot can’t, such as open a pill bottle and lift more weight.

EL-E’s arm has very stiff joints, like a traditional factory robot. When people interact with EL-E, it can sense that they’re making contact but is unable to smoothly adapt to their touch. On the other hand, the two-armed robot has flexible joints that act like springs. This enables its arms to be pushed in a desired direction and smoothly respond to contact with people and the world.

Kemp hopes future robots will be able to help with communication and even transferring, such as from a bed to a wheelchair.

When assistive robots do become widely available, Kemp suspects they won’t be exactly like EL-E but a close relative. Kemp expects them to be smarter and more capable of interacting with others.

See a video of EL-E in action

“I will be disappointed if within the next 10 years, we don’t see this type of technology helping people in homes,” he says. “My hope is that it would be much sooner than that.”

Kemp estimates that EL-E (which is not for sale) currently costs about as much as a “luxury automobile,” but he expects the price to drop if it reaches production and becomes more successful in the marketplace. He hopes prices ultimately will be comparable to “a nice power wheelchair.”

“I’m confident that robots are going to be able to help provide care, and hopefully make it so a caregiver does not have to be around all the time,” he says. “We believe in this technology, and in the long term, it can really make a difference in people’s lives.”

Kemp welcomes suggestions for other types of assistance the robot should offer. Contact Georgia Tech’s Healthcare Robotics Lab at hrl@lists.gatech.edu or visit www.healthcare-robotics.com for more information.

by Amy Labbe

Bob Paulson, pictured with wife Maureen and the couple’s dog, Opal, wrote his memoir using an ERICA eye-tracking computer system.

By all accounts, 72-year-old Robert “Bob” Paulson has led an interesting and accomplished life. His is the tale of a Kansas farm boy conquering the Big Apple and creating a beautiful family with whom he’s shared the best life has to offer.

Some might say a diagnosis of ALS in 1996 rendered his good life bad. But Paulson will tell you, using the eye-tracking device that’s become his voice, that’s definitely not the case.

Paulson’s memoir, Not In Kansas Anymore, about life on the farm, in New York City, and with ALS, currently is available online at Amazon, Target, and Barnes and Noble. It was written entirely “by eye,” and is testimony to Paulson’s belief that life with ALS still has plenty to offer.

From the country to the Big Apple

Born in 1937 and raised on a farm near Lindsborg, Kan., young Paulson milked cows, fed pigs and gathered eggs. He climbed trees, studied by the light of a kerosene lamp, and attended school for his first five years in a one-room schoolhouse. He and his mother sold eggs and cream to pay for his music lessons.

In 1959, Paulson earned a B.S. in nuclear engineering from Kansas State University, went to work for the Atomic Energy Commission and started classes at Georgetown University Law School.

Studying law was “delightful,” Paulson writes, after the math and science of nuclear engineering. He especially enjoyed the “real people with real problems and practical, common-sense (most of the time!) solutions for governing their conduct towards each other.”

Upon graduation in 1963, Paulson hired on with the New York patent law firm Morgan, Finnegan, Durham and Pine, where he would eventually be made a partner and where he remained for more than 40 years.

Paulson met and married Maureen Dowling, and they had three sons. The family bonded over a mutual love of tennis, spring breaks in Florida, and summers in Westhampton Beach on Long Island where they kept a vacation house.

“Those moments with the family on the beach or playing tennis are the fondest of my memories,” Paulson says.

Enter ALS

Around 1995, experiencing unexplained leg and stomach-muscle fatigue, Paulson found himself unable to jump, and stumbling and falling. Walking became increasingly difficult.

ALS was diagnosed in 1996, and Paulson began using a cane and then a wheelchair. An aide helped him shower, dress and get to his office, and another assisted him at work until his retirement in 2003.

Six months later, pneumonia and respiratory failure necessitated an emergency tracheostomy, ventilator and feeding tube, and Paulson spent the next several years getting used to his new life-support equipment. In 2007, he lost his ability to speak, and looked for a new way to communicate.

A technological fix

Paulson’s fondest memories center around his family. He’s pictured here with wife Maureen, sons Luke, Jake and Josh, and daughter-in-law Tammy.

Paulson obtained an ERICA eye-tracking computer system from Eye Response Technologies in 2007.

He wryly notes that he “missed the computer age” and had to learn even the basics of “computer word processing lingo.” His success has convinced him that eye-tracking devices “can be successfully used by anyone [who has] difficulty using their hands or [who] has no speech.”

Eye tracking takes a while to master he says, noting that the most important concerns are proper position of the computer screen, accurate calibration of the camera, and ensuring the device is set up for the user’s strongest eye.

At first he thought of his computer as “a simple communication device,” on which he spent time “slowly typing out rudimentary needs, questions or answers.” He soon learned, however, that it’s “much, much more.”

Paulson’s system allows e-mail communication and Internet access to “news, research, stock market portfolios and, as I did with my book, drafting, saving, editing, sending and receiving documents.”

Now, he says, “I almost have to say that my computer has become my best friend. I interact with it nearly six hours every day. I ask questions and get phenomenal answers. It sends mail to me and delivers my letters anywhere. My computer alone has been largely responsible for giving me a quality of life that makes life definitely worth living.”

Writing and publishing

Around the end of 2007, with prodding from Maureen and friends who had heard his stories about growing up on the farm, Paulson began to organize his thoughts for the book that eventually would become Not in Kansas Anymore.

The writing was relatively easy, Paulson recalls. “I was simply writing down thoughts I had kept in my mind throughout the years.”

At first he wrote about individual events in no special order. After completing about 40 such “vignettes,” he consulted with an editor friend who helped him organize and edit.

Paulson then began collecting pictures, while Maureen put together a team of family members and friends to manage the business end of the publication.

Friends who own a small publishing company recommended self-publishing as the fastest, easiest and least expensive way to proceed. Not in Kansas Anymore eventually was self-published through Gemma B. Publishing of Winnipeg, Manitoba, Canada.

Paulson notes that although most of his team worked “long distance and solely by computer,” they were able to put the final product together “with little difficulty.”

It took about 14 months from the time he first began writing to the finished product. He worked on it “four to six hours most every day.”

Life lessons learned and shared

Paulson encourages readers “to take advantage of any and all opportunities that present themselves, as … no one knows where or how high these building blocks will take us.”

As he has learned, and as he shares with his readers through his memoirs, “A diagnosis of ALS need not be a death sentence. With the technology available today, mobility and communication are virtually unimpeded, even for those on life-support ventilators. I say that, at its core, life is the ability to understand and communicate, and ALS leaves those abilities intact.

“I have no use of my hands, arms or legs and cannot speak. But I understand as fully as ever, and with my computer I can communicate as well as the next person.

“Life is good.”

by Alyssa Quintero

Gaining access to assistive technology (AT) devices can be an arduous task, especially when funds are low and insurance coverage isn’t available.

Equipment loan and recycling programs aid people who want to try out different devices, or who need to borrow equipment while they’re waiting for a device to be approved by insurance, or who can’t afford to pay out-of-pocket.

MDA offers reused/recycled AT through its loan closets. Depending on availability, people registered with MDA can borrow — for free and for as long as they need them — used communication devices, computers, power wheelchairs, scooters, lifts and other technology. (Equipment must have been prescribed through an MDA clinic.)

MDA’s loan closets also enable people to try expensive technical equipment before making a decision. This is especially valuable with an eye-tracking communication system, where time is needed to ensure it’s a realistic option for the user.

When equipment is no longer needed, families are encouraged to return it to MDA for use by others.

Borrowing independence

For Tara Webb of Arroyo Grande, Calif., an equipment loan program has helped her reclaim independence — and her voice.

Webb, 56, who cannot speak or use her arms and legs due to ALS, was denied Social Security benefits and doesn’t qualify for Medicare, so she relies on her private insurance for coverage. Unfortunately, her insurer has denied coverage for a communication device, despite appeals.

Luckily, the loan program had a device for Webb to borrow every time she needed something, “which made the frustration with the insurance company a bit more tolerable,” Webb wrote via e-mail.

During this time, Webb borrowed a Lightwriter and a Mercury device that she accessed via a foot switch. When her foot muscles deteriorated, she was put on the waiting list for an eye-tracking communication system.

After quite some time, an ERICA eye-tracking system became available, but Webb was unable to use it. So, she went back on the loan closet waiting list for a MyTobii system.

’Back on the radar’

After a year on the waiting list, Webb learned that the loan closet had a used MyTobii P10, a speech-generating device that also acts as a fully functioning computer.

It was donated to the loan closet after the previous owner died; he received the device through insurance, and used it for almost two years to generate speech and play chess.

“It’s important for people to realize that [communication] systems that are no longer needed and just taking up room in their home could be life-changing for someone else if they choose to donate the equipment,” said Amy Roman, an augmentative communication specialist and speech-language pathologist at the Forbes-Norris MDA/ALS Research Center in San Francisco.

Since November, Webb has spent “almost every waking hour” on the device. She communicates with her nurses and visitors, writes e-mails, explores the Internet, shops, reads the newspaper, keeps in touch with her college-age daughter and monitors her Facebook page, where she has 130-plus friends.

“I’m able to e-mail my doctors, health care providers and business people directly with questions, concerns or problems,” Webb said. “I don’t have to do everything secondhand. I can carry on normal conversations, and I feel like I’m participating in my life and not just lying here watching everything pass me by. I feel alive and happy again.”

Now, she says, “the whole world is at my fingertips, actually at my eyes.”

Adds Roman, “She immediately starting shooting e-mails out to people, and all the neurologists were so happy to hear from her. It was dramatic to have Tara back on the radar.”

Tara Webb, who received a diagnosis of ALS in March 2004, uses a MyTobii eye-tracking communication device that she’s borrowing from an equipment loan program. Webb, who spends most of the day in bed, can produce speech, and communicate with doctors and caregivers. She also stays in touch with family and friends via the Internet.

“I would be totally depressed and isolated again without it,” Webb says of her communication device, calling the loan program “a lifesaver.” She plans to continue donating unused equipment back to the loan closet, adding, “I plan to keep ‘paying it forward.’”

More help available

In addition to MDA’s equipment loan program, State Assistive Technology Act programs provide short-term equipment loans to people who want to try a device, or who need a device while they wait for insurance approval or financial assistance.

AT Act programs also offer recycling/reutilization programs that sanitize, refurbish and repair donated equipment (including durable medical equipment, and communication and mobility devices), that is then donated or sold at an affordable price. (Services and equipment availability vary from state to state.)

To locate the AT Act Program in your state, visit Resna’s National Assistive Technology Technical Assistance Partnership at www.resnaprojects.org/nattap/at/statecontacts.html, or call (703) 524-6686.

Some states participate in equipment exchange programs, which are much like online marketplaces with classified ads. These help match people who need equipment with people who have equipment to donate or sell.

The national Pass It On Center offers an online directory of reuse and exchange programs that’s searchable by state at www.passitoncenter.org. The resources include the state AT Act reuse programs, as well as programs offered by local nonprofit organizations, centers for independent living (CILs) and more. For more information, call (800) 497-8665.