by Bill Greenberg

"In my world of painting I feel no pain. I have no problems. I am in a world without limitations."
- Dan Beacome, 1950-2002

"Pride of the Yankees"   by Bill Ross

Fifteen works of art from the nationally renowned MDA Art Collection will be displayed in the exhibit, called "Transcending Barriers to Creativity: Featuring Artists With ALS," which will also present works by local artists with ALS.

Among the works to be shown is Dan Beacome's "Old Barn," a painting that has special significance for Bronxville, N.Y., artist William Ross.

Ross is expected to attend the opening of the exhibit, which will include his newest donation to the MDA Collection, "Pride of the Yankees," and two other Ross paintings. (See "Bill Ross and the Art of Living With ALS" December 2001.)

"Pride of the Yankees" depicts legendary New York Yankees first baseman Lou Gehrig standing before several microphones, waiting for the crowd's applause to die down before delivering his now-famous farewell address.

Ross' opaque watercolor painting eloquently portrays Gehrig's consummate humility as he prepares to utter the immortal words, "Today, I consider myself the luckiest man on the face of the Earth."

After his diagnosis in January 1999, Ross worried that ALS would take away the use of his hands, and with it the ability to paint. Then in April 2000, Ross received his copy of Quest, MDA's bimonthly magazine, which featured a photograph of Beacome on the cover - painting with the brush held in his mouth.

With Beacome's example and the help of Hiroshi Mitsumoto, director of the Gehrig center, Ross learned how to mouthpaint.

"Pride of the Yankees," completed early this year, is one of the first results.

"Breathtaking Metamorphosis" by Erin Brady Worsham

Worth a Thousand Words...

Behind every work of art in the exhibit is a story as compelling as those of Beacome and Ross.

For example, Erin Brady Worsham's "Breathtaking Metamorphosis" illustrated a story she wrote for the June 2001 issue of Quest, called "Life on the Vent." The article describes the effects of ALS on the Nashville, Tenn., woman's ability to breathe, and chronicles her experience with a tracheostomy.

Fred Siwak of Rockport, Mass., will be represented by a linoleum block print, "Dragonflies." (See "New England Artist," October 2001.)

In October, less than four months before Beacome lost his 23-year battle with ALS, he wrote these words to describe the process of learning how to paint with the brush in his mouth:

"It has been a continuous experiment, but I've thrived on the challenges. There have been many failures along the way, but they only serve to make the victories sweeter! I hope I've been able to help others see things that way."

Dan Beacome

Beacome, who received his diagnosis in January 1979, didn't discover his gift of painting until he'd already lost the use of his hands. In 1986, Beacome moved into the Michigan Masonic Home in Alma.

"I was 36 years old, and my disease was progressing," he wrote. "Doctors didn't think I would last a year."

At the Masonic Home, he decided to try painting. Beacome recalled, "We spent several days experimenting with tools and techniques, until we reached a point where I was able to push paint around on a canvas."

In 1992, Beacome's "Old Barn" became one of the first pieces accepted by the MDA Art Collection. Now in its 11th year, the Collection comprises some 280 pieces of art, representing all 50 states.

"Knowing that my painting gives others pleasure, and perhaps inspires others to try to fight their demons, makes me feel good," Beacome said.

It's a sentiment that could be echoed by all of the talented artists represented in "Transcending Barriers."

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May Is ALS Awareness Month

We'll also be announcing some new publications in the next issue of The MDA/ALS Newsletter. That issue will include our annual list of MDA/ALS clinical care and research centers, as well as the services, publications and other materials available through MDA's ALS Division.

Watch for the May issue of The MDA/ALS Newsletter, or visit www.als-mda.org, and see what's new from the world leader in ALS research and services.

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ALS Centers Get Boost From New York Gala

Wings of Hope took place at the famed Tavern on the Green restaurant on Nov. 8 and raised some $650,000 for MDA's ALS research program.

An award of $324,000 was given to the Eleanor and Lou Gehrig MDA/ALS Center at Columbia Presbyterian Medical Center in New York, which is directed by Hiroshi Mitsumoto.

Mitsumoto, in turn, presented the event's Diamond Award to Jeffrey Rothstein, director of the MDA/ALS Center at Johns Hopkins University in Baltimore. (A colleague accepted on Rothstein's behalf.)

The Diamond Award recognized Rothstein's outstanding contributions to ALS research, and carried a cash prize of $320,000 which the center will use to fund grants for cutting-edge ALS research.

Toni Diamond of Southwick, Mass., and her husband, Warren Schiffer, initiated the MDA event, which included a dinner and auction. Diamond, who is affected by ALS, is a former flight attendant for United Airlines.

Diamond received a Spirit Award during the evening, as did Michael Beier of New York, director of Equity Trading for Credit Suisse First Boston. Beier, who received a diagnosis of ALS in December 2000, will chair the 2002 gala this fall, which will carry the new name of Wings Over Wall Street.

Beier and Mitsumoto were recently elected MDA vice presidents.

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ALS Research Roundup

Oxidative Stress, Copper May Be Wrong Suspects in ALS

SOD1 (short for superoxide dismutase 1) is an enzyme that uses copper to break down superoxide, a byproduct of cell metabolism. When superoxide and other oxygen-based chemicals build up in cells, they can cause damage through a process called oxidative stress. Early theories proposed that mutant SOD1 simply fails to protect against oxidative stress, but it's become clear that the mutant protein gains a new, mysterious function that's toxic to motor neurons.

MDA devotes more than $5.5 million a year to ALS research and services — more than $135 million since 1950, and more than any other U.S. national voluntary, private-sector health agency.

A more recent theory holds that mutant SOD1 binds to copper in a way that causes the enzyme to work backwards - producing rather than destroying superoxide - but others say there's little evidence of increased oxidative stress in mice that develop ALS because of SOD1 mutations ( "Solving the SOD1 Puzzle," February 2001). So, a group at Johns Hopkins University in Baltimore - including Jeffrey Rothstein, co-director of the university's MDA/ALS Center - tested the theory by eliminating the copper from mutant SOD1, thus blocking the enzyme's ability to handle superoxide and other oxygen-based chemicals.

Rothstein and his group carried out their test by creating mutant SOD1 mice that lack a protein called CCS, which is required for delivering copper to SOD1. As expected, getting rid of CCS blocked SOD1's oxidative capacity.

But it didn't affect the onset or progression of disease in the mice, suggesting that copper and oxidative stress don't have a primary role in SOD1-related ALS. The study was published online by Nature Neuroscience in March.

The results imply that, at least for SOD1-related ALS, copper-binding drugs and antioxidants might not be as promising as once thought.

Studies Question Timing of Ventilation, Feeding Tubes

Respiratory Intervention

In the Oct. 15 issue of the Journal of the Neurological Sciences, researchers reported that seven ALS patients who received early respiratory intervention felt better than a control group, as measured by a "vitality scale" the authors employed.

The patients received noninvasive ventilation (respiratory support without tracheostomy) when their forced vital capacity was between 70 percent and 100 percent of normal. Forced vital capacity (FVC) is the amount of air a person can forcefully exhale after taking in as deep a breath as possible.

The researchers found that measuring blood oxygen overnight and strength of inhalation may be more sensitive measures of early problems in breathing in ALS than FVC.

Blood oxygen content is measured by oximetry, in which a painless electronic sensor is worn on a finger or toe. The ALS patients who received earlier ventilation showed an oxygen reading of less than 90 percent for 1 minute (normal is 95 percent or higher).

In the study, six other people with ALS received "standard care," starting noninvasive ventilation when the FVC dropped below 50 percent of normal.

Jeffrey Rosenfeld, director of the MDA/ALS Center at Carolinas Medical Center in Charlotte, N.C., and Robert Miller, who directs the MDA/ALS Center at California Pacific Medical Center in San Francisco, were among the investigators. The authors caution that the study is small and needs to be verified in a larger sample.

Gastrostomy Tubes

In another study, investigators reporting in the Feb. 12 issue of Neurology found that inserting feeding tubes, even in the face of relatively severe respiratory impairment, may still be fairly safe and may prolong life.

The 1999 practice parameter suggests gastrostomy tubes should generally be avoided in patients with FVC values of less than 50 percent of normal, because the procedure is riskier.

The study looked at 33 people with ALS, all of whom had low FVC values, including one as low as 7 percent.

No deaths or injuries occurred during the tube insertion procedures. Twenty people survived an average of six months after the insertion, and 13 survived an average of eight months. The investigators conclude that the tubes, which can provide good nutrition for severely weakened ALS patients who have trouble eating, may have improved survival.

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ALS Studies, Clinical Trials Need Volunteers

Buspirone Study

Buspirone (Buspar) is on the market as an anti-anxiety agent, although the mechanism by which it works isn't clear. It's now being studied in ALS because it appears to either mimic or stimulate the activity of neurotrophic factors, natural nervous system chemicals that are believed to keep nerve cells healthy.

Mice with ALS that were treated with buspirone showed improvements in lung function compared to untreated littermates.

Genetic Factors in Sporadic ALS

This large-scale study still needs participants to help in the search for genes that may play a role in seemingly nongenetic (sporadic) ALS. The study is also examining environmental risk factors.

It requires blood samples and the completion of a questionnaire, but no travel is necessary.

Myotrophin Back Again

You may remember Myotrophin, an experimental ALS drug that was tested in two clinical trials in the 1990s with contradictory results. The U.S. Food and Drug Administration recommended a "tie-breaking" trial, and one is now open for recruitment at 16 centers.

Myotrophin is a brand name for a neurotrophic factor called IGF1, or insulin-like growth factor 1. The drug is injected under the skin twice a day.

Contact study coordinator Julie Haubenschild at Mayo Medical Center in Rochester, Minn., at (507) 266-8087, (507) 284-5821 or haubenschild.julie@mayo.edu.

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Public Service Still Defines Maine Man Mayo

by Bill Greenberg

Joe Mayo works as clerk emeritus of the Maine House of Representatives

Mayo is 42 now and, having received a diagnosis of ALS in 1999, he isn't quite so healthy. But, though he no longer walks, has difficulty speaking and receives nourishment through a feeding tube, the desire to help others remains a driving force in Mayo's life.

Putting the Public First

A year after he graduated from the University of Maine with a bachelor's degree in accounting and finance, Mayo ran for the House seat left vacant by his father's death.

Mayo's wife, Rebecca Wyke, recalls, "Joe simply went door to door, talking to as many people as he could, and he won. After that, he ran all of his campaigns that way."

Mayo quickly became known for his expertise on tax issues, as well as the complexities of the state budget.

"But I would say that Joe's real expertise is people," says Michael V. Saxl, longtime friend who's now speaker of the Maine House. "Even if you have deep, heartfelt policy differences, Joe can put you at ease immediately with a smile, and with his nonadversarial approach. He's always been very successful at bringing people together."

Beyond the Legislature, Mayo has brought his knowledge of budgetary and tax issues to the table as moderator of town meetings and school budget meetings in his small, coastal town of Thomaston. He's also led efforts to bring better health care services to his community.

In 1992, after serving in the House for some nine years - including four years as majority whip - Mayo resigned his seat to become the clerk of the House, a post to which he was elected by the House members.

"If you have an interest in the Legislature as an institution, it's a highly respectable place to spend your time," Speaker Saxl explains. "The clerk's job is quite simply to manage the House."

Millie MacFarland served as Mayo's assistant clerk, and succeeded him as clerk when his ALS forced him to step down in December 2000. Mayo now serves the Legislature as clerk emeritus, a title of honor that the House created especially for him, MacFarland said.

Both Saxl and MacFarland emphasize that clerk emeritus is far from being an honorary title.

Saxl explained that, even though Mayo comes into the office just three days a week, "I talk to him every single day about where we are and where we're going. He continues to write draft memos on how to address many different issues that we're facing, and invariably he's always correct."

MacFarland adds, "I rely on Joe for so many things. I can't say enough about the tremendous support that Joe's been for me as a friend and also as a colleague."

Scholarship and Support

When Mayo's diagnosis of ALS was confirmed in the summer of 1999, there were no support groups in his part of Maine. So Mayo simply started his own to help both patients and their families.

Morgan Kelly says her father, Raymond, who recently lost his battle with ALS, found great strength in his friendship with Mayo.

"My dad was always really happy to see Joe," she says. "Joe is just an amazingly brave person."

Last year, Mayo's friends established a scholarship fund to honor him. In December, at a meeting of Mayo's support group, Morgan Kelly became the first recipient of the Joseph W. Mayo ALS Scholarship Fund, receiving a check for $2,500.

"I am thrilled that the generosity of my friends has allowed me to help such a wonderful young woman continue her studies," Mayo writes from his home in Augusta, where he now lives with his wife and their three children.

Mayo said the fund has raised more than $50,000 to help children and grandchildren of people with ALS in Maine, New Hampshire and Vermont.

On March 12, 2001, Joe Mayo did something he hadn't done in years, when he addressed the House in support of the Act to Improve End-of-Life Care in the state.

Actually, he sat silently while Rebecca read his prepared statement. The bill was intended to expand eligibility for hospice services to those covered by both private health insurance and Medicaid. As his words carried through the chamber his many friends and colleagues sat in quiet contemplation.

Saxl says that, as powerful as Mayo's argument was that day, it was Mayo's trademark diligence that ultimately resulted in the act's passage.

"It was during budget negotiations between the House and Senate, and we were at an impasse," the speaker recalls. "As part of bringing the two houses together, we required that the Senate support this legislation."

But a transcription error threatened to derail the whole act.

"We were on the floor, about to pass the budget," Saxl explains. "And Joe was rereading the entire budget to make sure we had everything right. When he got to the hospice section he found that the reviser had missed a whole portion in the drafting."

Saxl held up the session until an amendment could be crafted and passed, restoring the missing section of the act - and the budget was passed.

The Fight Continues

Mayo hopes for a cure to be found for ALS, though he knows it will probably come too late for him.

"I have made peace with my illness," he writes. "To fight it would be a waste of time, and time is too precious to me."

He participates in WGME-13's broadcast of the MDA Jerry Lewis Telethon. "I believe that MDA is conducting important research to find cures for people with neuromuscular disease," he explains.

Mayo's keeping his finger on the pulse of state government, and trying to help as many people as possible along the way.

"I think it is important for everyone to focus on the business of living. To love their families, enjoy their friends, contribute to their community."

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Communication Is Key: Larry Cisneros Keeps Channels Open in Work and Life

by Tara Wood

"My immediate response was, 'No way, I never played baseball,'" Cisneros, 63, said.

Want to do your part to advance ALS research? People with ALS are needed to take part in clinical trials or ALS studies. Some studies also need family members to participate. Go to www.mda.org/research/ctrials.aspx and click on "clinical trials and studies" to find out which studies you're eligible for.

At the time, Cisneros didn't fully understand what ALS was, but his reaction was indicative of a man who's been determined to maintain a positive attitude and high quality of life in the face of the disease.

Cisneros, a retired telecommunications specialist who until recently lived in the Dallas area, first received the diagnosis in July 2000.

The discovery of his ALS was another low point in a year that he and his wife, Sandy, have described as "a bummer": 2000 saw Sandy begin a battle with breast cancer that included chemotherapy and radiation treatments.

"Most of the year 2000 was dedicated to getting me through a pretty horrendous experience," Sandy Cisneros said. "By the time I got my feet on the ground and got some hair back on my head, we certainly got into facing his ordeal."

Cisneros uses a power wheelchair since he can no longer walk, has lost some of his right hand function, and has been experiencing weakness in his shoulders and arms. His speaking and eating aren't affected, and in fact, his "appetite is too good," he jokes.

Sandy and Larry Cisneros

Changes Are Plentiful

The loss of those abilities has forced some role reversals and major life changes for the couple, who have been married for 38 years and have two adult children and two grandchildren.

First, Larry retired two years earlier than expected. Then they put their treasured Rockland, Texas, home, which sat alongside a golf course, on the market because it required too much upkeep and was largely inaccessible to Larry's wheelchair.

They'd just begun investigating housing in Florida, where they could be closer to their kids, when the Texas house suddenly sold, months earlier than they'd expected.

Finding themselves without a home, they began a four-week "nomadic journey," driving around the country to visit friends and family.

Meanwhile, they arranged to have a new but smaller home in Florida built with wide doorways and other accessible features. The home should be completed in August, and until then, they're staying with relatives in Twentynine Palms, Calif.

Through it all, Sandy, a retired nurse, has had to assume many new responsibilities, Larry said.

"Not just lifestyle changes and being the primary caregiver, but her role in our marriage has changed," Larry said. "I did the driving on long trips, worked on cars or dealt with the service people, did repairs around the house, and took care of the flower beds and lawn. That is no longer."

For example, during their road trip that stretched from St. Louis to California, Sandy logged over 40 hours of cockpit time, covering over 2,300 miles in their minivan.

"I'm now in the role of co-pilot," Larry said.

But you're not likely to catch the couple complaining about their situation, and they say that help they found in an MDA support group was crucial to their adjustment.

"It's so reassuring to know that the feelings you have and the problems you're facing are not unique to you," Sandy said.

They've reached a point at which they can look at their situation and feel grateful for the experiences they've shared, including the successes in Larry's professional life.

'Houston, We Have a Problem'

Larry spent 36 years in the telecommunications industry, including 12 years with Communications Satellite Corporation (COMSAT) during what he calls the "glory days of satellite telecommunications."

COMSAT was one of many communications providers for NASA during the Gemini and Apollo space programs. As a supervisor, his role was to "facilitate continuous communications through the Pacific regions satellite." He had that job during two historic flights - Apollo 11 in 1969 (the first walk on the moon) and Apollo 13.

"For Apollo 11 it was my shift that set up the video and audio equipment in preparation to receive the 'moon walk' television signal from Australia," he said. "I remember that the actual moon walk started just minutes after my team went off shift. We stayed over to assist if necessary but really to watch the live historical event."

The Apollo 13 mission in 1970 started out typically, and there was minimal television coverage, he said. Then, "the routine mission went critical with the damage to the spacecraft. NASA issued alerts and television broadcast networks ordered up more television feeds."

(After oxygen tanks on the spacecraft exploded, a lunar landing was aborted. With great effort, the astronauts returned safely to Earth.)

"With all of the advancements in technology there was little we could do except to ensure the communications for NASA was not interrupted and do our job with diligence," he recalled.

Finding Strength in Each Other

Ironically, the Cisneroses have learned that maintaining communication is still a key to their happiness.

Larry was at first hesitant to tell his wife that he had ALS, not wanting to "upset the apple cart" while she battled her own illness. But they lean on each other, and take comfort when friends say they're praying for them.

"We work real hard to support each other, and I think we have also been blessed with a family that is incredibly supportive," Sandy said.

Larry credits his wife as his main source of strength, describing her as a woman who continues to bring order to chaos. "She painted a clear vision for the future and accepted challenges brought about by this change in life," he said.

The couple has this advice for others who are coping with ALS: "Keep talking, allowing yourself and your partner time to cry or vent anger," Larry said. He added, keep family and friends in the loop.

MDA has helped them get organized, find resources and anticipate changes, like getting a head start in ordering a motorized wheelchair, they said.

The Cisneroses believe a positive attitude is central to a better quality of life, and as they get ready for a new episode they work to make the most of every day.

"Life has been so wonderful to me," Larry Cisneros said. "If you look at the opportunities that I had, my exceptional wife, loving children, great family support and wonderful friends, what else could I ask for? At some point in life there has to be 'a little milk spilt.'"

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Equipment Survey Can Help With Planning

by Chris Medvescek

Now a little light has been shed on the last of those questions. A group of physical and occupational therapists have conducted a pilot survey of the types of equipment used by people with ALS, and how useful they find each item at various stages of the disease. The survey also looked at methods used to obtain equipment.

The Adaptive Equipment Use Survey was conducted last spring by physical and occupational therapists at the MDA/ALS Center at Johns Hopkins University in Baltimore and the Wake Forest University Medical Center ALS Clinic in Winston-Salem, N.C. With the assistance of MDA offices in Maryland and Virginia, survey forms were sent to 200 people with ALS across the country. Of that number, 102 were returned.

"As clinicians, we were frequently frustrated with the lack of information for both patients and caregivers regarding the effectiveness - usefulness - of equipment prescribed or obtained to help manage symptoms," said survey coordinator Brenda Shaeffer, a physical therapist at the Hopkins center. "This pilot study will be a first step to providing people with ALS with evidence-based information to make the most appropriate decisions regarding equipment use."

The information also will help justify equipment purchase by insurers and other funding sources.

Bath Benches Most Valuable

Survey respondents rated their disease progression using the ALSFRS (Amyotrophic Lateral Sclerosis Functional Rating Scale). This 10-question form assesses a person's ability to perform daily living tasks. The highest score (40) indicates normal function, while the lowest (0) indicates no independent function.

The survey showed that the greatest equipment use is by people in the 10-29 ALSFRS score range, with lesser needs at either end of the scale. Augmentative communication devices were most useful for people with scores of 10-19, although approximately a quarter of those scoring 20-29 also reported needing the devices.

Bath benches and augmentative communication devices were rated the most essential of the eight categories of equipment, followed by (in order): power beds, power wheelchairs, walkers, patient lifts, manual wheelchairs and canes.

Paying for It

Most people answering the survey said they obtained their equipment through insurance or private funds.

About 14 percent obtained manual wheelchairs or walkers with the help of organizations such as MDA.

Loan closets were used primarily for manual wheelchairs (19 percent), lifts (25 percent) and bath benches (15 percent).

Most communication devices were obtained with personal funds or private health insurance. (In 2001, Medicare began covering augmentative and alternative communication devices.)

While this preliminary study has value, its authors caution that it's too small to reliably predict equipment needs. They hope to expand the survey to more people.

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Medicare Guidelines Revised on Cough Device

Photo courtesy of J.H. Emerson Co.

Medicare has reclassified these devices, sometimes called in-exsufflators, cofflators or cough simulators, to allow for specific coverage of them, instead of lumping them in with other durable medical equipment. The new Medicare code for the item is E0482.

These devices, such as the CoughAssist product of the J.H. Emerson Co., deliver air under positive pressure and then rapidly switch to negative pressure. They can be useful in ALS and other neuromuscular conditions that involve severe weakness of the diaphragm, rib muscles or abdominal muscles, which compromises coughing and airway clearance. A doctor's prescription is required.

The Emerson company can be reached at (800) 252-1414, (617) 864-1414, info@jhemerson.com or at www.jhemerson.com.

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Rare Diseases Acts Introduced in the House

The Rare Diseases Act of 2002 (H.R. 4013) makes permanent the Office of Rare Diseases at the National Institutes of Health. The Rare Diseases Orphan Product Development Act of 2002 (H.R. 4014) funds the FDA's Orphan Products Grant Program for drug development.

ALS is among the 6,000 rare diseases covered by the acts.

Diane E. Dorman, vice president for public policy for the National Organization for Rare Disorders, is optimistic that both House bills will pass, especially if they attract a sizable number of co-sponsors. She urged people to contact their representatives about co-sponsoring both bills, noting that "25 million people (with rare diseases) speaking with one voice can bring about a profound change."

In other legislative action, an ALS activist warns that the 15 percent cut in Medicare services scheduled to take effect Oct. 1 could imperil home health care benefits for some people with ALS.

David Jayne of the National Coalition to Amend the Medicare Homebound Restriction (NCAHB) encouraged people to contact their representatives about the importance of home health care coverage, and ask for the elimination of the cut in those Medicare benefits. See www.als-mda.org/publications/als/als6_3.html#homebound.

The cut, a result of the 1997 Balanced Budget Amendment, "makes no adjustment for more costly or more vulnerable patients and therefore will fall heaviest on the most costly patients - those who suffer from chronic illness, more serious illness, as well as those in rural areas," Jayne said.

Jayne, who has ALS and lives in Atlanta, founded the NCAHB to protest Medicare homebound regulations that prevent people receiving home health care benefits from leaving home except for brief periods.

"While broader coverage under the homebound definition is extremely important, it could be rendered meaningless if the covered services are not funded," he said.

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