Telethon Will Highlight MDA’s ALS Research and Services

Stuart and Lisa Nichols of Houston, with Stanley Appel, chief of neurology and director of the MDA/ALS Center, Methodist Hospital, Houston. Stu Nichols will be profiled on this year's Telethon.

by Christina Medvescek

The 40th annual MDA Jerry Lewis Telethon this Labor Day weekend will draw the attention of some 45 million viewers across the country, and another million around the world, to the fight against ALS.

Beginning at 9 p.m. EDT on Sunday, Sept. 4, the 21½-hour broadcast will feature interviews and profiles of many individuals with ALS, as well as information about promising ALS research and MDA ALS services.

MDA’s ALS Division Co-chairpersons Christopher and Reda Rice return for their third visit to the national Telethon, which originates this year from The Beverly Hilton in Beverly Hills, Calif. Chris Rice, 40, received an ALS diagnosis in 2001. He and his wife Reda, of Houston, work throughout the year to raise awareness of ALS and MDA’s efforts to find a cure. Both are frequent contributors to the MDA/ALS Newsmagazine, writing about issues of coping and caregiving. (See May the Telethon Bring Awareness.)

Nationally recognized ALS authority Stan Appel, director of the MDA/ALS Center at Methodist Hospital in Houston, and an MDA Board Member, also will appear on the national broadcast, discussing ALS research and the services available at the 35 MDA/ALS centers across the country.

MDA Board Member and former Olympian Bart Conner will speak about promising advances in ALS gene therapy. MDA-funded scientists have delivered vascular endothelial growth factor (VEGF) as a gene or protein to mice with ALS, which showed strength improvements. It’s believed a lack of VEGF may contribute to the development of the disease.

Stuart Nichols, 53, of Kingwood, Texas, will be profiled on the national broadcast. A global financial accounting manager for Exxon-Mobil, Nichols received an ALS diagnosis in 2004, and was featured in the June issue of the MDA/ALS Newsmagazine (“Staying in the Game”). His wife, Lisa, and two grown children, Andrew and Whitney, will appear with him in the profile.

Of Local Importance

Many more individuals and families affected by ALS will be featured during the local segments of the Telethon that occur each hour. These appearances are an effective way to help the public realize that ALS can strike anyone, including people within their own communities.

“ALS devastates not only the individuals who have it, but their families as well,” said Jerry Lewis, MDA national chairman and Telethon star. “We’ve lost far too many people to this terrible disease. By taking their stories to the public, we hope to generate the support necessary to help MDA stop ALS and other neuromuscular diseases.”

Noting that Eleanor Gehrig (widow of baseball great Lou Gehrig) was one of the founding forces of MDA in the early 1950s, Lewis vowed, “We’re going to fight this battle until it’s won.”

The Telethon will be broadcast Sept. 4-5 on some 190 “Love Network” stations around the country, as well as on cable in Canada and worldwide via streaming video, at www.mda.org. For local times and stations, call your local MDA office or check TV listings.

Jerry Lewis to Receive Emmy The Board of Governors of the Academy of Television Arts & Sciences will honor Jerry Lewis this September with the coveted Governors Award — an Emmy that honors “achievements of current distinction, especially those that exemplify pro-social or pro bono publico contributions.” The Awards Committee stated that it “unanimously recommends that the 2005 Governors Award be given to Jerry Lewis for his work in connection with the Muscular Dystrophy Telethon . . . Jerry Lewis is the host and guiding spirit of the telethon and fundraising efforts of the Muscular Dystrophy Association, and despite battling his own illnesses in recent years, has never missed a telethon. . . Lewis has helped lead a battle that has resulted in increased life expectancy and improved quality of life for children and adults suffering from neuromuscular diseases.” The award will be presented on the Telethon and at the Primetime Creative Arts Emmy Awards on Sept. 11.

MDA Opens 35th ALS Center The MDA ALS Division announces the designation of the Oregon Health & Science University in Portland as the site of its 35th MDA/ALS center. MDA/ALS centers focus on service and research, and offer the latest therapies using a team approach for people with ALS. The centers also help people learn to better cope with the disease. The director of the new center in Portland is Jau-Shin Lou, associate professor of neurology and co-director of MDA’s outpatient clinic at the university. Lou conducts extensive research in the areas of ALS and Parkinson’s disease. A complete list of MDA/ALS centers and contact information is available at www.als-mda.org/clinics/alsserv.html.

ALS Research Roundup

by Margaret Wahl

Depression Doesn't Dominate in ALS

Only 15 (19 percent) of 80 people with late-stage ALS recently surveyed met standard criteria for a diagnosis of depression, say investigators at the Eleanor and Lou Gehrig MDA/ALS Research Center at Columbia University in New York.

Of those 80, 61 were interviewed at monthly intervals at least twice. In that group, 35 (57 percent) never met depression criteria, while five (8 percent) were consistently depressed. The other 21 people (34 percent) were depressed only at some of the monthly assessments.

The investigators, who published their results in two articles in the July 12 issue of Neurology, concluded that depression is the exception, rather than the rule, in late-stage ALS; and that, when it occurs, it’s often transitory.

They didn’t find that spiritual beliefs, the presence of a spouse as a caregiver, depression in the caregiver, financial status, or participation in a hospice program were an influence on depression status.

The researchers also examined factors that may influence a person with ALS’s decision to hasten death. Of the 80 people surveyed, 53 died during the study period. Of these, 23 (43 percent) had said they thought about ending their lives; 10 (19 percent) had expressed to others a wish to die; and three (6 percent) had actively hastened their deaths.

Participants who expressed a wish to die didn’t differ from others in disease severity, age, gender, race, education, support from Medicaid or Social Security Income (SSI), hospice participation, living at home or in a nursing home, or whether they had a spouse as a caregiver.

The study did confirm greater hopelessness and interest in suicide, as well as a lower importance attached to religion, in those who expressed a wish to die.

The authors say that a desire to hasten dying when one has a terminal disease may not be a feature of depression but may be part of a broader phenomenon they call end-of-life despair.

Neurologists Richard Olney and Catherine Lomen-Hoerth of the University of California at San Francisco, in an accompanying commentary, suggest exploring whether depression treatments have an effect on the wish to die.

They also consider why religion and spirituality, which apparently help cancer patients, didn’t offer protection against a wish to die in the ALS patients studied.

They suggest that a more sensitive measure of “comfort in religion,” as well as more careful probing for subtle cognitive changes, might shed light on this.

They conclude by saying, “Whether an ALS patient’s wish to die is more strongly influenced by depression or end-of-life despair remains to be determined. What is more remarkable is that a majority of ALS patients have a more positive attitude toward life even as the inevitability of death is imminent.”

Spastin Gene Implicated in Juvenile-Onset ALS

A mutation in a chromosome 2 gene for a protein called spastin has been implicated as a potential cause of, or risk factor for, a juvenile-onset (before age 25) form of ALS. The spastin protein is involved in the functioning of nerve cell fibers.

In the July 12 issue of Neurology, Thomas Meyer at Charité University Hospital in Berlin, and colleagues, describe a 73-year-old man with ALS symptoms dating back to age 24 and no family history of ALS.

The mutation they found in one of his spastin genes wasn’t found in any of the 200 people in the comparison (control) group; nor was it found in eight of his relatives whose genes were screened.

Other mutations in the spastin gene can cause the neurological disorder spastic paraplegia.

Does Diaphragm Stimulation Work in ALS?

Rhythmic stimulation of the diaphragm through electrodes — “pacing” the diaphragm — may improve air exchange in ALS, says Raymond Onders, associate professor of surgery at Case Western Reserve University in Cleveland.

Onders has implanted electrodes in the diaphragms of three people with ALS, all of whom had lung capacities that were 53 percent of normal or less. He says the electrodes send signals through what remains of their phrenic (diaphragmatic) nerve fibers.

Two months after implantation, Onders reports, the rate of decline in respiratory capacity has slowed, all patients are able to vocalize the “ah” sound longer, and all report feeling better. Onders says the system is safe but that long-term gains can’t be assessed until more time has elapsed.

Other experts, however, voice concerns.

“This is not a good idea in my opinion, and the long-term results may show more rapid progression,” says Greg Carter, a physical medicine and rehabilitation specialist who co-directs the MDA/ALS Center at the University of Washington in Seattle.

“Phrenic-nerve pacing requires healthy motor neurons, which is not the case in ALS. It may work for a short time, but the effect will be short-lived. There is a very real danger, in my mind, of accelerating motor neuron death ... due to overtaxing the cells.”

Walter Bradley, a neurologist who directs the Kessenich Family MDA/ALS Center at the University of Miami, says he’s “not as worried about the potential damage in ALS as about the likelihood that it will not work.”

Onders, who hopes to study 10 people with ALS, says he welcomes inquiries from patients and professionals. He can be reached at (216) 844-5797 (Cleveland) or at raymond.onders@uhhs.com.

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National ALS Database Proposal Before Congress

by Chris Medvescek

An investment in knowledge always pays the best interest, observed Benjamin Franklin. To that end, Congress is considering creation of a national system to collect and store information on the prevalence and incidence of ALS in the United States.

The ALS Registry Act (S. 1353), sponsored by Sens. Harry Reid (D-Nev.) and John Warner (R-Va.), would establish a national ALS registry under the auspices of the Centers for Disease Control and Prevention. A House version of the bill is expected to be introduced in early September by Rep. Eliot Engel (D-N.Y.).

It’s hoped that investing in a national ALS database (a proposed $25 million for the first year) will pay off in increased knowledge about the disease, more research into possible genetic and environmental factors, and enhanced efforts to find treatments and a cure.

Information to be compiled by the national registry would include: the number of people in the United States living with ALS; environmental and occupational factors that may be associated with the disease; and the age, race or ethnicity, gender and family history of individuals with ALS. Other information also may be gathered at the discretion of an advisory committee to be made up of ALS organizations, researchers, federal agencies, clinicians, and people with ALS and their families.

In addition, the ALS Registry Act would establish a secure system for putting people with ALS in contact with scientists studying the environmental and genetic causes of motor neuron disease or conducting clinical trials on therapies.

The proposed national registry would coordinate with appropriate existing state, local and federal registries, such as the Department of Veterans Affairs ALS Registry, the DNA and Cell Line Repository of the National Institute of Neurological Disorders and Stroke Human Genetics Resource Center, and the Agency for Toxic Substances and Disease Registry.

 

Vent Update

In “Safe Harbor” (July), I stated that the cost of vent rental and supplies was $300 to $400 a month. That amount was based on my Medicare statements, which said I “may be billed” that amount. However, after not getting any bills for the last several months, I checked with my equipment provider and was told I had no outstanding balance.

Unsure of what was happening, I asked for input from other vent users. I received only four replies but all said they were not billed for any amount Medicare didn’t pay.

Apparently it is common practice for equipment providers to write off any amount not paid by Medicare and any supplemental insurance. I cannot guarantee that everyone on Medicare will have the same experience but I didn’t want anyone to worry about the cost before checking with their local respiratory care company.

Diane Huberty
Fort Wayne, Ind.

May the Telethon Bring Awareness

by Reda Rice

As the 2005 Telethon approaches, I realize more than ever that ALS awareness is still needed.

My husband, Chris, reminds me of this each time we go to a restaurant. He walks in with his cane, is able to eat his food on his own, but at the end of the meal the waitress assumes he’s mentally incapable of paying the bill, so she hands it my way. This is an example of lack of awareness.

Webster’s dictionary defines awareness as: to be conscious, cognizant, alert, informed, mindful and aware of danger; antonym: oblivious.

What struck me most was the antonym, oblivious. That’s truly the opposite of what we want … for people to be oblivious of the 30,000 Americans who live with ALS; for people to assume there’s something mentally wrong with those in wheelchairs or those who have speech problems.

I’m embarrassed to say that before my husband received his diagnosis, when I was 35, I was oblivious myself. I was living in my own world, one that didn’t include disease and disabilities. Now that I’m no longer oblivious and in the dark, don’t I have a duty to share my awareness with others? This is the question I challenge you to ask yourself.

WHERE does awareness come from? It must come from the source. Messages coming from people living with ALS and their families can be powerful and educational. Awareness can be heartfelt and hopeful. To be a source, we must be willing.

HOW can we give awareness? Through involvement. It starts close to you, with your family, friends, work, church, school and community. It evolves through activities that cause others to notice. The Jerry Lewis MDA Telethon on Labor Day weekend is one such event. We can volunteer to answer phones at the local TV station where the Telethon is televised, or we can help raise money. Each of our efforts, no matter how small, brings needed awareness.

WHEN do we give awareness? Well, the old saying says, “There’s no time like the present.” When we get involved, we’re fighting against people being oblivious to ALS. Contact your local MDA office today. I promise it will bless you more than you’ll ever know.

Chris and Reda Rice of Houston are co-chairs of MDA’s ALS Division.

Luckiest Man: The Life and Death of Lou Gehrig

by Kenneth Plax

Luckiest Man: The Life and Death of Lou Gehrig, by Jonathan Eig, 420 pages, 2005, $26.00. Simon & Schuster, SimonSays.com.

Jonathan Eig’s fine account of the life of Lou Gehrig fractures the man of marble. In his stead emerges a prodigiously talented athlete but a diffident man whose clear-eyed stoicism in the face of physical decline and death restore heartfelt tragedy to Gehrig’s story.

Eig offers a convincing psychological depiction of Gehrig as a man impelled by both a powerful work ethic and an equally powerful respect for authority instilled in him by his mother, Christina, who, haunted by the deaths of her other children, both doted upon and overwhelmed her only surviving child. Gehrig, in turn, remained devoted to Christina. Indeed, Eig implies it took Gehrig’s relationship with his wife, Eleanor, to bring him to full maturity.

The work ethic combined with the respect for authority produced a dutiful employee, a team player and a fierce competitor.

Eig economically recounts Gehrig’s career, extraordinary even in an era of baseball giants; rightly, Eig calls Gehrig the best first baseman ever to play the game. Gehrig’s famous farewell, in Eig’s rendering, is moving. Striking an admirable balance between game details (including an appendix with career statistics) and context, Eig generates strong narrative force.

Of particular interest is the story of Gehrig’s battle with ALS, illuminated by a cache of correspondence Eig discovered between Gehrig and his doctor, Paul O’Leary, of the Mayo Clinic.

Although many of the issues surrounding treatment remain regrettably as unresolved today as they were in 1939, the amount of information the person with ALS now receives is substantially different from that afforded to Gehrig.

At the strong urging of Eleanor Gehrig, O’Leary didn’t inform Gehrig there was no cure for ALS or that his long-term prospects were grim; indeed, he continually offered inadequate, baseless or just plain wrong information calculated to inspire false hope. Throughout his illness Gehrig remained upbeat and cooperated with prescribed treatments; Eig suggests, however, that Gehrig was complicit in the surrounding conspiracy of cheerfulness. As Tommy Henrich, one of Gehrig’s former teammates expressed it, “He knew.”

The Lou Gehrig Jonathan Eig gives us is one who achieved physical grace on the field and grace of character off it — heroism with a human face.

Although Eig doesn’t detail events subsequent to Gehrig’s death, it would be remiss not to acknowledge Eleanor’s response to her husband’s tragedy — devotion to both the memory of Gehrig and the fight against Lou Gehrig’s disease. In the early days of the Muscular Dystrophy Association she worked very closely with current President & CEO Robert Ross, serving as the campaign chairperson, and was very active on MDA’s behalf.

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