ALS: Anyone’s Life Story

by Bill Norman

To commemorate ALS Awareness Month, MDA will celebrate the stories of 31 individuals affected by ALS. “ALS: Anyone’s Life Story” will feature a different story each day in May on MDA’s ALS Web site.

The series was inspired by Augie Nieto, who serves as co-chairperson of MDA’s ALS Division, along with his wife, Lynne. Nieto often speaks of how ALS caused him to change his life focus from striving for success to instead working for significance.

Among those featured in the series, you’ll meet Marilyn Cooper, Northridge, Calif., who tells us that after her diagnosis she went back to college and earned a master’s degree in counseling. “I read non-stop and have started a book club that has been going strong for seven years,” she says.

Angelo Sciulli, Lancaster, S. C., received his diagnosis in 1998, but this summer he’s going fly fishing in Colorado. He even found time to publish a book in 2005 about his life with ALS — described through photography trips to places such as Kenya, Ecuador and Newfoundland.

“I’ve always held the opinion that life is lived between one’s ears. Nothing has both challenged and proven this belief more acutely than ALS,” says Robert Harr, Dewitt, Mich. “One’s mind is the wellspring of all action in every aspect of life.”

“ALS has changed me for the better,” says Mary Kay Stillwell, Wichita, Kan. “I’ve gone from being a strong-willed, independent and controlling individual to a strong- willed, appreciative person who delegates jobs.”

Charles Dyer, Jonesboro, Ga., had always loved hunting. He says, “I was never very good at bird hunting until now. Thanks to the way I shake when I hold the gun, my aim covers the whole sky, and the birds don’t stand a chance.”

Having to choose from among many, many stories of strength, humor, perseverance and love wasn’t easy; but it was a reminder that ALS stories are often inspirational.

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MDA Commemorates ALS Awareness Month

by Kathy Wechsler

It’s May, and MDA is in full swing as it commemorates the 16th annual national ALS Awareness Month. The goal is to spread the word about the devastating disease, and about the health care services, research and public education programs of MDA’s ALS Division.

PSAs

To campaign for increased public awareness of ALS, the Muscular Dystrophy Association is promoting a series of public service announcements (PSAs) featuring some of the people with ALS whose stories are told in “ALS: Anyone’s Life Story”.

Publications

As part of the same campaign for public awareness, MDA is issuing a new MDA ALS Division brochure, a concise description of MDA’s ALS program. The brochure includes a summary of the services MDA offers, Web site addresses and contact information.

MDA is also offering “Milestones in ALS Research.” “Milestones” provides a timeline that traces significant events in the history of ALS and important research efforts supported by the Association.

Chats

MDA will sponsor two special ALS Research Updates chats during ALS Awareness Month. One will be devoted to recent research advances, while the other will address upcoming research efforts; one will occur during the first week in May, while the other will occur during the last week. The times and dates will be posted on the MDA Web site. The chats will be open to anyone who has registered, and anyone can register immediately.

Local Events

Throughout the month, MDA offices across the country hold ALS-centered events and seminars. For more information on an event in your area, contact your local MDA office.

Portland, Ore.

The Portland MDA office has published a special ALS Awareness Month newsletter highlighting a number of people with ALS in the community, including Jeff Young, who is profiled in MDA’s online “ALS: Anyone’s Life Story” series May 6. Portland’s MDA office also is sending letters to all Oregon and Southwest Washington neurologists informing them of services offered by the MDA ALS Division. A series of ALS Awareness dinners will be held in the homes of families affected by ALS.

Houston

During May, TV station KPRC (a member of the “Love Network” that broadcasts the Jerry Lewis MDA Telethon) will air an ALS story featuring a local family served by MDA. In addition, Houston’s MDA office is sending letters to local families affected by ALS recapping services offered by MDA’s ALS Division. On May 12, the ALS Support Group will meet at Spring Shadows Pines. After a joint meeting for people with ALS and their caregivers, the two groups will meet separately.

Buffalo, N.Y.

MDA Day at the Bisons will take place at Dunn Tire Park May 9. John Berkhoudt, who has ALS, and his barbershop quartet, the Friends of Harmony, will perform the national anthem and throw out the first pitch at the Buffalo Bisons minor league baseball game.  Prior to the game, there will be a tent party, and MDA families can purchase tickets at discounted prices.

Phoenix

The ALS Life Planning Conference will be held at St. Joseph’s Hospital May 11.  Dietrich Stephan from the Translational Genomics Research Institute (TGen) will speak on genetic predisposition to sporadic ALS.

Knoxville, Tenn.

The local MDA office will hold an ALS Awareness silent auction during the May 12 Tennessee Smokies baseball game. Baseball memorabilia will be auctioned off.

Columbus, Ohio

The Living with ALS: A Daily Journey seminar, to be held at the Concourse Hotel May 19, will explore the issues of everyday life with ALS. Speakers will include physical, occupational, respiratory and speech therapists; neurologists; and Medicare and Social Security experts. The event is free and open to the public.

Southern California

The Conference of Southern California at the Anaheim Convention Center May 5 will focus on ALS research breakthroughs. Keynote speakers include MDA ALS Division Co-Chairperson Augie Nieto, Hans Keirstead from the University of California at Irvine, and Sean Scott from the ALS Therapy Development Institute. Marilyn Cooper, a member of the MDA National Task Force on Public Awareness, will speak about her life since her ALS diagnosis 32 years ago.

Honolulu

The annual ALS seminar will have a new twist this year. Taking place at Queen’s Conference Center May 19, the event will be a spa day aimed at showing people how to pamper their loved ones who are affected by ALS. Free and open to the public, the spa day will concentrate on accessible travel, fashion and beauty, health and medical issues, and law and finance.

Hartford, Conn.

Throughout May, area MDA clinics will post in their exam rooms information about ALS and MDA’s recent research breakthroughs. The one-page sheets are available for visitors to take home. The May 16 ALS support group will have a special seminar on the importance of planning. Speakers will include financial and legal experts who have ALS themselves.

The Field of Dreams fund-raising event will be held at Joe Black’s Restaurant & Pub May 2 and 3. Corporate sponsors who come to the event donate funds for ALS research minutes by sponsoring 20 minutes ($1,300) for a single play, 40 minutes ($2,600) for a double play and 60 minutes ($3,900) for a triple play.  The top 20 fund-raisers will receive two tickets for VIP skybox seats at a minor league baseball game.

Shawnee Mission, Kan.

During the May 17 ALS support group, pictures will be shown of the April 9 signing of a proclamation announcing May as ALS Awareness Month in Kansas by Gov. Kathleen Sebelius. People with ALS will be invited to the May 20 Kansas City T-Bones baseball game, where one family will get to throw out the first pitch. Information about MDA’s ALS Division will be distributed.

Baltimore

The Baltimore MDA office will hold its MDA/ALS Spring Fling Picnic at Keswick Home May 10. The evening of ALS awareness and family fun will be held during the season’s last MDA/ALS support group meeting. The May 12 MDA/ALS Caregiver Cookout, which will be held at the home of MDA/ALS Outreach Committee member Pat Fraher, will give caregivers encouragement, support and understanding about living with a loved one with ALS.

El Paso, Texas

May 18 is Lou Gehrig’s Awareness and Fire Fighter Appreciation Night at El Paso Diablos’ Cohen Stadium, and one ALS family will throw out the first pitch. There will be New York Yankees items for auction. After the baseball game, spectators can gather on the field to watch “Pride of the Yankees,” the Hollywood account of Lou Gehrig’s life, which will be shown on a screen placed on the pitcher’s mound.

Denver

On May 11, the symposium An Afternoon with the Experts will be held in the conference area of the Denver Zoo. A panel of researchers will discuss ALS research breakthroughs and a current clinical trial, and introduce patients involved in the trial. The conference, complete with tickets to the zoo, is free. Call ahead for reservations. For more information, call Linda Johnson at (303) 691-3331.

by Margaret Wahl

Caregivers May Be More Discouraged Than People With ALS

A study conducted at the University of Torino in Italy has found that when 31 people with ALS and their caregivers were interviewed separately as the disease progressed, the caregivers reported a significant increase in depression and a sense of being burdened. In contrast, those with ALS didn’t report a change in their quality of life or an increase in their level of depression.

People with ALS and their caregivers report different levels of depression as the disease advances.

Adriano Chio and colleagues, who published their findings in the March 20 issue of Neurology, conducted structured interviews of the patients and caregivers at two points during the course of the disease, approximately nine months apart. Paid caregivers were excluded, as were ALS patients with obvious cognitive impairment. (The study originally had 55 couples, but 20 patients died, and four withdrew for other reasons.)

At the initial interview, caregivers reported a better quality of life than did patients; but in the second interview, the patients reported a higher life quality than did their caregivers.

Caregivers reported a significant increase in their perceived burden and a reduction in their quality of life. Lack of time for oneself was the main source of caregiver dissatisfaction.

In this study, the investigators say, the patients seemed aware that their caregivers were becoming more burdened over time, but their sense of themselves as a burden didn’t change.

The study’s authors suggest that people with ALS may, at least in some cases, lack empathy for their caregivers and say this could be because of reduced insight, related to subtle cognitive changes. They say more careful examinations of the mental status of those with ALS might help caregivers by providing them with deeper knowledge of the persons they care for.

“Psychological support for ALS caregivers is needed, and  it should be supplied by ALS centers,” Chio said.

The incidence of ALS may differ among ethnic groups, say researchers in Ireland and the United States who reviewed 40 years (1966 through 2006) of ALS population studies.

Cultural differences may influence willingness to acknowledge mental distress.

Simon Cronin at Beaumont Hospital in Dublin and colleagues, who published their results in the March 27 issue of Neurology, found that the incidence (number of new cases per year) of ALS may be lower in African, Asian and Hispanic populations than it is in Caucasians (non-Hispanic whites).

However, the authors note several possible flaws in their conclusions. Among them are different methods used in the various studies; variation in the average age of death among populations, leaving fewer people of ALS-susceptible age in some areas compared to others; and differences in access to medical care, leading to underdiagnosis of ALS in some countries and among U.S. minorities.

The investigators say finding ALS incidence differences, if they exist, could provide further clues to both genetic and environmental contributors to ALS.

A variation in ALS incidence correlated with an ethnic group “would support the notion that genetic factors play a dominant role in the pathogenesis [origin] of the disease,” they write. If the risk of ALS is higher in a particular group, that population should be prioritized for study because their higher “genetic burden” might make detection of ALS-causing mutations easier, they say.

Identified differences in various populations might also find that some environmental factors, such as dietary habits or exposure to toxins, contribute to ALS.

When 92 people with end-stage ALS in three countries were compared, those in the United States reported the least amount of distress. Those in Israel reported the most, and German patients were between the other two groups.

Investigators at Columbia University in New York, the University of Munich (Germany) and Tel Aviv (Israel) University, who published their findings in the March 27 issue of Neurology, say patients at the three sites were similar with respect to social and demographic features, disease severity and nearness to death. (Those who opted for long-term ventilation via tracheostomy weren’t included.)

Steven Albert at Columbia (now at the University of Pittsburgh) and colleagues found that Israeli patients were the least likely to report religiousness or spirituality. However, even when the researchers took that factor out of the mix, reported distress levels by nationality didn’t change.

The nature of care provided, however, may have been a contributing factor. All three sites were multidisciplinary ALS centers, but they differed somewhat in their approach to care. German patients were less likely to use noninvasive ventilation than were the other two groups. Americans were routinely referred to hospice care, and nearly 80 percent used it, while the Israeli system didn’t provide hospice services. U.S. patients may also have been more aware of the option of long-term ventilation, which may have been comforting, even if it wasn’t chosen.

Nevertheless, the authors say they’re not certain why the Israelis reported severe distress and Americans reported so little. “More generally,” they say, “there may be country-level effects on patient willingness to report negative states or accept descriptions of states of mental health.”

This Year in Research

by Margaret Wahl

The year since May 2006 has witnessed several steps in the evolution of ALS research. Results aren’t yet available for several causative factor and treatment studies, but in many cases, they’re expected soon.

MDA’s “whole-genome association” project conducted at the Translational Genomics Institute (TGen) in Phoenix yielded some potentially important and previously unidentified differences between the DNA of ALS-affected and unaffected study subjects. These results will likely provide raw material for a number of future investigations.

To block post-DNA genetic instructions (RNA), “antisense” RNA can be used to target “sense” RNA. Researchers hope to block erroneous, ALS-causing instructions for the SOD1 protein.

This year, with funding made possible by MDA’s Augie’s Quest initiative, MDA also teamed up with the Cambridge, Mass., ALS Therapy Development Institute. This state-of-the-art laboratory specializes in efficient testing of the biochemical effects of compounds of interest in cells alone and in mice with ALS.

In another area, a technology known as “antisense,” which blocks genetic instructions, has matured to the point that an MDA-supported clinical trial of antisense in a familial form of ALS is contemplated for the near future. In this trial, an antisense compound will block incorrect and highly toxic instructions for the SOD1 protein, a known cause of ALS.

Also of note are recent findings that inflammation, a natural process that occurs when the body feels under siege, isn’t just a marker of ALS but is likely a contributor to it. In ALS, inflammation is in the nervous system and is carried out by cells known as microglia. They’re likely to become targets of research and treatment trials soon.

Here’s a summary of where we are on several fronts:

MDA ALS DIVISION —
Research, Services, Information

by Amy Labbe

The Muscular Dystrophy Association’s ALS Division offers the most comprehensive range of services of any voluntary health agency in the nation. The Division’s programs include an aggressive worldwide research program dedicated to finding causes, treatments and cures; top-notch medical care at clinics staffed by leading ALS experts; and print, Internet and video material on managing daily life with ALS.

For more than 50 years, MDA has led the world in ALS research and services, investing some $210 million. Last year MDA spent $18 million on its ALS program. It’s projected that MDA’s ALS research expenditure in 2008 will exceed $24 million.

Research

MDA‘s ALS Division supports a worldwide program of ALS research, from basic science to therapy development to clinical trials of potential treatments. MDA funds leading researchers seeking the causes of, and treatments and cures for, ALS.

The MDA Web site offers information about participating in a clinical trial.

“This Year in Research,” provides a useful overview of both recent and ongoing research in ALS.

Medical Care

MDA maintains 225 hospital-affiliated clinics and 37 designated MDA/ALS centers nationwide, providing specialized medical services for people affected by ALS. These facilities are staffed by multidisciplinary teams of health professionals skilled in the diagnosis and medical management of ALS, including symptom control, medical interventions and therapies to help maintain the highest possible quality of life throughout the course of the disease.

Other Services

Through its local offices, MDA offers support groups for those with ALS and their caregivers.

People with ALS can borrow wheelchairs and other durable medical equipment from MDA’s equipment loan closets to help with special circumstances or day-to-day living. MDA also provides financial assistance to help fund the cost of wheelchairs, leg braces and communication devices, as well as wheelchair repair.

To find your nearest MDA office, call (800) 572-1717.

Information

MDA is the nation’s best source of up-to-date news about ALS research developments, services and clinical trials. Its print and Web publications also provide comprehensive information on dealing with medical, financial, practical, emotional, and other challenges encountered by those with the disease.

The first stop for information is MDA’s ALS Division Web site or your local MDA office.

At the Web site, in addition to news, you’ll find information and access to regularly scheduled online chats geared toward caregivers and those dealing with ALS. You can also see transcripts of past chats.


Publications

Each local MDA office, as well as MDA National Headquarters, offers an assortment of ALS-specific publications, many of which can also be found on the MDA/ALS Web site.

Everyday Life With ALS: A Practical Guide: This 150-page illustrated book provides basic information on saving energy, home modification, respiratory and speech equipment, mobility equipment, exercise and more.

Facts About Amyotrophic Lateral Sclerosis: An introductory pamphlet for those with new diagnoses.

Los Hechos Acerca de la Esclerosis Lateral Amiotrófica: The Spanish translation of “Facts About ALS.”

MDA ALS Division: A description of MDA’s ALS program.

MDA/ALS Newsmagazine: Published 10 times a year, the magazine features news on ALS research, profiles of people with ALS, and insightful articles on health care and caregiving.

Meals for Easy Swallowing: A book of recipes and feeding tips. No longer in print, but available online.

Milestones in ALS Research:  A timeline of important research funded by MDA.

Quest: MDA’s award-winning, bimonthly national newsmagazine includes articles on all aspects of living with neuromuscular diseases, information on helpful products and research updates.

When a Loved One Has ALS: A Caregiver’s Guide: No longer in print but available online.

Videos

Videos available from local MDA offices offer helpful guidance on ventilation options.

Breathe Easy: A Respiratory Guide for People Living With Neuromuscular Diseases: This educational video explores options for assisted breathing devices and techniques.

Breath of Life: This version of “Breathe Easy” is geared for use by physicians and other medical professionals.

Family & Caregiving Resources for People With ALS

by Alyssa Quintero

MDA/ALS Centers

Thirty-seven facilities at major medical institutions have been designated MDA/ALS Centers, indicating that they’re focal points of MDA’s ALS program, although all MDA clinics serve people with ALS.