by Christina Medvescek

Striking people of every ethnic, cultural and economic background, ALS is an equal-opportunity disease.

And the ways people cope with the disease can vary widely based on their cultural backgrounds.

As director of nursing at Miami’s MDA/ALS Center, Gonzalez is a master problem-solver.

“It’s important for those of us working with people with ALS to know about different cultures,” says Ginna Gonzalez, director of nursing at the Kessenich Family MDA/ALS Center at the University of Miami.

Gonzalez recently was honored as the distinguished lecturer at the Sixth Annual Vicki Appel MDA/ALS Patient Care Conference in Houston. This “nurse of the year” honor is given to an outstanding MDA/ALS center nurse coordinator.

Gonzalez, who helped start the Kessenich center’s multidisciplinary facility in 1998, has made patient education a priority at the center by initiating a Web site, a newsletter and patient support groups. She’s a member of the National Neuromuscular Nursing Advisory Board, the Society of Gastroenterology Nurses and Associates, and the Association of Hispanic Nurses.

At the MDA/ALS center, Gonzalez sees her primary role as that of problem solver, whether it’s finding funding for in-home assistance for a person with ALS, or locating an examining room big enough to accommodate all 10 members of an extended family.

MDA's ALS Division maintains 32 MDA/ALS centers across the country.

Born and educated in Bogota, Colombia, Gonzalez is particularly sensitive to the ways in which culture affects patient care.

Living in culturally diverse Southern Florida, Gonzalez has seen a number of such situations.

For example, many large Hispanic families insist doctors not tell patients they have ALS, in an effort to protect them. An Asian ALS patient who indicated in the emergency room he didn’t want to go on a ventilator was overruled by his family — the nucleus of decision-making in that culture.

A Jewish patient couldn’t attend an ALS support group held on a Saturday, a Jewish holy day. A Muslim patient had trouble finding home health workers willing to abide by his prohibition against wearing shoes in his home.

Cultures in which extended family and religious organizations play pivotal roles often refuse hospice services, misunderstanding what’s being offered, Gonzalez has found. African-American followers of the religion Santeria may avoid going to doctors at all, preferring to seek help in the rituals of their religion.

Some faiths and traditional healers offer “treatments” –- effective or not — to diseases that so far have stymied modern medicine, Gonzalez notes.

“I had a patient from Cuba who was treated for two weeks with flowers and chicken blood. He came back and said, ‘It didn’t work, I’m still sick.’

“But I told him, ‘At least you tried.’” The patient continued treatment at the ALS center.

More Education

Health care workers are likely to go wrong if they don’t involve family or church in decision making. They should also avoid trying to force patients to accept care they may be resisting for cultural reasons, Gonzalez says.

Becoming aware of the different ways in which culture and health care interact may result in better access to medical services for many people with ALS, she says. Also important is recruiting more minority health care providers and giving other professionals cross-cultural training.

“We need to know how people are thinking to know why they’re rejecting hospice or not going to a doctor,” she says. “As health care providers, we need to educate the patient and family according to their culture.”

ALS Online

Check out the new and improved MDA/ALS Division Web page at www.als-mda.org. The change in design makes it easier to find what you’re looking for, and the layout is easy on your eyes. Visit the page to learn about the programs and services of MDA’s ALS Division.

The ALS “disease page” on MDA’s main Web site has also been renovated. The newly modified page defines the disease and provides easy links to other ALS information on the site. You can go directly to news articles about research, profiles of people with ALS, MDA’s ALS publications, clinical trials and more. It’s at www.mda.org/ disease/als.

Watch www.mda.org/chat/calendar.html for announcements of new and ongoing online chats for those with ALS, including expert-led chats on medical and research topics.

ALS RESEARCH ROUNDUP

by Margaret Wahl

Indinavir Shown Ineffective in ALS

The drug indinavir (brand name Crixivan), used to treat human immunodeficiency virus (HIV) infections, was found ineffective in ALS, investigators announced on April 27 at the annual meeting of the American Academy of Neurology in San Francisco.

Hiroshi Mitsumoto

Stephen Sclesa, director of the Neuromuscular Division at Beth Israel Medical Center in New York, presented the results. Hiroshi Mitsumoto, who co-directs the Eleanor and Lou Gehrig MDA/ALS Research Center at Columbia Presbyterian in New York, was also on the study team.

In a clinical trial comparing people with ALS who were given indinavir and others given a placebo, investigators found no significant differences in physical function, general muscle strength or breathing muscle strength.

Retroviruses, including HIV, have been implicated as a possible cause of ALS. In addition, drugs like indinavir have cell-preserving properties that some investigators have thought might be useful in ALS.

Military Service Is Apparent ALS Risk Factor

Men who served in the U.S. military from the World War I era through the Vietnam era appear to be more likely to develop ALS than their counterparts who didn’t enter the military, says a study presented at the April meeting of the American Academy of Neurology.

But combat doesn’t seem to change the degree of risk.

There was no difference in ALS incidence between soldiers who fought in either world war, or in Korea or Vietnam, and those who served during peacetime.

The study was directed by Marc Weisskopf, an epidemiologist at the Harvard School of Public Health in Boston. Weisskopf told the Associated Press that the study offers no hints about how military service might increase the risk of ALS. Some theoretical possibilities include more exposure to heavy metals such as lead, infection exposure or extreme physical exertion, he said.

Military service appears to increase the risk of ALS by 60 percent. In the general population, about two people out of 100,000 develop ALS each year. The increased risk means that about three people per 100,000 in that group would develop ALS in a given year.

A separate study, published in the Sept. 23 issue of Neurology, found that veterans who served in Southwest Asia during the Gulf War in the early 1990s had about twice the risk of developing ALS as those in the military at the same time who didn’t go to the Gulf. (See "Gulf War Vets," October 2003.)

No Enteroviruses Found in ALS-Affected Tissue

In 2000, French researchers intrigued the ALS community by showing that 15 out of 17 spinal cord samples taken from ALS patients contained evidence of an enteroviral infection. Only one out of 29 samples from people who didn’t have ALS showed evidence of such a virus. (See “The ALS/Virus Connection,” February-March 2000.)

The polio virus, Coxsackie viruses and the ECHO group are enteroviruses.

Now, a group of researchers in part supported by MDA has failed to duplicate the 2000 results in a carefully controlled study. The team included MDA grantee Martina Berger of the University of California at Irvine, who was on the 2000 study team; and Robert Brown, Raymond Roos and Benjamin Brooks, directors of the  MDA/ALS centers in Boston, Chicago and Madison, Wis.

The researchers examined spinal cord samples from 24 people who had died of ALS and compared them with 17 samples from people who had died of non-ALS causes.

Two laboratories independently examined the samples and neither found enterovirus material in either set of samples. They did, however, find enteroviral evidence in five other spinal cord samples (two from people and three from mice) to which enteroviruses had purposely been added. The latter finding demonstrates that the enteroviral detection techniques were working properly, researchers said.

The authors of the paper, published in the April 27 issue of Neurology, note that they haven’t proven beyond a doubt that enteroviruses don’t have a role in ALS, but that their findings weaken the argument for an ALS-enterovirus connection.

The investigators noted that they didn’t address the possible roles of other viruses and other transmissible agents in causing ALS.

Blood Biomarker Study Needs Participants

Merit Cudkowicz

Finding unique biochemical markers of ALS in the blood, spinal fluid or other tissues would make diagnosing the disease and following its progress much easier. It would also help in evaluating the effectiveness of experimental treatments.

Earlier this year, researchers announced they’d identified abnormalities in the fluid surrounding the brain and spinal cord that appear to be unique to ALS (see “Spinal Fluid Changes,” January 2004). Merit Cudkowicz, an MDA grantee at the MDA/ALS Center at Massachusetts General Hospital in Boston, was part of that study team.

Now, Cudkowicz, in concert with Metabolon, a Durham, N.C., biotechnology company, will compare blood samples from 100 people with ALS with samples from people with other nerve and muscle diseases and a group with no specific disease. All participants must be older than 20, and all blood samples must be drawn at Mass General.

For information, contact study coordinator Kristyn Newhall at (617) 726-0653 or knewhall@partners.org, or go to www.mda.org/research/ view_ctrial.aspx?id=65.

ALS: TAG LINES AND GAG LINES

At MDA's Los Angeles Gala in April, Kathryn and Jeff Repetto were presented with a puppy, which they've named Moose.

Jeffrey I. Repetto of Culver City, Calif., received the Karen Brown Courage Award at the MDA Gala in Los Angeles in April.

Repetto, a former advertising copywriter, learned he had ALS in August. Five weeks later, he and Kathryn Lundeen were married and honeymooned in Australia. Now 39, he shared his unique perspective on the disease, at the gala.

by Jeff Repetto

I come from the world of advertising. In my training, we’re taught to think a little bit differently about things. And since ALS causes paralysis but leaves the mind intact, it gives you a lot of time to think. Maybe too much time.

After I was diagnosed, the same tag line kept running through my head: “ALS: It’s not just for baseball players anymore.”

And it hasn’t stopped there.

1. I thought it might be fun, enlightening even, to share some of these random thoughts. Put these thoughts together and you get a snapshot of what my world is like.

I’m not a doctor, but I’m pretty certain that people who drive sports cars are not handicapped. Why is this important? Because when the last handicapped spot is taken at Ralph’s by a guy with a nice tan driving a 500SL, something isn’t right.

So, I’ve come up with a plan to solve this nagging social problem. It’s called the handicap face-off. When all the handicapped spots are filled, drivers will be requested to return to their cars for a comparison of ailments.

ALS always wins.

2. Oprah rocks, but Ellen is funnier. And who watches Maury, Rikki or Montel anyway? And these are just three of the mysteries of daytime TV. Does Bryman College have fraternities?  Will you really be happier as a trained dental technician?

As far as I can tell, daytime TV is designed for women with erectile dysfunction who pay far too much for their car insurance.

3. My wife and I have begun to pay strange men to see me naked.  The worst part is, they don’t seem to enjoy it.

I don’t enjoy it much either — and not for the reason my wife supposes, namely that with my ego I think they should be paying me. Instead, it’s because the presence of our custodial caregivers reflects my increasing helplessness.

It’s hard to accept assistance with tasks that one used to have a reasonable proficiency with, like eating, bathing and going to the bathroom. Although I will say it’s great to have another Lakers fan in the house.

4. It’s great that we’re here tonight to raise money to find a cure for ALS. We can use every penny.

In fact, I have a theory: If every dollar used to develop and promote Viagra had instead gone toward ALS research, I’d be getting a prescription from my doctor so I could play tennis with my wife — and some of you would be doing a lot more reading in bed.

5. My wife and I are big shoppers. Here’s just a partial list of what we’ve bought in the last eight months:

A Dodge Caravan with automatic wheelchair ramp, a hospital bed, three canes, a tall walker, a manual wheelchair, a power wheelchair, a wheelchair lift, a commode chair, a shower chair commode, bed pans, an elevated toilet seat, an elevated toilet seat with arms, four portable urinals, sleep boots, an easy-lift recliner chair, a breathing machine, a cat bag to carry it in, a neck brace, a Hoyer lift, easy-grip silverware, and a male girdle, which I like to call a guydle.

My new tag line for ALS? “It’s not just a disease, it’s a lifestyle.”

6. Over the last seven years, the friends I made at Dailey and Associates went from co-workers to my second family.

Just two weeks ago they got together and raised over $23,000 for MDA. And that’s just one small example of the love and support they’ve shown me over the last eight months.

I used to complain about going to work sometimes, but now I’d give anything to go.

7. I was asleep, but now I’m awake.

I’m awake to how many friends I have, what wonderful people they are, and how much they truly care about me.

And I’m awake to my family. To my father, who has opened up to me in ways he never has before. To my mother, who has gone to such great lengths to show her love and support. To my sister, who I know would trade places with me in a New York minute.

My illness is tragic, but it has allowed me to receive gifts of love from those who mean the most to me. If you’re wondering about the meaning of life, it’s simple: To love and be loved. The ultimate wake-up call and my friends and family taught me that.

8. I don’t deserve a courage award, but I know someone who does.

Less than two months after I was diagnosed she stood before friends and family and said these words: “For richer or poorer, for better for worse, in sickness and in health.”

Never have these words meant so much. My wife, Kathryn, married me knowing what lay ahead. It was an act of loyalty, love and yes, of courage, for which I will never be able to repay her.

It’s hard to believe, but sometimes ALS stands for “A Lucky Son of a Bitch.”

Peter Donnelly, Repetto's best friend, holds the Karen Brown Courage Award that Repetto received at the gala.

9. I want to thank each and every one of you for supporting MDA, because they have supported me. Even with a great company and incredible friends and family to help me there have been many times during this process when Kathryn and I haven’t known where to turn.

But with one call to Morris Lawson [MDA Health Care Service Coordinator in Santa Monica], the appointment would be made, the doctor found, the problem solved.

With your generous support, I know that we will find a cure for this terrible disease. While I am pleased to be here tonight to receive this award, I know that you join me in the fervent hope that I am the last person to be so honored.

The Long and Winding Road:

Applying for Social Security Benefits Now a Little Easier for People With ALS

by Christina Medvescek

When you decide it’s time to jump off the work train, there are a couple of government safety nets ready to catch you. Recent changes have made these nets a little stronger for people with ALS.

Social Security Disability Insurance (SSDI) and Supplemental Security Income (SSI) both offer benefits to people of any age with ALS (see “Definitions”). But in the past, applicants with ALS had to show certain disabling signs before they were approved for benefits.

Under new regulations, applicants with ALS only need a formal medical diagnosis of the disease in order to be considered “disabled” by government standards.

Expediting Claims

Although most SSDI applicants with ALS were approved the first time they applied (about 96 percent in 2002), some were denied and had to undergo the time-consuming process of appeal. And time is a precious commodity to people with ALS.

This situation was dramatized by the late John Hunter, a Litchfield, Ohio, man with ALS who crusaded for changes in the law after his initial SSDI application  was turned down because he was still walking. (See “Social Security Changes,” September 2003.)

Last summer the Social Security Administration (SSA) revised its regulations to “simplify and expedite” the application process for people with ALS. For those applying for SSDI, this means the shortest possible wait for benefits. (It generally takes 60 to 90 days to process SSDI applications, and about five months to get a check.)

The old regulations required applicants with ALS to have “significant bulbar signs” (i.e., difficulty in chewing, swallowing and speaking) or “disorganization of motor function” (inability to walk effectively or use both arms). Under the new regulations, a valid diagnosis of ALS is sufficient to prove disability, regardless of how impaired the applicant is at the time.

The other requirements to qualify for SSDI and SSI still apply (see “Definitions”).

For SSI applicants, the new regulations mean that an ALS diagnosis gives them a “presumptive disability.” They can immediately receive monthly SSI payments for up to six months while their claims are being processed. SSDI applicants who also qualify for SSI can receive SSI checks while waiting to get an SSDI check.

If a claim is rejected for some reason, applicants don’t have to pay back the SSI money they’ve received.  

This regulation change isn’t the first time SSA has recognized the need for speed for people with ALS. In 2000, Congress eliminated the Medicare waiting period for SSDI recipients with ALS, allowing them to receive Medicare coverage immediately rather than waiting two years as most other SSDI beneficiaries must do.

What You Need to Apply

The new regulations, listed in the Social Security “Blue Book” under Part III, Listing of Impairments (Section 11.00, Neurological), require applicants to submit “a clinically appropriate medical history, neurological findings consistent with the diagnosis of ALS, and the results of any electrophysiological and neuroimaging testing.”

Current tests used to diagnose ALS (and rule out other diseases) include EMG (electromyography) and nerve conduction studies, blood tests, and an MRI of the brain and/or spinal cord. A copy of the neurologist’s notes also is needed. Documents submitted to SSDI must be originals or certified by the doctor’s office.

For More Info

SSA recommends applying for disability benefits as soon as you’re eligible. Start the process by learning more about the basic requirements and all the documents needed to apply. It’s possible your spouse and minor children also may qualify for benefits.

After learning the basics, write down any questions, then get in touch with an SSA representative by phone or in person. You may start the process before you have all your documents gathered.

Application also can be made online at the SSA Web site.

Two free SSA publications outline the basics in clear, supportive language: “Understanding the Benefits,” Pub. No. 05-10024; and “Disability Benefits,” Pub. No. 05-10029.

These are available at www.ssa.gov, at your local SSA office or by calling (800) 772-1213.

Social Security Definitions SSDI (Social Security Disability Insurance, or Title II of the Social Security Act) • Provides monthly income to people who worked in jobs in which they paid Social Security taxes, and are no longer able to work because of disability • Requires a certain number of “work credits” (based on your years of employment and how much you earned) • Requires that you meet the government’s definition of “disabled,”  which for people with ALS is a formal medical diagnosis of the disease • Pays an average monthly amount of $862 for a single person and $1,442 for a disabled worker with a spouse and dependent child SSI (Supplemental Security Income, or Title XVI) • Is available to people who never worked, or worked but didn’t pay Social Security taxes, or didn’t work enough in recent years to meet the “work credit” requirements of SSDI • Helps very low-income people who are 65 or older, blind or disabled • Requires that your financial resources (savings and assets) not exceed $2,000 and that you meet the government definition of disabled • Pays a set monthly payment of $564 to an eligible individual and $846 to an eligible couple.

TOP

Alan Houghton: A Strong Mind

by Kathy Wechsler

New York oncologist Alan Houghton knows both sides of the doctor-patient relationship.

There’s only so much that medical science understands. A lot of dealing with an illness like this involves not just the medical profession, but family and friends,” says Alan Houghton of New York, who received an ALS diagnosis in 1993.

“Those people are probably more important than the medical profession-side.”

Oncologist Houghton has developed this opinion after 11 years on both sides of the doctor-patient equation.

Career Continues

Houghton, 57, stopped seeing cancer patients a few years ago when mobility became too difficult, but he continues to be involved with patient care. Working with a group of physicians, Houghton reviews the histories of new patients, makes recommendations, and approves experimental treatments.

“I’ve been a doctor who took care of patients with cancer and many with advanced cancer, and so that gave me a very different perspective of having an illness,” said Houghton, who uses a power wheelchair and controls the joystick with his chin.

Even without seeing patients, Houghton wears many hats.

At New York’s Memorial Sloan-Kettering Cancer Center, he’s chairman of the immunology program, and head of the melanoma disease management team, which is made up of surgeons, oncologists and researchers. Houghton is also a professor of medicine and immunology, and chairman of the immunology graduate program at Cornell University. He’s also editor of a medical research journal.

These positions don’t require Houghton to be on the move all the time but allow him to use his expertise in oncology, he says.

Cerebral Focus

“In a way, ALS has helped me, because it’s caused me to focus more on my research,” said Houghton, who’s working to develop vaccines for cancer. “I can’t use my arms and legs, but I use my brain more. It’s allowed me to be more cerebral, rather than physical.”

Since his speech is only slightly affected by ALS, Houghton uses voice recognition software and other devices to stay as active as possible.

“I know keeping busy is very important for me in terms of what I call ‘not becoming my disease,’” he said. “I know I have ALS, it’s part of what I am, but I haven’t become ALS — it’s just part of my life.”

Houghton and his wife, Meryl, have two sons, Colin, 20, and Sean, 17. His family is his main source of emotional support, he says. Meryl is involved in an MDA caregivers’ support group and writes a column for the local MDA/ALS newsletter.

In his free time, Houghton reads e-books and listens to music. He also uses the Internet to find information about his ancestors.

Celebrating Accomplishments

Houghton was one of 10 people with ALS who received state MDA Personal Achievement Awards in 2003.

“Being accepted was a thrill for me,” Houghton said. When staff at the local MDA office asked if they could nominate him, he said, “Sure, I would like to do anything I can for people with neuromuscular diseases.”

Through the Eyes of a Wife … Thanks

by Reda Rice

Christopher and Reda Rice, ALS Division Co-chairpersons. Chris received an ALS diagnosis in late 2001. The Rices have three children.

Through the eyes of a wife, a man with ALS is one of the most special people in the world. Unbeknownst to himself, he’s a huge blessing in many people’s lives.  He is proof that God uses all things for good.

A father with ALS, like my husband, Chris, brings his children more gifts than he can ever know:  Through his behavior, Chris gives our children lessons in patience and putting others first, in taking turns and waiting for yours. He also gives them the  enjoyment of simple things like sitting and hugging, or feeling love with just a glance.  

In a world filled with endless options for entertainment, instant gratification and a tendency to be self-centered, these gifts are invaluable.

A man or woman with ALS gives young colleagues, family and friends a generous spirit, a sense of gratitude and a new perspective on life.  This perspective isn’t focused on what one has or wants, but on what they can do for others.

The person with ALS is an inspiration to others with the disease.  People with ALS form a support system for each other and share vital information for living. Their humble nature and stubborn tenacity are contagious, helping each other to keep spirits high.

Through the eyes of this wife, people with ALS have taught me that my life wouldn’t be complete without faith and hope. Those are two things I pray I never lose.

If you have ALS, I hope you’ll focus on these positive blessings that you pour out to so many.  Know that you are loved and appreciated, and that the way you face your circumstances has a lasting effect on generations to come.

So, thank you for helping heal the world from itself.  May you all hang on for you own personal healing.  

And for my sweet husband, Chris:  I’m so proud of you and my love grows for you more each day.