Colorado Couple to Co-Chair MDA’s ALS Division

Steven and Jennifer Bishop

Those who meet Steven and Jennifer Bishop are struck by their positive and courageous outlook on life — an attitude they’ve gained while facing the challenge of amyotrophic lateral sclerosis.

The Bishops, who display a unique spirit and a keen ability to articulate their experience, have been tapped to serve as co-chairpersons of MDA’s ALS Division.

The residents of the Denver suburb of Arvada, Colo., will help raise awareness of MDA’s leadership of the battle against ALS through public appearances, speaking engagements and public service announcements.

“We’re truly honored, and see this as our new life mission,” Steven Bishop said. “It’s our goal to make Americans as familiar with ALS as they are with diseases like Parkinson’s or Alzheimer’s.”

Steven Bishop, 37, received a diagnosis of ALS in March 2001 and is still ambulatory. He has a background in sales in the telecommunications industry, and Jennifer Bishop worked in social services before becoming a stay-at-home mom to their son Christopher, 4 — their “main source of joy.”

The Bishops will relate their personal experience with the disease, and speak about the vital help and information they’ve found through an MDA support group in Denver.

“The greatest thing about our support groups, and probably any support group, is you are there with people who are going through your exact situation, but they’re dealing with it in different ways,” Jennifer Bishop said.

As co-chairpersons, the Bishops hope their efforts will stimulate more support for MDA’s ALS research program and help other families cope with the disease’s challenges.

“This has really given me a chance to understand what living is all about,” Steven Bishop said of the disease. “At the end of the day, if I’m able to have more positive emotional feelings than negative emotional feelings, then I don’t care what tomorrow is, because I’m not guaranteed that anyway.”

Watch for more about the Bishops in upcoming issues of this newsletter and in other MDA materials.

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ALS Research Roundup

by Margaret Wahl and Dan Stimson

Doctors Discuss ALS Treatment Trends at MDA Gathering

Last month, some 180 MDA-associated physicians and other health professionals gathered in Tucson to focus on major trends in the medical care of ALS and other neuromuscular disorders. Several MDA-supported ALS researchers provided status reports on their work as well.

Robert Miller, who directs the MDA/ALS Center at California Pacific Medical Center in San

Stanley Appel

Other MDA/ALS center directors explored details of daily care in ALS. Terry Heiman-Patterson, director of the MDA/ALS Center of Hope at Drexel University in Philadelphia, presented data on how noninvasive (without tracheostomy) ventilation can improve quality of life and extend survival. Sleep quality, endurance, shortness of breath, speech and cognitive function can benefit from such ventilation, she said.

Several doctors expressed the view that ALS is a treatable disease. Jeffrey Rosenfeld, who directs the MDA/ALS Center at Carolinas Medical Center in Charlotte, N.C., emphasized the desirability of putting in a feeding tube for needed calories and fluids early in the disease course, rather than viewing this intervention as an “end-of-life” measure.

Edward Kasarskis, at the University of Kentucky at Lexington, said the ALS disease process probably increases patients’ caloric needs by a number of mechanisms, making reduced food intake a serious problem.

Other physicians emphasized the need to support the patient and family throughout the course of ALS, including providing appropriate end-of-life care.

Hiroshi Mitsumoto, director of the Eleanor and Lou Gehrig MDA/ALS Center at Columbia Presbyterian Medical Center in New York, noted the need to constantly evaluate and update standards of care with respect to diagnosis, medication use, nutrition, breathing and quality of life. Mitsumoto co-chaired the conference with Stanley Appel, who directs the Ronny & Linda Finger MDA/ALS Center at Baylor College of Medicine in Houston.

On the scientific front, Don Cleveland, in the Department of Cell Biology at the University of

Hiroshi Mitsumoto

Appel noted that cells of the immune system known as microglia are activated in the nervous systems of those with ALS and that their presence could be an important clue to disease causation and treatment. The anti-inflammatory drug celecoxib (Celebrex), now being tested in ALS, may work in this pathway.

Genetic and environmental factors were discussed by several researchers. Gulf War participation and exposure to agricultural chemicals likely increase the risk of developing the disease, it was noted, while some studies have also implicated exposure to lead, high dietary fat intake and smoking as ALS risk factors.

Variations in several genes, in addition to the well-known SOD1 gene flaws, have been identified as possible causes of ALS or ALS susceptibility.

ALS Researchers Meet in Melbourne and Review Drug Progress

Researchers convened in Melbourne at the 13th International Symposium on ALS/MND, hosted by the Motor Neurone Disease Association of Australia, Nov. 17-19.

The following were among the highlights of this meeting:

Stem Cell Safety Trial

A procedure in which bone marrow stem cells were taken from seven Italian ALS patients and implanted into their spinal cords appears to be safe and well tolerated, physician-investigator Letizia Mazzini announced. Establishing safety was the purpose of the trial.

The seven patients, who apparently received stem cell transfers in October 2001, had no major problems, with the exception of pain after surgery.

Marinol

Robert Miller

Robert Miller, director of the MDA/ALS Center at California Pacific Medical Center in San Francisco, and Deborah Gelinas, director of the MDA clinic at the same institution, were among investigators who found that a marijuana-derived compound appears to relieve symptoms in ALS.

The compound is dronabinol (brand name Marinol), made by Unimed Pharmaceuticals (part of Solvay), and it’s approved by the U.S. Food and Drug Administration for treatment in AIDS and cancer.

In the three-month study, involving 20 people with ALS, the drug was well tolerated. Improvements in sleep, appetite and spasticity (muscle tightness) were noted, and the researchers said the compound merits further study for its possible symptom relief and perhaps as a neuroprotective agent.

For more information, see www.marinol.com.

Oxandrolone

Jeffrey Rosenfeld, who heads the MDA/ALS Center at Carolinas Medical Center in Charlotte,

Jeffrey Rosenfeld

Oxandrolone is a synthetic steroid of the “anabolic” (tissue-building) type. It’s similar in structure to the male hormone testosterone.

Muscle strength was checked every three months for a year in participants who took 10 milligrams of oxandrolone twice a day. The muscle groups that were weakest (less than 40 percent of normal strength) at the outset didn’t decline during the study period, while similarly weakened muscle groups in those receiving a placebo as part of a separate study did.

The muscles that were rated as stronger (more than 40 percent of normal strength) at the trial’s outset did, however, get significantly weaker during the study. The drug didn’t help with weight maintenance or respiratory capacity, but was well tolerated with few side effects.

The authors say that oxandrolone “may have a selective benefit in the weakest muscle groups” in ALS. They’re undertaking further studies to test oxandrolone in conjunction with other compounds.

Creatine

A study of 175 people with probable or definite ALS at University Medical Centre in Utrecht and the Academic Medical Centre in Amsterdam in the Netherlands found “no evidence for a beneficial effects of creatine on survival, disease progression or symptoms.”

In this startlingly disappointing study, participants were randomly assigned to receive either 10 grams a day of creatine or a placebo.

“A difference in survival in favour of creatine could not be proven,” the authors write in their summary. “The rates of decline of functional measures were not significantly different between the groups.” The trial was stopped when it became clear that the creatine wasn’t making a difference.

Neurodex

The drug Neurodex (also called AVP-923 and made of a combination of dextromethorphan and quinidine) appears to decrease episodes of excessive laughing or crying that sometimes occur in ALS, according to a study by Avanir Pharmaceuticals.

The unwanted, inappropriate displays of emotion, termed pseudobulbar affect, probably occur in ALS because of a loss of control of some parts of the brain over other parts as a result of nerve-cell degeneration.

Seventy people with ALS took capsules of Neurodex containing 30 milligrams of dextromethorphan and 30 milligrams of quinidine. Control groups took each of the substances alone.

“The results of the study demonstrate that AVP-923 was statistically significantly more effective than its components in the treatment of pseudobulbar affect,” the investigators write in their study report.

Avanir plans to conduct a trial of AVP-923 in people with pseudobulbar affect associated with a variety of conditions. (Check www.mda.org/research/ctrials.aspx for updated trial information.)

Scientists Report Progress Toward Drug Therapies

A t the 32nd annual Society for Neuroscience meeting, held last month in Orlando, Fla., several research groups reported new leads on potential drug treatments for ALS.

NINDS Screen Starts to Pay Off

Jeffrey Rothstein at Johns Hopkins University in Baltimore and Jari Koistinaho at the University

Jeffrey Rothstein

Glutamate provides a “go” signal to motor neurons (muscle-controlling nerve cells) in the spinal cord, but becomes toxic with prolonged exposure. Normally, proteins called glutamate transporters vacuum up excess glutamate, but there’s evidence that people with ALS have a deficiency of one of these transporters called EAAT2 — leaving their motor neurons vulnerable to glutamate’s toxic effects.

Riluzole (Rilutek) works by inhibiting glutamate’s release from brain cells. Unfortunately, it slows the disease by just a few months.

With an eye toward more powerful treatments, Rothstein is searching for drugs that stimulate EAAT2, and Koistinaho is after drugs that protect neurons from a lethal dose of kainate, a glutamate analogue.

Their work is part of the Neurodegeneration Drug Screening Consortium (NDSC), a group of nearly 30 labs in the United States and abroad working together to screen over 1,000 chemicals for activity against ALS, Huntington’s disease, Parkinson’s, spinal muscular atrophy and spinal-bulbar muscular atrophy. The National Institutes of Neurological Diseases and Stroke (NINDS) organized the effort and selected the chemicals, most of which are FDA-approved drugs.

The screen has started to bear fruit, but the researchers involved are keeping quiet about the results while they prepare their work for scientific publication.

Without giving away any details, Rothstein and Koistinaho reported that their screens have turned up nearly 100 hits. Rothstein, who co-directs the MDA/ALS Center at Hopkins, found 22 chemicals that stimulate at least a threefold increase in EAAT2 levels when tested in rat spinal cords. Using a similar protocol, Koistinaho found 75 chemicals that decrease kainate-induced neuronal death by at least 50 percent.

Iron, Antibiotics Show Promise

Two research groups have tested new drugs and drug combinations in SOD1 mice, which carry a mutant version of the SOD1 gene linked to familial ALS.

M. Flint Beal at the Weill Medical College of Cornell University in Ithaca, N.Y., showed that the mice live several days longer if they’re treated with iron-porphyrin.

The compound is related to a chemical found in red blood cells and is believed to counteract oxidative stress, a buildup of oxygen-based free radicals. Iron-porphyrin extended survival of the SOD1 mice when given before or after disease onset, improved their motor performance, and reduced the levels of malondialdehyde (an indicator of oxidative stress) in their spinal cords.

Beal’s group also tested the effects of inhibiting matrix metalloproteinases (MMPs), enzymes that activate proteins on the outer surface of cells. One type, MMP-9, is elevated in the spinal cords of SOD1 mice, possibly leading to the activation of tumor necrosis factor alpha (TNF-alpha), a cell surface protein that contributes to inflammation and cell death.

When Beal’s group deleted both copies of the MMP-9 gene in SOD1 mice, survival was extended by 31 percent. Treating the mice with an MMP inhibitor extended survival by just 10 percent.

Finally, Jean-Pierre Julien at McGill University in Montreal tested a triple-drug cocktail consisting of riluzole, minocycline and nimodidpine.

Minocycline, an antibiotic believed to inhibit apoptosis (cell suicide), extends survival in SOD1 mice by about three weeks and is being tested in ALS patients in an MDA-funded clinical trial (see “Large Minocycline Trial Opens,” November 2002). Nimodidpine is used to treat brain hemorrhage, and works by blocking the entry of calcium into neurons — thought by some ALS experts to be an early event in motor neuron death.

SOD1 mice given all three drugs when they were in the early stages of ALS lived about six weeks longer than untreated mice did. Since all three drugs have FDA approval in different diseases, the triple-drug cocktail could be fast-tracked into clinical trials.

No ALS ‘Cluster’ Found at Kelly Air Force Base

When 39 cases of ALS were identified in 1999 among current and former employees at Kelly Air Force Base in San Antonio, area residents and experts wondered what that might mean.

But a thorough investigation, conducted by the Air Force Institute for Environment, Safety and Occupational Health Risk Analysis, and the San Antonio Metropolitan Health District, has failed to confirm the existence of an ALS “cluster” at the base.

The study results are published in the November issue of the Journal of Occupational and Environmental Medicine.

The investigators looked at records of 31,811 civilian employees who worked for at least a year at the base between 1981 and 2000. (The base closed in July 2001.)

Some 3,264 deaths occurred in this group through Oct. 31, 2001. Mortality from all causes of death and all cancers combined was significantly less than expected when compared with either U.S. or Texas mortality rates as a reference group.

The study did find evidence of increased mortality from breast cancer, but it isn’t known whether this increase is related to workplace or other factors.

Neurologist Merit Cudkowicz, a staff physician and researcher at the MDA/ALS Center at Massachusetts General Hospital in Boston, was among the consultants for this study.

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Protection and Prevention Are Keys to Comfortable Skin

by Christina Medvescek

People with ALS need extra care to keep their skin healthy and comfortable.

First, there are the changes wrought by the disease. Skin becomes extremely fragile whenever there are nutrition or breathing problems. ALS also seems to change the biochemical properties of collagen and elastin, which run in thin fibers through the middle layer of skin called the dermis. As ALS progresses, these fibers become irregular in size and shape. Blood vessels in the dermis also display irregularities and protein deposits.

Then, there are the common itchy or painful ailments brought on by weather and immobility. Caregivers can take several steps to ease skin woes for loved ones with ALS.

Dry, Itchy Skin

Winter air sucks moisture from the skin. To put it back, try these tips:

Put moisture into the air.
Use a humidifier or place a bowl of water on the heating vents.

Avoid hot baths or showers.
Hot water strips the skin of moisture; use lukewarm instead. A 15-minute lukewarm soak saturates the skin, giving it that “prune” look. If a hot soak is a must, use bath oil. It’s not necessary to take a full bath every day; a “parts” cleanup with a sponge usually is sufficient.

Pat dry, don’t rub.
Skin specialists compare rubbing with a towel to rubbing with sandpaper. Patting the skin leaves more moisture behind.

Use heavy, greasy lotions. Immediately after patting, seal in moisture with the greasiest lotion tolerated.

Shave with lotion.
It’s much gentler than foam. When washing, use mild or soap-free cleansers. Avoid deodorant soap.

Wash itchy scalps.
This complaint comes up regularly among ALS patients. Try using cooler water in shampooing, changing hair products, moisturizing the scalp, using anti-itch or antifungal shampoos, and consulting a dermatologist.

Fungal Infections

“It’s ironic that you would develop ‘jock itch’ as a result of being confined to a wheelchair,” says

To combat infections:

Dry off completely.
After gently washing and drying, dry the area further with a hair dryer on the cool setting.

Wear absorbent clothing.
Look for materials that draw moisture away from the surface of the skin.

Use medicated remedies.
Avoid cornstarch, talcum or other nonmedicated powders that can encourage fungus when they get damp.

Try herbal remedies.
Some tried-and-true remedies include applying apple cider vinegar or vitamin E; eating six cloves of fresh garlic or six to nine garlic capsules a day; eating yogurt with live cultures; and taking more B-complex vitamins.

Pressure Sores

Whenever skin is under prolonged pressure, tiny blood vessels are compressed, the supply of oxygen and water is interrupted, and skin starts to die. People who are immobile should have their skin checked thoroughly at least once a day. An area that stays red longer than 15 minutes after pressure is removed is the beginning of a pressure sore. (For dark-colored skin, look for areas that are darker or purplish-blue in color.)

To prevent sores:

Keep pressure off bony areas.
Use padding to protect the most common pressure points (see graphic, above). When making a foam pad, don’t cut out the center (as is done for bunions or corns), as this can further decrease circulation.

In bed, real or synthetic sheepskin pads are effective, as are “egg crate” foam mattress pads.

Comfort Food for Skin Skin is best nourished from the inside out, through healthy foods and eight to 10 glasses of water a day. Because vitamins and minerals work cooperatively to aid skin, start with a daily multivitamin-mineral supplement. From there, follow healthy eating practices: plenty of fresh fruits and vegetables, whole-grain breads and cereals, cooked dried beans and peas, milk, and extra-lean meat or fish. Skin care experts recommend including one linoleic acid-rich food in the daily diet, such as safflower oil, nuts, avocado or seeds. Other essential fatty acids, which help moisturize skin, are found in soybeans, salmon, tuna, shrimp and corn oils. Repairing skin damaged by pressure sores requires extra protein, zinc, and vitamins A, C and K. Vitamin C also builds collagen, which is adversely affected by ALS.

Beware of recliners.
In sitting, the coccyx (tailbone area) takes the brunt of pressure. “As soon as mobility is affected, a pressure relief cushion, like a ROHO or Jay cushion, is a basic necessity,” Huberty says. Ordinary foam or air cushions “just won’t do the job.”

Huberty warns that recliners aren’t meant for people with limited movement and can cause bad pressure sores, especially when people sleep in them at night. Outfit the chair with a proper pressure cushion and keep a close eye out for problems.

Ideally, immobile people need to be turned every two hours. At a bare minimum “one or two position changes a night will greatly reduce the risk of pressure sores,” Huberty says. A programmable automatic turning mattress can regularly shift an occupant’s weight, helping caregivers get some sleep.

Don’t drag the skin.
“Shearing force” is created when one layer of skin moves but the other doesn’t. This happens when a person is dragged or sits up in a hospital bed raised to greater than 30 degrees.

Be as active as possible.
Nancy Schuman, neuromuscular nurse practitioner at the MDA/ALS Center at the University of Washington in Seattle, says the center doesn’t see many pressure sores among ALS patients. She credits education and a philosophy that “encourages patients to be active and not to be in bed, which is probably the worst thing a person can do.”

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Are You Eligible for VA Benefits?

by Bill Greenberg

According to the most recent U.S. census, some 25 million Americans are considered veterans of the U.S. armed forces. For those who’ve served our country in the military, the Veterans Administration offers a variety of programs, ranging from education to home loans and even burial.

For veterans with disabilities, the VA offers assistance with vocational rehabilitation, and assistive technology items such as home modifications and adaptive automobile equipment. It also offers comprehensive health care services, via a nationwide network of VA medical centers in all 50 states and in U.S. territories.

Eligibility

Getting the benefits to which you’re entitled can be a complex process, but the initial question of eligibility is simple: If you served in any branch of the armed forces on active duty and left under honorable conditions, you’re eligible for at least some VA benefits.

If you were discharged before September 1980, it doesn’t matter how long you served. If you were discharged after that, you need to have served a minimum of two years.

Those who served in the National Guard and military reserve units may be eligible as well. If your unit was mobilized by federal order, and you served the full duration of that mobilization, you’re eligible.

All you need in order to establish your eligibility is a copy of your DD-214 U.S. Military Discharge form. If you can’t locate yours, you can request duplicates from the branch in which you served.

Disability Defined — VA Style

Once you’ve established eligibility, the next important question is whether your disability is service-connected. But even if it isn’t service-connected, you could still be eligible for VA assistance.

If you received your diagnosis of ALS while you were on active duty, or if you can prove that you were experiencing symptoms — either while on active duty or for up to two years after your discharge — your ALS will be considered service-connected.

In addition, Congress is considering legislation that would add ALS to the list of diseases “presumed to be service-connected when incurred by veterans of the Persian Gulf War.” (See “VA Studies Show,” and “Congress Considers Gulf War Benefits,” February 2002.)

Even if you can’t establish a service connection to your ALS, you may still fall under the VA’s “catastrophically disabled” classification, making you eligible for some VA benefits.

‘Priority Groups’

Your “priority group” is determined as part of the initial eligibility process and directly affects the level of medical benefits you’re entitled to receive. It’s important to remember that your priority group designation can change as your symptoms increase in severity.

The first three priority groups are for veterans with service-connected disabilities, ranked by the extent of the disability. Group 3 also includes recipients of the Purple Heart and those who were discharged from active duty because of a disability they incurred or aggravated in the line of duty.

Priority groups 4 through 7 don’t require that your disability be service-connected.

Group 4 includes veterans who are considered “catastrophically disabled”— meaning they’re permanently unable to carry out some activities of daily living without assistance.

Group 5 involves low-income requirements. Group 6 includes certain war veterans, but not Gulf War veterans.

Group 7 has an income ceiling and requires recipients to make specified co-payments. You can expect to pay $7 for a month’s worth of any prescription drug, even Rilutek; $15 to see a primary care physician; and $50 to see a specialist, such as a neurologist.

For inpatient hospitalizations, you’ll pay the going Medicare deductible rate plus $10 for the first 90 days you’re in the hospital. Nursing home services are also available for the Medicare deductible plus $5 per day.

Getting Started

To find out what benefits you may be eligible for, you can contact the VA directly. The Web site, www.va.gov, contains hundreds of pages of information about benefits and an online application form. You can call the VA Enrollment Center at (877) 222-VETS (8387), or the main VA benefits information line at (800) 827-1000.

And there’s another way.

Chartered by Congress in 1946, the Paralyzed Veterans of America is a nonprofit organization that features a network of service officers across the country. Most of them work out of regional VA offices or VA medical centers, assisting veterans with mobility disorders.

Henry Bodenbender, the PVA’s director of medical services, says PVA service officers are uniquely suited to help you identify the VA benefits to which you’re entitled — and get them.

“The service officer is an advocate, so he’ll fight for whatever you need because they know the system. Most PVA service officers have been there for years.”

To contact the PVA, call (800) 424-8200 or visit www.pva.org.

“We’re finding a lot of people who just come in to get their medications,” Bodenbender says. “The VA puts out more money for drugs now than they have in the past, because the drugs are so much more expensive.”

If you’re a veteran of the U.S. armed forces, VA benefits represent the richly deserved thanks of a grateful nation. You’ve earned them.

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AAC Device Pops the Question — on the Telethon

by Tara Wood

J ames “Darrick” Bingham has a particular zest for living that even ALS hasn’t been able to contain.

Wendy Buster and James Bingham with Brandon and Andrew

Bingham, 33, is said to almost always have a smile on his face, and he’s known for his tendency to do “doughnuts” (spin in fast, small circles) with his power wheelchair whenever there’s a reason to celebrate.

That’s why, except for an initial shock, no one was truly surprised that the Fremont, Calif., man took a major life step during his Sept. 2 appearance on the KTVU broadcast of the Jerry Lewis MDA Telethon in San Francisco.

Bingham proposed on the air to his longtime sweetheart, Wendy Buster, and he did it with a flair unique to a person with ALS: He spoke the words, but also used his augmentative communication device (the Portable Impact by Enkidu Research) to make sure his message was clearly conveyed.

Buster wasn’t in the studio because she had to work, but she quickly got word of the live, televised proposal. She relayed her enthusiastic acceptance with a telephone message to Bingham’s sister.

The family later called the station to say that Buster had accepted, and the Telethon hosts shared the good news with viewers.

The betrothal brings Bingham and Buster full circle, as they already have two sons and now they’ll become a complete family. After living two hours apart, Buster and the children, Andrew, 11, and Brandon, 8, moved in with Bingham at his mother’s home in November.

They’re looking at a January wedding date.

“She wanted to give me the gift of marriage,” said Bingham, who received an ALS diagnosis in February 2000. Bingham’s speech is affected by the disease, and he uses a power wheelchair for mobility but can take a couple of steps with support.

He first felt symptoms of weakness and had trouble with his hands when he was working as a plumber.

In addition to his strong religious faith that “sustains me,” Bingham credits the Forbes Norris MDA/ALS Research Center in San Francisco with having a large and positive impact on his quality of life.

“Forbes Norris is always one step ahead,” Bingham said.

MDA has helped him with the purchase of several types of assistive equipment, and connected

Carolyn Smith, Bingham’s mom, said her son’s decision to make a new start in life with a marriage proposal in the face of ALS helps illustrate his desire to live life to the fullest.

“That’s what life’s really all about. He was so thrilled to be on that Telethon. He was just ecstatic.

I don’t think he slept the night before — he had this big grin on his face,” Smith said.

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MDA Opens 29th ALS Center in Nashville

T he ALS Division of MDA has opened its 29th MDA/ALS research and clinical center.

The new center is located at the Vanderbilt University Medical Center in Nashville, and directed by neurologists Gerald M. Fenichel and Jane Howard. Those wishing to obtain more information or to schedule an appointment at the center should call MDA’s office in Nashville at (615) 832-5005.

MDA/ALS research and clinical centers provide ALS patients with care from physicians, a nurse coordinator, physical therapist, occupational therapist, speech therapist, and other professionals. In addition to clinical services, the Nashville team also conducts ongoing ALS research.

For a complete list of MDA/ALS centers, go to www.als-mda.org/clinics/alsserv.html.

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