JERRY LEWIS RESPONDS TO '60 MINUTES'

On Nov. 22, the CBS program "60 Minutes" aired a segment about Dr. Jack Kevorkian, including a tape of his administering a fatal injection to a man with ALS. In response to the program and subsequent media coverage of it, MDA National Chairman Jerry Lewis sent the following letter to the executive producer of "60 Minutes."

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NEW TRIAL TO INJECT BDNF INTO SPINAL CORD FLUID

The pharmaceutical companies Amgen and Regeneron have announced the opening of a new trial of BDNF for ALS.

BDNF -- brain-derived neurotrophic factor -- was previously tested and found ineffective in the disorder. However, the new trial will administer the experimental drug directly into the fluid surrounding the spinal cord, in contrast to the previous trial, in which it was given as subcutaneous (under the skin) injections. The method ("intrathecal" delivery) is an attempt to improve the absorption of BDNF by the nerve cells in the brain and spinal cord ("motor neurons") that are affected in ALS.

The experimental drug is based on a natural substance that improves survival of nerve cells subjected to adverse conditions in laboratory experiments. The trial will be conducted at 16 centers in the United States and Europe, will last 18 months and will be placebo-controlled (meaning one group will be given an inert substance and be compared with the group receiving BDNF).

For information about the trial, call Amgen at (800) 772-6436 or check Amgen's Web site, www.amgen.com.

The principal U.S. investigators for the trial are:

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STUDY SHOWS ADULTS CAN MAKE NEW BRAIN CELLS

Recent findings challenge the long-standing idea that human nerve cells (neurons), unlike most other cells of the body, are incapable of regeneration after infancy and thus are particularly vulnerable to degenerative disorders.

A new study reports the discovery that at least some new nerve cells may be produced in very low numbers throughout the normal human life span. New neurons have been found in a region of the brain involved in learning and memory called the "hippocampus" and in another region beneath the lining of the brain cavities.

Researchers Peter Eriksson of Göteborg University in Sweden and Fred Gage of the Salk Institute in La Jolla, Calif., reported in the November issue of Nature Medicine the discovery of newly born neurons in the brains of five adults ages 57 to 72.

The researchers were able to identify the newly born neurons in people who had taken a particular chemical involved in cancer diagnosis. The chemical, which measures how fast cells are growing, is incorporated only into the genetic material of dividing cells. Cell division is the mechanism by which the body creates new cells.

The researchers found the chemical in some brain cells, which was convincing evidence that those were newly produced cells.

"The good news," says Daniel Drachman, codirector of the MDA/ALS Center at Johns Hopkins University School of Medicine, "is that this work demonstrates that neurons are in fact produced throughout life in adult humans. The concept that you get your complement of neurons at birth and then it's downhill all the way is no longer so clear."

The confirmation of adult brain cell regeneration in humans may eventually allow researchers to understand how to stimulate neural cell division for the treatment of neurodegenerative diseases or traumatic brain injuries.

In addition to long-term benefits, this finding has spurred speculation on the therapeutic possibilities of transplanting healthy neurons capable of cell division into the brains of people with neurodegenerative disorders such as Parkinson's or Huntington's diseases.

Drachman offers a note of caution about the transplant idea for ALS. Although the idea that such neurons could be transplanted for therapeutic purposes is an interesting one, he says, it may have no bearing on the treatment of ALS for a long time.

"Researchers did not look for neuron birth in the region of the brain involved in muscular control or in the spinal cord," Drachman points out, "both areas that would be of great importance for people with ALS. The phenomenon of adult neural cell division may be restricted to the hippocampus."

Drachman also points out that it is unclear whether or not the newly born neurons form functional connections within the brain.

The real significance of this finding likely lies in the potential for replacing damaged cells.

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PEOPLE WITH ALS SHARE STORIES IN NEW BOOK

The personal accounts of people affected by ALS are always gripping. David Feigenbaum of Virginia Beach, Va., has compiled over 30 such stories, mostly gathered from the Internet, in Journeys With ALS: Personal Tales of Courage and Coping With Lou Gehrig's Disease.

The profiles in this book add up to a microcosm of those affected by ALS -- a range of ages, both sexes, all backgrounds. Most of the accounts were written by the person with ALS, a few by family members. Some are long-term survivors, while others progressed quickly.

Each story is exemplary in some way, depicting victories over fear, disability and time. The authors have in common the shared experience of shock at their diagnoses and prognoses, followed by a process of learning to accept and manage the effects of ALS. Feigenbaum's subtitle is most appropriate.

Feigenbaum himself, a father in his 50s looking forward to enjoying the "empty nest" years and fruits of a flourishing rare coin business with his wife, shares his story of ALS, too. The stories are quite human and could offer others with ALS both emotional encouragement and practical advice, about drug trials, eating, communicating, travel and other adjustments. The book could also be helpful to family members who are seeking a way to cope with the disorder.

The book is available from DLRC Press, P.O. Box 61661, Virginia Beach, VA 23466; (757) 473-1130; info@davidlawence.com; for $19.95 plus $5 shipping and handling.

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PENICILLAMINE NOT HELPFUL SO FAR IN GENETIC ALS

Studies in mice with the SOD1-related form of genetic ALS have suggested that the drug penicillamine, which sticks to a copper molecule that forms part of the SOD1 protein, might help people with this form of the disease. Mice with this genetic ALS survived slightly longer when taking this drug.

However, penicillamine so far hasn't shown this kind of beneficial effect in six people with SOD1-related ALS at Massachusetts General Hospital in Boston, say neurologists Merit Cudkowicz and Robert Brown, MDA research grantees at that institution. ALS progression continued in all the patients studied, Cudkowicz said.

The doctors caution that it's hard to conclude anything definite from such a small study, especially since there was no control group (taking an inert substance) with which to compare the treated group.

"One cannot draw any firm conclusions about this treatment without a large, controlled study," Brown noted. "Indeed, on the basis of an anecdotal trial of a small number of patients, one might have incorrectly concluded that riluzole was of no benefit in ALS." (Riluzole has a modest effect on survival in ALS and is marketed as a treatment for the disease.).

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WHEELCHAIR SELECTION: An ALS Patient Guide

by Marilyn Trail and Peggy Ingels

Selecting a wheelchair can be as baffling as buying a new car. Options vary for features, models, styles, accessories and, of course, cost.

Because ALS causes progressive muscle weakness over months or even years, seating needs often change. A patient with mild leg weakness may need a lightweight chair with a low back to take to the mall or the park; whereas the patient with more advanced weakness, including the upper body, requires more supportive features.

We're offering some guidelines for wheelchair selection based on our experience in working with ALS patients in the MDA/ALS Center in Houston.

You may ask, "How do I know if I need a wheelchair?" Questions to consider in helping to make this decision might include:

• Do you have frequent falls?
• Do you fatigue if you walk more than 350 feet?
• Are you reluctant to attend social outings because your legs "give out"?
• When you go to discount stores or the grocery store, do you use the store's scooter?

A lightweight, collapsible wheelchair is useful for frequent outings.

If you answer "yes" to any of the above, you may want to investigate the types of wheelchairs on the market, their features and their costs. Before purchasing a wheelchair, you need to consult with your therapist or physician.

Questions to consider when selecting a wheelchair include:

• Will you use the chair daily, or only for outings?
• Will the chair be your primary source of transportation?
• For how many hours a day will you sit in the chair?
• Are you still in the work force?
• Do you have the arm strength to propel a manual chair?
• Would purchasing two chairs during the course of your illness be an option?
• Are you alone for long periods of time during the day?

The Low-Back Manual Wheelchair

If you have good upper extremity muscle strength, but tire when walking long distances, you may want to consider renting a collapsible, lightweight, low-back wheelchair with removable, elevating leg rests and removable desk arms. This light chair can easily be put into the back of a car to take to the mall, church or theater. With this style of chair, you can push the chair (like pushing a grocery cart), wheel yourself until you feel tired or have a friend push you.

The High-Back Manual Wheelchair

The Metro by Everest & Jennings is an example of a foldable chair weighing only about 30 pounds.

If you have weakness in your arms, shoulders and/or neck, as well as in your legs, we recommend a high-back wheelchair to help provide maximum support and proper body alignment. Additional features should include a neck support and reclining high back to balance the head in the correct position. We also recommend arm troughs or a lap tray for comfortable positioning of the shoulders, arms and hands.

A firm back cushion or rigid insert is necessary to support the back and shoulders and to prevent a forward, rounded posture. A seat cushion, removable elevating leg rests and an anti-tilt device are also essential. This type of seating system also helps prevent shoulder problems that can result from muscle weakness. If you plan to purchase only one wheelchair, this is the type we strongly recommend.

For safety, anti-tilt devices on the high-back manual wheelchair are essential.

The High-Back Motorized Wheelchair

Questions to ask yourself before considering a power chair include:

• Do you work or go to school?
• Are you alone for long time periods?
• Do you have the financial resources for this purchase?

Everest & Jennings' Lancer 2000 with LeBac is a power chair incorporating adjustable head and leg supports among other features.

With the advancement of electronic technology, multiple options (such as chin and eyebrow switches) are now available to propel and position a power chair. If you decide to purchase a motorized chair, we recommend that it be a high-back chair with reclining or tilt features. It should also have a headrest, removable elevating leg rests and removable armrests.

For optimum posture and comfort, firm back and seat cushions are necessary.

Other things to consider include the weight of the chair. An adapted van or lift system is needed if you are going to use the chair outside the home.

Look for a chair that offers options for expanded features that can be added as needs change. Motorized chairs, like automobiles, come with many "bells and whistles." The more options you choose, the higher the price.

We recommend that the high-back motorized chair have tilt or reclining features.

The Scooter Dilemma

The hybrid scooter has a covered base with three to six wheels. The scooter is lighter in weight than a high-back power wheelchair and tends to be more maneuverable. It can be disassembled and placed in the back of a car. The steering mechanisms are on the armrests (we don't recommend a scooter with handlebar steering).

Many ALS patients say they want a scooter rather than a wheelchair because the word "scooter" doesn't have as strong a connotation of disability.

However, wheelchairs are costly and you'll want a mode of transportation that can meet your long-term needs. A conventional scooter won't do this, especially if you have significant upper extremity and trunk weakness. The chair portion offers less support than high-back wheelchairs, thus rendering it obsolete if trunk and upper body weakness develops.

Since ALS is a progressive disease, and seating systems are costly, we encourage you to plan ahead when selecting a wheelchair. Work with your therapist, physician and wheelchair vendor to select the seating system that best meets your needs.

This story first appeared on the MDA/ALS Center Web site at www.bcm.tmc.edu/neurol/struct/als/als1.html. The site is maintained by staff of the Ronny and Linda Finger MDA/ALS Center at Baylor College of Medicine in Houston. More information about wheelchair types and options appears in MDA's bimonthly magazine, Quest.

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DESIGNER WHO HAD ALS HONORED IN N.Y. EXHIBIT

Marc Harrison, who taught at the Rhode Island School of Design (RISD) for almost 40 years before losing his life to ALS on Sept. 22 at age 62, is one of a number of design pioneers whose work is being featured in a major exhibition at the Cooper-Hewitt National Design Museum in New York City.

Unlimited by Design, the first museum exhibition of universally designed residential interiors, opened Nov. 17 at the Manhattan museum, a branch of the Smithsonian Institution. Universal design is a concept that promotes accessibility and ease of use as standards that can benefit everyone.

The Maxi Kitchen from RISD. Photo by Mark Johnston

A highlight of the exhibition is the Universal Design Kitchen created by RISD students under Harrison's direction. Harrison was a senior industrial design professor at RISD. Notable chef Julia Child also added expertise to the project.

The kitchen was conceived to allow customization for different users while minimizing movement and effort and creating "comfort zones" that place cooking tools within easy reach. RISD students analyzed every movement involved in preparing a spaghetti dinner and incorporated the insight they gained into the design.

"We're unaware of the excessive and repeated steps we go through every day to complete simple tasks like making breakfast or lunch," Harrison once said.

According to members of the design team, Harrison's ALS, which was diagnosed in 1996, had a "profound impact" on the project. Harrison used a power wheelchair.

However, Harrison was interested in universal design long before his own mobility became limited due to ALS. He attributed his lifelong concern with the needs of people with disabilities to a childhood accident involving a traumatic brain injury. Products he designed have been in use for decades and range from turnstiles to supercomputers.

Years ago he contributed to the development of the Cuisinart food processor, originally designed to accommodate people with arthritis and visual impairment. In accordance with the notion of universal design, the processor went on to achieve broader success with the nondisabled population.

The inclusion of his students' kitchen design in the Cooper-Hewitt exhibit is the latest honor associated with Harrison's name. Over the years, he received a number of awards for excellence in design education.

Two months before his death, Harrison, who was a resident of Portsmouth, was named recipient of MDA's 1998 Personal Achievement Award for Rhode Island. The award, which recognizes the accomplishments and community service of people with ALS and other neuromuscular diseases, was presented to Harrison in the Universal Design Kitchen at RISD.

MDA Senior Vice President and Executive Director Robert Ross hailed Harrison as a leader whose achievements had a major, positive impact on the lives of the disabled and nondisabled alike.

Harrison had been profiled on the 1997 local broadcast of the Jerry Lewis MDA Telethon aired on WLNE-TV.

The Cooper-Hewitt Museum, at the corner of 91st Street and Fifth Avenue in New York, is fully accessible and is closed Mondays and federal holidays. The exhibit will continue through March 21. General admission is $5.

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YOUR LETTERS MATTER

NOTE FROM MDA SENIOR VICE PRESIDENT AND EXECUTIVE DIRECTOR ROBERT ROSS:

The following letter was received at MDA National Headquarters in Tucson, Ariz., accompanied by a generous donation to support MDA research and services.

It means a great deal to MDA when people like the Alperts let us know our efforts in the war on ALS are appreciated. Kind words like the ones that follow make us want to redouble our efforts and intensify our long-standing commitment to do everything possible to help families fighting ALS.

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ALS -- NOT CURABLE, BUT TREATABLE

by Edward Oppenheimer

Many diseases can, of course, cause death if not treated. High spinal cord injury, a serious infection, an appendicitis, etc., can kill when neglected. When treated, these are still serious conditions, but usually life can continue and death can be prevented.

It's true that the average length of life of people with ALS today is about four years. However, this reflects the fact that people with ALS often don't have optimal treatment and resources.

If ALS is to be fully treated, people with the disease should have:

• adequate information about the treatments available
• access to health professionals who are interested in and experienced with all the available options in the care of ALS
• the encouragement to go forward
• the needed resources to go forward
• the desire to use available care

When the patient lacks the desire to use available care, after having been fully informed and provided with a positive approach from professionals such as doctors, nurses, physical and occupational therapists, social workers and others, not proceeding is acceptable. However, when any of the needs cited are insufficiently filled, not treating ALS to the fullest is regrettable. Ideally, this situation shouldn't exist.

What is "fully treated" ALS? The answer is my "wish list" for all people with ALS:

1. A positive and experienced team approach to assist patients and their caregivers in solving any of the problems that typically occur

This means a patient should have access to a team of adept and enthusiastic health professionals who are available even when the patient isn't taking part in a research protocol. The team should include professionals who can make home visits and coordinate care as needed.

2. Very good nutrition to maintain appropriate weight

At some point this may include using a gastrostomy tube (feeding tube) if swallowing problems occur.

3. Regular social interaction with friends and the community

It's vital that the person with ALS get out of the house by using mobility assistance, stay engaged in living and maintain ways to communicate even if speech becomes difficult. Good alternative and augmentative communication devices are essential.

4. Personal assistance to cover each 24 hours as needed, without placing an undue burden on family members

5. Vigorous treatment for infections, particularly respiratory infections

This can often be provided at home with appropriate antibiotics when needed. Medication can be given by mouth, via a feeding tube or even intravenously, depending on the person's condition.

6. Monitoring of breathing capacity so noninvasive assisted ventilation can be available when capacity is decreased and related symptoms are present

An ALS patient can receive mask-delivered or other noninvasive ventilation as long as this works, and then shift to tracheostomy (invasive ventilation, via a tube into the trachea) if that becomes necessary. Equally important are good attention to effective coughing and clearing of secretions, and prevention of aspiration (inhaling particles, such as food, into the lungs). The decision to stop using a ventilator at any time, or to use only noninvasive ventilation and not go on to tracheostomy ventilation, should be made by the patient.

7. Access to ALS research centers and protocols when desired, and access to excellent ALS care even when there is no desire to participate in research

8. Very good supportive care

9. Good symptom-relieving care when major aspects of treatment are no longer desired or appropriate

When most of these aspects of care are available to people with ALS, they can often continue living in a productive way and avoid life-threatening complications related to ALS most of the time. ALS motor neuron impairment may become severe, but life and spirit and social ties can continue.

Ideally, people with ALS should have choices. They should know that there are options that will allow living to continue even if the resources needed are considerable.

Edward Oppenheimer of the Southern California Permanente Medical Group is an associate clinical professor of medicine at the University of California at Los Angeles and is a pulmonary consultant to the American Academy of Neurology's task force on clinical practice guidelines for ALS. He has also been a consultant to MDA.

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HELP FIGHT ALS TODAY AND TOMORROW

Many people who know the devastating effects of ALS are providing lasting support for MDA's battle against the disease. Through your will, you can designate a gift to MDA earmarked to support ALS research or services.

Bequests to MDA can be made with cash, securities, real estate, or other property. You can bequeath a percentage of the entire estate to MDA or make a bequest of the residue, donating property remaining after all bequests to family and others have been satisfied. You may also name a memorial gift in honor of a family or individual.

To give what remains of your estate after other bequests have been satisfied, just include the following language in your will:

"I give, devise and bequeath all (or a specified fraction of) the rest, residue and remainder of my estate, whether real or personal, of every kind and description, and wherever situated, to Muscular Dystrophy Association Inc., a New York not-for-profit corporation having its principal office at 3300 East Sunrise Drive, Tucson, Arizona, 85718-3208, for its program of research and services related to amyotrophic lateral sclerosis."

To give a dollar amount or percentage of your estate:

"I give, devise and bequeath the sum of $________ (or ________ percent of my estate) to Muscular Dystrophy Association Inc., a New York not-for-profit corporation having its principal office at 3300 East Sunrise Drive, Tucson, Arizona, 85718-3208, for its program of research and services related to amyotrophic lateral sclerosis."

Your attorney or financial adviser can help you work out the details of a bequest to MDA's ALS program. For more information, call MDA's Planned Giving Department at (800) 572-1717.

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NFL STAR REMEMBERED

Former teammates of NFL defensive lineman Glenn Montgomery, who died in late June at the age of 31 from ALS, are keeping his memory alive by sponsoring a golf tournament in May with the Houston Gulf Coast MDA office.

Montgomery, a graduate of the University of Houston, was with the Houston Oilers from 1989 until he was traded to the Seattle Seahawks in 1996. He retired from the NFL in the fall of 1997 after receiving a diagnosis of ALS and devoted much of his time to public speaking in order to help others with ALS and raise public awareness of the disease.

Montgomery and Stanley Appel, director of the Ronny & Linda Finger MDA/ALS Center at the Baylor College of Medicine, were featured on a TNT postgame show in the fall of 1997. They discussed the debilitating effects of ALS and possible treatments. During the powerful segment, Montgomery announced his plans to advocate for families affected by the disorder and to encourage other football players to help with the fight against ALS.

"Glenn in the last several months did a spectacular job of increasing awareness of the need for research," Appel told the Houston Chronicle. "He was a very courageous guy, a man whose drive and motivation and will to succeed were unparalleled."

Many of Montgomery's former teammates say, despite the onset of his illness, his competitive spirit never left him. He fought ALS with the same ferocity he demonstrated during his eight-year NFL career.

In the months before his death, Montgomery had been working with a Houston television reporter on a series about the effects of the disease. The Houston Muscle Team Dinner, held in August shortly after Montgomery died, was dedicated to the New Orleans native. The event attracted more than 450 people and raised $107,000 to benefit MDA programs throughout the Texas Gulf Coast area.

Montgomery's teammates plan to hold the golf tournament to honor the NFL player they will remember as "the fighter."

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The Association welcomes gifts for ALS research honoring significant occasions of achievement. These gifts may be made in tribute to special people or to mark such events as anniversaries, birthdays, weddings, graduations or retirements.

THE ALS NEWSLETTER
Muscular Dystrophy Association
National Headquarters
3300 East Sunrise Drive
Tucson, Arizona 85718-3208

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