Jerry Lewis MDA Telethon Will Raise Funds for ALS Research and Services

The upcoming Jerry Lewis MDA Telethon will air live for 21 ^1/2 hours starting at 9 p.m. EDT on Sunday, Sept. 3. The Telethon will raise funds to support MDA research seeking treatments and cures for ALS and related neuromuscular diseases. It will also support MDA's network of some 230 hospital-affiliated clinics and 21 MDA/ALS centers.

Lewis, MDA's National Chairman, has hosted the Labor Day weekend broadcast for all of its 35-year history. This year, Lewis hopes to exceed by one dollar the 1999 total in pledges and contributions of $53.1 million.

Lewis will be aided by longtime Telethon anchor Ed McMahon and co-hosts including "Entertainment Tonight" correspondent Jann Carl, comedian Norm Crosby and NBC talk show host Cynthia Garrett. They'll share duties introducing performers and interviewing families affected by neuromuscular disorders.

This year's Telethon will be available for viewing in its entirety on the Internet, at www.mda.org, through streaming media provided by GlobalMedia.com and RealNetworks. The Telethon will be Web-enhanced, meaning it will offer MDA and Telethon-related content on demand and interactive features such as the ability to pose questions online to the MDA Telethon team.

The Telethon is carried on some 200 MDA "Love Network" stations nationwide. Check local listings for time and station in your area.  

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Theory May Brighten Outlook for Treating Late Stages

A recent study challenges conventional views about the way neurons (nerve cells) die, and provides an encouraging outlook for rescuing neurons during advanced stages of ALS.

Many scientists have theorized that, in neurodegenerative diseases like ALS, neurons die because they accumulate damage over time. (In ALS, motor neurons, nerve cells that control voluntary muscle, die, leading to gradual paralysis and other disease symptoms.)

This "cumulative damage" theory predicts that, at later stages of a neurodegenerative disease, neurons will become sicker and die at a steadily increasing rate. In that case, treatments aimed at rescuing the sickly neurons would become less effective with time.

But the new study, published last month in the British scientific journal Nature, disputes the theory, by showing that neurons actually die at a constant rate in many neurodegenerative conditions. Based on their findings, the authors propose that in diseases like ALS, each neuron dies from a single "hit" - a destructive event that occurs at random, sending a neuron down an irrevocable path to death.

If true, this "one-hit" theory means that the ability to save an individual neuron from death doesn't decrease with time. Though someone in the late stages of ALS may have lost many neurons, the surviving neurons could be saved if the single, fatal hit could just be prevented.

The authors of the new study, led by Rod McInnes at the University of Toronto, examined the time course of neuronal death for Huntington's disease and Parkinson's disease in humans, for retinal degeneration in mice, and for isolated neurons treated with toxic levels of the neurotransmitter glutamate.

The authors speculate that their results probably apply to other diseases, including ALS and Alzheimer's disease. Some MDA-funded scientists studying mechanisms of neuronal death in ALS find that idea persuasive.

"The strength of the paper," Robert Friedlander of Harvard Medical School in Boston said, "is that they looked at many different [conditions], and they found very similar things in terms of the rate of [neuronal] death." And McGill University's Heather Durham, in Montreal, agreed that "it's probably true that once [neuronal] death starts in ALS, it follows a similar pattern."

Friedlander questioned some aspects of the study. For instance, the study relies heavily on mathematical models to interpret rates of neuronal death. The "mathematical assumptions that they make really need to be further validated," Friedlander said.

Durham suggested the study oversimplifies neurodegenerative diseases by focusing on neuronal death as the main problem. "In ALS, there may be a lot of [neuron] dysfunction ... in addition to neuron loss," she pointed out.

Despite these shortcomings, Friedlander and Durham agreed that future studies should determine how well the one-hit theory fits the time course of neuronal death in ALS. If it does fit, said Durham, the theory won't necessarily change therapy aimed at curbing neuronal death in ALS, but it "gives us hope that it would work better [even] as the disease progresses."  

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What Everyone With ALS Should Know About Breathing

by Edward Sivak, M.D

Edward Sivak

Edward Sivak is a professor of medicine and a pulmonologist associated with the MDA/ALS Center at the State University of New York in Syracuse.

Q: Does ALS affect breathing?

A: Most people with ALS, sooner or later, have some breathing difficulties. This is because the muscles involved in respiration - moving air into and out of the lungs - gradually weaken. In some people, this occurs early in the disease course, while in others, it occurs later.

Unless the patient has another disease in addition to ALS, there's no direct involvement of the lungs themselves in the beginning. Lungs can be affected later, however, secondarily to the muscle weakness that leads to collapsed air spaces, or atelectasis.

Q: What happens if respiratory muscles become too weak to move enough air in and out of the lungs? Do you suddenly stop breathing?

A: No. Respiratory impairment in ALS is a gradual process. You can slowly lose respiratory function and may not even realize it's happening.

Q: What should I look for?

A: People with ALS, if they're still able to exercise or propel a manual wheelchair, may feel short of breath with exertion.

Shortness of breath when lying down (orthopnea) is common, but it can be hard to recognize for what it is. In a horizontal position, the weakened respiratory muscles are further compromised by gravity. People with weak breathing muscles feel anxious when they lie down.

As respiratory muscles weaken, delivery of oxygen to the lungs and expulsion of carbon dioxide from them become progressively impaired. This is particularly so during sleep, a time when respiratory efforts are normally reduced compared to when we're awake. If oxygen is inadequate during sleep, restlessness, light sleep and frequent waking or near-waking occur.

Here, too, you aren't likely to recognize that the problem is your breathing unless you're schooled in respiratory physiology. You may just feel that you're not sleeping very well, or notice that you're dozing off a lot during the day. You may notice that mental processes are "blunted" or "fuzzy," or that you often have a headache when you wake up. This last is thought to be due to a buildup of carbon dioxide.

Q: Are there medications that can make respiratory problems worse?

A: Yes. Unfortunately, they're often the very ones prescribed for the patient who appears to be anxious and not sleeping well - the person who probably has unrecognized underventilation (hypoventilation).

Almost all medications prescribed for insomnia or anxiety can depress respiration, affecting blood oxygen and carbon dioxide levels in the person with ALS. I can't think of any that are safe, especially if the underlying respiratory problem isn't being treated.

The best way to evaluate insomnia or anxiety in an ALS patient is to look for a respiratory problem first. If that's already being treated and respiratory function is being monitored, then some anti-anxiety medications, if they're needed, can be used - with caution.

Q: What should I do if I suspect I have a breathing problem?

A: First, of course, see your doctor. Many tests can be done, ranging from the very simple to the very complicated, to check the status of your respiratory efforts.

One simple test has you blow into a machine called a spirometer that measures how much air you can move in and out of your lungs. This measurement should always be taken in both the sitting and supine (back-lying) positions to determine the strength of the diaphragm muscle.

Another test uses an electrode placed on the finger or earlobe to measure the amount of oxygen in the blood. The device is called an oximeter.

Your doctor may also want to measure the concentration of dissolved oxygen and carbon dioxide in your blood, a test known as blood gas levels.

These tests may show normal or nearly normal values when taken during the day, but if there are symptoms of inadequate respiration, an overnight test is usually warranted. In my practice, I have a low threshold for ordering what's called a sleep study or polysomnography study. The patient is asked to stay overnight in a sleep lab, where electronic sensors monitor his or her brain waves, heart rate, respiratory movements (the rise and fall of the chest and abdomen), and blood levels of oxygen and sometimes carbon dioxide.

It may seem like a lot of trouble, but a sleep study is worthwhile because it picks up low oxygen levels or retention of carbon dioxide during the deeper phases of sleep, which is when they first occur in ALS. A sleep study can also reveal that deep sleep levels aren't being achieved.

Q: If I have low oxygen or high carbon dioxide, what can be done about it?

A: At our clinic, we offer the patient what's known as noninvasive positive pressure ventilation. NIPPV delivers either a set volume or a set pressure of air (not oxygen, just room air) into the lungs via a mechanical device. There are a number of these devices on the market now, and each one has some advantages and disadvantages. The details are best addressed on an individual basis with your physician and respiratory therapist.

Noninvasive Ventilation

The word "noninvasive" means that the air is delivered by a mask over the nose, mouth or both. "Invasive" ventilation means that air is delivered through a surgical hole in the throat, called a tracheostomy (or trach).

NIPPV offers the person with failing respiratory muscles a kind of splint for these muscles, propping up their function with a little extra power. NIPPV doesn't have to be used all the time. It's usually needed during the sleeping hours at first and then extended into daytime use, sometimes for only short periods at first and then for longer periods.

Q: Is NIPPV for everyone?

A: No. Each case is individual, and not everyone tolerates this form of ventilation. People without teeth, with short jaws, or with weakness of the mouth and throat muscles may have special problems using a mask.

Q: Is NIPPV a permanent solution?

A: Unfortunately, no. Eventually, in ALS, the time usually comes when the respiratory muscles have deteriorated to such an extent that breathing can no longer be maintained with NIPPV. At this point, the person has to decide whether or not to move to invasive ventilation. Trach ventilation delivers air more directly to the lungs, bypassing the mouth and throat and any leaks that may occur along the way from the machine to the trachea (windpipe). Therefore, it's usually more effective for end-stage respiratory muscle degeneration than is NIPPV.

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With Faith and Rilutek, 'Miss Mary' Keeps Going

Mary Boyter

Tenacious like a bulldog. The glue that holds things together. The epitome of hope and faith.

An angel.

That's how a friend describes Mary Boyter, a Shreveport, La., woman who received a diagnosis of ALS in 1994.

But Boyter simply describes herself as somebody who has been very blessed and is charged with a mission: to help others with the same diagnosis.

Boyter's ALS symptoms have progressed slowly and allowed her to remain involved in her church, community and family. So much so, in fact, that she was recently given the 2000 MDA Personal Achievement Award for Louisiana for her efforts.

"God is doing this for me so that I can encourage others that it can be done," said Boyter, who is affectionately known by most as "Miss Mary."

It's been nine years since Boyter, 66, began to experience symptoms that interrupted her usually active lifestyle.

"I've had the symptoms since 1991. Since then, in '91, '92, '93 and '94 I fell and broke a bone every year. I was a jogger, I played tennis, I bowled, and I thought I was getting clumsy," she said. A double fracture in her ankle and changes in her speech forced her to seek answers from medical professionals.

Cookies & Coping

Yet Boyter hasn't stopped moving, helping and praying since she received her diagnosis. She makes frequent visits to homes of people living with ALS, as well as nursing home residents and people who are ill. Her visits can include kind words, tips for coping or homemade cookies she brings to share.

ALS causes her difficulty with climbing stairs, and her sweet Southern drawl has also been slowed. But that doesn't keep her from taking speaking engagements around her community as part of her church ministry or on behalf of MDA.

For MDA, Boyter volunteers for many fund-raising events and shares valuable tips for living and coping at MDA support groups.

This Labor Day weekend she'll again be volunteering at the Jerry Lewis MDA Telethon, doing "whatever they ask me to do. I'm everywhere from the kitchen to the desk," she said. She also works in the Telethon office on the Friday before the broadcast.

Boyter also takes care of her mother, who lives in a nearby nursing home, and keeps in touch with her three adult children who live close by in Louisiana and Texas.

She works four days a week at the Caddo Parish Sheriff's office. There, at the jail's visitation desk, her duties include computer tasks, answering phones, entering people in and work on reports and files. Her past assignments had her working in identification, organized crime, and court divisions.

Conferring Credit

How does she stay strong in the face of ALS?

"God just gives me the energy and the motivation," she said. "When I speak to people or different groups I always tell them God gets credit first, then the MDA, and then the medication, in that order."

Her medications include Rilutek and amantadine, a drug commonly prescribed for people with Parkinson's, and daily vitamins.

The best prescription, though, is a positive attitude, Boyter said.

When she speaks to others coping with ALS, she advises being flexible to adjust to a changing disability, to avoid bitterness and to stay active as long as possible.

"You have to be motivated," Boyter said. "If you sit and dwell on the things that could be or couldn't be, you will never make it."

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Chats Under Way, New Groups Sought

MDAchat is thriving, and you can still get in on the action.

The chats - specialized discussion groups via MDA's Web site - are an opportunity to share points of view, experiences and interests.

Current MDAchat groups include the Caregiving Connection, held the third Tuesday of each month; Kick Back, a potpourri session about living with a wheelchair and more, the first Tuesday of each month; and Positive Thinking, about maintaining a good attitude, held every Thursday.

Chat organizers are seeking people to form MDAchat leadership host teams to assist ongoing groups or cover specific topics. New groups focused on ALS are invited.

To get involved, you can volunteer to set up a team, ask two or three others to join, and complete and submit a new group application. Applications, schedules and transcripts of previous chats are available on MDA's Web site at http://www.mda.org/chat/.

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21st MDA/ALS Center Designated

MDA has inaugurated a new center in the war on ALS, the MDA/ALS Center at Mount Sinai Hospital and Medical Center in New York. The new center is the 21st such multidisciplinary facility in the United States maintained by MDA to serve people with ALS.

The center will be co-directed by Charles Warren Olanow and Dale J. Lange. Olanow is chairman of the Department of Neurology at Mount Sinai and chief of its neurology service. Lange is associate professor of neurology at Mount Sinai and director of its division of neuromuscular disease.

The new center can be contacted by calling (212) 241-8674 or e-mailing warren.olanow@mssm.edu.

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ALS Exercise Video Available

"Wellness in Motion," a 45-minute video, presents a gentle exercise program for people who are in the early to middle stages of ALS. Its creator, physical therapist Betsy Lindsey, notes that the video should serve as an addition to daily activities and those prescribed by a doctor or therapist.

Lindsey is associated with the MDA clinic at University Medical Center in Tucson, Ariz. She's a member of the National Neuromuscular Nursing Advisory Board and is directing a research project on the effects of exercise in ALS.

"Wellness in Motion" was produced by the University of Arizona Biomedical Communications Department and was funded in part by Rhone-Poulenc Rorer, now Aventis Pharma, makers of Rilutek.

The cost of the video is $6. Checks, made payable to the University of Arizona, should be mailed to: UA Health Sciences Center, Department of Neurology, 1501 N. Campbell Ave., Tucson, AZ 85724-5023, Attention: Betsy Lindsey, PT.

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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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