FAQ about ALS (Lou Gehrigs Disease)
These three are our "Men in Black". They make sure all questions are answered. My son Allan, nephew Brian and son David.
ALS/MND FREQUENTLY ASKED QUESTIONS
This “ALS/MND Frequently Asked Questions” is posted here at sci.med.diseases.als “every now and then”. The purpose of this FAQ is to help spread information about the illness, to promote understanding and interest. These are also the aims of the many ALS/MND Societies around the world, among them ALS Canada, who were a driving force in the establishment of this newsgroup.
This existence of this FAQ is due to the hard work of Mike Beal, who identified the need for a FAQ for this newsgroup, undertook the big task of assembling it, regularly posting it, and maintaining it. The material was compiled from many sources, including and especially other “on-line PALS” (“persons with...”). I copy this material from Live&letdie who has taken up this task to maintaun these questions for you.
It is our hope that the FAQ will encourage participation at this newsgroup; so, please post questions, comments, criticisms, and suggestions for changes to the group for discussion.
The Frequently Asked Questions:
WHAT IS ALS/MND? WHAT ARE THE SYMPTOMS OF ALS/MND? WHAT IS THE AVERAGE LIFE EXPECTANCY? HOW COMMON IS ALS/MND? HOW IS ALS DIAGNOSED? WHO IS LIABLE TO GET ALS/MND? WHAT DOES “AMYOTROPHIC LATERAL SCLEROSIS” MEAN? ARE THERE DIFFERENT TYPES OF ALS/MND? WHEN WAS ALS FIRST DISCOVERED? WHAT CAUSES ALS/MND? CAN YOU “CATCH” ALS/MND? WHAT ABOUT ENVIRONMENTAL CAUSES? IS THERE HOPE FOR PEOPLE WITH ALS/MND? WHAT CAN I DO TO HELP? WHERE CAN I LEARN MORE?
The Short Answers (details follow):
WHAT IS ALS/MND? Amyotrophic Lateral Sclerosis, aka Motor Neuron Disease, is a fatal, incurable neuromuscular illness.
WHAT ARE THE SYMPTOMS OF ALS/MND? Weakening and wasting of muscles.
WHAT IS THE AVERAGE LIFE EXPECTANCY? Two to five years from diagnosis.
HOW COMMON IS ALS/MND? About 7 in 100,000 population will have ALS/MND at any given time.
HOW IS ALS/MND DIAGNOSED? No definitive diagnosis: patients fitting a profile are tested to eliminate other possibilities.
WHO IS LIABLE TO GET ALS? Anyone, but mainly over 40’s.
WHAT DOES “AMYOTROPHIC LATERAL SCLEROSIS” MEAN? The words come from the Greek; and describe the muscle-wasting, and scarring of the spinal cord.
WHY IS IT ALSO KNOWN AS “LOU GEHRIG’S DISEASE”? After perhaps its best known victim, a great 1930’s baseball player.
ARE THERE DIFFERENT TYPES OF ALS/MND? Yes: Guamanian, a localized form Familial, an inherited (relatively low incidence) form Sporadic, all the rest.
WHEN WAS ALS/MND FIRST DISCOVERED? In 1869.
WHAT CAUSES ALS/MND? We don’t know.
CAN YOU “CATCH” ALS/MND? No.
WHAT ABOUT ENVIRONMENTAL CAUSES? Possibly (probably?)
IS THERE HOPE FOR PEOPLE WITH ALS/MND? Yes. Recent work has produced drugs which seem to slow the progression.
WHAT CAN I DO TO HELP? Volunteer. Spread the word. Give money, for patient support and for research.
WHERE CAN I LEARN MORE? On-line; hundreds of sites, some listed here. And from ALS/MND societies and support groups all over the world, listed here.
And, The Details:
WHAT IS ALS/MND? ALS/MND—Amyotrophic Lateral Sclerosis, aka Motor Neuron Disease—is a rapidly progressive, fatal neuromuscular disease. It attacks motor neurons in the spinal cord and lower brain, which transmit signals from the brain to the voluntary muscles throughout the body. When motor neurons die as a result of ALS, the ability of the brain to control muscle movement is lost. When muscles fail to receive messages, they weaken, atrophy, and die. There is no known cure.
ALS/MND is also known as Lou Gehrigs Disease, and in French, maladie de Charcot.
WHAT ARE THE SYMPTOMS OF ALS/MND? The groups of muscles affected and the order in which they are affected varies from one person to another. Early symptoms usually include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and uncontrollable periods of laughing or crying. The hands and feet may be affected first, causing difficulty in walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes.
About 25% of patients have bulbar (throat) onset, which means that voice and swallowing are first affected. About 50% have arm onset, and 25% leg onset.
The disease frequently takes its toll before being positively diagnosed: many patients are significantly debilitated before learning that they have ALS/MND. Muscle wasting gradually spreads to the muscles of the trunk of the body, and the disease eventually affects swallowing, chewing and breathing. Complete paralysis eventually results, usually occurring within two to five years of diagnosis. When the diaphragm is attacked, the patient is unable to breathe for him/herself, and faces permanent ventilator support in order to survive. ALS/MND attacks only ‘motor’ neurons. Sight, touch, hearing, taste, smell and muscles of the eyes and bladder are generally not affected. Sexual function and drive are not affected. The mind is not affected, and remains sharp despite the progressive degenerating condition of the body. Dennis Kaye, a Canadian ALS victim, wrote an inspirational book on his struggle: “Laugh? I Thought I’d Die”. He said of his like: “With keen mind and open eyes We watch ourselves die.”
WHAT IS THE AVERAGE LIFE EXPECTANCY? This is between two and five years for the newly diagnosed person. Improved medical care is resulting in longer and more productive lives for people with ALS/MND. Twenty percent will live more than five years and up to 10% will survive more than ten years.
HOW COMMON IS ALS/MND? Once thought rare, ALS/MND is now fairly common.
In terms of INCIDENCE (how often does it strike), a normal population produces about 2 new cases per 100,000 every year. (A 1995 Dalhousie University Study found in that there was a 2.03 per 100,000 population incidence in Nova Scotia.)
(As ALS/MND is terminal and incurable, death rates are very close to incidence rates.)
According to the United States National Institute of Health, some 5,000 people in the U.S. are newly diagnosed with ALS each year. (That’s about 13 new cases every day!)
A recent study conducted by the V.A (Veterans Administration) and major research centers in the U.S. have shown a likelyhood that by serving in the military, you are 5x more likely to contract ALS.
Many scientists believe the incidence of ALS/MND is increasing, though some feel this may be due to improved understanding of the illness and better diagnostic techniques. In terms of PREVALENCE (how many are affected at any one time), ALS/MND affects about seven to eight people out of every 100,000.
It is estimated that as many as 30,000 Americans have the disease at any given time, and that some 300,000 Americans who are alive and apparently well today will die with ALS. In Canada, from a population of about 30 million, two to three Canadians die every day of ALS/MND. Over 2,500 Canadians currently have ALS. Some 20,000 to 25,000 Canadians who are alive and apparently in good health will die of ALS/MND.
HOW IS ALS/MND DIAGNOSED? At present there is no definitive means of diagnosis of ALS/MND. Most diagnoses are made by eliminating all other possibilities—ailments whose symptoms resemble those of ALS/MND. Neurologists use a number of clinical tests to establish a profile, including blood testing, EMG, MRI, etc.
WHO IS LIABLE TO GET ALS/MND? Anyone can get ALS/MND. Most who develop ALS/MND are between the ages of 40 and 70. There are, however, cases of the disease attacking persons in their twenties and thirties. Generally though, ALS/MND occurs in greater percentages as men and women grow older.
ALS/MND strikes men slightly more frequently than women. About one in 800 adult men, and one in 1,200 adult women die of ALS/MND.
ALS/MND occurs throughout the world with no racial, ethnic or socioeconomic boundaries.
90% of ALS/MND cases are people with no family history of the disease. Ten percent of the cases are classified as familial or inherited ALS/MND.
WHAT DOES “AMYOTROPHIC LATERAL SCLEROSIS” MEAN? “A-myo-trophic” comes from the Greek language. “A” means no or negative. “Myo” refers to muscle, and “Trophic” means nourishment---“No muscle nourishment.” When a muscle has no nourishment, it “atrophies” or wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that nourish the muscles are located. As this area degenerates it leads to scarring or hardening—“sclerosis”—in the region.
WHY IS IT ALSO KNOWN AS “LOU GEHRIG’S DISEASE”? Lou Gehrig, “The Iron Horse”, was a baseball superstar in the late 20’s and 30’s, one of the “Golden Ages” of baseball. After 14 glory-filled seasons, Lou Gehrig’s career, and his life, were cut short by ALS/MND. New York born-and-bred Lou Gehrig had brief but impressive call-ups with the New York Yankees in 1923 and ‘24, and made the big club for good in 1925. On the first of June, manager Miller Huggins called on Gehrig to start at first base for the injured Wally Pipp. So began one of sport’s truly amazing feats. On May 2, 1939, an ailing Lou Gehrig would take himself out of the lineup. In between, Gehrig appeared in every one of the Yankees’ 2,130 regular-season games.
Though today Lou Gehrig’s name may bring “the illness” and “the streak” to mind, he was in his day a true superstar. His accomplishments will forever keep him among the legends of the game. Gehrig hit for power, hit for average, and was a consistent run producer. He was twice named the American League’s Most Valuable Player. In his 14 full seasons the Yankees won seven World Series championships. The Wall Street Journal recently ran an article which stated that statistically, as measured by “contribution to team success” Lou Gehrig was the greatest player of all time, and that his compensation in today’s market would exceed $10 million per year.
Many call the Yankees “Murderers’ Row” team of 1927 the greatest team in history. In that year, Gehrig’s teammate the legendary Babe Ruth hit 60 home runs, a mark only once equaled (by Roger Maris with 61 in 1961, in a longer season). And in that year Lou Gehrig batted .375, hit 47 home runs with 52 doubles and 18 triples. He drove in 175 runs, and was named League MVP. He repeated as MVP 1936. In 1934, Gehrig won the Triple Crown for leading the league in batting average, home runs, and runs batted in: .363, 49, and 165.
Lou Gehrig batted .295 in his rookie year and his last full year, 1938; the only full seasons he hit under .300--in 1930 he hit .379. Over his career he hit 23 grand slams, a record to this day. He averaged an astonishing 147 RBI’s per season. As the 1939 season opened, Lou Gehrig was an obviously sick man. He would play in only eight more games. In Washington on April 30, he singled for career hit #2721. That hit was his last. Two days later, at Detroit’s Briggs’ Stadium, he took himself out. He played in an exhibition game against the Yankee farm club in Kansas City, then flew to Rochester for examination at the Mayo Clinic. On June 19, his 36th birthday, he was finally diagnosed with Amyotrophic Lateral Sclerosis. (The NY Times called the illness “a form of infantile paralysis.”)
On the fourth of July, 1939, 60,000 packed Yankee Stadium to honor a hero. And on June 2, 1941, just short of his 38th birthday, Lou Gehrig died.
Lou Gehrig’s consecutive game streak, one of the greatest records in the history of sport, was never so much as approached until Baltimore Orioles’ shortstop Cal Ripken Jr. surpassed it in 1995. The occasion generated a great deal of much-needed publicity of the illness, and was marked by the founding of a research fund in Mr. Ripken’s name.
(Nathan Brown has a terrific tribute page to this great man at http://www.nmia.com/~browns/gehrig.htm )
ALS/MND claimed the life of actor David Niven, US Senator Jacob Javits, and afflicts physicist Stephen Hawking. Jon Stone, co-creator of Sesame Street, died in 1997 of ALS/MND. Michael Zaslow, popular American daytime drama actor, was diagnosed with ALS in 1997.
ARE THERE DIFFERENT TYPES OF ALS/MND? There are three common classifications, and numerous sub-categories: Sporadic: the most common form of ALS/MND, Familial: less than 10% of ALS/MND cases suggest genetic inheritance, Guamanian: a high number of cases of ALS/MND occur in Guam and the Trust Territories of the Pacific.
WHEN WAS ALS/MND FIRST DISCOVERED? ALS/MND was first described in 1869 by Jean-Martin Charcot, a trail-blazing French neurologist.
WHAT CAUSES ALS/MND? The cause of ALS/MND is not yet known. While many theories are being researched, at present neither a cure nor a means of prevention is known.
In 1993, scientists announced in a paper published in the British journal “Nature” that they had isolated the gene associated with about 20% of the cases of the inherited form of the disease. While only 10% of ALS/MND patients have this genetic predisposition, there is no evidence of a clinical difference between the familial and the sporadic forms of the illness.
A currently favored theory combines genetics and toxicity (poisons): that sporadic ALS/MND occurs in those with a genetic predisposition to motor neuron degeneration, and that the illness is then triggered by environmental insult. In Nov 1996, Dr. Jeffrey Rothstein of Johns Hopkins University found that 42% of sporadic ALS/MND patients have a defect in the gene that controls the protein EAAT2, a protein which helps to regulate the brain’s glutamate levels, well known to be a factor in motor neuron degeneration.
WHAT ABOUT ENVIRONMENTAL CAUSES? The very high incidence of ALS/MND on the island of Guam, in Western New Guinea and on Kii peninsula of Japan may provide some clues about environmental influences. Heavy metals such as lead and mercury are suspected causes, as is aluminum, which can poison the body and cause ALS/MND symptoms.
CAN YOU “CATCH” ALS/MND? ALS/MND cannot be “caught”—it is not contagious. Though some scientists believe it is possible that ALS/MND is caused by a slow-acting or latent “virus”, there is absolutely no fear that it is contagious: there is no increased incidence among medical personnel who deal with ALS/MND patients.
IS THERE HOPE FOR PEOPLE WITH ALS/MND? ALS/MND wreaks a devastating effect on patients as well as their families. As they struggle to cope with the prospect of advancing disability and death, it consumes their emotional and financial reserves. It is a costly disease in its later stages, demanding both extensive nursing care and expensive equipment. But there is always hope. Recent medical discoveries have led to new drugs which seem to slow the progression of the disease. Others have shed light on factors which will encourage the regrowth of nerve cells. Advances in knowledge of other neurological diseases also continue to shed light on causes and treatment of ALS/MND.
WHAT CAN I DO TO HELP? ALS/MND takes a tremendous toll on the physical, financial, and emotional resources of its victims and their families. ALS/MND societies and support groups around the world do much to help, but there’s always need for more. If you know someone with ALS/MND, offer your time to relieve the family members of the constant strain. Or just to talk, to listen, to be a friend.
Spread the word. We must continue to raise public awareness of this devastating killer. Get involved, find out, write, phone, talk, shout about it.
Give money. Patients often need expensive home renovations, specialized equipment, therapy, and nursing care, for an open-ended time frame. And science needs funding to intensify the search for cause and cure, so we can eliminate this nightmare.
WHERE CAN I LEARN MORE? The Internet and World Wide Web contain a tremendous amount of information on ALS/MND. Support groups, universities, medical and scientific associations, drug companies, and many individuals are represented, most with links to other sites. A separate FAQ on ALS Internet Resources will be posted regularly along with this FAQ.
