Frequently Asked Questions (FAQ's)
The existence of these FAQ's is due to the hard work of Mike Beal.
- 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?
- Is ALS painful?
- Who Is Liable To Get ALS/MND?
- What Does "Amyotrophic Lateral Sclerosis" Mean?
- Why Is It Also Known As "Lou Gehrig's Disease"?
- 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?
What Is ALS/MND?
Amyotrophic Lateral Sclerosis, a.k.a 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 Gehrig's 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!)
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 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.
Is ALS painful?
Some of the pain ALS may cause are pressure sores, muscle aches or cramps,
swelling feet, and muscle contractions.
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, a famous baseball player in the U.S. during the 1930's, became afflicted
with ALS/MND. He was known as baseball's "Iron man", playing in 2,130 consecutive
games at first base for the New York Yankees. Lou Gehrig was a true superstar.
But, at the peak of a glorious career, he was diagnosed as having Amyotrophic
Lateral Sclerosis. He died two years later at the age of 38. Lou Gehrig's consecutive
game record, one of the greatest marks 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.
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 recently of ALS/MND.
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 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.
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.
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.
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.
- 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
Michael Beal 1954 - 2003
Diagnosed ALS - 1994