OVERVIEW AND FACTS
What is ALS?
Amyotrophic lateral sclerosis (ALS), often called “Lou Gehrig’s disease,” is a progressive and invariably fatal neurodegenerative disease. ALS attacks the nerve cells (neurons) that control voluntary muscles causing the neurons to die. When the motor neurons die, they can no longer send impulses to the muscle fibers that normally result in muscle movement and the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively depleted, patients in the later stages of the disease may become totally paralyzed.
Amyotrophic lateral sclerosis/ALS/ Lou Gehrig’s disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.
Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, and swallowing or breathing. Limbs begin to look “thinner” as muscle tissue atrophies.
Why is ALS called “Lou Gehrig’s Disease”? Lou Gehrig was a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. That term is often used by Americans, but ALS has different names in different countries. The French refer to it as Maladie de Charcot, after the man who discovered it. People in England and Australia call it Motor Neurone Disease (MND). Whatever it’s called, the disease and its results are the same.
Facts about ALS:
• ALS is not contagious
• ALS can strike anyone and has no racial, ethnic or socioeconomic boundaries
• Approximately 5,600 people in the U.S. are diagnosed with ALS each year
• As many as 30,000 Americans may have ALS at any given time
• The life expectancy of an ALS patient averages about two to five years
• More than half of all patients live more than three years after diagnosis, but many people live with quality for five years and more
SYMPTOMS AND TYPES
The symptoms at the onset of ALS may be so slight that they are frequently overlooked. Some of the most common symptoms include following:
• muscle weakness in the hands, arms, legs or the muscles of speech, swallowing or breathing
• twitching and cramping of muscles, especially those in the hands and feet
• loss of the use of the arms and legs
• difficulty in projecting the voice and “thick speech”
• shortness of breath, difficulty in breathing and swallowing are common in more advanced stages
Although there are many commonalities, symptoms will vary from person to person. Common tell-tale signs may be tripping or stumbling, trouble lifting, uncontrollable periods of laughing or crying or slurred speech. Progressive muscle weakness and paralysis are experienced by all people with the disease and the hands and feet are usually affected first.
Most of the weakening of the muscles starts on the trunk of the body, but will eventually begin to affect speech, swallowing, chewing and breathing. When the breathing muscles become affected, the patient will need a ventilator to breathe.
Unlike some other neuromuscular diseases, the person’s sense of sight, touch, hearing, taste and smell are not affected.
There are three classifications of ALS:
• Sporadic – the most common form of ALS in the United States accounting for 90 to 95% of all cases.
• Familial – occurring more than once in a family lineage. This accounts for a 5 to 10% of all cases in the U.S. but there is a 50% chance each offspring of a parent with ALS will inherit the gene mutation and may develop the disease.
• Guamanian – found in people of Guam and the Trust Territories of the Pacific in the 1950’s.
TREATMENT AND CARE
Unfortunately, there are no treatments to slow or halt the progression of ALS. There are drugs and devices available to help control symptoms and make living with the ALS easier.
Rilutek (riluzole) is an approved drug for the treatment of ALS. This drug seems to prevent the damage that can result from the nerve cell being overexcited by glutamate. There are potentially damaging side effects on the liver with this drug.
Physical therapy to slow the muscle deterioration is used in early stages of the disease.
There are medications and supplements available to relieve stiffness in the limbs and throat, slow muscle decline and to slow weight loss and ease cramps. Antidepressants are often prescribed to help with the depression that is often present with this fatal disease.
Scientists and researchers are always looking for new medicines and treatments for ALS and, ultimately, to unlock the cause of the disease to find a cure.
LIVING YOUR LIFE
While ALS is not as common as many diseases, there are many well-known people who have had the disease.
• Hall-of-Fame baseball player Lou Gehrig
• Hall of Fame pitcher Jim “Catfish” Hunter
• Senator Jacob Javits
• Actors Michael Zaslow and David Niven,
• Sesame Street creator of Jon Stone
• Television producer Scott Brazil
• Boxing champion Ezzard Charles
• NBA Hall of Fame basketball player George Yardley
• Pro football player Glenn Montgomery
• Golfer Jeff Julian and golf caddie Bruce Edwards
• British soccer player Jimmy Johnstone
• Musician Lead Belly (Huddie Ledbetter)
• Photographer Eddie Adams
• Entertainer Dennis Day
• Jazz musician Charles Mingus
• Composer Dimitri Shostakovich
• Former vice president of the United States Henry A. Wallace
• U.S. Army General Maxwell Taylor
No matter if you are a world famous athlete or the average person on the street, a diagnosis of ALS is devastating. As a progressive disease, the person with ALS will weaken and lose more and more muscle strength and independence as the disease progresses. From the muscle weakness of the early symptoms to the progression of weakness and paralysis of the muscles of the limbs and eventually the speech, swallowing and eventually breathing, the person will ALS will require increasing levels of care.
Another large part of living with disease is the use and cost of medical care, home medical equipment and the cost of home health caregivers. It is important to know as much about your health plan coverage and other programs for which you may be eligible as you can. You will need to be your own best advocate or the advocate for someone with the disease. Contact the Social Security Administration, Medicare, Veterans Affairs and any other group or organization that may be of assistance. Use the internet to find more information and support.
The cause of ALS is not completely understood making it that much harder to diagnose. There is currently no one test to establish the diagnosis of ALS. Doctors often use observation, family history and ruling out other diseases before a diagnosis can be established. A comprehensive diagnostic workup may include:
• electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)
• blood and urine tests including looking for certain proteins and other indicators
• spinal tap
• magnetic resonance imaging (MRI) and other x-rays
• myelogram of cervical spine
• muscle and/or nerve biopsy
• neurological examinations
There are other diseases that have similar symptoms to ALS that are more treatable and have a better outcome. The ALS Association recommends that a person diagnosed with ALS seek a second opinion from an ALS “expert” – someone who diagnoses and treats many ALS patients and has training in this medial specialty.
ASSISTANCE AND COMFORT
If you or someone you care about is living with ALS, we encourage you to learn as much about the disease as possible and seek help when you need it. There is more information available from the groups below. You can click on the links below, write or call them to receive more information.
• The ALS Association’s Care Services electronic newsletter, The Exchange, is published monthly. Sign up to receive The Exchange
• ALS Association Certified Centers and Clinics who work closely with our chapters
• There are many books for and about ALS patients and their family caregivers. Use the ALS Association’s FYI Information Index to find out more.
• Support groups give a place for family caregivers to meet one another and offer each other mutual support and practical problem solving strategies. Find your ALS local support group.
• Most ALS Association chapters have loaner equipment such as lifting devices, wheelchairs and communication devices can make a significant difference to the family caregiver and the ALS patient.
• For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute’s Brain Resources and Information Network (BRAIN) at:
P.O. Box 5801
Bethesda, MD 20824
• The following organizations are also great resources for people dealing with ALS.
P.O. Box 425783
Cambridge, MA 02142