ALS

ALS (commonly known as Lou Gehrig’s Disease) - Questions & Answers
(taken from the ALS Survival Guide)

What is ALS?
ALS is a fatal neuromuscular disease characterized by progressive muscle weakness resulting in paralysis.

What do the letters ALS stand for?
ALS stands for Amyotrophic Lateral Sclerosis. Like many medical terms it comes from Greek words.

  • A—without
  • Myo—muscle
  • Trophic—nourishment
  • Lateral—side (of the spinal cord)
  • Sclerosis—hardening or scarring

Is there a cure for ALS?
There is no cure for ALS, but there is a great deal of promising research going on now.

How long do people with ALS live?
50% of ALS patients die within 18 months after diagnosis. Only 20% survive 5 years and 10% live longer than 10 years. Persons with ALS who go on a ventilator may live for many years. Improved treatment is allowing ALS patients to live longer than before.

What are the symptoms of ALS?
ALS symptoms may include tripping, stumbling and falling, loss of muscle control and strength in hands and arms, difficulty speaking, swallowing and/or breathing, chronic fatigue, and muscle twitching and/or cramping. ALS is characterized by both upper and lower motor neuron damage. Symptoms of upper motor neuron damage include stiffness (spasticity), muscle twitching (fasciculations), and muscle shaking (clonus). Symptoms of lower motor neuron damage include muscle weakness and muscle shrinking (atrophy).
In "ALS: A Comprehensive Guide to Treatment", Dr Mitsumoto lists upper motor neuron signs as spasticity, hyperreflexia, and pathological reflexes (Babinski's sign - the one where they run an object from the heel to the big toe). Lower motor neuron signs are listed as muscle weakness, truncal muscle weakness, muscle atrophy, fasciculations, hyporeflexia, hypotonicity or flaccidity, and muscle cramps. Bulbar signs are listed as dysarthria (speech problems), dysphagia (swallowing problems), and sialorrhea (drooling).

What treatments are available?
There is one FDA approved drug, Rilutek, which slows progression of the disease. There are a number of other medications to help relieve symptoms of ALS. Therapies, supplements, and proper nutrition can be part of a treatment plan.

How common is ALS?
The incidence of ALS is about two per 100,000. This means that for every 100,000 people, two will get ALS. The prevalence of ALS is about 11 per 100,000. This means that if you look at a population of 100,000 people, 11 of them will be living with ALS. In the United States, approximately 30,000 people are living with ALS. There are about 5,000 new cases per year, or 15 per day.

Are there different types of ALS?
There are three types of ALS. The most common type is called sporadic ALS. This type of ALS is not hereditary and accounts for 90% of ALS cases. Familial ALS is hereditary, is passed on by a dominant gene and accounts for nearly 10% of ALS cases. The third type of ALS is called Guamian and is related to the high incidence of ALS on the island nation of Guam.

Is ALS always a fatal disease?
ALS is almost always fatal. There are rare cases where the disease progression plateaus or stops. There are a few cases of people reporting a reversal of symptoms. If an ALS patient opts for a ventilator, he or she can live for many years with the disease.

Do ALS patients suffer painful, suffocating death?
In the majority of cases, ALS patients die painlessly and peacefully, often in their sleep. If an ALS patient does not opt for a ventilator to assist breathing, death is usually a result of insufficient oxygen which leads to a build-up of carbon dioxide in the blood. The build up of carbon dioxide has a narcotic effect on the patient making him or her sleepy. Many ALS patients also opt for medications to ease breathing discomfort and anxiety. ALS patients often choose to die in a hospice setting or at home where they can be comforted by their loved ones.

What is the cause of death for ALS patients?
A common cause of death among ALS patients is respiratory failure or cardiac arrhythmias due to insufficient oxygen. Another common cause of death is respiratory infection such as pneumonia. The risk of respiratory infections increases as weakened diaphragm and chest muscles make it more difficult to clear the lungs.

Is ALS a disease of the muscles or nerves?
ALS is a disease of the motor nerves, specific nerve cells in the brain and spinal cord that control voluntary movement. Motor nerves attach to muscles and when the motor nerves gradually degenerate and die, the muscles no longer receive nerve impulses. As a result of the nerve death, the muscles atrophy and waste away. When an ALS patient first notices neurological symptoms, more than half of the motor neurons may already be dead. The body has a remarkable ability to compensate for nerve loss and new nerves grow at the same time other nerves are dying. Eventually, nerve death becomes so pervasive that muscle paralysis is the result.

Which muscles are affected first?
Most ALS patients first notice muscle weakness in either the arms or the legs (32 percent in the arms and 36 percent in the legs.) This is called limb-onset ALS. Approximately 25% of ALS patients have difficulty speaking as their first symptom. This is called bulbar ALS because it involves the corticobulbar area of the brainstem. ALS is a very variable disease, and there are also cases affecting breathing first, without any other symptoms. Approximately 7 percent have difficulty breathing (dyspnea) as their first symptom.

Are there muscles that are not affected?
ALS does not affect all muscles. Bowel and bladder control remains intact as does sexual function. The heart muscle is unaffected. The muscles of the eyes are the last ones affected and, in some cases, are not afflicted.

How does ALS typically progress?
ALS progression can vary considerably with different patients. A common progression is as follows: difficulty walking resulting in the use of a cane followed by a walker and then a wheelchair. As the legs get weaker so do the arms and hands. The patient loses the ability to write, type, and feed themselves. As the muscles of the limbs become weaker, difficulties in speaking and swallowing begin to occur. This may result in the need for an augmentative communication system in order to communicate and a feeding tube in order to get adequate nutrition. Typically, the ALS patient has a gradual decrease in lung vital capacity as the muscles of the chest and the diaphragm begin to weaken. When lung vital capacity drops below 50%, the ALS patient needs to consider using a ventilator in order to stay alive. This progression may be as quick as six months or as long as several years.

Does exercise help slow ALS progression?
Exercise does not slow down the wasting of muscles due to ALS. Heavy exercise is not recommended because of the fatigue it causes on already weakened muscles. Light exercise such as walking, swimming, and stretching can help maintain strength in the muscles which are not yet affected by ALS. When muscle atrophy becomes severe, the only recommended exercise is range of motion to preserve joint flexibility.

Does ALS affect speech?
ALS does affect speech and swallowing when the disease involves the corticobulbar area of the brainstem. As the muscles of the mouth and tongue weaken, speaking becomes increasingly difficult. Speech therapy may be helpful to an ALS patient and can enable the person to be understood even with a weak voice. Eventually, ALS patients lose the ability to speak and must rely on an augmentative communication device in order to communicate.

What is Bulbar ALS?
Bulbar refers to the brain stem where motor neurons are concentrated. Bulbar ALS is the type of ALS where speaking and swallowing difficulties are the first symptoms. Many patients with bulbar ALS are unable to speak or eat but are able to walk and write. Bulbar-onset ALS patients generally have a faster disease progression than do limb-onset patients. Limb-onset patients eventually develop bulbar symptoms as their disease progresses.

What is the average age of ALS onset?
The average age of ALS onset is 55. ALS can affect people at any age, and cases have been found in persons as young as 12 and as old as 98. 80% of ALS cases begin between the ages of 40 to 70. There appears to be a trend of more younger patients in their 20's and 30's being diagnosed with ALS

How do ALS patients eat when they lose their ability to chew and swallow?
As muscle weakness makes eating more difficult, dietary consistency changes are needed. Gradually the diet includes softer foods and eventually may consist of puréed foods. When eating becomes too tiring or causes problems with choking, many ALS patients opt for a feeding tube. This is a relatively simple procedure which can greatly extend the quality and quantity of an ALS patient.

Do all ALS patients become bedridden?
Many ALS patients do not remain bedridden even though they may be totally paralyzed. Specially designed wheelchairs and lightweight, portable ventilators allow the patient to be mobile. Improvements in technology allow paralyzed ALS patients much more freedom of movement than in years past.

What other health problems are caused by ALS?
There are several health problems that can occur as a result of the immobility caused by ALS. All of these health problems are treatable. They include: skin breakdown which can lead to bed sores; problems with constipation and painful joint contractions resulting from lack of movement.

Is depression common among ALS patients?
Depression is common among ALS patients and may be more common among caregivers. Depression can be minimized through the use of antidepressant medication, counseling, and a strong network of supportive family and friends.

Is ALS an expensive disease?
ALS is a very expensive disease. The only FDA approved medication, Rilutek, costs approximately $700 a month. Other medications to treat ALS symptoms are also expensive. Adaptive equipment is expensive. A power wheelchair with tilt and recline features may cost $17,000. A home health aid for 10 hours a day may cost more than $30,000 a year. When an ALS patient goes on a ventilator, he or she will require skilled nursing care. This may cost in excess of $250,000 a year. Insurance companies may pay some of these expenses. Medicare and Medicaid may pay some of these expenses

Are there any famous people who have or had ALS?
The most famous person living with ALS is noted British physicist Stephen Hawking. He has been living with ALS for more than 35 years. He is able to move only two fingers. Other notable people who have had ALS are actor David Niven, baseball players Lou Gehrig and Catfish Hunter, U.S. Senator Jacob Javits and Soap Opera star Michael Zaslow.

Additional Links

Recommended Library Resources

  • Video: Maintaining Quality of Life: A Patient’s Perspective
  • Book: Tuesdays with Morrie by Mitch Albom