To help assist in the
understanding of this devastating disease, I have put together this
information sheet. It is in a Question/Answer format for easier reading.
For additional information, I have compiled a list of ALS links. ALS
Internet Resources
Email a friend about Rockin' For a Cure
Amyotrophic Lateral Sclerosis (ALS)
is a fatal neuromuscular condition characterized by progressive muscle
weakness,
muscle wasting, fasciculations and increased reflexes, resulting in total paralysis of all voluntary muscles. OVERVIEW: ALS is a rapidly progressive, fatal
neuromuscular disease that 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 because of
ALS, the ability of the brain to control muscle movement is lost. When
muscles fail to receive messages, they weaken, atrophy, and die. The most basic activities and
function of daily living, such as, walking, talking, breathing, writing,
reaching, bending, squatting, jumping, twisting, pushing, pulling,
grabbing, pinching, squeezing, holding, lifting, all become extremely
difficult tasks, even monumental, and eventually can not be done at all. There is no known cure.
What do the letters ALS stand for?
ALS stands for Amyotrophic Lateral Sclerosis. Like many medical terms, it comes from Greek words. "A"
= without
When was ALS first discovered?
ALS was first described in scientific literature in 1869 by
the French neurologist Jean-Martin Charcot.
ALS is commonly known in the US as Lou Gehrig’s Disease. In England, it is known as MND, (Motor Neurone Disease). The French refer to ALS as Maladie de Charcot.
There is no cure for ALS
How long do people with ALS live?
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, but once on a
ventilator, 24-hour care MUST maintained by qualified nursing care.
Dropping things, tripping,
stumbling and falling. Abnormal fatigue of the arms and
legs. Loss of both muscle control & strength in the hands, arms,
feet and legs. Difficulty with speaking, swallowing and breathing. Muscle twitching and cramping. Chronic fatigue Brisk reflexes Uncontrollable periods of laughing
or crying. 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).
Is ALS painful?
Some of the
effects of ALS may be painful. Common pains as a
result of ALS include pressure sores, muscle cramps, joint contractures,
constipation, burning eyes, swelling feet, and muscle and joint aches.
Is ALS hereditary?
This is called
familial ALS. Generally, we define familial ALS as two or more cases in
the same bloodline. In familial ALS, the disease is autosomal dominant,
meaning that if a parent has ALS, their children have a 50% chance of
inheriting the defective gene. While the risk of inheriting the
defective gene is 50% for each child of an affected person, not all
people with the defective gene will develop the disorder. Twenty percent
of familial ALS cases have a specific gene defect in the SOD (super
oxide dismutase) gene. 90% of ALS cases
have no familial link, and they are called sporadic ALS.
What causes ALS?
Researchers know that an excess of a neurotransmitter called
glutamate clogs the synapse of the nerve cell preventing transmission of
neural impulses. The cause of this neurotransmitter problem remains a mystery.
Men are much more likely to get ALS than women. Older people are more likely to get ALS than younger people.
Does ALS affect the mind?
It is now known that some ALS patients do experience dementia,
but it is unknown whether ALS was the cause of the dementia. Past ALS literature describes the mind as being
"completely
unaffected." Many current ALS specialists are starting to take a much
closer look at this statement. Many are reconsidering.
Does ALS affect
the bladder and bowel?
But as research
continues on, this belief is changing rapidly. Many ALS patients
are now reporting that there is a lack of bowel control.
Early ALS symptoms
may be similar to a number of other neuromuscular diseases. Diagnostic
tests include MRI (magnetic resonance imaging), EMG (electromyogram),
muscle biopsy, and blood tests. In order for a definitive diagnosis of
ALS to be made, damage must be evident in both upper and lower motor
neurons. When three limbs are sufficiently affected, the diagnosis is
ALS.
How long does it take to diagnose ALS?
Many of the initial symptoms are similar to other
neuromuscular diseases. Diagnosing ALS is a process of elimination, which is time consuming.
What treatments are available?
It is Rilutek, manufactured by Rhone-Poulenc Rorer http://www.alsinfo.com/rilutek_facts_care
The drug, an anti-glutamate, extends survival in ALS patients
by a mere three months. The drug was approved by the FDA in December
1995 and became available by prescription in January 1996. Rilutek is the
only FDA approved drug, which has shown to slow progression of the
disease in only some patients.
Are there any medications to stop the progression of ALS?
A number of drugs
are available to help manage the symptoms of ALS. Vitamins and antioxidants are taken by many ALS patients in an effort to slow down the disease.
How common is ALS?
This means that for every 100,000 people, two will get ALS.
The prevalence of ALS is about eight per 100,000. This means that if you
look at a population of 100,000 people, eight 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. (As ALS is terminal and incurable,
death rates are very close to incidence rates.)
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?
There are a few rare cases where the disease progression
plateaus or stops. Concerns of accurate diagnoses have been suspect in these rare
cases. If an ALS patient opts for a ventilator, he or she can live
for many years with the disease under the care of qualified nursing
personnel.
What is the cause of death for ALS patients?
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?
Motor nerves
attach to muscles and when the motor nerves gradually degenerate and
die, the muscles no longer receive nerve impulses. Because 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?
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?
Bowl 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?
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.
Does exercise help slow ALS progression?
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.
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 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?
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 there
20's and 30's being diagnosed with ALS
A higher incidence
of ALS is correlated with exposure to agricultural chemicals and
solvents.
Why is ALS called Lou Gehrig’s disease?
How do ALS patients eat when they lose their ability to chew and swallow?
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.
Is there equipment available to help ALS patients breathe?
Do all ALS patients become bedridden?
What other health problems are caused by ALS?
Is ALS an 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.
Also visit www.madisonscout-volunteers.org A Benefit for ALS Research
For more information please
contact Mike Anello 414-483-1510
or email below navigate |
