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Sep 3rd´10 |
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What is ALS?
ALS stands for Amyotrophic Lateral Sclerosis, sometimes called Lou Gehrig's disease. It is a rapidly progressive and fatal neuromuscular disease that is characterized by degeneration of a select group of nerve cells and pathways (motor neurons) in the brain and spinal cord. This loss of motor neurons leads to progressive paralysis of the voluntary muscles. The heart is not a voluntary muscle, and therefore, remains unaffected by the disease.
However, since breathing is controlled voluntarily by the chest muscles, death usually occurs when the chest muscles are no longer able to help the lungs achieve adequate oxygenation. Generally, there is little impairment of the brain or the senses.
"Amyotrophic" means:
A = absence of
myo = muscle
trophic = nourishment
lateral = side (of spine)
sclerosis = hardening or scarring
ALS is not contagious, but it is fatal. For the most part, the battle is short, with 80% losing their lives within three to five years of diagnosis. While between 10% and 20% live ten years or more after diagnosis, others live only a few months. While the cause is unknown, research is being conducted in areas relating to genetic predispositions, viral or infectious agents, environmental toxins and immunological changes.
For some people, the muscles for speaking, swallowing or breathing are the first to be affected. This is known as bulbar ALS. The term "bulbar" refers to the motor neurons located in the brain stem, that control the muscles used for chewing, swallowing, and speaking. ALS symptoms, and the order in which they occur, vary from one person to another.
In 85% of cases, ALS affects the lower portion of the spinal cord first. This is known as limb onset ALS. In these cases, muscle weakness, cramps and weakened reflexes affects the muscles in the arms and legs as the first signs of ALS. The rate of muscle loss can vary significantly from person to person with some patients having long periods with very slow degeneration.
Is ALS a rare disease?
ALS is not considered a rare disease. Approximately 2,500-3,000 Canadians currently live with ALS. Two or three Canadians lose their battle to this devastating disease every day.
"ALS is clearly the most common cause of neurological death on an annual basis," Dr. Michael Strong, clinician at the University Health Sciences Centre and research scientist at the Robarts Research Institute, London, Ontario.
What causes ALS?
We don't really know what causes ALS, but we do know that it can strike any adult at any time. While the usual age at onset is between 45 and 65, people as young as 17 have been diagnosed in the past. Between five and 10% of ALS cases are found in the same families, meaning that they are "familial", and are definitely linked genetically. But for the most part, diagnosis is sporadic and we don't know how it is caused.
What are the early symptoms?
ALS usually becomes apparent either in the legs, the arms, the throat or the upper chest area. Some people begin to trip and fall, some may notice muscle loss in their hands and arms and some find it hard to swallow and slur their speech.
ALS is difficult to diagnose. There is no specific test available that will either rule out or confirm the presence of ALS. Diagnosis is usually made through a 'diagnosis of exclusions'. Neurologists conduct a number of tests, thereby ruling out other disorders that may cause similar symptoms, such as strokes or multiple sclerosis and if nothing else is positive and yet the symptoms continue to worsen, ALS is often the reason.
What are the effects of ALS?
Because ALS frequently takes its toll before being positively diagnosed, many patients are debilitated before learning they have ALS. The disease usually does not affect the senses - taste, touch, sight, smell, and hearing - or the mind. ALS has a devastating effect on patients and their families. As they cope with the prospect of advancing disability and eventually death, it consumes their financial and emotional reserves. It is a costly disease in its later stages, demanding both extensive nursing care and expensive equipment.
What can be done about ALS?
There is no known cure at this time and very little in the way of treatment that will have an effect on the disease itself.
Is there hope for people with ALS?
Research is looking to find not only the cause of the disease so that a cure can be developed but also other medications or treatments that can help until a cure is found. With improved knowledge about ALS, healthcare providers and families can help people living with ALS live life more fully. The services offered by the ALS Society of New Brunswick help improve the quality of life for those who live with ALS and their families.
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