by Kimberley Allen R.N.
Amyotropic Lateral Sclerosis (ALS), frequently referred to as Lou Gehrigs disease is a member of a group of diseases known as motor neuron diseases. It is a progressive and inevitably fatal neurolgical disease in which the nerve cells that control voluntary muscles gradually deteriorate and die.
It is estimated that currently there are between 20-30 thousand peoople in the US suffering with ALS with another 5,000 diagnosed every year. ALS affects people of all races and ethnic backgrounds and is one of the most frequently diagnosed neuromuscular diseases worldwide. The symptoms of ALS usually appear between the ages of 40-60yrs, however it can affect any age and it is more common in men than women. Most cases of ALS, 90-95%, appear at random, and there is no clearly defined cause or risk factor associated with the onset of symptoms. However, there is a small percent, 5-10%, that are inherited.
There are currently several researchers that are studying possible causes of ALS including chemicla imbalances and gene mutation. Usually the first symptom of ALS is weakness affecting only one leg, or one arm, as the symptoms of ALS start on one side of the body then slowly progresses to affect the rest of the body. the rate at which ALS progresses depends on the rate at which the motor neurons die. As the motor neurons die they stop sending the signals to the muscles telling them to move. Over time there are no neurons left to tell the muscles to move causing them to get weaker and shrink. Depending on which part of the body is affected you might first notice some awkwardness when walking or running or start tipping and stumbling more than you used to. The symptoms don’t always appear in the legs first, you could start experiencing difficulty with tasks using manual dexterity such as buttoning a shirt, or writing, some first experience difficulty with speech and swallowing. Though the progression of the disease and the order in which symptoms appear vary from patient to patient the results is always the same. Eventually patients will be unable to stand or walk and will at some point require 24 hour care. It is important to remember that the disease affects motor neurons only, taking away the patients ability to move, it does not affect cognitive function so these patients are fully aware of what is happening to their body’s which causes anxiety and depression.
There is currently no cure for ALS, treatments are primarily focused on relieving symptoms and improving quality of life. For this reason it is important that health care providers clearly explain the disease and how it progresses as well as any treatment options early so that the patient and family can make informed decisions about their care in advance.
Early in ALS physical and occupational therapy can help you maintain strength and mobility, supportive equipment and devices can help you with your activities of daily living allowing you to remain independent longer. Speech therapy can help you maintain your ability to communicate after speech problems start, there are also medications that can make breathing easier as the weakness progress to the chest muscles. All patients will eventually lose their ability to breathe on their own and will require mechanical ventilation for survival.
There is a new drug available called riluzole, it’s the first drug to be approved by the FDA to treat ALS. This drug has slowed the progression of the disease in some patients. It is believed to work by reducing the levels of glucamate which is a chemical messenger in the brain.
Recieving a diagnosis of ALS is devastating for both the patient and family, it’s important to take time to absorb the info and grieve. Once you have over come the initial shock the most important thing you can do is remain hopeful. The disease varies from patient to patient some live much longer than statistics indicate. Also there is continuing research being done relating to all areas of ALS including treatment. Joining a support group can help the patient and family to understand and cope with the changes. It’s a good idea to sit with your family and discuss what and how much care you want in the future. This allows you to be in control and make that choice. Determining where you want to spend your final days in advance can help you and your family to concentrate on enjoying the time you have instead of worrying about what to do in the future.
Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at firstname.lastname@example.org.