Amyotrophic Lateral Sclerosis (ALS)

 

By Victor Montour

Amyotrophic Lateral Sclerosis or better known as ALS is a progressive neurodegenerative disease that affects the nerve cells in the brain and spinal cord. A-myo-trophic comes from the Greek language. “A” means no. “Myo” refers to muscle, and “Trophic” means nourishment – “No muscle nourishment.” When a muscle has no nourishment, it “atrophies” or wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening (“sclerosis”) in the region.

ALS

The progressive degeneration of the motor neurons in ALS eventually leads to the demise of the motor neurons. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. Voluntary muscle action is progressively affected, people may lose the ability to speak, eat, move or breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control.

There are two different types of ALS, sporadic and familial. Sporadic which is the most common form of the disease in the U.S., is 90 – 95 percent of all cases. It may affect anyone, anywhere. Familial ALS (FALS) accounts for 5 to 10 percent of all cases in the U.S., Familial ALS means the disease is inherited. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease.

Recent years have brought a wealth of new scientific understanding regarding the physiology of this disease. There is currently one FDA approved drug, riluzole, that modestly slows the progression of ALS in some people. There is not a cure or treatment that halts or reverses ALS.

Who gets ALS?

Based on U.S. population studies, a little over 5,600 people in the U.S. are diagnosed with ALS each year. (That’s 15 new cases a day.) It is estimated that as many as 30,000 Americans have the disease at any given time. According to the ALS CARE Database, 60% of the people with ALS in the Database are men and 93% of patients in the Database are Caucasian.

Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. However, cases of the disease do occur in persons in their twenties and thirties. Generally though, ALS occurs in greater percentages as men and women grow older. ALS is 20% more common in men than in women. However with increasing age, the incidence of ALS is more equal between men and women.

There are several research studies – past and present – investigating possible risk factors that may be associated with ALS. More work is needed to conclusively determine what genetics and/or environment factors contribute to developing ALS. It is known, however, that military veterans, particularly those deployed during the Gulf War, are approximately twice as likely to develop ALS.

Facts You Should Know

  • ALS is not contagious.
  • The life expectancy of a person with ALS averages about two to five years from the time of diagnosis
  • Once ALS starts it always progresses, eventually taking away the ability to walk, dress, write, speak, swallow, and breathe and shortening the life span.
  • ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries and can affect anyone.
  • Military veterans are approximately twice as likely to develop ALS.
  • The onset of ALS often involves muscle weakness or stiffness as early symptoms. Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.
  • There can be significant costs for medical care, equipment and home health caregiving later in the disease.

ALS and Hospice Care

The nurses at Front Range Hospice know most people with ALS experience several, if not many, of the following symptoms: respiratory insufficiency, fatigue, problems with mobility, pain, dysarthria, dysphagia, problems with secretions, involuntary emotion expression disorder (IEED), depression, insomnia, and constipation. These symptoms and less common symptoms are addressed by our hospice nurses along with possible interventions to maximize ones comfort and dignity.

If you would like more information about ALS or would like more information about Front Range Hospice please call 303-957-3101 or 970-776-8080 or email us at info@frhospice.com

 

 

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About Front Range Hospice- Legendary Care

Front Range Hospice is a center for excellence in providing end-of-life care and we continue to strive to keep our company achieving distinction. Visit us at www.frhospice.com.
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