Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
Why Write About ALS?
After going through test after test for the last couple of years, a dear friend has received the news that she has ALS disease. After years, she finally got an answer about what was happening to her health. I guess that this is my way of coming to terms with the fact that I will be losing my friend and, by letting, others know about this terrible disease and its effects on everyone.
Statistics of the Disease
According to the Mayo Clinic-“Worldwide, ALS occurs in 1 to 3 people per 100,000. In the vast majority of cases – 90 to 95 percent – doctors do not yet know why ALS occurs. About 5 to 10 percent of ALS cases are inherited.”
Most commonly, the disease strikes people between the ages of 40 and 70, and as many as 30,000 Americans have the disease at any given time.
The Mayo Clinic states these are the established risk factors for ALS:
- Heredity–Up to 10 percent of the people who have ALS inherited it from their parents. If you have this type of ALS, your children have a 50-50 chance of developing the disease.
- Age–ALS most commonly occurs in people between the ages of 40 and 60.
- Sex– Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.
The Mayo Clinic also states that there are also environmental factors for ALS, which is:
- · Smoking
- · Lead exposure
- · Military service
Note: The reason that military Service is among these is the exposure to certain metals, chemicals, traumatic injuries, viral infections, and intense exertion.
Tests and Diagnose: It is difficult to diagnose ALS because it can present itself like other neurological diseases. The tests are to rule out other diseases.
- · Electromyogram-Measures the electrical discharges produced in the muscles.
- · A Nerve Conduction Study-Measures the strength and speed of the body’s nerve signals.
- · MRI– Gives detailed images of the brain and spinal cord.
- · Blood and Urine Tests-Can help eliminate other possible illnesses or causes of the signs and symptoms.
- · Muscle Biopsy-Eliminates other muscle diseases.
Early signs and symptoms of ALS start in the hands, feet and limbs and include:
- Difficulty lifting the front part of your foot and toes (foot drop)
- Weakness in your leg, feet or ankles
- Hand weakness or clumsiness
- Slurring of speech or trouble swallowing
- Muscle cramps and twitching in your arms, shoulders and tongue
This is a progressive disease and it will spread to all parts of the body affecting chewing, swallowing, speaking, breathing and paralyzing all muscles.
The progressive complications are:
- · Breathing-Paralyzes the muscles used to breath.
- · Eating and Drinking-Paralyzes the muscles used for swallowing and the aspiration of food, liquids or secretions go into the lungs causing pneumonia.
- · Dementia-The ability to remember and make decisions will become difficult and will result in “front temporal dementia”.
Medications and Treatments
The first and only medication that doctors use to slow down the effects of ALS in some people is “Rilutek” that has been approved by the Food and Drug Administration. A doctor may prescribe other medications to provide the relief of the effects of ALS.
- · Muscle cramps
- · Constipation
- · Fatigue
- · Excessive salivation
- · Excessive phlegm
- · Pain
- · Depression
- · Physical Therapy-Will help to maintain as long as possible muscle strength, cardiovascular fitness, motion and well-being.
- · Occupational Therapy-Helps show how to use a braces, walker, wheelchair and ramps.
- · Speech Therapy-Maintaining communication is a major issue to make sure caregivers clearly understand what the patient needs. So this therapy helps with alternative methods of speech such as; alphabet board, pen and paper, speech synthesizers, and computer-based equipment.
Care, Support and Decisions
- · Grieving-Both the patient with ALS and their families and friends will go through a grieving period. Ignoring the disease and the result will not make it go away.
- · Outlook-Be hopeful some ALS patients have been known to live up to 10 years which is longer than the 3-5 years after the signs start. A good outlook can make life easier.
- · Physical Changes-Look at the physical changes in a different fashion like you the physical changes we all have as we age. They will be easier to accept and live with.
- · Life Choices-While the ALS patient still has their mental awareness they can be proactive in regards to how they will live their life towards the end.
Such as; medical care, life-extending procedures, where and how they are going to spend their last days, and funeral arrangements.
Everything that will need decided towards the end of the illness. This way the patient will have some sort of control on the rest of their life while they still can.
- · Support-This is the most important one of all to get support for both the patient, their family and friends to deal with this disease. There are support groups that can be attended and even online support groups. It is important for everyone to know and feel that they are not alone.
The loving support of family and friends for the patient is worth its weight in gold to them. Just knowing that they do not have to worry about everything until the end is priceless.
Call them, visit them and love them regardless of their physical state. They might still know that you are there loving and supporting them.
ALS Association Foundation: http://www.alsa.org/about-us/
Mayo Clinic: http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/DS00359/DSECTION=tests-and-diagnosis