What is ALS?
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. It is also called Lou Gehrig disease or motor neuron disease. ALS means that over time neurons stop working and die back. In ALS, the neurons in the brain, spinal cord, and those outside of the spinal cord that go to muscles, waste away.
The disease causes muscle weakness that gets worse over time. It can affect the arms, hands, legs, or feet as well as the muscles used to swallow or breathe. People with ALS have difficulty with things like walking, standing, reaching for the phone, or using a fork to eat because the motor nerves that allow movement are affected.
ALS is a terminal disease because there are no medicines or treatments that can stop the dying back of the nerves going to the muscles. A person with ALS will slowly get weaker and eventually die.
The progression and symptoms of ALS are different for everyone. Some people have long gaps of no progression. Some progress very slowly while others have faster progression. No matter how your disease progresses it is very important to get your care from an ALS interdisciplinary team. This will not only help prolong your life but improve your quality of life as well.
In the beginning, you may not notice the slight changes that occur. As time goes on the symptoms become more noticeable.
The first symptoms of ALS can vary between people. Symptoms that begin in the arms or legs are referred to as limb onset ALS. They include:
Muscle twitching (fasciculations) or muscle cramping
Weakness in the arms, legs, hands, or feet
Clumsiness or falling
Symptoms related speech and swallowing are known as bulbar onset ALS. They include:
ALS affects the motor nerves but does not cause any change to sensation, hearing, smelling, vision, or taste. Usually bladder and bowel function is normal.
The precise cause of ALS is not clear and very few risk factors for developing the disease have been identified.
It is estimated that about 30,000 people in the U.S. are living with ALS.
About 5,000-6,000 people are diagnosed each year.
Most patients with ALS are white, and more than half are men.
The average age at diagnosis is age 55.
Most patients are between ages 40 and 70 when diagnosed. However, ALS can occur at any age.
For unknown reasons, military service is a risk factor for future diagnosis. Several studies have documented an increased risk for veterans to be diagnosed with ALS. It is now accepted that a U.S. veteran who served between 1910 and 1982 is about 1.5 times more like to be diagnosed with ALS compared with someone who was never in the service. This risk does not depend on how, where, or when you served.
ALS is not contagious.
Forms of ALS
Most patients with ALS have sporadic disease. This means they did not inherit the disease from their parents and are unlikely to pass it on to their children. A small number of patients with ALS have familial ALS. This is an inherited form of ALS that is passed from parent to child. Some of these mutations have been identified and researchers are working on developing treatments with this information.
ALS can be difficult to diagnose because there is not one specific test to confirm that a person has the disease. Doctors use a physical exam to look for features that are characteristic of the disease. They also do blood tests to rule out disorders that can mimic ALS.
The work-up also includes these tests to rule out other conditions:
Because ALS can be so difficult to diagnose, it can take an average of 1-2 years to reach a diagnosis of ALS. Therefore, it is important to see a neuromuscular specialist. This type of doctor specializes in diseases of the peripheral nervous system and muscles.
Treatment of ALS
The treatment of ALS is divided into two categories:
Symptomatic treatments. These try to help any problems you may develop from the disease. This would include drooling, pain, cramping, difficulty sleeping, depression, increased crying or laughing (pseudobulbar affect), difficulty walking, swallowing, and breathing. You will be given durable medical equipment to help you keep as much independence as possible.
Veterans will also be provided grants to help with the following:
Adaptive wheelchair vans
Daily home health assistance
Skilled nursing care when appropriate
Modifying their homes to improve accessibility
Monthly financial compensation to help provide additional care
Modifying therapy. This means using medicines to slow down the disease. There are two medicines currently available that have been shown to slow down the progression of the disease. The effect of these medicines is mild.
Riluzole (Rilutek) is a medicine you take by mouth twice a day. It can have some effects on the liver and digestive system. Your healthcare provider should monitor your liver function tests before starting the medicine and every 3 months after starting it for the first year.
Endaravone (Radicava) is an IV medicine that must be given every day for 2 weeks. Then you are off the medicine for 2 weeks. This cycle is repeated for the rest of a person’s life.
Living with ALS
ALS is a life-changing diagnosis. Getting the right care requires medical experts from different fields working together. This is often called a multidisciplinary team. People with ALS who receive care from ALS centers that have a multidisciplinary team have a higher quality of life.
The life expectancy after diagnosis is 3 to 5 years. Half of people will live longer than 3 years after being diagnosed. However, some people with ALS will live longer. About 10% of patients will live for 10 or more years after their diagnosis.