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Amyotrophic Lateral Sclerosis (ALS)

by Katharina Cangl


What is ALS?

Stephen Hawking, Lou Gehrig, and Jim "Catfish" Hunter have two things in common. First, they were diagnosed with ALS. Second, their battles with the disease brought international awareness to this devastating condition.


But what is ALS in the first place? ALS (amyotrophic lateral sclerosis) is a condition that disrupts the communication between your nerves and your muscles. The nerves in question are called motor neurons. When you want to move a muscle, for instance, to walk, chew, or breathe, these neurons tell your muscles to make this voluntary movement.


ALS blocks motor neurons' ability to communicate with muscles, thereby preventing your muscles from moving. The symptoms progressively worsen, eventually leading to paralysis and the inability to perform basic tasks like breathing and chewing.


What are the stages of ALS?

As mentioned before, ALS is a progressive disease that can be divided into three stages.


In the early stage, the first noticeable symptoms include muscle cramps, muscle tightness, and slightly slurred speech. These symptoms are not alarming, so you and your primary care physician might not consider ALS immediately. This is why most people go undiagnosed until they experience the following symptoms:


  • Muscle weakness

  • Labored breathing

  • Difficulty speaking

  • Trouble swallowing

  • Muscle twitches


This early stage typically lasts for approximately 9 months to 1.5 years.


Next is the middle stage, which lasts a few months to a year. During this stage, your symptoms worsen and spread to different parts of your body. It is during this stage that you may undergo your first ALS procedure, such as the insertion of a feeding tube. These symptoms characterize the second stage of ALS:


  • Difficulty chewing or swallowing

  • Trouble speaking

  • Weight loss

  • Neuropathy

  • Muscle cramps

  • Difficulty breathing


As a result of gradually losing control over your body, you may also become prone to depression and anxiety. It is important to have a good support system in place to help you get the assistance you need.


Finally comes the late stage, typically reached after a little over two years. Initially, this stage results in the following symptoms:


  • Chewing is impossible; a feeding tube is needed

  • Speech is impossible; alternative communication methods may be required

  • Weak chest muscles can cause breathing issues and increase pneumonia risk

  • Poor breathing can lead to fatigue, confusion, and headaches; ventilation is required


As time goes on, your symptoms worsen further, causing the following:


  • Malnutrition from swallowing difficulties

  • Pulmonary embolism (lung blood vessel blockage)

  • Cardiac arrhythmias (irregular heart rhythms)

  • Pneumonia from food or liquid aspiration


At this point, your doctor’s primary focus will be to make you as comfortable as possible.


What causes ALS?

10 percent of the time ALS is inherited. The remaining 90% occurs randomly. 


ALS can be the result of environmental factors, like lead and mercury as well as physical trauma. This is why ALS often occurs in veterans.


Most people develop this disease between the ages of 40 and 70, however it can occur earlier. 60% of ALS patients in the US are male. It is unknown why, but 93% of people with this disease are caucasian. 


Is ALS treatable?

There is no cure for ALS. However, the progression of the disease can be slowed with medications such as Riluzole and Edaravone. Riluzole has proven very effective and increases life expectancy by 25%. Medication doesn’t reverse the symptoms that have already occurred, so it’s essential to start it as soon as possible.


Additionally, breathing therapy, physical therapy, and speech therapy can help keep you comfortable and independent as the disease progresses.


Unfortunately, the life expectancy for ALS is only between 3 and 5 years.


But remember, there are always people who beat the odds, like Stephen Hawking, who lived over 50 years with ALS. Thanks to modern medical research, new medications are being developed that have the potential to checkmate ALS once and for all in the future.


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