ALS awareness: What happens as muscles deteriorate?

Beloved actor Eric Dane was famously known for his role in a popular medical drama. He experienced a dramatic change in his own life when he was diagnosed with amyotrophic lateral sclerosis (ALS), also referred to as Lou Gehrig’s disease. Dane recently succumbed to his illness, but his ALS diagnosis brought awareness that will have a lasting impact.

ALS is a neurological disorder where nerve cells deteriorate over time and cause muscle weakness. The cause of ALS is still undetermined, but some genetic, environmental and lifestyle factors, even possibly smoking cigarettes, may play a role.

Three important facts to know about ALS:

1. Your thumb is the most impacted finger

Muscle weakness slowly spreads throughout your limbs when you have ALS. People with ALS will experience muscle weakness that may include or progress to involve their hands, arms, legs and feet. The muscles that control swallowing, speaking and breathing will also experience muscle weakness.

Split hand syndrome is often associated with ALS. This syndrome characterizes how the hand looks when ALS causes the thumb muscles to become weaker and thinner as they deteriorate. Typically, you will notice the thumb is weak from the loss of muscle and outstretched from the rest of the hand due to weakness. Researchers have found that ALS typically affects the thumb side of the hand before the pinky side of the hand.

“A study found that 70% of people had split hand syndrome when they were diagnosed with ALS,” says Dr. David Randall, a neuromuscular medicine physician at Advocate Health Care. “There are many potential causes of split hand syndrome, so its presence alone doesn’t necessarily equate to an ALS diagnosis. Plus, there are still a number of people with ALS who don’t have the syndrome.”

He adds that evaluation and testing will determine if the thumb weakness is from ALS or a different cause.

2. ALS progresses differently for everyone

Some people with ALS have a more rapid progression of symptoms than others. ALS symptoms in general can appear in varying orders.

“Every person’s experience with ALS looks different,” Dr. Randall says. “People with ALS often only live three years after receiving their diagnosis. Others may live five years or more than 20 years. Unfortunately, we can’t predict that. We can, however, try to slow the progression of the disease with medications, treat the symptoms and preserve your muscle strength for as long as possible, such as with physical therapy.”

3. ALS deaths are often due to respiratory failure

In addition to affecting your limbs, ALS also weakens the bulbar muscles, the muscles you use to speak, swallow and breathe.

“People often pass away from ALS when the diaphragm and chest muscles weaken and therefore breathing becomes incredibly difficult,” Dr. Randall says. “When you breathe in, your chest muscles contract. If you don’t have control over these muscles, your breathing becomes less effective at moving air and can lead to respiratory failure.”

Your chest muscles also control your ability to cough. Coughing is an important way that your body clears irritants from your airway. If you are unable to do so, this can lead to severe respiratory infections that lead to respiratory failure.

What to do if you think you have ALS

If you notice ALS symptoms, such as progressive weakness in your arm, leg or bulbar muscles, abnormal muscle thinning or muscle twitching, schedule an appointment with a neurologist. They will review your symptoms and run any necessary tests. There isn’t a single test that detects ALS. Instead, testing helps rule out other conditions that may mimic the presentation of ALS.

“Your doctor will evaluate all your symptoms through a careful review of your medical history, a detailed neurological exam and testing,” Dr. Randall says. “The testing typically consists of blood tests, imaging and a nerve conduction study. Additional testing may be necessary based on the results.”

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