Straight Talk About ALS

ALS, or amyotrophic lateral sclerosis, is a disease that slowly destroys motor neurons – lessening muscle function. 

Motor neurons carry impulses from your brain to your spinal cord, and then from your spinal cord to muscles throughout your body. As motor neurons die, your brain loses the ability to tell your muscles what to do.

ALS is also known as Lou Gehrig’s disease, named for the legendary New York Yankee who was forced to retire from baseball due to his symptoms.

Who Gets ALS?

Anyone can get ALS. It usually strikes between age 40 and 70. The average age of diagnosis is 55. 

ALS is 20 percent more common in men. But with age, the gender gap lessens. More than 90 percent of ALS patients are Caucasian. Military veterans, particularly those in the Gulf War, are twice as likely to develop ALS. The reason is unknown.

What Causes ALS?

There are two types of ALS, sporadic and familial. Up to 95 percent of cases are sporadic. This means the disease occurs at random with no clear reason or family history. 

Up to 10 percent of cases are familial, which means the disease is inherited from a parent. Genetic testing can determine if you have inherited the genetic mutation, but the test results are not always straightforward. Genetic counseling (without testing) can help you better understand the disease and decide if testing is right for you. 

As motor neurons die, your brain loses the ability to tell your muscles what to do.

What Are the Symptoms?

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The earliest symptoms are usually muscle weakness or stiffness, but the signs are different for everyone. You might have trouble lifting a spoon, or your vocal pitch may change when you speak. Stumbling, dropping things, slurred speech and uncontrolled laughing or crying are other common early symptoms. 

ALS typically does not affect intelligence, thinking, seeing or hearing. Muscles of the bladder are also not affected, in most cases.

How is ALS Diagnosed and Treated?

Diagnosing ALS is difficult. In the early stages, ALS symptoms are very similar to other, more treatable diseases and disorders. Because there isn’t a test to confirm ALS, doctors typically run tests to rule out other diseases and disorders before making an ALS diagnosis.

“The current treatment for ALS usually involves treating symptoms and weakness and eventually the problems with patients’ breathing and swallowing,” says Bryan Ludwig, MD, of the Clinical Neuroscience Institute. Treatment for the symptoms is aimed at helping patients live with the disease. But there is no cure. 

“There is one medicine we use to slow the progression of the disease,” Dr Ludwig adds. Physical therapy and speech therapy may prolong independence and improve quality of life. 

Dr. Bryan Ludwig talks about treatment of ALS. Click play to watch the video or read the transcript.

How is ALS currently treated?

The current treatment for ALS usually involves treating their symptoms, their weakness, and then eventually the problems with their breathing, the problems with their swallowing... There is one medication out there at this point that we use to try to slow the progression of the disease, but there are not many treatments available beyond that and most of it involves just trying to support the patient as best we can as they continue through the progression of the disease.

 

As the disease progresses and your motor neurons die, you gradually lose the ability to speak, eat, move and breathe. Most people with ALS die from respiratory failure within three to five years.

What About Research?

Scientists are working vigorously to understand ALS, develop effective treatments and find a cure. Even though ALS affects a relatively small number of people, Dr. Ludwig said, “the way it affects patients is so profound and so devastating that we need to put all our energy and effort into looking for a cure.”

“Research is where it starts. There has been an enormous amount of research already, but this disease process is so complex and so variable across different patients that it makes it difficult to study.”

Dr. Bryan Ludwig talks about ALS research. Click play to watch the video or read the transcript.

Why is research so important in the continued and future treatment of ALS?

I think research is critical to trying to find a cure to ALS. Even though it's not a large number of patients, the way it effects patients is so profound and so devestating that we need to put all of our energy into looking for the cure. Research is where it starts, and there has been an enormous amount of research already, but this disease process is so complex and so variable across across the different patients that get it, it makes it difficult to study. And the amount of energy and resources needed to do it requires a lot of financial support, so it's a wonderful thing that's going on right now for the ALS Foundation to gain all of this financial support to hopefully push us into a new understanding of ALS.

 

The popular “Ice Bucket Challenge” in 2014 raised $115 million to fund ALS research efforts. The ALS AssociationOff Site Icon offers information about ongoing research and clinical trials.

Small Steps: Don’t Sweat Small Memory Lapses.
Forgetting a name or date is pretty common as you age, as long as it’s not disrupting your life.