May Is ALS Awareness Month
By Teresa Otto, MD
“I consider myself the luckiest man on the face of the earth,” baseball great Lou Gehrig said when he retired in 1939. Even though he had just been diagnosed with ALS, a disease commonly called Lou Gehrig’s Disease, Gehrig was grateful for a great career and a fully led life.
Gehrig died about two years later, at age 37.
With ALS Awareness month in May, let’s look at the disease’s symptoms, diagnosis, treatment options, and ways to save on ALS medications with prescription drug cards.
ALS—A Disease of Several Names
ALS is short for amyotrophic lateral sclerosis. That’s a mouthful but descriptive. Broken down to its Latin roots, amyotrophic means no (a) muscle (myo) nourishment (trophic). Without nourishment, the muscle atrophies or shrivels. “Lateral” designates the area on the spinal cord the disease affects. “Sclerosis,” or hardening of the tissue, tells you what the disease does to the spinal cord.
ALS causes progressive skeletal muscle weakness due to nerve damage in the spinal cord and brain. If the nerves heading to your muscles fail to work, your muscles won’t get any signal to contract or relax. The muscles just won’t work. This leads to progressive extremity weakness and breathing difficulties.
ALS is also called Lou Gehrig’s disease. Diagnosed on his birthday, June 19, 1939, Gehrig delivered his famous “luckiest man” line in his home ballpark, Yankee Stadium, during his retirement speech. He became the face of the disease and was honored by having his jersey number retired.
Lou Gehrig Day
In 2021, the Major League Baseball network named June 2 Lou Gehrig Day. Each year, it’s a day to remember Gehrig and bring awareness to ALS.
Why June 2? Gehrig became the Yankee’s starting first base player on June 2, 1925. And he passed away on June 2, 1941.
While ALS is commonly called Lou Gehrig’s disease, famous people who have had ALS include: physicist Stephen Hawking, New York State Attorney General, and U.S. Senator Jacob Javits, actor David Niven, “Sesame Street” creator Jon Stone, and baseball Hall of Famer Jim “Catfish” Hunter.
ALS Awareness Month
Although Lou Gehrig is specifically honored in June, May has been ALS Awareness month for the last 30 years.
May brings awareness to all affected by ALS, not just those known on the world’s stage. The entire month serves to shine a light on people with ALS from all walks of life.
According to the ALS Association, “Every 90 minutes, someone is diagnosed with the disease, and someone passes away from it.” Data compiled from the National ALS Registry’s 2015 entries identified more than 16,000 people in the United States living with ALS that year.
Although the disease is rare, it is devastating and has no cure. At least not yet. The ALS Association is on a mission to find a cure. Through awareness and the financial support it generates, the ALS Association funds research and advocates for people with ALS year-round.
Who Gets ALS?
The article published from 2015 data provides some answers to that question.
ALS primarily affects those over age 40. The 70 to 79 age group has the highest incidence of ALS, with 20 of 100,000 people affected. Overall, the prevalence is about 5 per 100,000 people.
The data from 2015 shows some differences in ALS cases across the population, though:
- Men are affected more often than women (6.4 to 4 per 100,000 people).
- ALS prevalence in white people vs. black people is 5.4 to 2.3 per 100,000 people.
- Prevalence varies by U.S. region — Midwest has the highest prevalence (5.5 per 100,000 people), and the West has the lowest (4.4 per 100,000 people)
Nine out of 10 people diagnosed with ALS have no family history; 10% of people have familial or inherited ALS. Apart from the inherited cases, ALS has no known cause but may be triggered by genetics and environmental factors.
Researchers continue to search for environmental factors. It seems to be sporadic, although military veterans have a higher chance of contracting ALS than the general population. As such, the Department of Veteran Affairs regards ALS as a service-connected disease. Researchers continue to look for factors including exposure to toxins, heavy metals, viruses, and strenuous physical activity that may explain the higher prevalence of ALS in military veterans.
Symptoms of ALS
Symptoms generally begin in muscles in your hands, arms, feet, and legs. The onset of symptoms can be quite gradual and include:
- Muscle twitches or cramps in your arms and tongue
- Difficulty speaking, trouble swallowing or choking on food
- Hand weakness with difficulty holding on to things
- Weakness in your legs and feet with difficulty walking or climbing stairs
- Tripping, stumbling and falling
- Difficulty doing everyday activities — dressing, eating, bathing
Within the last 10 years, healthcare providers and family members have noted about half of the people with ALS show mental changes:
- Behavior changes include inappropriate laughing, crying, and yawning
- Impaired thinking
If you or your loved ones notice these symptoms, a visit to your primary care provider is in order. No one test can determine whether you have ALS, although a recent study shows promise. Researchers found a protein normally located in nerve and brain cells in skin cells in people with ALS. This may serve as an easy way to make an early diagnosis if additional studies confirm the findings.
Until that time, though, a thorough neurologic exam and testing are in order. Tests rule out other causes of your symptoms and may include:
- Blood tests to check for thyroid disease or abnormal calcium levels
- Urine tests to check for heavy metals or toxins
- X-rays or MRIs looking for spinal cord or nerve damage
- Studies looking at nerve impulse conduction
- Collecting a muscle or nerve sample for examination
- Spinal tap to look for abnormalities in the fluid that bathes the brain and spinal cord
The thinkALS tool on the ALS Association website helps primary care physicians and neurologists diagnose ALS sooner, start treatment earlier, and hopefully slow disease progression.
Treating ALS symptoms involves a team effort with your physicians and healthcare providers using a multi-pronged approach. Physical therapists can help strengthen your muscles with gentle exercises. Occupational therapists recommend assistive devices that help you walk and maintain your balance. Nutritionists help you maximize your calorie intake with foods that are easy to chew and swallow.
Speech therapists help with swallowing and voice exercises. They can also assist with voice banking, a way to create a “personalized synthetic voice” for future use should you lose your ability to speak.
Many people seek counseling or pastoral care.
The Federal Drug Administration (FDA) has approved several disease-modifying drugs for use in people with ALS. While they do not cure the disease, they can prolong your life and/or reduce the disease’s symptoms:
- Radicava (edaravone): scavenges free radicals (trash generated from a cell’s energy production) to help keep nerve cells healthier
- Rilutek (riluzole): stops the release of a cellular chemical that’s harmful to nerve cells
- Tiglutik (riluzole): a thickened, liquid form of riluzole
- Exservan (riluzole): formulated as a film placed on your tongue. It can be used for anyone unable to swallow.
Medications play a role, too, in alleviating symptoms. These are some of the more commonly used drugs:
How to Save Money on Your Prescriptions
Getting an ALS diagnosis can be overwhelming, confusing, and heartbreaking. Visits to specialists and therapists fill your calendar. Medication costs add up quickly.
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Prognosis of ALS
ALS is a fatal disease, with respiratory failure as the usual cause of death. The average life expectancy after diagnosis is three years, but there are plenty of examples of people who threw these statistics a curveball: 20% of people will live longer than five years, 10% longer than 10 years, and 5% longer than 20 years.
And then there’s Stephen Hawking. He lived 55 years after his ALS diagnosis and, in that time, made countless contributions to science and our understanding of the universe.
The ALS Association currently supports 162 ALS research projects in 11 countries worldwide. Researchers are investigating everything from possible environmental triggers to new medications.
Research is also geared toward developing assistive devices such as speech-generating devices, eye gaze control systems, and brain-computer interface systems that allow a person to control a computer with his or her brainwaves.
Ways to Show Your Support During ALS Awareness Month
The ALS Association states its “mission is to discover treatments and a cure for ALS, and to serve, advocate for, and empower people affected by ALS to live their lives to the fullest.”
To show support for people with ALS and the work the ALS Association does on their behalf, you can:
- Volunteer at a local chapter.
- Sign up for a fundraising Walk to Defeat ALS. Annual walks have generated $276 million used entirely for advocacy, research, and services for people with ALS.
- Advocate by asking your congressional representatives to fund ALS research.
- Donate to the ALS Association on its website.
Teresa Otto, MD, is a freelance medical writer on a mission to inform readers about the positive impact of good nutrition and a healthy lifestyle. She is a retired anesthesiologist who practiced in Billings, Montana, for most of her career. She graduated from the University of Washington School of Medicine in Seattle and did her anesthesia residency and fellowship at New York University and Columbia-Presbyterian in New York.