ALS Treatments and Medications

You probably know ALS (amyotrophic lateral sclerosis) as Lou Gehrig’s disease. He was a major league baseball player for the New York Yankees who had been one of the greatest sluggers, with 493 home runs, before being forced to retire in 1939 after developing ALS. On Jul. 4, 1939, Gehrig announced to a crowd of 62,000 fans that he was leaving baseball because of the disease. He died less than 2 years later, on Jun. 2, 1941. He was 36 years old.

Most people had never heard of ALS at that point, and it became forever associated with Gehrig. Lou Gehrig isn’t the only famous person to develop ALS. Stephen Hawking, the renowned physicist; Jim “Catfish” Hunter, another professional baseball player; and Senator Jacob Javits, are just a few other notable people who had ALS.

Today, ALS affects as many as 30,000 people in the United States, and approximately 5,000 new cases are diagnosed each year. Although it can appear at any age, most diagnoses are made in people between the ages of 40 and 70 years old. It is most common in those older than the age of 60.

In ALS, motor neurons progressively degenerate, or die, and as that happens the brain’s ability to send messages to the muscles via the spinal cord decreases until it eventually stops altogether. When the muscles stop receiving signals, they become weak and twitch. Eventually, they atrophy, or waste away. Voluntary muscle movement is used in speaking, chewing, running, writing, exercising, and most other activities requiring movement.

There are two main types of ALS:

A third type is found on the island of Guam, where it is known as lytigo-bodigo. Symptoms start when people are in their 40s and 50s. As the disease progresses, usually when the people are in their 60s, they develop shaking and lack of coordination, as is often seen in Parkinson’s disease. Finally, cognitive problems associated with dementia occur.

Everyone does not experience early ALS in the same way. Some people might have symptoms first in their arm or leg, called limb onset. Others might have initial symptoms in the face and throat muscles and have difficulty with speech or swallowing. When it appears in the facial muscles it is called bulbar onset. The early symptoms depend on which nerves and muscles are affected first. As the disease progresses, additional muscle groups are involved until all muscles in the body are affected.

For some people, the first sign of ALS is uncontrollable weeping or laughing—called the pseudobulbar affect. This symptom may continue throughout the progression of the disease.

Although anyone can develop ALS, there are a few risk factors:

Veterans are twice as likely to develop ALS than non-veterans. The reasons for this aren’t fully understood but it could possibly be because of exposure to lead, pesticides, and other environmental toxins. It is recognized as a service-connected disease by the U.S. Department of Veteran’s Affairs.

There is no single laboratory test to diagnose ALS. It is often diagnosed based on a detailed history of symptoms, observation by a physician, and by exclusion (ruling out other conditions). Your doctor will ask the following questions if ALS is suspected, according to Dr. Geraci:

Before making a diagnosis, your doctor might suggest tests to rule out HIV, human T-cell leukemia, West Nile virus, multiple sclerosis, post-polio syndrome, and Kennedy’s disease.

Some of the tests performed during the diagnostic process include:

As the disease progresses, your doctor should continue to perform neurological exams on a regular basis to note the progression of the disease and determine how quickly symptoms are worsening.

Most people with ALS die from respiratory failure, usually within one to five years of the onset of symptoms. About 10% of people survive for 10 years or more.

There are two medications—Tiglutik and Rilutek (riluzole)—used to treat the main symptoms of ALS. Other treatments and medications might be used to manage muscle cramps, excess saliva and phlegm, involuntary episodes of crying or laughing, pain, depression, sleep disturbances, and constipation.

In addition to medications, other therapies can be helpful:

Physical therapy can help enhance independence and safety. Low-impact exercise, such as walking, swimming, stationary bicycling strengthens muscles, improves cardiovascular health, and helps fight fatigue and depression. Physical movement also improves range of motion, prevents spasticity and minimizes the shortening of muscles.

Occupational therapy might be used to help with practical issues such as ramps, braces, walkers, wheelchairs. Therapists might also help with other assistive devices and provide ways to better manage daily tasks, such as dressing and hygiene.

Speech therapy can sometimes help with communication difficulties. Therapists might also introduce computer-based speech synthesizers and teach caregivers ways to communicate with their loved ones.

Nutritional support might be helpful when chewing and swallowing become difficult. Nutritional therapists can help caregivers plan and prepare meals that provide calories, fiber, and fluids while decreasing the chances of choking.

In the advanced stages, some people might require ventilators that inflate and deflate the lungs.

ALS research for more effective treatments is ongoing, including through clinical trials. Here are some resources to find a clinical trial near you:

Tiglutik and Rilutek (riluzole) are in a class of medications called benzothiazoles. This medication works by changing the activity of certain natural substances in the body that affect nerves and muscles, and helps slow down the progression of the disease and prolong survival up to 12 months. Although the mechanism for improvement is not fully understood, this medication does sometimes provide improvement in bulbar and limb function during the early stages of the disease, according to a report published in 2018. It also might extend life during the late stage of the disease.

Radicava (edaravone injection) is in a class of medications called antioxidants. It might work to slow nerve damage associated with ALS symptoms. This medication can only be taken intravenously and must be administered by a medical professional.

A third medication, Nuedexta (dextromethorphan HBr and quinidine sulfate), is in a class of medications called antiarrhythmics. It does not work on the muscle symptoms of ALS but can treat the pseudobulbar affect, sudden and frequent outbursts of crying or laughing, which may be seen in people with ALS.

This is not a complete list of side effects. Consult with your healthcare provider or pharmacist to learn more about each medication.

There are also some aids that can help you maintain your independence as long as possible according to the Muscular Dystrophy Association.

The ALS Association also suggests using electronic communication devices. These can be stand-alone or might work in conjunction with a computer, tablet, or cell phone.

Frequently asked questions about ALS

Some people first notice difficulty with simple, daily tasks, such as buttoning a shirt, writing, or turning a key in a lock.

Symptoms do not always follow the same pattern. They might appear in the limbs for some people and others might have initial symptoms in the face and throat. As the disease progresses, additional muscle groups are involved until all muscles in the body are affected.

For some people, the first sign of ALS is the pseudobulbar affect, uncontrollable weeping or laughing.

The FDA also approved the medicine Radicava in 2017. At the time, this marked the first new treatment in 22 years.