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.

What is ALS?

ALS stands for amyotrophic lateral sclerosis, a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord, according to the ALS Association. Motor neurons start in the brain and are sent to the spinal cord, which sends them to muscles throughout the body. These motor neurons are responsible for voluntary muscle movement and muscle control.

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:

• The most common type is called sporadic and it accounts for 90% to 95% of all cases. It can affect anyone, anywhere, at any time.

• Familial ALS accounts for 5% to 10% of all cases. This type is inherited. People with this type have a 50% chance of having a child with the gene mutation who could develop the disease.

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 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.

How is ALS diagnosed?

“ALS is typically diagnosed by neuromuscular neurologists,” according to Anthony Geraci, MD, Director of Neuromuscular Medicine at Northwell Health. “Early signs are very broad and include subtle weakness of a limb such as a mild foot drop that causes you to occasionally trip, or decreased dexterity of the fingers, for example when typing on a keyboard. Weakness can also start in larger muscle groups, and the patient could first notice trouble getting out of a chair or raising their arms to brush their hair. Some patients have a first symptom of slightly slurred speech or coughing when swallowing liquids. Muscle twitches and cramps can also be an early sign.”

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.

• Age: The disease typically affects people between the ages of 40 and 70

• Gender: Men are slightly more likely to develop ALS, however as age increases, this difference lessens

• Ethnicity: People who are Caucasian and non-Hispanic are more likely to get the disease than other ethnicities.

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:

• Do you trip when you walk?

• Do you have trouble rising from a chair?

• Do you have trouble grasping a pen and writing?

• Has your handwriting changed?

• Have you or those close to you noticed slurred speech?

• Have you been experiencing muscle twitching or cramps lately?

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:

• Electromyography (EMG) to detect electrical activity of muscle fibers

• Nerve conduction study (NCS) to measure electrical activity of nerves and muscles

• Magnetic resonance imaging (MRI) to rule out spinal cord tumor, herniated disk in the neck, syringomyelia, or cervical spondylosis

• Blood and laboratory tests might be used to rule out other diseases

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.

ALS treatment options

There is no cure for ALS and there are no effective treatments that can halt or reverse the progression of the disease.

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:

• ALS Association and Northeast ALS Consortium

• Clinicaltrials.gov

• Center Watch

• Centers for Disease Control and Prevention

ALS medications

There are currently two medications approved by the U.S. Food and Drug Administration (FDA) for the treatment of ALS.

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.

What is the best medication for ALS?

Only three medications have been approved by the FDA for the treatment of ALS symptoms. Your doctor will work with you to determine what combination of medications is best for you.

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