ALS Life Expectancy

Amyotrophic lateral sclerosis (ALS) life expectancy varies, but someone diagnosed with ALS is expected to live from two to five years. Some people with ALS do live much longer, however. About 10% of those with the condition will live 10 years, and 5% will live for 20 or more years.

There is no cure for ALS, but there are some treatments that may slow the loss of physical function and improve the quality of life for people impacted by it.

This article briefly explains what an ALS diagnosis means, and what you can expect in terms of ALS progression and its complications. It discusses medication and other treatment interventions.

What Is ALS?

ALS is a neurodegenerative condition affecting the brain, spinal cord, and muscle activity. It is also known as Lou Gehrig's disease, named for a baseball player who died of ALS in 1941.

There are different classifications of neurodegenerative disorders, with ALS defined as a transactivation response DNA binding protein 43 (TDP-43) proteinopathy. This describes the abnormal function of proteins that lead to symptoms including muscle weakness and atrophy.

ALS also has additional subtypes, depending on how it evolves in the body and where impacts are located originally. For example, bulbar ALS is associated with head and neck weakness and less positive outcomes (life expectancy may be two years or less).

Spinal onset impacts begin in the limbs, with some evolving evidence that these might be considered two different diseases for treatment purposes.

The two types arise from different genetic pathways, but genetics is only one factor in the development of ALS. More study is needed to fully understand what causes ALS.

How ALS Affects the Body

Early symptoms can include:

• Muscle stiffness or cramping
• Muscle spasms with limited control
• Swallowing problems (dysphagia)
• Persistent and progressive difficulty speaking clearly, including slurring words for no reason

Beyond the first symptoms, ALS continues to progress. People typically have trouble lifting things, or using their hands for tasks. Their balance and gait will change, with increased risks of trips and falls. Work, school, home, and hobbies become a challenge. Over time, the effects of ALS include:

• Difficulty standing and walking
• Inability to get out of bed
• Trouble with eating and nutrition
• Challenges with words and speech
  

Death from ALS is usually caused by respiratory failure, due to the muscles that allow for breathing becoming paralyzed. Both non-invasive and invasive ventilation can ease breathing, but ALS will continue to progress.

It's also common for people living with ALS to experience anxiety and depression, because they remain aware of their condition. It's more rare for people to experience dementia-like symptoms.

Some people don't fall neatly into either spinal or bulbar subtype and experience other symptoms. About 5% of people have difficulty breathing, morning headaches, mood changes, and sleep disruptions with their ALS.

ALS Life Expectancy

The prognosis for ALS is poor. ALS is a progressive condition that is fatal within five years for 80% of the people impacted by it.

Factors That Affect Life Expectancy

ALS can be considered either familial (a genetic influence) or sporadic, meaning there is no known reason. Studies do suggest that environmental factors linked with sporadic ALS cases include:

• Toxic exposure
• Infectious agents
• Viruses
• Physical trauma
• Poor diet
• Smoking

For example, a biotoxin called Beta-methylamino-L-alanine (BMAA) is found in plants, food, and seafood. Exposure has been associated with certain types of ALS.

Researchers increasingly find evidence that specific genes are involved with specific types of ALS, with gene C9orf72 the most common culprit implicated in up to 40% of cases in families. It's also associated with 6% of sporadic cases. Other factors include:

• Age: People tend to be diagnosed between the ages of 40 and 70, with an average of 55.
• Sex: People assigned male at birth are more at-risk, but there's less difference with increased age.
• Race: White people are diagnosed with ALS more often than Black people.

Any of these factors may play a role in a specific ALS diagnosis and affect life expectancy. Another factor is the time it takes to receive an accurate diagnosis, which averages 11 months after the first symptoms. An early diagnosis can affect treatment and prognosis.

Treatments

While there is no cure for ALS or ways to stop the progression of the disease completely, there are some treatments that may improve life expectancy and quality of life.

Medication

Medications used to treat ALS include:

• Rilutek (riluzole) has been in use since 1995 and has been shown in randomized double-blind clinical trials to prolong life expectancy for approximately two to three months, but it did not relieve symptoms. This medication also goes by the name of Tiglutek when in a liquid form and Exservan when used as an oral film, both of which are easier for people with problems swallowing.
• Radicava (edaravone) is a free-radical scavenger medication that was approved in 2017. In the phase 3 trial that led to its licensure, it lessened the decline in physical functioning by 33% in a very select group of people who were in the study. However, researchers caution that those results may not be generalizable to everyone with ALS.
• Nuedexta (dextromethorphan hydrobromide/quinidine sulfate) may help improve bulbar motor function (important for speech and swallowing). In particular, it may help to better speech control with fewer pauses, but more study is needed.
• Relyvrio (sodium phenylbutyrate/ taurursodiol), was approved in 2022. In a randomized, double-blind, placebo-controlled trial, patients who received Relyvrio experienced a slower decline in physical functioning than those who received a placebo. Relyvrio can be taken orally or through a feeding tube.
• Qalsody (tofersen) was approved in 2023 for the treatment of adults with ALS who have a mutation in the superoxide dismutase 1 (SOD1) gene. In a randomized, double-blind, placebo-controlled trial, patients who received Qalsody experienced less nerve injury and neurodegradation than those who received a placebo. Qalsody was approved under the accelerated approval pathway and will undergo continued clinical trials to confirm its clinical benefit.

Noninvasive Ventilation

The use of noninvasive ventilation (NIV) is a cornerstone of ALS treatment. The primary mode of NIV is positive-pressure ventilation, which is basically breathing through a mask that fits over the mouth and nose.

Ultimately, more invasive ventilation through a breathing tube down the throat or via a hole in the front of the neck (tracheostomy) may become necessary.

Diaphragmatic Pacing

The FDA has approved the NeuRx diaphragm pacing system, which uses implanted electrodes to cause the diaphragm to contract and create a breath.

This device is also used for people with spinal cord injuries. If severe respiratory failure has not yet occurred, this device allows people to breathe without a ventilator for up to four hours per day.

Even though the FDA has approved its use, some healthcare providers question its usefulness for ALS. There are specialists who consider it can be harmful.

Percutaneous Endoscopic Gastronomy (PEG)

A feeding tube is placed into the stomach and leads out of the body through the abdominal wall. Although studies have failed to show a consistent impact on survival time, it is considered to be a safe nutritional protocol in patients with a functional gastrointestinal system.

Because a PEG tube bypasses the mouth and throat, the risk of choking and inhaling food and medications into the lungs is minimized. Liquid tube feedings, medications, and water can all be given by PEG tube.

Complications

Complications can arise from the neurological damage of the disease process or from treatments:

• Choking, due to impaired swallowing
• Shortness of breath, due to the atrophy of breathing muscles
• Risk of falling, due to weakness and atrophy of the muscles
• Nutritional deficiencies, due to trouble eating
• Infections, from medical devices like ventilators, feeding tubes, and urinary catheters
• Anxiety and depression, related to an unpredictable and terminal illness
• Inappropriate emotional outbursts, related to damage to that part of the brain

Cognitive changes and dementia also can occur as ALS progresses.

Improving Quality of Life

People living with ALS may benefit from several types of therapy. These can include:

• Physical therapists can help a person with ALS to do physical exercise and stretching that strengthens functioning muscles, decreases spasticity, and doesn't overwork their muscles. The goal is to preserve the strength and flexibility they still have.
• Occupational therapists can help with assistive devices that improve functionality and increase independence, such as ramps, braces, walkers, and wheelchairs.
• Speech therapists can help teach adaptive strategies to speak more loudly and more clearly. As speech deteriorates, there are adaptive devices like computer-based speech synthesizers using eye tracking technology and even brain-computer interfaces that can control equipment like wheelchairs using only brain activity.

Summary

ALS life expectancy remains comparatively low, with most people living just a few years after diagnosis. Yet many factors, including the type of ALS (genetic or sporadic) and other factors and influences (environmental exposure, age, military service) can affect the prognosis.

Advances have been made in recent years in the support and treatment for ALS. Researchers have also made advances in understanding the disease process of ALS, which will hopefully lead to new treatments. Quality of life interventions also are important for people living with ALS.

Talk to your healthcare provider about what you can do to live more comfortably with ALS and care for those who are living with its challenges. Be aware that support groups and other resources can help with navigating this difficult illness.