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  • Roberta Flack, known for songs such as “Killing Me Softly,” has been diagnosed with ALS.
  • Her spokesperson said this week that the disease has left her unable to sing.
  • ALS causes nerve cells that control voluntary muscles to stop working and die.

Grammy-winning musician Roberta Flack has been diagnosed with amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig’s disease.

This progressive nervous system disease has made it “impossible [for her] to sing and not easy to speak,” her spokesperson said in a statement sent to CNN on Monday. “But it will take a lot more than ALS to silence this icon.”

While ALS is a relatively rare disease, notable athletes, entertainers, politicians, and thousands of Americans have been affected by this disease.

Awareness of ALS has increased in recent years, in part due to the Ice Bucket Challenge, which helped raise millions of dollars for research, according to the ALS Association.

ALS causes nerve cells that control voluntary muscles to stop working and die. This can lead to weakness and paralysis in the muscles of the hands, arms, legs or feet, as well as in the muscles that control speech and swallowing.

There is no cure for this fatal disease.

ALS is a progressive nervous system disease that affects nerve cells in the brain and spinal cord, causing the cells to deteriorate and eventually die.

The disease specifically affects motor neurons, which control muscles that govern voluntary movements. When motor neurons die, the brain is no longer able to activate those muscles.

This can cause specific muscles to become weak and leads to paralysis. As a result, people may lose the ability to move, eat, speak and breathe.

In 2017, there were an estimated 18,000 to 32,000 cases of ALS in the United States, according to the Centers for Disease Control and Prevention.

The most common initial symptom of ALS is muscle weakness — which is usually painless.

Dr. Stephen Goutman, director of the Pranger ALS Clinic and associate director of the ALS Center of Excellence at Michigan Medicine in Ann Arbor, said muscle weakness typically starts in one region of the body and then spreads to other areas.

Symptoms of ALS generally start gradually, but can vary among people, depending on which motor neurons are affected. Symptoms include:

  • weakness or fatigue in the legs, feet, arms or hands
  • tripping and falling
  • dropping objects or other hand clumsiness
  • muscle cramps or twitches
  • slurred speech
  • swallowing difficulty
  • uncontrollable crying or laughing

Goutman said ALS doesn’t just affect the motor neurons. Many people with the disease also experience cognitive changes such as decision-making difficulties, personality changes or irritability.

Due to the degeneration of the motor neurons, ALS eventually affects chewing, swallowing, speaking, and breathing.

“Because of the progressive nature of the disease, it is unfortunately fatal, mostly because of the difficulties with breathing,” said Goutman.

On average, people with ALS live from 3 to 5 years after they develop symptoms, although some people live longer.

ALS doesn’t usually affect bladder control, vision or other senses because these are controlled by different types of neurons.

Certain factors increase the risk of developing ALS, including:

  • Heredity. About 5-10% of ALS cases occur within families and are caused by genes known to be linked to ALS.
  • Age. ALS typically occurs in people between 55 and 75 years of age, although cases can occur in younger people.
  • Sex. Men have a slightly higher risk than women of developing ALS. Familial ALS occurs equally often in men and women.
  • Genetics. Some studies suggest that 60% of the risk of non-inherited ALS is due to genetic factors. People who have these gene variations may be more likely to develop ALS.

In addition, research has found a possible link between ALS and certain environmental factors. More research is needed to fully understand these connections.

  • Smoking. Some studies indicate that smokers have a higher risk of developing ALS than people who never smoked.
  • Environmental exposures. Research suggests a possible link between ALS risk and exposure to heavy metals (such as lead and mercury), chemical solvents, radiation, pesticides and other toxins.
  • Military service. Studies indicate that people who served in the military have a higher risk of developing ALS compared to those who did not serve in the military.
  • Occupation. Research suggests that the ALS risk is higher among people with certain jobs, such as construction, manufacturing, mechanical or painting.
  • Contact sports. Studies have found a higher risk of ALS among people who play competitive contact sports that involve repetitive head and neck trauma, such as hockey, football and soccer.

Three drugs have been approved by the Food and Drug Administration (FDA) to slow down the progression of ALS:

  • Riluzole (brand name Rilutek)
  • Edaravone (brand name Radicava)
  • Sodium phenylbutyrate and taurursodiol (brand name Relyvrio)

“These medications essentially do the same thing, in that they slow down the progression of the illness and probably extend the lifespan a little bit,” said Dr. Adam Quick, a neurologist at The Ohio State University Wexner Medical Center in Columbus.

“These drugs work in slightly different ways,” he added, “so the hope among ALS specialists is that we may be able to use all three medicines at the same time in a particular person, and slow down the progression of the disease more than with an individual medicine by itself.”

These medications may also work better when given earlier in a person’s illness. However, one of the challenges with ALS is that it takes about a year after symptoms first appear for someone to get diagnosed, said Goutman.

But “our hope is if we can shorten the time to diagnosis, we can eliminate some of the frustration experienced by patients, and also ideally start therapies sooner,” he said.

Currently, there’s no way to screen widely for ALS, so physicians rely on people to reach out if they have possible symptoms.

Goutman encourages people to contact their doctor if they experience any concerning changes in their daily functioning — and to not assume that these changes are just the result of aging.

None of the FDA-approved ALS drugs stop or reverse the progression of the disease, but other treatments are available that can provide relief from symptoms and improve people’s quality of life.

“People get excited about new medications,” said Quick, “but a lot of the really helpful things right now are just supportive care.”

For example, physicians may prescribe medications to provide relief from symptoms that commonly occur in people with ALS, such as muscle cramps, fatigue, pain, depression, and sleep problems.

In addition, therapies are available to help improve a person’s physical functioning, including:

  • Breathing care
  • Physical therapy
  • Occupational therapy
  • Speech therapy
  • Nutritional support

Goutman said noninvasive ventilation is very effective for managing respiratory symptoms. This treatment uses a mask or other interface to help people breathe, instead of requiring a surgical opening in the trachea.

“Noninvasive ventilation actually extends life, maybe better than some of the drugs that we have,” he said. “It also improves quality of life.”

Quick said in addition to breathing support, lifestyle changes such as improved nutrition and regular physical activity can improve a person’s lifespan and comfort.

“These kinds of non-pharmacological treatments are really helpful,” he said. “We try to emphasize these in our clinic, because I think they are as important of an aspect of managing ALS as the pharmaceutical options.”

He also thinks that people with ALS do better when they are seen in a multidisciplinary ALS clinic, because they can visit with a physician, physical therapist, occupational therapist, and dietitian all in one place.

Thanks to increased funding for ALS research, scientists are developing and testing other potential drugs for ALS. “So I think there’s a lot of hope on the horizon,” said Goutman.

These medications will first need to be tested in clinical trials to see how much they benefit people with this disease.

Goutman and his colleagues are also trying to identify environmental risk factors for ALS, work that could one day help reduce people’s risk of developing the disease.

In a study published earlier this year, they found that people with ALS reported higher workplace exposure to metals, particulate matter, volatile organic compounds and combustion pollutants before their diagnosis, compared to people without ALS.

These exposures occur in “production” jobs such as manufacturing and welding.

“If we can identify who’s at highest risk of getting ALS, we can start thinking about what behavioral interventions we can do to lessen their risk,” said Goutman.

Additional work is needed before scientists can pinpoint how much ALS risk is linked to certain levels of exposure to different toxins, he said.

Quick encourages people with ALS to take part in clinical studies, and others to donate to this kind of research.

“At Ohio State, we really value research studies and we try to get our patients actively enrolled in them as much as possible,” he said, “because we really want to do better for people with ALS — as a medical community and as a society.”