ALS: Why is Everyone Doing the Ice Bucket Challenge?

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Is your Facebook feed flooded with videos of friends and celebrities doing the “Ice Bucket Challenge”? Everyone from Bill Gates to Mark Zuckerberg has dumped buckets of ice water over their heads in an effort to raise awareness for ALS. But how much do you know about the disease behind the cause?

What is ALS?

ALS, otherwise known as amyotrophic lateral sclerosis, or motor neuron disease, is possibly the most famous uncommon disease in the world today. First described in the nineteenth century by the great French pathologist, Jean-Martin Charcot, ALS achieved infamy first when Yankee Hall-of-Famer Lou Gehrig (after whom the disease is still often called “Lou Gehrig’s Disease”) contracted it, and later, theoretical physicist and cosmologist Stephen Hawking. Today, we are still looking for a cure, and people everywhere are dumping buckets of ice water over the heads as a spur to challenge others to do the same and donate to the cause.

How common is ALS?

ALS is fortunately rare, affecting no more than three out of every 100,000 persons worldwide. Most cases are what we call sporadic, meaning they do not arise in families. But about 10 percent of cases can be traced to a variety of genetic defects.

What are the symptoms of ALS?

The major initial symptoms include slowness of gait and other movements, spasticity, weakness, muscle fasciculations, and difficulty with speech and swallowing.

How is it diagnosed?

The diagnosis of ALS is made by a clinical evaluation including medical history intake and physical examination. The diagnosis is confirmed by electrodiagnostic testing of nerve and muscle function, which shows the characteristic pattern of motor neuron dysfunction. Many, often less common, disorders can mimic some of the features of ALS, and electrical testing can usually distinguish these. In some patients, when the diagnosis is still in doubt, MRI imaging of the brain and spinal cord and a lumbar puncture (otherwise known as a spinal tap) to obtain cerebrospinal fluid, can be useful in ruling out other confounding diagnoses.

What causes ALS?

The initial symptoms are caused by destruction of the motor neurons which are the nerves that control movement and all of our muscular processes, but other brain regions may be involved as well.

Age, a family history of ALS, and tobacco smoking are all recognized risk factors. The underlying cause of the motor neuron destruction is not understood, although many theories have been proposed and are currently being studied. Until we get a better handle on why this devastating process begins and progresses, it will be nearly impossible to find a cure.

How does ALS progress?

Over time, people with ALS become disabled by muscular weakness and atrophy, and progressive difficulties with swallowing can lead to aspiration of mucus into the lungs and resultant pneumonia, as well as dehydration and malnutrition. Eventually, breathing can become impossible, and respiratory failure is the most common cause of death.

How long can people live with ALS?

We used to think that all patients pursued a relentless, rapidly downhill course, but we now know that the trajectory of the disease, while inevitably one of decline, varies greatly from patient to patient. The average patient survives three to five years from the time of diagnosis, but a subset of patients—about one in 10—lives beyond 10 years. And rare individuals, like Hawking, can live beyond 20 years.

 Are there any treatments?

Only one drug, riluzole, has been shown to slow the inevitable decline of ALS, and the effect is a small one. Its precise mode of action is not well understood. Treatment at present is therefore almost entirely symptomatic. Careful attention to respiratory function is key, and ultimately, invasive ventilation with a breathing machine becomes a necessity if the patient opts for aggressive intervention. As the disease progresses, feeding tube placement can be offered to sustain adequate nutrition.

Many medications are available to help thin and reduce the thick mucus that can obstruct breathing. Many patients with ALS experience severe pain from their muscle spasticity and spasms, and medications can be helpful here as well. Multidisciplinary clinics specializing in ALS, which bring together neurologists, physical therapists, mental health workers, dieticians, nurses and respiratory therapists have been shown to prolong life, improve quality of life, and provide important psychosocial support.

 What can I do?

ALS is a terrible disease, but thanks to the efforts of dedicated researchers and the millions of people who don’t mind a bath of ice water, progress is being made. Although a cure is not yet in sight, aggressive and compassionate care can help improve and prolong the lives of those unfortunate enough to have been stricken.

To learn more about ALS, donate to the cause, or read about the Ice Bucket Challenge, visit www.alsa.org.

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The One Medical blog is published by One Medical, an innovative primary care practice with offices in Boston, Chicago, Los Angeles, New York, Phoenix, the San Francisco Bay Area, Seattle, and Washington, DC.

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