All about ALS, the rare disease behind the ice bucket challenge

Despite several clinical trials on animals, only one approved drug can treat the infected

 
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By Jyotsna Singh
Published: Tuesday 26 August 2014

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The ice bucket challenge that saw celebrities pouring ice and water over themselves and US president Barack Obama reach for his wallet to make a donation was started by the ALS Association in the US to raise awareness about the disease. The initiative is also aimed at collecting donations that can be used to eradicate amyotrophic lateral sclerosis (ALS) that affects nearly 30,000 US citizens.

The challenge dares nominated participants to be filmed while a bucket of ice-cold water is being poured on them or donate $100 to the association. The person posting the video tags others and they have to dare to do the same.

Although pouring ice or ice-cold water on one's body has nothing to do with treatment of ALS, the videos of celebrities taking up the challenge, posted on social media, have created a buzz about the three-week event. In India, Bollywood celebrities like Sonakshi Sinha, Akshay Kumar, Riteish Deshmukh, Sunny Leone, Sidharth Malhotra and Bipasha Basu joined the race.

Despite all the videos going viral and many talking about it, the mystery around ALS remains; not many know what the disease is all about.

What is ALS?
ALS is a neurological disease that affects functioning of muscles adversely. In later stages, the disease may lead to complete paralysis.
 
Also called Lou Gehrig's Disease, ALS affects nerve cells in the brain and the spinal cord. Motor neurons that, under normal condition, travel from the brain to the spinal cord and from the spinal cord to the muscles throughout the body start degenerating. The progressive degeneration eventually leads to the death of these neurons and in their absence, the ability of the brain to control muscle movement is lost. With voluntary muscle action gradually affected, patients eventually suffer paralysis.
 
The condition does not affect all the muscles of the body. It affects motor neurons that provide voluntary movements (like a man’s effort to climb up and down the stairs) and power to muscles. Since muscles in the heart and digestive system are of a different kind and their function is not voluntary, but automatic, they do not get affected by ALS.
 

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What does amyotrophic lateral sclerosis mean?
The word a-myo-trophic has been derived from Greek language. "A" means no or negative. "Myo" refers to muscle, and "trophic" means nourishment. In short, it means "No muscle nourishment." When a muscle has no nourishment, it wastes away.
 
“Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located.
 
As this area degenerates, it leads to scarring or hardening ("sclerosis") in the region.
 
Early symptoms of ALS include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. The limbs too become thinner.
 

Unfortunately, the magnitude of ALS is not completely understood. At present, only one US FDA approved drug, riluzole, modestly slows the progression of ALS. A few other drugs are under clinical trials.

While most of the celebrities in the US have joined the ice bucket challenge, Canadian-American actor Pamela Anderson has a different take. She posted on Facebook that she cannot support the challenge because the drugs are first tried on animals, for which they are put through cruel tests.

"In recent experiments funded by the ALS Association, mice had holes drilled into their skulls, were inflicted with crippling illnesses, and were forced to run on an inclined treadmill until they collapsed from exhaustion. Monkeys had chemicals injected into their brains and backs and were later killed and dissected,” wrote the star actor.

“What is the result of these experiments (other than a lot of suffering)? In the past decade, only about a dozen experimental ALS treatments have moved on to human trials after being shown to alleviate the disease in animals. All but one of these treatments failed in humans—and the one that “passed” offers only marginal benefits to humans who suffer from ALS," she added in her post.

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