“My expectations were reduced to zero when I was 21. Everything since then has been a bonus.” ― Stephen Hawking
Until a month back, amyotrophic lateral sclerosis (ALS), a form of motor neuron disease, was more immediately associated with the famous theoretical physicist Professor Stephen Hawking. However, today the ALS Ice Bucket Challenge-or as many social media users know it, the #ALSIceBucketChallenge-is everywhere. The idea sparked by an ALS patient named Pete Frates, caught on with athletes in Boston and spread like wildfire.
The Ice Bucket Challenge relies on a simple dare: you get 24 hours to post the video of yourself being drenched by a bucket of ice water on social media (for example, Facebook, YouTube or Twitter)- and tag three (or six, or as many as you can think of !) of your closest friends to do likewise- or donate $100 to ALS charity of your choice. In many cases, participants do both, they keep the cycle going by daring others and fork over the donation as well.
ALS is a neurodegenerative disease that affects a person’s ability to move voluntarily. The disease, also known as the glass coffin, stops the individual from voluntarily eating, walking and talking, but not from thinking or feeling. Thus, a “glass coffin” effectively translates to the condition wherein the sufferers are ‘locked in’ to their bodies.
Now coming to the popular question,
What is ALS?
Each muscle in our body is controlled by motor neurons that reside in the brain (in the frontal lobe, to be precise). These are controlled electrically and are synaptically connected to motor neurons that reside lower down in the brain- along with the motor neurons in our spinal cord. ALS is the disease that weakens the motor neurons (either in brain or spine, or even both).
In 2006, it was determined that ALS was determined by the accumulation of abnormal proteins, called 43 kDa transactive response (TAR) DNA-binding protein (TDP-43), in the brain. 10% of ALS is genetic and based on a gene mutation. There are now multiple genes that have been identified as potentially causing the disease, for example, chromosome 9 open reading frame 72, or C9orf72 , or gene that encodes the enzyme copper-zinc superoxide dismutase 1 (SOD1), or even TAR DNA-binding protein 43 (TDP-43). The remaining 90% occurs at random with no clearly associated risk factors. Researchers from the Scripps Research Institute and the Mayo Clinic developed a new drug that combats molecular contributors to ALS. Knowledge of specific genes helps to identify specific mechanisms in the brain, and would potentially give us targets for therapies.
The widespread campaign to “raise awareness” has enlisted the help of seemingly every A-Lister on the planet, including Mark Zuckerberg, Obama, Bill Gates to our very own Bollywood stars-Rahul Bose, Bipasha Basu, Abhishek Bacchhan and Riteish Deshmukh and tennis players Rohan Bopanna and Sania Mirza, to name a few. The donations to the ALS Association have risen to $21 million over the last few weeks! It’s just an incredible example of a fundraiser campaign that has exploited the power of social media to the fullest. The ultimate aim is to transform this large-scale awareness into actual research that brings relief to ALS patients.
[Note: The quote is taken from The Science of Second-Guessing,New York Times Magazine Interview, December 12, 2004.]