Imagine if a close family member or friend was diagnosed with amyotrophic lateral sclerosis. The statistics flocking into your mind would include the debilitating symptoms, the understanding of knowing they may only have three to five years left to live, and the realization that the person you love will never be the same.
Amyotrophic lateral sclerosis (ALS or Lou Gehrig’s Disease) is a neurodegenerative disease that affects roughly 30,000 individuals in the United States every year. The disease is “characterized by the massive loss of motor neurons in the spinal cord, brain stem and cerebral cortex.” This loss in function of motor neurons leads to stiffness, weakness, muscle cramps, decreased reflexes and eventually the inability to breath, leading to the tragic cause of death: suffocation. The eventual dysfunction of the motor neurons can be contributed to a neurotransmitter toxicity, (glutamate toxicity), with the major dysfunction involving excitotoxicity (too much calcium being released in the cell, leading to cell death) in the AMPA and NMDA receptors.
The AMPA receptors found in the cell membrane seem to be impacted due to the fact that when the channel for calcium to influx into the cell stays open, or “unedited”, the channel doesn’t have the ability to control the amount of calcium let into the cell, as mentioned before, leading to excitotoxicity and death of the motor neuron.
What has recently been reviewed is the impact the NMDA receptors (the main receptor for the excitatory neurotransmitter glutamate) may play in regards ALS. Similar to AMPA receptors, calcium may enter the cell at a rate that leads to excitotoxicity. This excitotoxicity results in calcium release from the endoplasmic reticulum (ER), eventually leading to misfolded proteins. The structure of a protein is the entire basis of the function to the protein. With misfolded proteins, the function is compromised, giving rise for the cell’s inability to carry out its function, eventually leading to cell death.
Even if all of this information was known, the news of the diagnosis would probably leave you with one statistic looming over all the rest.
There is no cure.
Even with certain treatments, such as Riluzole and Memantine, by inhibiting NMDA receptors, the symptoms of the disease are only prolonged by two to three months. If this were the case, what would you do? Research is heavily funded dependent on the number of individual’s impacted. Some diseases most impacted by people include lung and heart disease, breast cancer, and Alzheimer’s disease. ALS is not amongst the top in the list. So what to do?
Raise awareness.
The ice bucket challenge has that connotation for that exact purpose. Dumping buckets of ice water over the head has swept through nations of bringing awareness to the neurodegenerative disease of ALS. The challenge went viral on social media just last year. The challenge is to film proof of ice water actually being dumped on the head, posting the video to social media, and then nominating friends and family to participate within 24 hours or forfeit by donating money to research funding of ALS.
But even if ALS is not among the top diseases to impact people in the world, I don’t believe that should make it any less important in finding a cure. ALS, as with any major disease, impacts more than just the individual. Entire families, friend circles and communities are impacted by the horrific and debilitating symptoms associated with these neurodegenerative diseases. Regardless of how many are affected, I still believe ALS should receive research funding in order to find a cure. Donate regardless of if you complete the Ice Bucket Challenge or not. If that means put your money where the ice is, do that!
ALS: Put your Money Where the Ice is
