In the last few months, the Ice Bucket Challenge has swept the nation with everyone from celebrities to your neighbor – and likely yourself too – completing this “challenge.” While the Ice Bucket Challenge has done an amazing job creating awareness and donations for this debilitating disease, few people truly understand what is happening for patients with ALS. Or why raising funds for research is so important.
ALS is a fatal neurodegenerative disease that stands for Amyotrophic Lateral Sclerosis – more commonly called Lou Gehrig’s disease. It involves the death of motor neurons in a person’s brain and spinal cord, which in turn causes muscle weakness, problems with speaking and breathing, and even paralysis. Because only the voluntary muscles are affected (like the muscles in your legs and arms), patients do not typically have problems with their heart or digestive system. But the inability to move and be “trapped in one’s body” can be devastating. Breathing, although usually involuntary, we can consciously control, meaning that it is also affected by ALS. Eventually, the patient requires a ventilator to breathe.
According to the ALS Association, every year in the United States 5,600 people are diagnosed with ALS, and at this very moment 30,000 people in the country suffer from the disease. After diagnosis, patients typically live for two to five years. Although ALS can be familial, this happens only 10% of the time. For the other 90%, onset of the disease is completely sporadic.
Currently, there is no cure for ALS and only one drug approved for treatment – riluzole – but it only extends the patient’s life by a few months. Why is it so difficult to develop treatments for this disease? The actual “cause” of ALS is unknown. It is believed to be caused by an overabundance of glutamate, an important neurotransmitter in the brain. This excess glutamate causes a particular receptor, called an NMDA receptor, to become hyperactive. When this NMDA receptor is active more than it should be, it results in too much calcium flowing into the nerve cells which eventually contributes to the death of the motor neuron. Until relatively recently, scientists believed that a different receptor, the AMPA receptor, was the main receptor involved with ALS. The brain and the many pathways of different neurotransmitters are very complicated, which only makes determining what is causing ALS more difficult. Mutations in a certain gene, called SOD1, could also have an effect on the development of ALS.
Why is this important? Because there are so many different pathways and possible reasons for the development of ALS in a person, it is essential that we conduct more research to help to discover and determine the best way to treat ALS. Ultimately, a cure for ALS could be found, but only with continual raising of awareness and donations. When the Ice Bucket Challenge craze has died down – as it eventually will – it is important that we do not forget about ALS. It is not a disease that gets a great deal of attention, but that does not mean it isn’t serious. It is a disease that progresses quickly and one that we need to remain aware of. Remember why you completed the Ice Bucket Challenge – not just for likes on Facebook, but to ultimately help the many people who struggle to walk, talk, and breathe as a result of ALS.
ALS: More Than Just the Ice Bucket Challenge
