Behind the current entertaining challenge, which encourages the masses to dump ice cold water over their heads, is the devastating illness ALS (Amyotrophic Lateral Sclerosis), also known as Lou Gehrig’s Disease, after the famous baseball player who was afflicted with and ultimately succumbed to the disorder. It’s a progressive condition that begins with muscle weakness and rapidly evolves into a major disabling disorder. In addition to this, it carries a high mortality rate with an average survival of 3-5 years after initial diagnosis. In the U.S., 7,000 new cases are diagnosed every year and, unfortunately, scientists have yet to decipher the cause of the sickness. The Ice Bucket Challenge has provided a fun way of bringing attention to and creating awareness for ALS, but we shouldn’t forget that the serious disease for which the campaign was created is in considerable need of financial support; a necessary step for the continuation of essential research.
ALS is a degenerative disease of the nervous system responsible for the active decomposition and death of motor neurons (nerve cells that carry the signal from the spinal cord to the muscles, resulting in the manifestation of movement). When these cells collapse, the signaling process becomes aberrant and the muscles are unable to receive proper instruction on how to accomplish movement. Scientists have suggested that abnormalities in the mitochondria and DNA might play a role in ALS and, to date, the only confirmed risk factors are genetic predisposition and advanced age. The classic clinical presentation of patients with ALS includes: lack of coordination, loss of balance, development of a slow pace, weakness of the arms and legs, difficulty with speech and swallowing, poor cognition, and weakness of the diaphragm muscle which results in respiratory failure [the most common cause of death]. The diagnosis of ALS is mostly clinical and is based on a set of objective exam criteria. Additional testing may include electro-diagnostic studies (e.g. nerve conduction study) which can measure the capacity of a nerve to transmit signals. The treatment of ALS is mostly assistive, as there is no cure for this disease and therapy is geared toward improving the patient’s symptoms. This is generally accomplished by a specialized medical team that can provide physical, occupational, and respiratory therapy.
Lou Gehrig, ironically nicknamed the “Iron Horse” due to his durability and remarkable consecutive games streak, had a dramatic deterioration in speed and coordination in the year 1939. It was that year, at the all-too-young age of 36, when he was diagnosed with ALS. His wife was told that the cause of ALS was unknown and that despite the fact that it was a painless and non-contagious disease, it was a cruel condition characterized by the destruction of the motor nervous system. Maddeningly, not much has changed in 75 years as we have yet to find a finite cause or a cure. The Ice Bucket Challenge marketing campaign, however, has revived an interest in Lou Gehrig’s Disease. Research cannot be conducted without appropriate financial backing, therefore, whether you want to dump ice on yourself or not, you must remember that monetary contributions are the most effective way for the average person to make a difference in overcoming this aggressive and debilitating disease.