Buzz
Amyotrophic lateral sclerosis (ALS) is a degenerative disease that impacts the nerve cells in the spinal cord and brain. It’s a ruthless and swift affliction—patients can progress from early signs to death in typically just over three years. As the disease advances, the neural pathways of the affected individual deteriorate, eventually leading to respiratory failure.
In 2014, awareness of ALS peaked, and fundraising efforts set new records, but the average person still remains largely unaware of the disease.
10. ALS and Athletes
Athletes are more susceptible to ALS than the general population. The condition is sometimes referred to as Lou Gehrig’s disease, named after the legendary New York Yankees first baseman who was famously diagnosed with it.
For over half a century, Gehrig held the record for the most consecutive games played, a feat that earned him the moniker 'The Iron Horse.' But even his formidable endurance couldn’t protect him from ALS, and he bid farewell to his fans at Yankee Stadium on July 4, 1939. In his iconic farewell speech, he expressed that, despite his terminal diagnosis, he felt like 'the luckiest man on the face of the Earth.' Lou lived for just two more years, passing away from ALS on June 2, 1941, at the age of 37.
Sports certainly play a role in the rise of neurodegenerative diseases. A CDC study tracking 3,439 NFL players reveals that football players develop ALS at a rate approximately four times higher than the US national average. Concussions are believed to significantly contribute to the development of ALS. Gehrig, for example, suffered numerous concussions throughout his career (the MLB didn’t mandate batting helmets until the very year of his passing).
In recent years, the NFL has been implementing regulations to minimize the risk of brain injury from repetitive concussions. After linebacker Junior Seau—who was reportedly subjected to around 1,500 concussions throughout his career—tragically took his own life, his brain was examined and found to have chronic traumatic encephalopathy, a neurodegenerative condition akin to ALS.
This issue extends beyond the United States. Italian soccer players have been found to have an elevated risk for ALS, developing the disease at a rate six times higher than the average Italian. One theory suggests that this heightened risk is linked to players’ frequent exposure to ticks in the grass during games.
9. An Orphan Disease
ALS affects only 2 out of every 100,000 individuals, which classifies it as an orphan disease. Pharmaceutical companies have not yet prioritized it for research, primarily because there is little profit to be made. Meanwhile, more common conditions, from depression to erectile dysfunction, are the focus of substantial investments due to their lucrative returns.
As a result, there are very few treatment options available. The only approved drug for ALS is Riluzole, which was authorized by the FDA in 1995. It works by inhibiting sodium channels in neurons, slowing the progression of the disease. Riluzole is priced at approximately $10 per tablet, but its efficacy is questioned, often extending the lives of ALS patients by only two or three months.
8. The Mystery of Stephen Hawking’s Survival
Stephen Hawking stands as the most well-known living individual with ALS, a true outlier in the realm of medical anomalies. Diagnosed at the tender age of 21, he was given a grim prognosis with little hope for survival. Yet, more than fifty years later, he remains alive, defying every expectation. His work throughout this time has profoundly shaped human understanding of the cosmos.
Hawking's doctors do not reveal the specifics of his condition, leaving the exact reason for his survival unknown. ALS typically targets the motor neurons that control the diaphragm and muscles used for swallowing, leading to respiratory failure or malnutrition. However, Leo McCluskey, medical director of the ALS Center at the University of Pennsylvania, notes, 'If you don't have these two factors, you might potentially live for a prolonged period, even as your condition worsens.'
In recent years, Hawking has relied on a full-time ventilator, but he continues to remain active, even appearing as a voice guest on shows such as The Big Bang Theory. Thanks to his wealth and status, Stephen Hawking has received some of the best medical care available, which has surely contributed to his extraordinary longevity. However, ALS has ravaged his body: he is nearly entirely paralyzed and communicates at a rate of one word per minute using a device that interprets the movements of his cheek.
7. The Paralyzed Screenwriter
Stephen Hawking is not the only well-known individual with ALS who is determined to make the best of a challenging situation. Scott Lew, a screenwriter with several notable works, including the 2013 film Sexy Evil Genius, is battling the advanced stages of the disease. His physical movement is severely limited to only a few muscles in his face and eyes, and it requires his assistants four hours each day to help him get out of bed and prepare for his daily tasks.
Scott Lew was the subject of the short documentary Jujitsu-ing Reality, which provides a compelling and motivational portrayal of how one can continue to be productive while living with ALS. This documentary has been showcased at numerous film festivals globally.
Similar to Hawking, Lew has surpassed expectations, living with ALS for 11 years as of 2014. He keeps an active blog where he shares poetry, video clips, and personal thoughts. In one of his essays, titled 'A Good Night With ALS,' he writes about the experience of 'being buried alive in your own body,' yet he also finds moments of beauty in life, declaring: 'Every day is a chance to have fun and be creative.'
6. Conjugal ALS
While ALS is known to be genetic in only 5-10% of cases, the cause in most instances remains unclear. There have been instances where both partners in a marriage develop the disease, which is puzzling because ALS isn't contagious. Experts suggest these occurrences might just be coincidences, but the true reason is still uncertain. In some cases, one partner wasn't diagnosed until years after the other’s death.
Around the world, at least 20 documented cases of conjugal ALS have emerged, with examples including one each from India and Spain, two from Brazil, three from the United States, four from Italy, and nine from France. All of these couples had been together for at least a decade before their diagnosis.
5. Richard Olney
While athletes have been found to have a significantly higher rate of ALS compared to people in other professions, the disease affects individuals across a wide range of fields. This includes musicians like Leadbelly and Charles Mingus, politicians like former Senator Jacob Javits, and there are even rumors that Chairman Mao Zedong succumbed to ALS. However, the most ironically affected individual may be Dr. Richard Olney.
Dr. Richard Olney was a trailblazer in ALS research, founding the ALS Treatment and Research Center at the University of California, San Francisco. But in 2004, his health began to deteriorate. By 2005, one of the leading experts in ALS came to the grim realization that he himself had developed the disease.
Dr. Olney bravely battled ALS for eight years, experiencing its most debilitating symptoms only in the final stages. Even in his last days, he continued his research, collaborating on papers with the help of his son. He conducted interviews, aiming to raise awareness of ALS and his research center. Dr. Olney passed away on January 27, 2012, at the age of 64.
4. Cluster Outbreak
While the medical community has yet to pinpoint a definitive cause for ALS, compelling patterns suggest environmental factors may play a role. In particular, unusual geographic concentrations of the disease have emerged. In Montreal, three Ashkenazi Jews developed ALS within 18 months of each other, all residing in the same apartment building. In another instance, four individuals in rural South Dakota, three of whom lived within a few miles of each other, were diagnosed with ALS over the course of a decade.
Soldiers returning from the 1991 Gulf War have shown an unexpectedly high incidence of ALS. These veterans may have been exposed to a variety of chemicals during the war, including nerve agents, pesticides, and radioactive substances. Interestingly, countries such as Mexico, Italy, and Poland report lower-than-average rates of ALS.
3. The Ice Bucket Challenge Controversy
The Ice Bucket Challenge, a viral sensation, seemed to be a harmless way to raise awareness. However, some individuals have faced criticism for participating simply for fun rather than contributing to the cause. Celebrities, in particular, have been accused of using it as a platform for self-promotion. Notably, figures like Charlie Sheen and Steve-O, who are not typically known for taking serious matters seriously, have emphasized the importance of fundraising over raising awareness.
Another critique of the challenge has been its use of clean water, especially in regions where access to water is limited. While this criticism is often misplaced, as the water usage was minimal compared to other processes, it became a significant issue in California, where a severe drought had caused widespread concern.
Movie star Matt Damon addressed the issue of water waste by participating in the Ice Bucket Challenge using water from his own toilet. While it's unclear how much water was saved by this method, it gave Damon the chance to promote his own charity, Water.org. Damon explained, “Because I co-founded Water.org, and we envision the day when everybody has access to a clean drink of water—and there are about 800 million people in the world who don’t—dumping a clean bucket of water on my head seemed a little crazy.”
2. The Ice Bucket Challenge's Boundaries
Until just a few months ago, many people had never even heard of ALS. However, the Ice Bucket Challenge, which went viral in the summer of 2014, changed that. The exact origin of the challenge remains unclear, but versions involving cold water can be traced back to 2013. As of now, the ALS Association has raised over $100 million, representing a 3,500-percent increase in donations compared to the previous year.
Like most viral phenomena, the Ice Bucket Challenge will eventually fade, leaving researchers no closer to a solution. Dr. Jonathan Serody, a bone marrow researcher at the University of North Carolina, commented, “If a million people would donate $100 a year for 30 to 40 years, you might get a breakthrough for ALS. These fleeting trends that vanish after a few months won’t provide long-term help. Researchers need more dependable funding.”
The United States government, through the National Institutes of Health, is the largest contributor to medical research worldwide. However, over the years, it has drastically cut its budgets, and ALS research has been particularly affected—the NIH allocated $59 million for ALS research in 2010, but its contribution has decreased by a third in the past four years.
1. Organic Toxins
Seafood has long been criticized for its elevated mercury levels, which are expected to rise in the coming years. The mercury content is so high that many health experts advise pregnant women to limit their fish consumption to small portions. However, an even more concerning substance may be present in fish—a toxin known as beta-methylamino-L-alanine (BMAA). This compound occurs naturally and is produced by algae found in both soil and bodies of water.
While researching ALS, neurologist Elijah Stommel became fascinated by clusters of cases in specific geographic regions. When he compared the addresses of 200 of his patients, he discovered that many were situated near bodies of water. The area most affected was Mascoma Lake in Enfield, New Hampshire.
BMAA may have also contributed to the ALS-Parkinsonism-dementia complex (known as Lytico-Bodig disease) that affected the Chamorro people of Guam after World War II. The Chamorro people had a dietary habit of eating local fruit bats, which consumed cycad seeds that produce BMAA. This toxin accumulated in the bats' bloodstream, making the Chamorro people dozens of times more likely to develop ALS than the general population.
