Progress spurred by ALS Ice Bucket Challenge — and what remains to be done 10 years later
Last month the ALS Ice Bucket Challenge celebrated its 10th anniversary.
The fundraising idea was the brainchild of both the late Pete Frates — a Massachusetts native, lifelong Boston Red Sox fan, Boston College alum and scholarship baseball player — and the late Pat Quinn.
In 2012, at age 27, Frates was diagnosed with the debilitating, degenerative and invariably fatal motor neuron disease amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease). Quinn was diagnosed in 2013 at age 30; so when the pair connected, they decided to devise a way to raise awareness about, and research funds for, the disease.
Their inspiration for the ALS Ice Bucket Challenge: the tradition of dumping a big cooler of a sports drink on the head of a team’s football coach after a big victory.
In August 2014 along with fellow ALS patient, the late Anthony Senerchia, Quinn and Frates took the ALS Ice Bucket Challenge — which led to both a viral and fundraising phenomenon.
According to The ALS Association, the threesome inspired “over 17 million people around the world to dump ice water on their heads and donate to an ALS organization. The Challenge raised awareness of the disease worldwide and raised $115 million to support our mission ... [to invest] in ALS research and care for people living with the disease.”
Senerchia, the oldest of the triumvirate, died in 2017 at age 46. Frates died in 2019 at age 34 and Quinn died at age 37 in 2020.
(For longtime Palm Beach Post staffers and readers, the ALS-centric summer of 2014 hit especially close to home as that was when award-winning courts reporter — and best-selling author — Susan Spencer-Wendel succumbed to ALS in June 2014 at age 47 after a courageous and poignantly public three-year battle with the disease.)
What’s happened in the past decade
Most experts believe that the increased funding and awareness have made a discernible impact.
Last year, National Geographic reported that Ice Bucket Challenge proceeds “funded the work that led to the discovery of five new genes associated with ALS and novel drug therapies. In the last year alone, the U.S. Food and Drug Administration approved two new treatments for ALS, both developed with funds from the challenge. Today, researchers are considered closer than ever to making ALS a livable, and eventually, curable disease.”
As Calaneet Balas, CEO and president of The ALS Association, told USA Today in July she believes upcoming data will prove that researchers are making progress and are on the right track.
"Anecdotally, it seems the needle has moved. We're seeing it," she said, noting that she’s witnessed the previous two-to-five-year survival rate typical of 20th century ALS sufferers being elongated to a decade or more.
Unfortunately, today’s longer-surviving ALS patients are doing so in the latter stages of the disease — when they’re paralyzed, unable to speak and require financially crushing around-the-clock care.
ALS causes largely remain a mystery
ALS has been confounding doctors and researchers for more than a century.
It’s an exceedingly rare condition with CNN reporting in 2021 “the overall incidence in the United States is 1.5 to 2.2 cases per 100,000 people, but the odds go up with age.”
Approximately 10% of ALS diagnoses are deemed related to genetics. As USA Today noted, in around 1 out of 5 genetic cases of ALS, the gene responsible is SOD1 — and there’s now a treatment to target that specific gene.
As for the other 90% of ALS sufferers, the cause(s) remain a mystery.
Statistically speaking the disease occurs more often in men than in women and most often strikes between ages 55 and 75.
Head trauma, sports and ALS
But the reason ALS resonates so strongly in the sports world is that data has shown that competitive athletes — especially those in sports with higher risk for head trauma — are at far higher risk for diagnoses than the general population.
As that’s especially true for participants in our country’s most popular sport: football.
In December 2021, researchers from Harvard University and Boston University’s CTE (chronic traumatic encephalopathy) Center found NFL players are about four times more likely to die of ALS than the general population.
According to Harvard University, the main takeaways from the study, which appeared in JAMA Open Network in December 2021, were the following:
Between 1960 and 2019, 19,423 men played in the National Football League (NFL). During that time period, 38 were diagnosed with ALS and 28 died of the disease.
Among these current and former football players, the risk of developing ALS and dying of the disease was nearly four times higher than that of men in the general population.
NFL players who developed ALS had a longer average football career (seven years) than those without the disease (4.5 years).
Many NFL players were in their mid-30s at the time of their ALS diagnosis. This is quite a bit younger than is typical for ALS.
Harvard University did note that “importantly, this study did not assess why there might be a relationship between ALS and playing professional football. The study authors speculate that traumatic brain injury might be to blame.”
It should also be noted that this connection between head trauma and risk for ALS has been a working theory for Boston University’s CTE Center for more than a decade.
Since 2008, the institution’s researchers have been microscopically studying the brain tissue of deceased former professional, collegiate and high school football players, as well as those of others who’ve been exposed to repeated head trauma, such as boxers, hockey players, soccer players, rugby players and war veterans.
They were looking for evidence of chronic traumatic encephalopathy (CTE), which is characterized by the brain producing an abnormal protein called “tau.” When looked at under a microscope, this tau protein — which is detectable only during post-mortem exams — appears as brown “tangles” that strangle normal, healthy brain cells.
Previously associated only with elderly dementia patients, the discovery of tau in the brains of virtually all late ex-football players in every age range — including one who was just 17 years old — has clearly established the link between repeated head trauma and the risk for developing CTE.
The Boston University CTE Center says that those who develop CTE experience, among other things, “memory loss, confusion, impaired judgment, impulse control problems, aggression, depression, anxiety, suicidality, Parkinsonism and, eventually, progressive dementia.”
Tau in the spinal fluid
The Boston University researchers also have examined tissue samples of deceased CTE athletes who, as their motor neuron capabilities rapidly deteriorated, had been diagnosed with ALS. In all of the cases, researchers found tau protein present in the spinal fluid — likely having leaked from the brain.
Conversely, in late CTE athletes who had not displayed ALS-like symptoms, there was no tau in the spinal fluid.
What’s more, in tissue samples studied of late ALS patients who were not recipients of head trauma, there was no tau protein in the spinal fluid.
Thus, Dr. Ann McKee — head of the Boston University CTE Center — coined this condition “chronic traumatic encephalomyopathy” (CTEM), which is “likely caused” by repetitive head trauma.
At this stage, doctors don’t know why some folks with CTE have their tau protein leak into their spinal fluid and others don’t, but the supposition is that there’s probably some genetic predisposition for it.
And all of the tau-related information above means there's a potential irony to the condition also known as Lou Gehrig's disease.
The New York Yankees Hall of Fame slugger, who was nicknamed the Iron Horse because of his record-setting streak of consecutive games played (2,130 spanning the 15 seasons between 1925 and 1939), suffered at least five documented on-field concussions during that time — yet was back in the lineup the next day.
Considering what we now about the potential connection between repeated head trauma and development of ALS in younger men (Gehrig was diagnosed on his 36th birthday and died at age 37), it's certainly conceivable that Gehrig was afflicted with what McKee calls CTEM ― and that had post-mortem research been done on his brain and spinal fluid, it would have found tau protein in both.
But regardless of what caused Gehrig's fatal condition, what is clear is that now that we’re a decade removed from the summer of the ALS Ice Bucket Challenge, the initiative made a massive impact on the disease that bears his name — and yet there’s still so much more to do.
This article originally appeared on Palm Beach Post: Ten years later, did Ice Bucket Challenge pay off in ALS battle?
