Last summer, the ALS Ice Bucket Challenge went viral.
The rules were simple: If challenged, you were to donate $100 (or any amount you could) to the ALS Association, or you had to dump a bucket of icy water over your head.
There were flaws in the system because many people would rather dump the water over their heads than donate.
Some didn’t even look up what ALS was. But others, including celebrities, organizations and even average individuals, took the real challenge.
They donated to the ALS Association, they researched ALS and they stepped up to make a real impact.
Amyotrophic lateral sclerosis, or Lou Geurig’s Disease, is not a new disease.
It was first discovered in 1869 by Jean-Martin Charcot, a French neurologist.
It is a very debilitating disease; the body begins to “turn off” motor neurons in the brain, which prevents the brain from sending messages to the muscles.
This causes the muscles to degenerate, as well. The decline is slow and as patients start to lose motor skills, neurons die and more muscle control is lost. This eventually leads to paralysis and then death.
ALS was almost completely unheard of until 1939, when Lou Geurig, a world-famous baseball player, suddenly ended his career after being diagnosed.
Even so, the awareness of this disease fell into the shadow of other, more common diseases. That is, until the Ice Bucket Challenge propelled it back into the light.
The challenge produced a record number of donations to help provide specialty chairs for patients and fund research to help find a cure.
According to the ALS Association, about 5,600 people are diagnosed with ALS in the US each year and the patients generally survive three-to-five years with the disease.
This brings the total amount of Americans with ALS at any given point to nearly 30,000. Although that is high number when we look at it, compared to diseases like HIV (50,000 new cases in America each year), heart disease (735,000 heart attacks in America each year) or diabetes (1.5 million new cases in America each year), it is a drop in the bucket. It makes sense ALS is not on the forefront of our minds.
Another possible reason we don’t think about ALS is because our generation is not at high risk of contracting it.
Right now, our biggest threat is behind the wheel. Distracted driving (which includes drinking while driving and texting while driving) has the highest chance of taking young lives.
For the most part, ALS develops in people around the age of 55, but it can also happen between the ages of 40 and 70.
Rare situations have occurred where people develop ALS in their younger years, but for the most part, the risk of ALS increases as age progresses.
I am 25-years-old, and I have been living with Charcot-Marie-Tooth for 25 years. Unlike ALS, CMT is genetic, and it can show signs as early as the toddler years.
But like ALS, Charcot-Marie-Tooth is a neuromuscular disorder that affects the motor skills of those affected.
They are so similar, in fact, that Jean-Martin Charcot discovered both of them (although he had the help of Pierre Marie and Howard Henry Tooth, hence the name).
Charcot-Marie-Tooth is a neuromuscular disorder that affects the peripheral nerves.
The patients' brains work fine; they send the signal out to their hands or feet, but by the time the signal gets there, it’s short-circuited.
Think about a radio: You plug the radio into the wall and the radio plays. The socket is your brain, the wire represents your nervous system and the plastic coating is called the mylene sheath.
If you peeled away that plastic coating, symbolic of the degeneration of the mylene sheath, what would happen to the quality of the sound?
Although the wall (the brain) is still sending the right signals, it's not reaching the radio at the full potential.
This causes other problems. If the signals, small electric shocks, are not strong enough, they won't stimulate the muscles properly, which will inadvertently also cause degenerative muscles.
Scoliosis, often diagnosed with “unknown causes,” is a frequent side effect of CMT because the muscles supporting the spine are not strong enough for that job.
Muscle loss in the legs or feet cause other side effects, like drop foot, loss of balance or numbness. This muscle loss will eventually spread to the hands and arms.
Holding silverware, writing and the ability to tie your own shows will slowly fade away.
These are all trivial side effects if experienced on their own, but as the disease progresses and the symptoms worsen, daily life becomes a challenge.
And the worst part of all: no one (including many doctors) will ever understand what you are going through.
If the awareness of ALS is a drop in the ocean, Charcot-Marie-Tooth is a grain of sand in all the deserts.
Despite being highly genetic, it is passed down on the dominant gene, so there is almost no chance of being skipped. It is also rarely diagnosed and symptoms are not strictly defined.
Mild cases could go without ever being diagnosed, whereas severe cases could be misdiagnosed as muscular dystrophy.
Although the symptoms will progress through the lifetime, it does not impact the person’s lifespan. However, like ALS, there is no cure or even a proven treatment.
The Ice Bucket Challenge raised awareness and funds for ALS, not CMT. But that’s okay.
These neurological diseases, plus muscular dystrophy, muscular sclerosis and even Huntington's and Parkinson’s disease, are all intertwined and a cure for one could lead to a cure for them all.
You may not have heard of ALS before the Ice Bucket Challenge. You may not have a loved one suffering from it.
But the chances of being impacted by a neuromuscular disorder, like one of the above or many others, is not so unlikely. Our brains and our nerves are complicated.
There are teams of researchers working every day to figure out how they work, why they fail and how they can cure these diseases.
You might think a cure for ALS won’t impact you, and maybe it won’t. But, it will affect more than just the 30,000 people living with this debilitating disease at this very moment.
We need a cure for ALS so we can cure the world of all neuromuscular disorders, regardless of their awareness levels.
