A lot of people are jumping on board the ALS Ice Bucket challenge, ‘cuz it’s a weird, wild, trendy chance to give to charity. Some people that do this are all, “Hey, that’s what Stephen Hawking has, yeah? That guy’s a genius, pour some ice on me.” Others think, “Jeeze. ALS kills people? Gimme that bucket.” In the midst of all the freezing madness, a lot of the facts about ALS are being forgotten, omitted, or minimized. In light of this, let’s put down our buckets and talk about ALS, the ALS Ice-Bucket Challenge, and what it means as a fundraising trend.
What is ALS?
Here’s my really basic, really brief summary of ALS. It’s part of a group of neural-motor disorders, which I secretly wish would also be represented in all the awareness-raising with ice. More on that in a bit.
ALS–properly called Amyotrophic Lateral Sclerosis –-is a motor neuron disease, which affects voluntary muscle groups. In overly simple terms, ALS wrecks motor neurons, which the body uses to send signals of muscle movement between the body and brain. This means that the brain-body communication becomes distorted, slowed, and eventually halted, leaving the person paralyzed in many areas of voluntary movement. The disease is degenerative, and fatal, and often marked for its rapid progression, as those diagnosed with it are expected to live only 2 to 5 more years. As far as frequency goes, it is estimated that there are 30,000 people living with ALS at any given time. It should also be mentioned that ALS has a genetic component, with 7% of people living with the disease having family members that also had it.
Expanding Our Knowledge: Other Related Disabilities
ALS preys on the neurons that communicate between your brain and body. And while it’s an ugly beast, it’s not the only one. There are 6 other classifiable motor neuron diseases, of honorable, shitty mention. I’m writing them in the shortest way possible here, so that you’ll get a general feel for commonalities in motor neuron disabilities, and hopefully see why Science (not just the ALS association, exclusively), needs more money to both learn about this and other diseases.
PLS (Primary Lateral Sclerosis) This big guy can often be confused for ALS in its initial diagnoses, as first symptoms may look the same. Unlike ALS though, atrophy (the waning of muscle tone)is not common, as PLS mainly affects the neurons in the Cerebral Cortex, which progressively die, causing slowed and eventually stopped fine and gross motor abilities (and increased spasms and rigidity) in people with the disease. The progression is much slower than ALS, and people living with PLS often live 20 more years with the disease.
PMA (Progressive Muscular Atrophy) This one hones in on lower motor neurons only. This means that unlike the above PLS, upper motor neurons, such as the ones found in the Cerebral Cortex, are left untouched. Symptoms present as weakness and atrophy, with no spasms or rigidity present.
Progressive Bulbar Palsy This jerk sometimes turns into ALS, apparently. It is characterized by the slow degeneration of the brain stem. The prominent feature is that it ruins the nerves that control the face, tongue and throat, resulting in difficulty speaking, swallowing and chewing, with limb function difficulties likely to happen later on.
Post-Polio Syndrome In case you’d like a frame of reference, the guy who inspired the movie The Sessions (Mark O’brien, not John Hawkes) died from this in real life. Thanks to vaccinations, this disease has largely gone extinct, and is no longer fatal in most cases. When it does linger around, it only bothers those who’ve had Polio before, attacking whatever neuron activity might still be allowing them to move.
Kennedy’s Disease Here’s one I’ve never heard of before (reassuring, right?). This disease mainly affects men, and is highly genetic, as it is caused by genetic mutations that influence the Androgen Receptor. Because of this very specific effect, symptoms in men are erectile dysfunction, breast development, and atrophy of the testicles. It is not life-threatening, and only some people with it end up having to use a wheelchair due to other weaknesses, according to the inter-webs.
SMA (Spinal Muscular Atrophy) This fucking asshole of a disease affects the uptake of the muscle-building protein, SMN, as well as lower neuron motor functioning. This results in a lot of muscle weakening and wasting away, attacking the major muscle groups before the smaller ones. It has differing severities and 3 different types, and is very commonly diagnosed in kids. Later onset SMA is called Kugelberg-Welander Disease. SMA frequently falls under the Muscular Dystophy (so many muscular diseases…) umbrella too, because like MD, the muscles are affected due to a lack of a certain protein.
The bottom line here is that, while all these diseases differ from ALS, it’s important that we know where ALS fits in the puzzle, and to be aware that all of these diseases need further donations, understanding, and research. As you can see, 4 out of 6 types of motor neuron diseases are fatal. In the interest of well-rounded awareness, I’d encourage everybody throwing ice on themselves to think about how the people living with these diseases are impacted. Maybe contemplate how you would feel if suddenly your brain peaced-out on your body, and the ways it would change you(read: slow paralysis, dependence, etc). Don’t just do the ice dump for the sake of hard nipples, think about why you’re doing it.
I was gonna write about the disability/charity narrative coming through all of this icy noise, but I guess that’s another post.