This is an exciting and happy time for sufferers of ALS. Less than forty thousand people in America suffer from ALS, making it a rare disease. I too suffer from a debilitating rare disease. I have Ehlers-Danlos syndrome. Although I’m only 24, I suffer from a bevy of injuries and disorders. Like ALS, EDS is incurable, and all treatments are palliative. Nervous system involvement in EDS is very common. I personally know what its like to helplessly watch my muscles dysfunction. I have a foot long cyst inside my spinal cord (a condition known as Syringomyelia). Likely caused by excessive immobility and a herniated cerebellum ( a condition known as Chiari malformation), my syrinx has compressed many nerve roots in the spine, tingling, weakness, and cogwheel rigidity (although we’re still unsure of the exact cause of the cogwheel rigidity) in most of my muscles. In the three years since my diagnosis I have been lucky enough to see my disease pulled from obscurity to become relatively well-known in the health-care professions. Although directly that awareness has yet to personally benefit me, it represents an important milestone.
ALS as a name is much more well known in the general public than EDS, but EDS is much more easily identifiable and understood than ALS. The principle source of disability for most EDSers is joint injury and chronic pain. Almost everyone knows someone with Arthritis, and almost everyone has had a sprain, or tendinitis, and most know someone who has had re-corrective surgery (replaced hip, repaired rotator cuff tear or Labral tear). EDS can, and often does, get more complicated than that, but those basic pillars are identifiable. ALS is incredibly complex. It’s a difficult disease to explain, and very difficult to identify with. Most people one day will struggle with some sort of joint injury, and that well-known fact kind of equalizes the shock of seeing your fears realized in another human being. Most people will not struggle some day with motor neuron disease, and due to its visible effects, and the mystery that shrouds it, ALS often scares people away.
ALS is a visible disease (meaning its effects are visible, compared to EDS (my disease) which is most often characterized as invisible), so even if you can’t spot it when you see it, if you meet someone with progressive ALS, you will immediately know they’re suffering from something serious. It’s in the nature of visible diseases to polarize people, and with more serious diseases like ALS, that effect is almost uniform. Human beings have these interesting neurons called mirror neurons. When we talk about ‘living vicariously’, we’re dealing in mirror neurons. When you watch someone drink a glass of water, the exact same neurons activate in your brain that would have activated if you had actually drank a glass of water. When you watch a video clip of someone publicly embarrassing themselves, mirror neurons are responsible for that cringe effect – if you’re like me, then maybe you cannot even watch those types of videos because of that mirror-neuron effect. Likewise, when you see someone in pain, you feel their pain – an important mechanism underlying empathy. Because ALS is devastatingly visible, because it is so poorly understood by most people and so unidentifiable, and because it represents a deep fear most people share, when we see someone with ALS feeling their pain realizes those fears. Most people are risk-aversive, disproportionately overvalue the utility of their own health when they consider illnesses (have you ever given someone sick advice that you couldn’t follow yourself when you were sick?), and most people suck at on-the-fly probability and decisions making. If someone’s pain in particular is a trigger (which, generally, something as devastating as ALS usually is) it may cause a fight or flight response, intensifying the confusion; like the gut reaction I have to turn off an embarrassing video, even though I’m well aware I haven’t done anything embarrassing, and no one is watching me, most people in this situation will choose flight. Living with EDS is very lonely, I cannot imagine how much more lonely living with something like ALS must be.
The ice-bucket challenge has taken a disease destined to remain in obscurity, and positively framed it by associating it with the excitement of an innocuous challenge, bringing its awareness to the forefront of popular culture. Some people criticize the challenge for wasting water, and detracting from the seriousness of the disease, but I think the challenge has made a very scary disease seem less scary. It’s subtly introduced people to an object of fear and transformed that object of fear into something conquerable, something worth challenging ourselves with. Familiarity mediates fear. This challenge hasn’t ameliorated the fear we feel when we imagine ourselves living with ALS but its brought ALS to the EDS level; where people understand and can identify. Dumping ice water on your head is totally arbitrary and random, but it frames ALS much differently than it would be framed by individual people interacting with the disease, ALS, and with people who have ALS. It’s exciting to be nominated, its exciting to be part of a trend (which is low-risk and high-yield opportunity for social gain), and this trend is funny and allows room for creativity. It also leaves people with a sense of accomplishment and reward, a sense they’ve done some good. All of these things contribute to a radical re-framing of ALS and thus a radical shift in how people interact with ALS, and will interact with ALS in the future. It might seem like such a small thing, but it’s huge for those with ALS in so many ways. Even at its minimum potential for change, this challenge has done a lot.
Ignorance about a disease creates a self-sustaining cycle of suffering. Mirror neurons act rather indiscriminately, and so we not only imagine what it would be like generally to live with the restrictions ALS imposes, but we also feel the loneliness, the fear, the sadness, the frustration (with limitations, with fatality, with prolonged, intractable suffering) as we witness it. And that phenomenon drives a wedge between our desire to act compassionately and empathetically, and our own sense of mental stability and well-being. Unlike a movie that you can just shut off, the relationships we have with people are real, and elicit real emotional responses, which have powerful effects on our lives. Luckily, from my own experience, there are ways to mediate the social and emotional affects of suffering. When you know your society supports you and actively tries to understand your suffering you feel a sense of connection and justice with the community your disease tore you so violently from. This is important because without that understanding and support the person with ALS, or EDS, or any other chronic medical condition, can become lost; the rejection, exclusion, separation, and meaningless fatality and injustice added to the consequences of the disease itself leave little room for hope. People with disabling, chronic medical conditions have the (very same) potential to be just as inspiring, just as fun and just as enjoyable to be around as healthy people; they certainly have the time, in Thoreau’s words, ‘to acquire new value for each-other’.