Getting Better, One Day at a Time

A friend told me about eating out with her “sarcastic” friend—we all have one—when they saw a toddler, bundled up in winter layers, bounce off a closed glass door and fall because the child had not perceived the door.

The sarcastic friend said, sotto voce, “Get used to that, kid.”

Life is a clear, freshly cleaned, plate glass door that no one notices is a door, even with a shiny metal door handle at every-door-you’ve-ever-seen’s-door-handle-height on it, because we are too busy thinking about life until someone bonks into it. Loudly.

When are we too young to learn that? or too old to be reminded?

I bonked into my own life, repeatedly. Another friend has an analogy: Whenever he lived like he thought he was the “captain of his own ship,” he would run it aground, back it up, direct it in what he thought was a new direction, fire up the engines and re-launch full speed ahead, only to find that it was not a new direction at all and he had re-grounded it in the same spot, but deeper in the muck.

Ten years ago, my SMA symptoms were probably beginning to manifest themselves but I was still walking everywhere, and even if I did notice any changes I was not someone who was going to say anything out loud about them to another human being. I had terrible leg cramps. My right leg would spasm out from under me if I stood still for long, but it had done that for years; my reflexes would catch me and pop me back into place. That happens to everyone, right? I fell in my own apartment, a single-level, one bedroom, hard-to-fall-down-in apartment and twisted that ankle sometime in 2004. SMA? or something else?

It is possible it was my leg misfiring, a neuromuscular something-or-other, as well as something else. In 2004, I was still active in another disease, alcoholism; that stumble that I mentioned is not something that I actually remember as I was in a brownout that night, an ambulatory blackout. I remember awaking in pain and with a foot too swollen to put a shoe on it. Ice and aspirin and I was fine within days or never.

AA_Anniversary_Medallion_Cobalt_BlueHad my alcoholism not gone into remission four and a half years ago through a lot of work, I would not: be writing; know about SMA; know that I was born with SMA; or be walking at all, probably. I would not have the income that I have (Social Security Disability), which is a very small stipend but it is regular; I would not know eighty percent of the people I now call friends; I would not be in the relationship that has outlasted the few relationships I had ever adventured my way into and out of over the years.

I would not be walking because I would not know what was happening, would not have complained to anyone, much less a doctor, and probably would be on a walker by now instead of a cane. I would have silently suffered with a fear that my condition was one I “had drunk myself into,” which would probably frequently be a thought immediately preceding another guilt-riddled binge. (SMA is a genetic disease, and its symptoms would have appeared when they did even if I had been a teetotalling professional athlete.) When I was active, I liked feeling bad, feeling guilty, feeling self-pity, even, because I liked the relief for those feelings that I had in a bottle. I enjoyed feelings, good and bad, only insofar as I could suppress them.

Alcoholism is a disease, a psychological and physical one, in which craving supplants all emotions and that emotion directs all actions. All addictions seem to share that simple self-centered rule and draw vitality from this circular emotional logic. The solution is simple but difficult; for me it involved getting involved with life, doing for others, with others, and noticing that I am not the center of the universe and that you all are not my creations, figments of my imagination. The trick was getting me to want that, to notice that I did not know or had forgotten all this.

About ten years ago, maybe eleven, I tried to contact my future self, the 2014 edition of me with several years of sobriety. I called the A.A. hotline and some nice person listened to me for a bit and then he told me he would get me in the morning and bring me to a meeting. I of course did not go. The first step in recovery is to admit “we were powerless over alcohol—that our lives had become unmanageable.” I knew my life was becoming a wreck already, even with a good job, but … I knew nothing else. Ten years ago I was on Step Zero—I knew that MY life was wrong somehow—but I did not come into recovery until 2010; I do not wish those six years on anyone, even people I detest.

I do not wish SMA on anyone, even people I detest, too. The beautiful thing about bonking into real life is that the best people I know are alcoholics and addicts taking their recovery seriously and people with neuromuscular diseases—like SMA and SCA and Friedreich’s ataxia—people who expand their lives and their possibilities even as their boat changes course on them and coach me to do so, too.

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The WordPress Daily Prompt for November 15 asks, “Present-day you meets 10-years-ago you for coffee. Share with your younger self the most challenging thing, the most rewarding thing, and the most fun thing they have to look forward to.”

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Daily Prompt: George Takei Lost a Fan

The WordPress Daily Prompt for August 13 asks, “When was the last time a movie, a book, or a television show left you cold despite all your friends (and/or all the critics) raving about it? What was it that made you go against the critical consensus?”
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De gustibus non est disputandum. Loosely translated this means, “There is no reason to argue about matters of taste.” There is no fighting over subjective personal likes or dislikes such as colors or sounds. You can not successfully argue me into liking certain smells. I can not punch you hard enough to like what strawberries taste like. Being ticklish or not being ticklish is not an example to offer in a friendly “nature v. nurture” debate.

Comedy does not fall under the “can’t argue (or explain) taste” category of conversations. It is not a subjective taste. At worst, comedy falls in the “agree to disagree” territory—when it fails to entertain or amuse or even offends. But “agree to disagree” is a phrase that at least implies an acceptance of the possibility that opinions can differ. At best, the discovery that you and a new acquaintance share a sense of humor can seal the deal on a friendship for life. I do not know if you find what follows funny, but if you do, consider us friends (if you do not, that is fine, too; it is simply that these 16 seconds might be the quickest way to determine if you and I are potential friends) (yes, I am exaggerating):

Comedy often offends. To a degree, all comedy is offensive, as it shakes up and toys with perceptions; if you do not find the “Fish-Slapping Dance” funny, it may be because you find the waste of intellectual effort offensive. Or perhaps fish jokes generally turn you right off. “How is this funny?” becomes the same question as “Why is this funny?” “Why is this on my screen?”

Usually, “How” and “Why” are not the same question, not even related. How a car functions (fuel, pistons, wheels, tires, go go go) is not the same question as why I am using it (to go buy more fuel to drive somewhere else … um, I may need to look into this vicious cycle). The “Fish-Slapping Dance” actually can be seen as a depiction of the “How is this funny?” conversation. Michael Palin, the “little fishes,” dances the question, and then John Cleese, the “big fish,” delivers the only possible retort. “It is or it is not.” Splash.

“Because it is” is sometimes the only reply, but someone who replies with this is merely saying “Because it is funny to me,” as if to say that one can not argue about taste. That is true, but funny is not a taste. (“Funny tastes” might be a future “Daily Prompt.”) One who says this is falling prey to the logical fallacy that if something is amusing to him or her, and he or she rhetorically concedes that while YOU may not find it amusing (thus covering both bases), you, the non-amused one, are wrong. Wrong. And then you may find them theorizing that you have something rigid intimately involved with your colon. “Agree to disagree!”

So whose perceptions ought to be offended? When can offense be something that even the offended party finds amusing? I do not know if there can be a “unified field theory of offensiveness.” Or comedy. Myself, I am not a fan of “cringe comedy,” television programs or films depicting socially awkward situations in which two parties are in conflict and one party resides in an obviously superior social strata, yet they are the aggrieved party, and we the audience are supposed to laugh at the supposed aggressor, who may simply be a character depicted “doing their job.”

When all the roles are depicted as equal, scenes of bullies getting their comeuppance or returning the comeuppance can be sublime:

Poor Edgar Kennedy! Poor Harpo! Poor Chico! And they are all bullies! All three take turns being the most creatively worst person. They kick each other and destroy hats for no reason other than that is what no one in these positions ever does. They are equals. The comedy lies in the dance of disaster that unfolds for no reason. This is not cringe comedy, as all three get some sympathy and a head shaking laugh. But cringe comedy would happily use this bit (and it goes on much longer through the film, the essential “Duck Soup,” from 1933) and not get what makes it funny. (Larry David would be Chico Marx and his bald Edgar Kennedy would be some anonymous barista.)

Comedy works for me when it presents someone punching up at someone else, at a bully, at someone or something in power. It never works, and it is more likely to offend me, when it is a matter of punching down at someone or something.

(But isn’t Chico Marx’s mock-Italian accent [he was French-Jewish, by way of Brooklyn] offensive? I could reply with the “you have to know the context” argument, that his act was a commonly seen one in the era, that really, Groucho was the only Marx brother with an act unlike any other seen in the era, but if you find the accent offensive in the here and now of 2014 because it is not 1933, I really have no reply. If it is offensive to you, then it is.)

We all are members of interest groups. I am white, half-Jewish, half-Baptist, tall and thin, an alcoholic in recovery, and have a disease that is disabling me. What is the difference between this:

And this:

The second image is from the “Fight Ataxia Project,” a website for those people diagnosed with one of the many forms of ataxia (I was diagnosed with Friedreich’s ataxia for a year) and for their loved ones and caregivers. It is one of several t-shirt logos that the group is selling. When one has a neuromuscular disease like ataxia or spinal muscular atrophy, one trips a lot and one falls down. The t-shirt is for people with such diseases. It exists to show that we can laugh at (at) ourselves. With ourselves.

The first image is a surprise. Not in itself; I have seen it before. It falls into the general territory of hard-hearted internet memes like, “every poor person is gaming the system and has a better cell phone than me,” or “look at these out-of-perfect-physical-shape shoppers at Walmart! They dress funny,” or “disabled people are faking it.” I can only speak for me, but I am poor and you probably have a nicer cell phone than I have, I shop at Walmart and hate it, and I am disabled and wish I was faking it. Why would I?

But I am my own interest group here, obviously. Of course, as a disabled person (not in a wheelchair, and I avoid the word “yet,” but … you know) that must be why I must be offended, right? Nope. Not that. My offense is from the combination of the “miracle meme” insult itself—but it’s not that insulting and I wouldn’t be posting this long post merely about this image—with the “you can’t argue about taste” attitude many took towards it when it was shared by George Takei on his Twitter account last week. (There is a must-read piece—after reading mine, of course—by Scott Jordan Harris in the August 13 issue of Slate.) Yes, that George Takei.

This George Takei:

The wonderful George Takei. The heroic George Takei whom everyone loves, including me. Who has in the past taken down internet memes from his vast social media network when he and his ghost writers have come under criticism for offending. He did not take this one down when disabled rights activists complained (obviously, as I was able to post it here), and wrote this response:

Fans get “offended” from time to time by my posts. There hardly is a day where something I put up doesn’t engender controversy. Concerned fans, worried the sky may fall, ask me to “take it down.”
So I’m also going to ask them also to take it down—a notch, please.

What? Because this internet meme is ironic (a handicapped person stands up to get a bottle of booze fro the top shelf), see, and I do not get the irony because I am too close to the issue. You can’t argue taste, after all.

But wait, George Takei’s argument seems to be that you can argue taste. Many of my posts court controversy, he says, and I receive complaints every day, some of which I respond to, but the complaints from the disabled and their advocates, well, those do not balance out the sheer comedy of the miraculous alcohol post. The complaints of the sensitive disabled (if anything, the disabled are pretty thick-skinned) fell on his deaf ears. Which is a handicap.

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Added at 8:07 a.m., August 14:
George Takei posted this on his Facebook page this morning:

I’ve just come back from an extended trip to England, and I came home to a large number of fan emails concerning a meme I shared more than a week ago. In that meme, a woman in a wheelchair was standing up to reach for a bottle of liquor in the store, and the caption said something about a miracle in the alcohol aisle. To this I added a quip about her being touched by the holy spirits.

I did not expect the level of offense this meme caused. I had naturally just thought of those movies where the evangelical preacher miraculously cures someone who was disabled. What I’d never really considered before so many fans wrote in is how that portrayal of disabled persons is filled with ignorance and prejudice—two things I never want to promote, even inadvertently.

Now, before all of you go and start defending my right to post what I want, I want first to thank the many fans who wrote in with the hopes of educating me on the question of “ableist” bias. While I did not ever mean to suggest by sharing the meme that all people in wheelchairs cannot walk, or that they don’t need them despite the fact that they can stand on their own from time to time, I have taken the fan mail and criticism to heart.

After I’d posted the meme, I noted in the comments an inordinate amount of very uncivil behavior on the part of many fans, including both those who demanded I take it down and those who said I should leave it up. I also received a good deal of email IN CAPITAL LETTERS asking me if I would feel the same way if someone called me FAG or a JAP. Now, I took down the meme from my timeline shortly after it went up, but I admit I was decidedly irked by the tenor of some of those criticizing me. In that moment, I posted a follow up telling fans that perhaps they should “take it down—a notch” which, in retrospect, was not the most sensitive response.

The fact that I was surprised by the response the wheelchair meme received indicates that I do indeed lack knowledge, and some sensitivity, over what is clearly a hot button issue, and that I and others can take this as an opportunity not to dig in, but rather to open up to the stories and experiences of those in the disabled community. I appreciate those who took the time to write in. I wish I’d had the chance to respond sooner, but until today I was not able to go through all the mail I’d received.

So to those who were hurt by my posts on this issue, I ask you please to accept this apology. To those who think I shouldn’t have to apologize, I want to remind you that I get to decide what I apologize for, so there’s no need to come to my defense.

Very well then, carry on, friends. Carry on.

SCA or SMA? Ataxia or Atrophy?

“What a terrible thing to learn you have in the middle of your life.” That was my neurologist, my new neurologist, speaking.

In 2012 my first neurologist, Dr. M, diagnosed me with late-onset Friedreich’s ataxia, or at least he reported that he felt I have a form of spinocerebellar ataxia (SCA), a disease that has dozens of forms and is genetic in origin. Dr. M’s work led to insurance approval to conduct an expensive genetic test to identify which ataxia I have. (The approval would not have been granted frivolously, so I underwent three MRIs, one EMG test, many blood labs to eliminate Lyme disease and the many other viruses that could be a cause of my symptoms, and started seeing a cardiologist. The fact of my cardiomyopathy led to approval for the DNA test.) But Dr. M moved to a different practice and I met my new doctor, Dr. K, this month.

Dr. K reviewed the first doctor’s extensive notes and looked at me. “You don’t have Friedreich’s ataxia.” (Dr. M was not much for granting eye contact, so this moment of direct conversation surprised me.) Dr. K asked me about my symptoms and when they seemed to begin to appear, some of which I have written about elsewhere. Since 2005 I have been aware, at first dimly, of a mobility disorder developing in me; today, in 2014, I walk with a cane, stiffly, like I am wearing very tight jeans, have little sensation in my lower legs and even have moments of “body confusion” in which I think I am moving my right leg but my left leg moves. I sway when I stand and fall/walk into walls and my sense of where I am in the world contributes moments of comedy to my day.

normal brain

‘Abby Normal?’ Not my brain, but good-looking one like mine.

My MRIs show a spinal cord that is shedding its myelin sheath protecting the nerves inside, a couple vertebrae that have spontaneously fused—”When did you have this happen?” Dr. K asked me. “I don’t know,” I replied. “I’ve never had anything done on my back.” “Car accident?” “No.” “Okay, you have a spontaneous fusion”—and places where the nerves themselves are atrophying, withering. But my brain, my cerebellum specifically, it is a healthy brain. I have a fine-looking thinker. Thus, Dr. K posited, I do not have a spinocereballar ataxia, as my cerebellum is healthy.

I spoke in Dr. M’s defense. “I think he was surmising from the tests …” Do not use the words “think” or “surmise” with neurologists. “Ataxia,” Dr. K offered, “has been treated like an umbrella term for patients with movement disorders like yours. But it really is a specific disease and you do not have a spinocerebellar ataxia.” He turned to his computer and started typing what I thought would be yet more patient notes and requests for another battery of tests (Dr. M had generated several pages on my case), but then I noticed what looked like a Wikipedia page on his monitor.

“Based on your labs and the extensive deterioration in your spine and weakness in your legs, you have spinal muscular atrophy, I believe Type IV.” (I swear I heard him say “Type IV” with Roman numerals and in italics.) “Has anyone suggested this to you? No? It may be Type III, but I would say that you have a type called Kugelberg Welander, but very late-onset.” He looked back at me. “You have a genetic disease. What a terrible thing to learn you have in the middle of your life. Your symptoms are severe and I have not seen many adult cases, but you will live, I think, a long life. Your legs will continue to get weaker, but you will adjust.”

(How beautiful is it to have a doctor look in your eye and simply say, “What a terrible thing?” To my first neurologist, a terrific clinician and lab expert, I sometimes felt like I was an interesting case but basically a piece of meat that had the unfortunate habit of speaking.)

“You’re a writer? You’ll probably be teaching me about this by our next visit.”

Spinal muscular atrophy is a genetic disease, a muscular dystrophy that in most cases affects children, but not in my case. The vast majority of spinal muscular atrophy stories are incredibly sad: Type I strikes infants who do not ever control their own movements and eventually suffer respiratory failure and die before age 2. The second type strikes children before age 2 and renders them unable to walk, but most SMA Type II patients live into adulthood. Many children and young adults seen on the annual Muscular Dystrophy Association telethons through the years have had SMA Type II. Type III and Type IV—and there seems to be some debate as to whether there is a Type IV at all—are considered “late onset” and offer patients the lifelong challenge of impaired and deteriorating mobility and, eventually, respiratory difficulty. Since my symptoms struck in my mid-30s and grew aggressive after 40, I seem to be the very picture of an argument for naming a separate Type IV, if what I have is a spinal muscular atrophy.

Dr. K asked about family and friends. I mentioned something about having developed some friendships as a result of reaching out to the online ataxia community (Jason and Kristin: this is me talking about you). “But you don’t have ataxia!” he exclaimed. When he asked if I am still interested in a genetic test to definitively identify what it is I do have, I reminded him that I had been approved for a test for Friedreich’s ataxia. All that test would have shown us, he replied, is that I do not have FA, not what I have.

So one neurologist posited that I have a spinocerebellar ataxia (SCA) of unknown cause and unknown type and another that I have spinal muscular atrophy (SMA), which is rare when it strikes adults but has a relatively gentle prognosis, if a challenging one, with a future of ever decreasing mobility. I know that I do not know. My daily challenges are unchanged by either diagnosis. I could never wish either SCA or SMA on people I detest, but my disease leads me every day to deeper friendships and I hope more patience. Those are both good things. I think I laugh at myself more. I have to.

Since my preliminary diagnosis in 2012, I have been learning about the different ataxias and following the research progress that seems to be unfolding almost every day, especially as it affects Friedreich’s ataxia.

Both FA and the other SCAs and SMA are very serious rare diseases that all too frequently constrain, impair, or even end young lives. But late-onset genetic disease patients, like me, provide a useful, slow-motion version of their disease as it unfolds. As the doctor says in this video here embedded: “The SMA type 3 and type 4 patients are probably going to provide critical information as to the mechanisms of SMA. It is also possible, that a better understanding of the type 3 and type 4 patient, understanding why their disease is so much more benign than the type 1 and type 2 patient, might provide additional windows of insight into therapeutic strategies.”

I have signed up to be listed on the International SMA Patient Registry.

Rare Disease Day 2014

One friend has antiphospholipid antibody syndrome, which causes blood clots to form in any blood vessel in the body and leads to transient ischemic attacks (TIAs), or what she cheerfully refers to as “mini-strokes.” (Most people who have recurring TIAs call them mini-strokes; my friend has the happy quality of coming away from hers with anecdotes about the experience that make them almost sound like something I want to have, too.)

One of my closest friends has fibromyalgia, a disease of chronic pain with a “here today, gone tomorrow, here again and worse” pattern-less pattern of symptom presentation that sometimes renders her almost breathless with the pain. Everyone experiences physical pain, but a full-body stabbing pain with no traceable cause, and thus not many ways to treat it?

Other friends have multiple sclerosis, one whose symptoms are affecting his balance and control of his legs, and one whose hands are malfunctioning. One other friend has a form of epilepsy that is triggered by sleep deprivation yet also causes insomnia, which is one of the most vicious of vicious cycles I can imagine.

I have spinal muscular atrophy, type 4, a progressive, degenerative neuromuscular disorder. I have difficulty walking, because I am losing muscular control of my legs and my balance is affected because my legs from the knees down do not function.

Each of these conditions is a rare disease or a special form of a disease that makes it rare. Sadly, my personal, anecdotal experience with rare diseases is that they are not so rare, but each disease I mentioned above affects fewer than 200,000 Americans apiece, making each one a “rare disease.” However, there are almost 7000 officially recognized rare diseases in total, so rare diseases affect some 30 million Americans, or almost one in 10 people. Eighty percent of rare diseases are genetic in origin and fifty percent affect young children.

Rare_Disease_Day_animated-gif-400pxFebruary 28, today, is International Rare Disease Day. Rare Disease Day was first established in 2008 by EURODIS, the European Rare Disease Organization. In 2009, the National Organization for Rare Disorders (NORD) in the United States joined the effort to educate the public, advocate for better care, and shape policies to improve access to health care. This year’s theme is “Join Together for Better Care.”

 
Even though some 30 million Americans have a rare disease, and many millions more have rare diseases around the planet, each disease affects relatively few people, so rare diseases are also known as “orphan diseases.” The medical and pharmaceutical industries do not have a stellar history with treating rare diseases; drugs for rare diseases are called “orphan drugs,” because they do not address a family of disorders. In the video below, Sean Hepburn Ferrer, son of Audrey Hepburn and Mel Ferrer, addresses this issue. His mother died in 1993 from a rare form of cancer, Pseudomyxoma adenocarcinoma, that had not been researched in decades. A diagnosis was difficult to come by and treatments were limited to decades-old knowledge.

Those who care for and live with people with rare diseases are also affected by the disease and the sometimes grueling path to diagnosis. My family, my friends, and my girlfriend have walked with me every step of my path and keep me upbeat, encouraged, and sane. This day, honoring care for those with rare diseases, honors them, too. I love them.