Bits and Pieces: Arctic Vortex Edition

Okay, I know it’s considered terribly mundane and just downright boring to talk about the weather, but the winter here in NYC and in much of the Midwest and Eastern US has really been something else these last few months. Since weather reports here in The States only occasionally and very briefly touch on conditions outside of our borders, I’m only parenthetically aware that much of the rest of the world has also experienced extreme conditions this winter as well, but I do know that plenty of WK readers all around the world have been hit with some downright nasty tricks from mother nature this season.

Here in NYC, we’ve been subjected twice (so far) to a meteorological horror called the Polar Vortex, a huge mass of frigid air that descends from the North Pole, bringing with it plunging temperatures and loads of snow. In my previous 49 winters I don’t recall ever hearing anything about a Polar Vortex, though I do remember plenty of times freezing my ass off, so perhaps this is just a new name for an old song. Whatever the case, getting around in a wheelchair in temperatures fit for the Arctic can be brutal. Not that walking around when the wind chill hits instant frostbite territory is any great shakes, but at least the very act of walking generates body heat. Sitting in a wheelchair does no such thing, and the breeze added by zipping around at 7 or 8 mph only adds to the freeze.

Fortunately, I’ve got some very good cold-weather gear. Never mind high-tech fleece and modern materials like Thinsulate, my warmest piece of kit is an exact replica of the shearling jackets worn by US airmen fighting high in the skies above Europe during World War II. Back then the interiors of military aircraft were unheated, and at 20,000 feet the temperatures were routinely well below 0°F. My B-6 flight jacket is so warm that I can’t wear it in temperatures much above 25°F, and even when the temperatures hit the single digits a T-shirt and a thin wool sweater worn under the jacket are more than enough to keep me toasty. I also have a replica of the shearling hat worn by World War II flight crews, so decked out in my vintage military gear I can zip around the frozen city making believe my wheelchair is a P-51 Mustang fighter plane engaged in fierce dogfights with the mighty Luftwaffe (represented by oblivious pedestrians staring at their cell phone screens despite the freezy conditions) on the streets of New York. I’m proud to say this winter I’ve achieved the lofty status of “ace”, having bagged far more than the requisite five kills. Mwah hah hah.

Unfortunately, donning the flight jacket requires wrestling with my arch nemesis, the zipper. The freaking things are almost impossible to use when you’re down to only one marginally good hand, so I have to allot myself an extra 10 or 15 minutes of prep time just to get my goddamned coat on. Have I ever mentioned that having MS sucks? (I’ll be presenting some exciting zipper related news later in this post, I hope you can bear the suspense.) Luckily, for temperatures just a bit below freezing I have a very special pea coat that my mother-in-law, who is quite the whiz with a sewing machine, made into a Velcro fastened wonder. She removed the buttons and then sewed them on top of the coat’s now sealed buttonholes, affixing Velcro fasteners to the garment where the buttons would normally hold the coat closed. The black Velcro patches exactly match the color of the coat, making them almost invisible, and when I put it on the coat practically “buttons” itself. The buttons sewed over the buttonholes give the illusion that the coat is fastened in the customary fashion, giving no hint of the Velcro customization within. I made sure to get a military issue pea coat, so the thing is quite warm and sharp looking to boot. I’m pretty sure I have the only Velcro fastened pea coat in existence, and I’m positively tickled with my piece of unique outerwear. It’s like haute couture for gimps.

So, between my World War II flight jacket and my MS friendly Navy pea coat, I’ve got the problem of winter wheelchair attire practically licked. I’m sure this comes as a great relief to all of you, so rest easy, the Wheelchair Kamikaze will not be found frozen stiff in his chair on the thoroughfares of New York anytime soon. And the Luftwaffe will not be menacing the sidewalks of NYC, at least not on my watch. After all, terrorizing the streets of New York is my job.

But enough about me, there’s been a lot of interesting MS and disability related news lately, so forthwith comes my semi regular compendium of noteworthy tidbits… Onward, doggies, mush mush…

♦ There’s no denying that in addition to being a horrible disease, multiple sclerosis is also a burgeoning multibillion-dollar a year industry, with many of the drugs developed to combat the disease attaining “blockbuster” status. Ever wonder just how much money MS drugs bring in? Well, wonder no more, because here’s a list of the top 10 selling MS drugs of 2013, along with the cash flow generated by each (in American dollars):

#1: Copaxone-$4.3 billion

#2: Avonex-$3.0 billion

#3: Gilenya-$1.9 billion

#4: Tysabri-$1.7 billion

#5: Betaseron-$1.1 billion

#6: Tecfidera-$876 million

#7: Rebif-$622 million

#8: Ampyra-$302 million

#9: Aubagio-$226 million

#10: Extavia-$159 million

Add up all of that mazuma, and the top 10 selling MS drugs generated sales of roughly $14,000,000,000 last year. Yup, that’s a lot of zeros. Keep in mind, the sales of Tecfidera only represent about one quarter of the year, since the drug was first approved midyear and didn’t reach patients in large quantity until the fall of 2013. Additionally, Tecfidera was only recently approved for use in Europe (click here), so expect sales of the drug in 2014 to approach at least four or five billion dollars. Factor in all of the money made by MS neurologists, MRI facilities, infusion suites, medical laboratories and other MS related services and facilities,  and it's easy to see that multiple sclerosis has become quite the cash cow. I don't know about you, but the idea of "disease as industry" makes me a little bit queasy. For more info on each of the above drugs (click here).

♦ Speaking of blockbuster MS drugs, the Israeli pharmaceutical company Teva has received FDA approval for a higher dose Copaxone regimen requiring injections only three times a week (click here), as opposed to the daily injections patients currently on Copaxone must undergo. Copaxone was among the first of the disease modifying therapies to be marketed for MS, and was first approved in 1996. Since then, the drug has been shown in several studies to effectively reduce relapse rates and enhancing lesions for those RRMS patients on whom it is effective by about 35%. Recent open label studies looking at the long-term effects of the drug indicate that Copaxone may also positively impact the rate of disease progression (click here).

One may wonder why it took Teva 18 years to seek approval for a thrice a week version of the drug, since the new dosing schedule is obviously much more appealing than the old daily injection regimen. Maybe I’m a jaded cynic, but the fact that several companies are preparing to introduce generic Copaxone on the market might just have a little something to do with it. All of the generic versions of Copaxone are undergoing trials as once daily injections, so by getting approval of the three injections a week dosing regimen, Teva has made good old-fashioned brand-name Copaxone a much more appealing option from the patient perspective. Additionally, the success of Tecfidera, an oral medication, may also have played a role in Teva seeking a way to make the administration of Copaxone a less painful proposition. As was illustrated in the above list of top grossing MS drugs, Teva has about four billion reasons to try to protect their Copaxone franchise. In any event, this is good news for patients who have had success with Copaxone, who will now be required to give themselves 60% fewer injections. Of course, if there are any Copaxone patients out there who are masochists, this could be bad news, but hey, you can't please everybody…

♦ As I reported in my last Bits and Pieces post (click here), the powerful experimental MS drug Lemtrada was recently denied approval in the US by the FDA. The drug has been approved in most of the rest of the world, including, most recently, Mexico (click here). This action by the FDA has raised the ire of US MS patients, researchers, and clinicians alike (click here and here).

The FDA claims that it did not approve Lemtrada because the trials undertaken to prove the drug’s efficacy were poorly designed, but the FDA had previously approved the design of those very same trials. Lemtrada is a very controversial drug, as it carries a very high risk/reward profile. In trials undertaken by the drug’s manufacturer, Genzyme, as many as 50% of patients with highly active RRMS given Lemtrada were shown to have no evidence of disease activity (no relapses or new lesions) five years after they received their last dose of the drug. This is a startling success rate, but it comes at a price, as up to 30% of Lemtrada treated patients develop autoimmune thyroid diseases, and some develop a potentially fatal autoimmune blood disorder. It’s important to note that autoimmune thyroid disease is easily treated using hormone supplements, and the risk of the autoimmune blood disorder can be at least in part alleviated through careful monitoring.

Lemtrada is a powerful immunosuppressive agent which works by essentially rebooting a patient’s immune system. For patients with highly aggressive relapsing remitting disease, suffering some of the worst ravages of MS, the potential benefits of the drug could very well outweigh the known risks. I personally spoke to an MS neurologist who has my utmost respect about the FDA’s decision, and he was extremely dismayed by the agency’s actions, telling me of one patient who was being laid to waste by one of the most aggressive forms of relapsing MS. Not only did the drug stop the disease in its tracks, but the formerly MS ravaged patient recovered enough to actually be able to go back to work. Given this level of potential benefit, shouldn’t the decision as to whether or not to use Lemtrada be left in the hands of neurologists and the patients they treat? Seems the rest of the world thinks so, but not the FDA. Nope.

♦ In a disconcerting piece of MS news, a recent study has found that MS does indeed negatively affect life expectancy in the patients it strikes (click here). Researchers used insurance claims to identify a group of over 30,000 MS patients, and compared them to about 90,000 non-MS patients, finding that the MS patient population lived on an average six years less than their non-MS counterparts. The results of this study mirrored those done in some other parts of the world, but this was the first time such data was examined for MS patients in the United States. It’s important to note that this study looked at patient data from 1996-2009, and thus doesn’t include patients taking some of the newer, more powerful MS drugs. One can only hope that these drugs will positively affect MS patient longevity, and that future treatments will have an even greater significant positive impact. One can also hope that researchers concentrating on areas other than immunosuppression will come up with drastically more effective treatments that – gasp – actually address the root cause of the disease. Fingers crossed.

♦ In some better news, researchers in Australia have had initial success treating a patient with secondary progressive MS by increasing his immune response to Epstein-Barr virus (click here). EBV has long been suspected to play a role in the MS disease process, and this is the first research to directly target the virus in an attempt to alleviate multiple sclerosis. The research involved isolating a specific type of immune system cells, called “killer T cells”, from the patient’s blood, adding an experimental anti-EBV vaccine to them, and then growing more of them in the laboratory. The cells were then injected back into the patient over the course of eight weeks. The patient reported less fatigue, fewer leg spasms, better cognition, and improved use of his limbs. Of course, this was only one patient, and this research is in its very earliest stages. But hey, hope springs eternal…

♦ More news on the “infectious” front. Researchers following up on the discovery of a rare foodborne bacteria in an MS patient (click here) have infected rodents with that same bacteria, and found that the little varmints developed a disease very much like MS (click here). It’s long been suspected that infectious agents play some role in the MS disease process, a belief based on a convincing body of evidence. In fact, before attention shifted to the “autoimmune hypothesis” and pharmaceutical companies started making billions of dollars by producing drugs that tinker with the MS immune system, much if not most MS research was directed at finding the presumed infectious cause of MS. This area of research has been widely neglected for the past couple of decades, though, but interest in it seems to be picking up. From a patient perspective, this is a very good thing, for if one or more infectious cause or causes of MS can be positively identified we would finally be making great strides towards curing the disease. So, let’s pick up our MS research cheerleader pom-poms and shout in unison: “Frika Fraka, Firecracker, Shish Boom Bah, Infectious Agents, Infectious Agents, Rah Rah Rah!” Unfortunately, because of my debilitated physical state, I’m only able to pick up one of my MS research cheerleader pom-poms, but I expect the rest of you who are more able-bodied to do some vigorous two-fisted cheering.

♦ Although the above rhyme is about as poetic as I get, another member of the MS blogger community, Judith Mercado, is a far more accomplished poet, and has now published a book of her inspired work. Author of the blog “Peace Be with You” (click here), Judith writes her poetry in the ancient Japanese form of haiku, and I’ve long been a fan of her work. I was greatly honored when Judy asked me to provide a blurb for the back cover of her upcoming book, Peace on the Journey, and was of course happy to do so. If I may take the liberty of quoting myself, Judy’s “words resonate with wisdom and truth, and grace the reader with intimacy, honesty, and understanding.” While Judith’s blog often references her MS, the poems in Peace on the Journey are meant for a more general audience, anybody who travels on the sometimes gentle but all too often tumultuous path of life. Peace on the Journey is available in paperback and electronic Kindle version on Amazon (click here). Highly recommended.

♦ As promised, here is the earth shattering zipper news I promised the opening paragraphs of this post. An inventor seeking to help his physically challenged uncle has come up with “Possibly the Most Radical Innovation to Zippers in over a Century” (click here). Eventually joined by his mom and a mechanical engineer neighbor, inventor Scott Peters spent six years and went through over 100 prototypes before coming up with the Quickzip, a magnetically assisted zipper that only requires one hand to fasten. Be still my heart! The team of the inventors struck a deal with athletic clothing manufacturer Under Armour, which plans to start marketing Quickzip (now renamed Magzip) equipped clothing in the fall of 2014. I’m not one to encourage patent infringement, but I call on clothing manufacturers worldwide to meet this challenge and come up with their own easy fastening zipper innovations. Must I pick up my cheerleader pom-pom once again?

Here’s a rather, um, dramatic video produced by Under Armour highlighting the new technology…


RIP Heidi Sherman

My New Ride

Well, I got myself a new rig. Yup, after five and half years the time has come to put my old wheelchair out to pasture, and to welcome a new mechanical monster into the fold. I’m a bit of a sentimentalist, prone to developing emotional attachments to things animate and inanimate, so consigning my old chair to mothballs comes with mixed feelings. The old guy has served me well, and was, after all, my first wheelchair. Don’t we always hold a special place in our heart for our firsts? First base hit on a baseball diamond, first kiss, first boink, first wheelchair – wait a minute, one of these things is not like the others.

I find it almost impossible to believe that five and half years have come and gone since that old chair and I first made our acquaintances, but I guess that just goes to show that time flies even when all of your days are not exactly filled with wine and roses. Time definitely speeds up as you get older. These days, I’ll retire to the bathroom with a good magazine, and when I come out it seems several months have passed. I’m pretty sure Einstein noted this same phenomenon in his general theory of relativity, much of which I’m fairly certain he came up with while sitting on the throne. There’s a reason men spend so much time in the bathroom. Lots of heavy thinking going on in there. Trust me.

My new chair is the exact same model as my old chair, a Quantum Q6000Z (click here) with the high-speed motor package installed (hee hee). Unlike my trusty old friend, which had a static seat, my new mechanical wonder comes equipped with all the bells and whistles. The seat tilts, reclines, the leg rests extend and rise, and the seat can elevate 10 inches. With the seat reclined fully and the legs raised to their maximum height I can just about lie flat on the thing. It’s almost like having an easy chair on wheels.

All of this, of course, makes the chair much more comfortable for extended periods of use, but, alas, there’s the rub. Five and half years ago I didn’t need all of these fancy features because I was much more ambulatory than I am now. In fact, when I first got the old chair I didn’t even use it around the apartment, only employing it for outdoor excursions. These days my ability to walk has been reduced to attempting maybe five or six treacherous cane assisted steps, and I’m finding the new chair a much more hospitable environment in which to plant my backside for long stays. And although I do appreciate all of the new features, they also serve to remind me of the full-court press being put on by the disease, and just why they call progressive diseases progressive – they progress. I have a real bone to pick with whoever came up with this demented concept; I’d really like to give them a piece of my mind. Hey, hold on, thanks to MS, I already have. Dammit.

Naturally, the new chair is taking some getting used to. It’s a bit larger than the old chair, due to all of the extra seating motors and stuff, so it’s a lot trickier getting it around the tight corners in my apartment, and it’s not quite as responsive when trying to dodge pedestrians on crowded city streets. Hey, their problem, not mine. What’s a few ruptured Achilles tendons and smashed kneecaps amongst fellow New Yorkers? If pedestrians walking on the streets of New York insist on having their eyes glued to the screens of their cell phones, I refuse to take any responsibility whatsoever for whatever damage comes to them if they happen to crash into my speeding chair. I’m just a maniacal gimp gleefully careening through the streets of New York. I abdicate all culpability in the matter. After all, they’re the ones with working limbs. Or at least they were before running into me. Dammit.

This chair doesn’t seem to have the same range as my old chair, in which I could travel about 15 miles. Because of the very wintry conditions we’ve had lately, I’ve not taken the new chair out for an extended trek, but it looks like this chair’s range is significantly less than the old one. Of course, my body isn’t up to my marathon jaunts of old, as my “good” joystick controlling hand tends to want to stop working after shorter and shorter intervals, thanks to that whole progressive disease thing. Dammit.

Astoundingly, the list price of my new tricked out wheels came to an eye-popping $29,000. Yikes! I could buy a pretty decent car for $29,000, or even two economy cars for that same amount. I can only imagine the profit margins on power wheelchairs. How much could all of the parts cost? $10,000, max? I’d much rather have spent the money on a nice “preowned” five-speed convertible BMW, but these days I’d qualify more as luggage than driver, so that’s out. Dammit.

Thankfully, my wheelchair vendor got me a discounted price on the new rig, and my insurance company picked up the majority of the tab. Between the new chair, all of the diagnostic tests I’ve gone through to figure out the mysteries of my disease, and the myriad treatments I’ve tried in vain trying to tame it, I guess I could be the poster child for why healthcare costs in the US are absolutely insane. But hey, at 50 years old I think I’d be more a poster man than child, despite my hard fought battle to maintain my youthful demeanor. Thankfully, you’re only young once but you can be immature forever, and that’s exactly how I intend to continue to play it, with a hearty "hey diddle diddle and a nah nah nah" to boot. Perhaps I’m delusional, but looking at the new chair as a really slick shiny toy to play with makes the whole concept of “me in a wheelchair” much easier to swallow.

Hey, whatever it takes. Dammit.
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