Bits and Pieces: 1,000,000 Page Views Edition (Also: Lemtrada, Tysabri Risk/Reward, and Asinine Research)

Eyeballs (Photo credit: Skrewtape)

(If you have received this via email, the following post contains a slideshow and a video, which can be viewed on the Wheelchair Kamikaze website. Please (click here) to view the multimedia content on your web browser.)

Gadzooks! Zounds! Great Gooble Gobble! Late last week Wheelchair Kamikaze received its one millionth page view. 1,000,000! I’m absolutely gob smacked, even though I’m pretty sure about half a million of those page views can be directly attributed to my mother.

In the nomenclature of the Internet, each page view is just what it sounds like, an instance when somebody finds their way to a specific website through a search engine or link and gives it a peek. So, in the four years and 11 months since its inception, my modest little abode on the Internet has been looked at by folks 1,000,000 times, a figure which truly boggles my mind. People from all over the world have visited this place; the stats provided to me by Google show that in the last month alone Wheelchair Kamikaze has been frequented by people in (in alphabetical order): Australia, Belgium, Canada, China, Costa Rica, The Czech Republic, France, Germany, Iraq, Israel, Luxembourg, Malaysia, Mexico, The Netherlands, New Zealand, Norway, Poland, Saudi Arabia, Serbia, Spain, Sweden, Switzerland, Ukraine, United Arab Emirates, United Kingdom, The United States, and Venezuela. Holy crap!

In all honesty, I never expected more than a couple of dozen people to ever look at this thing, as when I started Wheelchair Kamikaze I wasn’t even all that sure of what a blog actually was. I’d been quite active on several online MS forums for a number of years, and during that time several fellow forum members intermittently urged me to start writing a blog, a notion to which I wasn’t all that favorable. I’d never really ventured into the “blogosphere”, and my conception of what a blog could be was fairly limited. In my mind a blog was pretty much just a sort of online diary, and I really didn’t think that what I did or thought would be of interest to anybody outside of the small sphere of human beings who actually knew me.

It wasn’t until my sorry ass landed in a wheelchair, and, at my wife’s urging, I attached a camera to that wheelchair and made a few videos of my wheelchair rides through Manhattan – which friends and family found amusing – that the idea of staking out my own virtual homestead took hold. Okay, I thought, a blog could provide me a place to house the videos and photos I took from my wheelchair, and maybe the occasional scribble or two, in a spot that would be easily accessible to the relatively few people who knew I existed. Never in my wildest dreams did I imagine that this site would provide a conduit through which a part of me could reach out and touch people all over the world, and that in turn these virtual connections would enrich my life in ways that are literally beyond words.

Wheelchair Kamikaze has provided a kind of method to the madness of my being stricken ill, and has many a time proven to be a lifeboat of sorts, helping me keep my head above the churning, tempestuous psychological waters of dealing with my chronic progressively disabling disease. For that I am more than grateful, and to all of the wonderful people who have contributed to those 1,000,000 page views I offer my most humble gratitude, which hardly seems sufficient given the remarkably positive impact creating this blog and interacting with those who view it has had on my life. It may be overstating it to say that Wheelchair Kamikaze has been my salvation, but it wouldn’t be overstating it by much. So, thank you, thank you, thank you.

Okay, with that bit of mushiness done with, let’s get to the business at hand, a rundown of various pieces of MS related news that have garnered my attention over the last few months or so. As usual, these pieces range from the sublime to the ridiculous, and I hope you’ll find them pertinent, useful, interesting, and/or amusing. On with the show…

♦ A new MS drug called Lemtrada has been approved for use in Canada (click here), Australia (click here), and the European Union (click here), but, surprisingly, not here in the United States, where the FDA rejected Lemtrada’s application for approval (click here). The FDA’s rejection comes as something of a shock, as it was widely expected that Lemtrada would receive the FDA’s authorization, despite the problematic side effect profile of the drug.

Lemtrada isn’t actually a new drug; in a previous incarnation, when it was called Campath, it had been used successfully to treat leukemia and lymphoma since 2001. The drug is a monoclonal antibody, a member of the same family of drugs as Tysabri, and works by dramatically depopulating immune system cells in treated patients. Lemtrada is so effective in wiping out its immune cell targets that it in effect prompts the body to “reboot” the immune system, much the same way that certain stem cell therapies attempt to do. The idea is relatively straightforward – wipe clean a person’s immune system, in the hopes that when that immune system is reconstituted it will no longer have an appetite for the patient’s own central nervous system cells. This relatively straightforward concept has proven somewhat tricky to pull off in practice, but Lemtrada does seem to accomplish this feat over an extended period of time.

When used to treat MS, Lemtrada is given intravenously in five day courses two or three times over a 12 month period. After this initial one-year period of dosing, clinical trials have shown Lemtrada to be remarkably effective when given to relapsing remitting patients with active disease. One study (click here) showed that five years after treatment 65 percent of such patients were free of clinically active disease, 72 percent were relapse free, and 87 percent of Lemtrada treated subjects were free of sustained accumulation of disability. In other words, five years after treatment, well over half of the patients given Lemtrada showed virtually no signs of multiple sclerosis activity – they were, for all intents and purposes, MS free.

What then, is the problem? Unfortunately, Lemtrada’s effectiveness comes at a price. Approximately 30 percent of Lemtrada treated patients develop autoimmune thyroid disease, which, though not to be pooh-poohed, can be effectively treated using conventional therapies. More disturbingly, some patients develop an autoimmune blood disease called immune thrombocytopenia (ITP), which, if not caught in time, is often fatal. Patients can be effectively monitored for ITP, but the fact that Lemtrada leaves patients susceptible to the potentially deadly disease is problematic, to say the least.

In those places where it has been approved for use, Lemtrada poses RRMS patients quite a dilemma. Is the prospect of being disease-free five years after treatment worth the risks associated with autoimmune thyroid disease and ITP? Quite the conundrum, but I would think that at least some folks being ravaged by highly active MS, experiencing relapse after crippling relapse, would certainly be willing to take the risk. As we’ve seen with Tysabri, patients can be quite tolerant of risk when a drug dramatically increases their quality of life. It will certainly be interesting to watch as the Lemtrada saga plays out in the regions that have given it approval.

Just as a side note, Genzyme, the drug company that manufactures Lemtrada, engaged in some sleazy activity several years ago by pulling Campath off the shelves when it appeared that the drug – newly named Lemtrada – would sail through the approval process for use as an MS therapy (click here). Why pull Campath off the shelves? Because Genzyme planned to dramatically hike the price of the drug when they changed its name from Campath to Lemtrada and switched focus from leukemia/lymphoma patients to those suffering from MS. When used to treat leukemia or lymphoma, a typical course of Campath treatment cost about $60,000. Since MS patients would need a far lesser dose of the drug, the cost for a course of multiple sclerosis treatment would only be about $6000. So, Genzyme made Campath unavailable and only planned to reintroduce it as Lemtrada once it was approved for use in MS patients, at a dramatically higher price, of course. 

Although the drug has been approved in Canada, Australia, and EU, those places restrict the price of drugs, something that is unheard of in the US, where drug companies can pretty much charge whatever they please. And now the FDA has failed to approve Lemtrada, foiling Genzyme’s Machiavellian business plans. What goes around comes around, as they say…

♦ Having mentioned Tysabri and the fact that it improves the quality of life for many of those taking it, here are two studies that illustrate just that. One study (click here) shows that Tysabri treated patients require less sick leave from work 12 months after initiating treatment. This retrospective study demonstrates that one year after initiating Tysabri treatment, MS patients required 33 percent less sick leave than before they started on the drug. Another study looked at MS patients requiring inpatient hospital stays, and found that Tysabri treated patients exhibited “significant reduction in the percentage of patients with MS related inpatient stays, MS related inpatient costs, and length of stay” (click here).

Most MS patients know that taking Tysabri carries with it the risk of developing a potentially deadly brain infection called PML. Now that Tysabri has been on the market for a considerable amount of time, we have reliable figures as to just what the risks are of developing PML while on the drug. The latest figures indicate that patients who are JC virus negative (JC virus is the pathogen that causes PML) have very little risk of developing the infection, about a 1 in 10,000 chance.

For patients who test positive for the JC virus, two factors come into play in determining their risk of developing PML. The first is whether or not they’ve previously been treated with immunosuppressive drugs, and the second is the amount of time they’ve been on Tysabri. JC virus positive patients who have been treated with prior immunosuppressants have a 1 in 556 chance of developing PML during the first two years of treatment, and a 1 in 89 chance in years two to four.

JC positive patients who have not been treated with prior immunosuppressants need to keep a careful eye on the amount of JC virus antibodies present in their blood, which can now be checked by blood tests. Depending on these levels, the chance of developing PML for these patients in the first year of Tysabri treatment ranges from 1 in 1000 to 1 in 10,000, in years two through four from 1 in 123 to 1 in 3333, and in years four through six from 1 in 118 to 1 in 2500.

The above figures can be viewed nicely in the slideshow below. Click the symbol in the lower right-hand corner of the slideshow to view fullscreen (if that doesn't work, right-click on the symbol and choose "open in new tab"). I’d advise all Tysabri patients to familiarize themselves with these numbers, and make risk/reward calculations based on their individual circumstances, in conjunction with their neurologists. Knowledge is power, people; arm yourselves accordingly.

Barts MSer PML Risk Guide Dec 2013 from Gavin Giovannoni

♦ One of the big problems I have with all of the currently available MS treatments is that they don’t do anything at all to address the root cause of the disease. How can they, since the root cause of MS remains completely unknown? Several recent discoveries may shed some light on that elusive cause, which increasingly appears to be at least partially infectious in nature. In one study, researchers found evidence that a soil-based bacteria, which has rarely if ever previously been found in humans, may be prevalent in MS patients (click here). Researchers reported that “that we identified this bacterium in a human is important enough, but the fact that it is present in MS patients is truly significant because the toxin targets the exact tissues damaged during the acute MS disease process.”

Another study found toxins secreted by bacteria associated with sinus infections in the cerebrospinal fluid of MS patients (click here). This is important because most blood-borne bacteria are blocked from entering the central nervous system by the blood brain barrier, nature’s way of keeping the brain and spinal cord isolated from the nasties that can infect other parts of the body. Infections in the sinuses, though, can circumvent the blood brain barrier by leaking directly from the sinuses into the central nervous system, opening up the possibility that nose to brain transport of bacterial toxins may play a key role in the MS disease process.

Though studies such as these are far from definitive, they do at least attempt to answer THE key question regarding MS: what in heaven’s name causes the freaking disease? There will never be a cure for the MS until that query is answered, and far too little time, effort, and money is currently being spent attempting to unravel this all-important mystery. One would think that such inquiries would be at the forefront of MS research, but instead, because most research is funded by pharmaceutical companies who must turn a profit to survive, the majority of MS research is currently targeted at finding new and better ways to suppress the immune system, resulting in ridiculously expensive drugs that may tame the disease but will never cure it. I’ll practice some self-restraint and stop this line of argument now, before I start ranting and raving and giving myself and my readers a migraine. Arghhh!

♦ Okay, on to one of my favorite subjects, the wonderful world of asinine research. Crack researchers in Germany have determined that spasticity is a problem for MS patients (click here). When I say “crack researchers”, I mean that the researchers must have been smoking crack. How else to explain them wasting time and precious research money conducting a study that confirms what is obvious to anybody suffering from MS spasticity, or anybody observing, even from a distance, somebody suffering from MS spasticity? The researchers could have spared themselves a lot of effort by just asking me or some of my MS buddies about spasticity. Spasticity sucks. End of study.

For those who are blissfully unaware of MS spasticity, the phrase refers to muscles that are rendered stiff and nonfunctional because they receive nerve signals to contract but not the requisite impulses to relax due to the fracked up nature of the MS ridden central nervous system, replete with faulty wiring and short-circuits. Spasticity can afflict almost any muscle in the body, and often causes considerable disability and pain. In fact, many MS patients consider spasticity their most troublesome symptom.

Now, through their earthshaking work, German researchers have, after fastidious and meticulous investigation, determined that “MS patients with spasticity suffer a significant burden because of resulting disabilities and reduced quality of life, especially in cases of severe spasticity”.

HOLD EVERYTHING!!! LIGHT MY PANTS ON FIRE!!! Do they mean to tell me that my twisted and clublike right arm and my clawlike right hand are not doing me any favors, and are in fact a “burden”? That my quality of life might be better if putting on a shirt, sweater, or jacket didn’t require my gimpified body to attempt the moves of a circus contortionist? That the “burden” of my disease would be less if I – gasp – didn’t have any spasticity? And all this time I’ve been gazing upon my twisted and useless appendages with such warmth and affection. Stupid me!

Let me save any researchers currently working on similar studies a lot of sweat and elbow grease. Not only does spasticity decrease quality of life, but weakness and paralysis can also be quite “burdensome”. Yup, arms and legs possessing all of the strength of a fart in a hurricane should not be counted as one of the pleasures of life. Also, bladder and bowel issues are not nearly as much fun as a night at the GiggleSnort Motel. That’s right, contrary to popular belief, urinary frequency and urgency don’t make for a whooping good time. Yes, I’d like to down a couple of pints of icy cold beer as much as the next guy, but I’d better be sitting on a toilet when I do so, because you could calculate the time it takes for the liquid to go from mouth to urethra with a stopwatch. Now, that might be the subject for some fascinating research.

Good grief…

♦ Being “retired” and grappling with a chronic debilitating illness leaves one with plenty of time to contemplate the mysteries of life, wondering just what the hell it’s all about. Gratefully, I came across the following video, in which Father Guido Sarducci (comic Don Novello) explains The Secret of Life. I actually recall the good Father doing this bit on Saturday Night Live back in the late 1970s, when the cast included such greats as John Belushi, Gilda Radnor, and Dan Aykroyd. I remember loving this monologue back then, and the intervening decades have done nothing to diminish its effect on my funny bone. Yup, life is a job, we’re all just here collecting our paychecks, hoping that our balance sheet comes out in the black when all is said and done…


Ciao for now…

'tis Better to Give… (Worthy MS Nonprofits for Holiday Giving)

English: Santa Claus as illustrated in , v. 56, no. 1449 (1904 December 7), cover. (Photo credit: Wikipedia)

(For those who receive these posts via email, this essay contains videos which can only be viewed on the Wheelchair Kamikaze website – click here)

Yes, it’s that time of year again: the holidays are upon us. It’s incredible how they always manage to just kind of sneak up on you even though it’s impossible to miss the signs of the season, most notably the endless stream of holiday themed advertisements and TV commercials that these days start running even before Halloween.

Maybe it’s precisely because of that constant commercial bombardment with manufactured Christmas cheer that the actual day of the holiday inevitably comes as something of an anticlimactic  shock. In an effort to simply maintain our sanity, perhaps we tune out all of the faux happy holiday chatter and go into a survival mode state of denial. After all, there are only so many Mercedes, BMW, Lexus, and Audi holiday advertisements one can watch before the brain simply perceives them all as white Christmas noise. And does anybody ever actually ever get a luxury automobile for Christmas? Anybody normal, I mean, folks in the “1%” not included? Back in 1981, my mother’s boyfriend gave me his recently deceased father’s 1970 Oldsmobile, but it wasn’t for Christmas and the car needed a new transmission and left rear quarter panel. Still, at 17, I was thrilled with my first set of wheels, and in my adolescent exuberance that 11-year-old Cutlass may as well have been Santa’s sleigh complete with flying reindeer.

Whatever the reason, I always seem to find myself scrambling to order last-minute Christmas gifts for the folks on my list, even though these days my schedule isn’t exactly bursting at the seams. Thank heavens for the Internet, where even gimps can spread Christmas cheer without much fuss. With just a few clicks of the mouse, it’s ho ho holy crap, I just maxed out my credit card. But far be it from me to play the part of Scrooge, as I do derive great satisfaction in giving gifts to the ones I love. In fact, I honestly much prefer giving gifts to getting them. These days, given my limited physical capacities, there isn't really much that I need, except maybe a brand-new central nervous system, which I can only imagine would be especially hard to wrap and would make quite a mess under the tree…

In the spirit of giving I thought I’d provide a list some lesser-known MS nonprofits that would greatly benefit from the holiday largess of MS patients and those who care about them. In the world of MS nonprofits, the National Multiple Sclerosis Society is the great big hairy ape in the room. Due to its ubiquitous MS Walks, high public profile, and aggressive fundraising, the NMSS has become the face of MS to the general public. When most people think about making a donation on behalf of MS patients, it’s to the NMSS that the money flows. The National Multiple Sclerosis Society is to MS nonprofits as Kleenex is to tissues, melding in the public’s mind as one and the same.

In reality, though, there’s a plethora of worthy smaller MS nonprofit organizations out there, many of them starving for funds and some of them doing incredibly important work in the nuts and bolts research trenches that will with any luck eventually cure this damned disease. Trust me, when the cure comes it will most likely be from one of these little guys, not from the monoliths most often associated with MS research, who, though well intended, may be just a little too invested in the status quo, even if on an organizationally subconscious level. I by no means want to disparage the work being done by the NMSS, and the folks I know who work for the organization are all extremely caring and dedicated people, but there are lots of other guys in the sandbox who tend to get crowded out by the well hewn NMSS fundraising machine.

Here then are a handful of nonprofit MS research groups whose voices are all too often drowned out by the booming fundraising megaphones of larger organizations. The below groups all get the much coveted Wheelchair Kamikaze stamp of approval, and I’d urge WK readers to request that their family and friends who might be inclined to make MS related donations this holiday season to consider the following groups in lieu of some of the more obvious candidates.

♦ The Myelin Repair Foundation (click here) – As its name implies, the MRF is entirely devoted to researching strategies and methods for repairing the damage done by the MS disease process. Founded by Scott Johnson, himself a PPMS sufferer, the Myelin Repair Foundation aggressively seeks to break down the barriers that slow down medical research by encouraging collaboration rather than competition, and actively partnering with research groups and organizations pursuing the tangible goal of myelin repair and neuroregeneration.

Now maintaining its own research laboratories, the MRF has set its goal to have a therapeutic agent that repairs MS nervous system damage available to patients by 2019. That may seem like a distant date, but it’s only five years away, and for decades patients have been told that a cure for MS will be had within 10 years. So far that promise has been nothing but a lie, but I’m confident that the MRF stands an excellent chance of turning promises into reality. I’ve had the pleasure of having dinner with Scott Johnson, and I can personally attest to the drive and dedication of the man and his organization. The MRF is already making great strides towards reaching their goal, a goal that once achieved will have tremendous positive impact on each and every patient stricken with multiple sclerosis.

I’ve previously posted the below video, but it’s exceptionally well done and conveys the mission and vision of the MRF in an extremely personal and emotional fashion, while memorializing my late friend and comrade in arms, George Bokos, The Greek from Detroit. I’m featuring it once again in the hopes that readers will forward it to friends and loved ones, some of whom will hopefully choose to help the MRF achieve its audacious goal.


♦ The Tisch MS Research Center of New York (click here) – The privately funded Tisch Research Center is an integral part of the MS clinic at which I am treated. Headed by Dr. Saud Sadiq, the Tisch Center is at the cutting edge of MS research, investigating and innovating paradigm shifting methodologies for combating multiple sclerosis and repairing the damage that the disease inflicts on its victims.

In extremely exciting news, the Tisch Center recently received FDA approval to begin only the second US trial using adult stem cells to repair damaged central nervous system tissues in MS patients. Utilizing breakthroughs made through years of intense research at the Tisch Research laboratories, the clinical trial will use stem cells specifically targeted at repairing nervous system damage (neural progenitor cells) injected directly into the spines of trial subjects in an attempt to achieve the Holy Grail of MS treatment, the regeneration of cells damaged or destroyed by the MS disease process. One of the trial subjects will be noted journalist and author Richard Cohen, husband of TV personality Meredith Vieira, who, along with Dr. Sadiq recently appeared on The Dr. Oz Show to talk about this groundbreaking clinical trial. You can view clips from The Dr. Oz Show featuring the trio (click here-part one) and (click here-part two).

Dr. Sadiq is my MS neurologist, and I can testify to the man’s obsessive passion for finding the cure for MS and his deep compassion for the patients he treats. Dr. Sadiq is a bit of a maverick and a very “outside the box” thinker, a fiercely independent physician who refuses to permit pharmaceutical representatives to even enter his clinic so as to keep clear of the influence of Big Pharma. Given the frustrating and unrelenting nature of my illness, I consider myself lucky to have Dr. Sadiq on my side, as I’m confident that if anybody will come up with the answers I seek, it will be The Big Guy (as I affectionately call him). The Tisch MS Research Center of New York is certainly worthy of whatever tax-deductible donations come its way, as in addition to stem cell research the center vigorously conducts a wide range of studies specifically targeted at finding a cure for MS. Hopefully, the upcoming stem cell trial will culminate in results that will forever change the way MS is treated, and for the first time restore function pilfered by the disease.

♦ The Accelerated Cure Project (click here) – The ACP is an organization dedicated to speeding up the pace of multiple sclerosis research and treatment by engendering collaboration among researchers through a variety of mechanisms. Perhaps the most valuable resource provided by The Accelerated Cure Project is the ACP Repository (click here), a storehouse of blood and spinal fluid samples collected from over 3000 subjects, along with voluminous data on the medical and familial history of those subjects. These samples and data sets are available to scientists and organizations conducting research that can positively impact patients with MS.

The ACP Repository contains samples of Wheelchair Kamikaze blood and spinal fluid, which I understand is being kept in lead lined containers under the watchful eyes of Seal Team Six. Heaven forbid any WK derived substances fall into the hands of nefarious evildoers, as pure bedlam would be sure to follow. Labrador Retrievers would take their rightful place as the dominant species on the planet, and hordes of crazed wheelchair drivers would terrorize any who dared stand in the way.

All joking aside, The Accelerated Cure Project is devoted to the all-important goal of eradicating multiple sclerosis. Along with the ACP repository, The Accelerated Cure Project maintains The Multiple Sclerosis Discovery Forum (click here), an online community connecting, educating, and challenging MS researchers worldwide. Although intended for MS researchers, The Multiple Sclerosis Discovery Forum is a valuable resource for anybody interested in the latest MS info and research findings. Additionally, the ACP maintains the OPT-UP program (click here), a wide-ranging clinical study designed to evaluate the effectiveness of MS drugs in real-world settings, identify predictors an early indicators of response to specific drugs, and detect biomarkers specific to progressive MS.

The Accelerated Cure Project is an exceptionally important resource for MS researchers around the globe, and the organization has provided specimens and data for over 70 groundbreaking MS research studies. The biosamples, metadata, and interactive resources provided by the ACP are playing a vital role in research that could very well unlock the answers for which we as MS patients so ardently hope.


Well, there you have it, three smaller MS nonprofits all well deserving of charitable donations this holiday season (or any season, for that matter).

Please allow me to wish Wheelchair Kamikaze readers a very Merry Christmas, a happy belated Hanukkah, a tremendously good Kwanzaa, a happy Festivus (for the rest of us), or just a particularly good couple of weeks for those who don’t celebrate any of the aforementioned holidays. And of course, may all of us have a New Year filled with abundant health, happiness, and laughter.

As Charles Dickens wrote in A Christmas Carol, “It is a fair, even-handed, noble adjustment of things, that while there is infection in disease and sorrow, there is nothing in the world so irresistibly contagious as laughter and good humour.”

Amen to that…

How Are You?

English: "Three Gentlemen Greeting Each Other" (Photo credit: Wikipedia)

How are you?

I’ve always hated that question. It’s asked countless times a day, most often in passing as part of a quick and breezy greeting. “Hey, how are you”, says one person acknowledging another, typically with all the sincerity of a society lady blaming a loud fart on the dog. For that very reason – that lack of any real authenticity behind it – I’ve long disliked this social grace, even back when I was healthy. The standard response to “how are you” is almost always “good” or “great” regardless of whether or not things are good or great. Sometimes the question is met with a tired attempt at sarcasm, “same shit, different day…”, or some equally worn-out rejoinder.

Now that I’m stuck with a chronic debilitating illness, I find this most frivolous of salutations especially grating. I’m sitting here half paralyzed with my ass firmly planted in a wheelchair, how the hell do you think I am? Footloose and fancy free?

Of course, one’s condition at any particular moment is all relative, and despite the fact that I am indeed sitting here half paralyzed with my ass firmly planted in a wheelchair there are naturally times that I am better off than others, but all in all my situation, even when broken down to its most basic elements, is a complex matter. Coming up with a response that summarizes my current state in just a few words is a virtual impossibility, and even on those occasions when I happen to be feeling pretty good or even great, a quick “good” or “great” just doesn’t seem sufficient to capture the manifold nuances of the situation. My usual response these days is a tepid “oh, I’m hanging in there, I guess”, but more and more, when I’m feeling particularly grumpy I find myself tempted to pin my interrogator against a wall with my 300 pound wheelchair and proceed to let them know precisely how I am.

So how am I?

Physically, I’m a wreck. My right arm and leg are completely useless, most often more hindrance than help, and my left side is gradually (but not gradually enough) spiraling the drain as well. Due to a rare and disastrous reaction to intravenous steroids called avascular necrosis, my hips and shoulders are quite literally broken, keeping me in a nearly constant state of pain. That very same pain keeps me from sleeping for more than an hour or two at a time before whatever position I’ve dozed off in becomes so excruciating it wakes me, so I’m sleep deprived and constantly exhausted. Whatever disease process has hacked away at my spinal cord has also taken a buzz saw to my endocrine system, sending my hormones completely out of whack, the physical impact of which can be debilitating, setting up a medical situation so complicated it’s proving almost impossible to untangle. For reasons unknown I get fevers almost every night, which don't play well with my extreme heat sensitivity. Other than that I’m as fine a specimen of human physiology as can be found on God’s green earth.

How am I?

Emotionally, hard as it may be to believe, I’m in a better place now than I was for most of the time I was healthy. Back then I was often wildly neurotic, angst ridden, and for reasons only my decades long list of therapists may ever know, perpetually intent on finding reasons to be miserable. Not that I was friendless or socially isolated, for despite all of my psychological foibles I managed to maintain a boyishness and wield a sarcastic wit, the combination of which came together to form a certain kind of charm (or was my thriving social life due to the fact that I developed the habit of pinning cash to my garments?). By and large, though, I was almost always in a constant state of discontent, an emotional expanse whose boundaries ranged from mild trepidation to downright anguish. Though I was always quick to laugh, quite often at myself, nasty little gremlins were always flitting around inside my brain, twiddling with knobs, switches, and dials labeled insecurity, anxiety, and self-doubt.

Now, physically saddled with a mysterious and chronic progressively debilitating disease, most of my old psychological kinks have somehow been vanquished. Not that I’ve become the poster boy for robust mental health, but it seems that having a genuinely horrendous problem to deal with has negated any pathological need I had for seeking out reasons to be anxious. Now that my overactive psychology has been given a tangible target on which to focus, albeit a dreadful one, I find my anxieties have quieted down considerably. There’s not much time or energy to be wasted on existential angst when your entire right side is doing its best impersonation of a mannequin. Not saying my situation doesn’t suck, as it sucks huge hairy monkey balls, but it definitely keeps me grounded in reality, and has given me reason to channel my energies towards self-preservation rather than self-doubt.

How am I?

Frightened. Nah, that’s too tame a word. Let’s try absolutely, completely, thoroughly scared shitless. That’s more like it. How does this omnipresent state of fear wash with my aforementioned newfound mental stability? Very easily; anybody who wouldn’t be scared shitless at the future prospects presented by a constantly progressing crippling illness would have to be considered a prime candidate for Basket Weaving 101 at an inpatient mental facility. Try as I might to stay centered and focused on the now, it’s almost impossible to completely shield oneself from glimpses of a potentially horrific future. And we’re not talking about an abstract threat here, as the reality of the situation has been personified by some of the most famous victims of the disease (Annette Funicello, Richard Pryor, etc.). Can there be any prospective future more dreadful than the real possibility of being reduced to a very alert brain miserably serving a tortured life sentence silently trapped inside a prison of completely useless flesh and bone? Enough said. I’d better stop now before I soil myself.

How am I?

Dauntless. Since my diagnosis almost 11 years ago, I’ve endured trials and tribulations that my former hypochondriacally neurotic self would never have imagined I could withstand – much less simply shrug off, as I have most of them. I’ve been poked, prodded, irradiated, pounded and punctured more times than I can count, all with nary a yelp of protest or consternation. I’ve been PET scanned, CT scanned, gallium scanned, and MRI scanned so many times that I now possess far more pictures of the inside of me than the outside of me. I’ve racked up well over a dozen spinal taps, had needles the size of fire hoses simultaneously stuck into the veins in both arms during plasmapheresis, and have orally and intravenously ingested all kinds of bizarre and potentially poisonous concoctions dressed up as medicines, the vast majority of which have been as effective as voodoo powder (maybe less so, as I haven’t yet tried voodoo powder). I’ve watched more and more and more of my body be transformed from vital to useless over the course of a short decade as this creeping paralysis has done its dirty work, enduring ever mounting indignities as the endless losses have piled up, and yet I’ve somehow been successful in fending off the impulse to call it a life and permanently take up the fetal position. Big ups to the powers of mindfulness and the teachings of the ancient Eastern philosophers.

How am I?

Angry. Pissed off at the universe for cursing me with this vexation, derailing my life just when it seemed I’d learned some hard lessons and things were finally going my way. Despite my inner demons I found myself a great girl and forged a successful career in a highly competitive industry, only to watch it all come crashing down around me. Well, all except the great girl, who’s so great that she miraculously hasn’t headed for the hills. I’m furious at modern medicine, which despite all of its whizbang technology and blaring headlines of paradigm shifting medical advances is left dumbfounded and rendered completely impotent by dozens of horrific maladies, the vast majority of which leave those whose job it is to cure them not knowing their asses from their elbows. I’m seething at so-called healers who are content to label diseases “autoimmune”, when it’s clear that an immune system gone haywire is a symptom of some much greater ill. Newsflash: we haven’t evolved over millions of years to simply have our immune systems wake up one day and decide, “fuck it, I think I’ll go rogue and just attack this son of a bitch”. I’m infuriated at a medical research model that has become so corrupted by the corrosive influence of big money that it’s completely lost sight of its primary objective: finding ways to eradicate diseases, not fancy new formulas for turning them into cash cows.

How am I?

Grateful. Despite all the terrors of and the destruction wrought by my disease, I’m cognizant that in its own twisted way getting sick was my ticket to freedom, and maybe even to a dash of wisdom. Yes, I’d found success in a “glamour” industry, but truth be told I always hated working. Though the positions I held required varying degrees of creativity, many of them found me stuck in buttoned-down corporate environments, the kind of places that the younger, more idealistic me proclaimed loudly to all who would listen that I’d never wind up. Some people flourish in such a business environment; I suffocated. I found the regimentation of the 9 to 5 lifestyle absolutely soul sucking, asphyxiating my spirit and smothering my passions. I somehow allowed myself to stray far from the path I had once sworn to follow, and found myself too trapped by the realities of adult responsibilities and my own fears to find my way back. My disease, or more precisely, the early “retirement” it forced upon me, turned out to be my emancipation.

Some find the transition to life on long-term disability nearly impossible. Not me. I took to this relative life of leisure as if I were to the manor born. I suddenly had the time to rekindle interests and appetites that had far too long been neglected; writing, photography, history, zombie movies, ancient aliens. Perhaps more importantly (but really, what could be more important than zombies and ancient aliens) I was afforded the perspective to examine the healthy life I was forced to shed, and thus come to fully understand just how trivial were most of the concerns that had previously tied me up in knots. Worries over relatively minor financial setbacks, social status, broken romances with the wrong people, fixations on material goods – all so pathetically frivolous in the face of the disease that now beset me. The Eastern philosophies I'd  read but had never been quite able to incorporate into my healthy daily life now became vital instruments of survival. That Siddhartha wasn't just whistling Dixie.

Perhaps the most important thing I've learned is that the greatest blessing on earth is a quiet night spent with the people you love who love you back (this includes dogs).

So, how am I?

Oh, I’m hanging in there, I guess…

Bits and Pieces: Tecfidera Poll Results Edition

(Photo credit: Ancratyne)

(For those readers who receive Wheelchair Kamikaze via email, this post contains a video that cannot be viewed in standard email formats. To view the video (click here) to open the Wheelchair Kamikaze website in your browser.)

Before we get going with this edition of Bits and Pieces, I’d like to thank all of the wonderful folks who left get well wishes in the comments section of my last post (click here) and who sent me emails full of warmth and support. Your words were appreciated profusely, and I only wish I’d been feeling well enough to respond to each message individually. I did read each and every one, though, and they were far better medicine than anything the doctors could ever prescribe.

I’m still on the mend, even after six weeks since I first took Acthar Gel, which precipitated my unraveling. My physicians are still rather confounded at my reaction to the stuff, and my blood work shows that my endocrine system remains in a bit of a tizzy. I’m feeling much better than I did about four weeks ago, but not nearly back to where I was before things went kablooey. Bit by bit, I suppose, and I’m sure my old friend creeping paralysis isn’t helping with the restorative process. As Samuel Goldwyn once said, in this life you’ve got to take the bitter with the sour…

Anyway, enough about me, as I’ve spent the last six weeks or so stuck in bed contemplating little else but my predicament. Far too much time spent gazing inward, and poking and prodding all the monsters that lie within. Figured a Bits and Pieces post would offer a potpourri of distractions to me and all the folks who read WK, so off we go with a collection of items of interest that were able to catch my attention these last several weeks. Hope you find them  informative and/or entertaining.

♦ First up, a look at the results of a poll that’s been running on Wheelchair Kamikaze for the last 3 ½ months or so, designed to get a snapshot view of the experiences of patients taking the new oral MS drug Tecfidera. If you are taking Tecfidera and haven’t yet participated in the survey, please feel free to do so now, as the poll is still “live”. The more respondents we get the more accurate the results will be, so please do take the time to answer this brief survey if you are a “Tec-y”:

How would you characterize the side effects you’ve experienced as a result of taking Tecfidera?


Have Tecfidera’s side effects forced you to stop taking the drug?


(This question should only be answered by those who answered "no" to the previous question) How would you characterize any benefits you’ve felt since starting Tecfidera therapy?


In order to view the results of the three poll questions, just click the “show results” link beneath each query.

Before I attempt to analyze the above numbers, please allow me a few words about polling bias and how it might affect these results. I know, you’re all probably thinking, “What a blowhard, what can the Wheelchair Kamikaze possibly know about the intricacies of polling?”. Believe it or not way back in the 1980s I worked for a political research firm that conducted well-publicized polls on a national level. In fact, we were responsible for the numbers reported on the NBC nightly news every evening during the 1988 presidential campaign, which pitted Michael Dukakis against George W. Bush.

I was hardly an executive in the firm, but I did supervise the “phone bank”, where 40 or 50 people dialed phones incessantly for five hours every night, asking the people on the other end of the line their preferences in the presidential race and their opinions on the issues of the day. Chief among my responsibilities was making sure that the people responding to our questions were representative of the population at large in respect to sex, age, race, income, and other demographic criteria. Obviously, a national poll with a disproportionate number of Southerners, or too few women, or too many Hispanics, would skew the results and lead to highly inaccurate conclusions. For any poll large or small to have a chance at being accurate the respondents to that poll must be representative of the target population as a whole.

This same element comes into play when looking at the little Internet Tecfidera poll I’ve been posting on this blog. Since there’s no mechanism to screen those responding, there’s no way to control for an over or under representation of the myriad variables among the folks who comprise the Tecfidera taking population. Furthermore, the simple fact that this is an Internet poll automatically skews the results, as it doesn’t capture the opinions and experiences of Tecfidera users who aren’t surfing the Internet. Of those who are, it’s only representative of the relative handful of patients who have stumbled upon the poll here on Wheelchair Kamikaze and have chosen to participate.

My hunch is that the very nature of a wide-open Internet poll such as this probably skews the results a bit towards the negative, since the many patients taking Tecfidera and experiencing few if any problems are very likely to be simply going about their lives and not bothering to search for Tecfidera info on the web or answering a blog questionnaire on the subject. This phenomena can be seen manifesting itself in the comments sections of the Tecfidera informational posts I’ve put up on WK (click here and here), which are primarily comprised of patients recounting their difficulties experiencing the side effects of the drug.

If one took these comments to be representative of the experiences of all Tecfidera patients, it would be easy to surmise that rather severe side effect reactions were the norm rather than the exception, which clearly isn’t the case. This in no way discounts the validity of the accounts of those commenting on my posts, but simply illustrates the skewed nature of much of the info you’ll come across on Internet MS forums, Facebook pages, and other online communities. Such outlets only provide a window on a subsection of the MS population, and all information and opinions gleaned from such sources, both positive and negative, needs to be viewed in that light.

Now, having said all that, I find the results of my little Internet survey to be quite interesting, especially when a bit of negative bias is taken into account. As of this writing, 557 patients had participated in the poll. Let’s have a look at the numbers.

The first question asked is, “How would you characterize the side effects you’ve experienced as a result of taking Tecfidera?” Fully 61% of those responding report experiencing few if any side effects (answering “none” or “mild”), while 23% of respondents report dealing with moderate side effects, and 14% characterize their side effects as severe.

That 14% of folks taking Tecfidera report experiencing severe side effects is concerning (representing approximately 1 in 7 patients), but, as I stated previously, I suspect these results are skewed somewhat towards the negative. Even so, the number does seem high, and is certainly higher than I expected when I first set up the poll. Still, the terminology involved is a bit subjective, in that people have different tolerances for the discomfort caused by side effects, and what one person terms “severe”, another might term “moderate”.

I think the next question demonstrates this subjectivity to an extent. When asked, “Have Tecfidera’s side effects forced you to stop taking the drug?”, 88% of those answering say no, and 11% report that they have indeed stopped taking Tecfidera due to the drug’s side effects. Since 14% reported severe side effects in the first question, it’s apparent that about 3% of those folks didn’t find their side effects to be severe enough to make them stop taking Tecfidera. This question doesn’t touch on a key element in determining whether or not a patient continues on with a drug, namely the length of time that side effects persist. It’s generally been reported that Tecfidera side effects dissipate after 6-8 weeks, so the possibility exists that some folks experiencing severe side effects might be able to stick out their discomfort long enough for those side effects to no longer be a problem.

Interestingly, the two major late stage trials of Tecfidera reported that only 2% of patients taking the drug had to stop due to intolerable side effects, the same number as those who were taking a placebo. Certainly, these trials were far larger and much more controlled than my rinky-dink little Internet survey, but the disparity in results is curious. While much of this difference can probably be attributed to the negative bias inherent in my Internet poll, it’s been reported that drug companies routinely fail to report on the results of people who drop out of their studies, a practice which, if true, is at best abhorrent. It’s been widely demonstrated that drug companies consistently suppress research results that don’t reflect positively on their products, publishing only favorable study results (a phenomenon called “publication bias”), so such charges wouldn’t surprise me. As I’ve stated time and time again, the mechanisms by which medical research is conducted and reported these days are almost completely dysfunctional, much to the detriment of patients and the doctors who treat them.

But I digress, as I can easily work myself up into a frenzy over pharmaceutical company monkeyshines. Before I start frothing at the mouth, let’s get back to looking at the results of my Tecfidera survey. The last question asked is probably the one that patients would ultimately deem to be the most important, “How would you characterize any benefits you’ve felt since starting Tecfidera therapy?”. After all, Tecfidera’s ability to control MS symptoms is the ultimate arbiter of its worth to patients.

Before delving into the numbers, it’s important to remember that I first posted this survey only a few months after Tecfidera was approved by the FDA, and it’s been shown that it takes about three or four months for the drug to fully take effect. Though I’ve no way of knowing this precisely, I’m sure many of those responding to the poll had not been on the drug long enough for it to reach peak effectiveness. Given that fact, I think the results conveyed are fairly impressive. Nearly 50% of respondents (46%, actually) reported experiencing some benefit from the drug, and 21% deemed those benefits to be “moderate” or “great”.

Since many of those responding did so within their first few months of taking Tecfidera, the fact that nearly half had already experienced some benefit is encouraging. Tecfidera affects the body on a number of levels: as an immunosuppressant, an anti-inflammatory agent, and as a potentially powerful antioxidant. Given this variety of mechanisms, one would think that the benefits of Tecfidera might increase with time, and indeed this is what the published research on Tecfidera has shown. It would be very interesting to conduct a poll that queried only folks who had been on Tecfidera for five months or longer, but given the vagaries of the Internet there’d really be no way to accurately capture such a population, at least not with the relatively simple tools currently at my disposal.

So, there you have it, my attempt at playing master researcher, and reliving my youthful sojourn into the world of quantitative research, when Dukakis battled the senior Bush for the Presidency of the United States (it really didn't turn out to be much of a battle, actually). I hope these survey results, and my analysis of them, are helpful to folks currently taking, or who are considering taking, this increasingly popular new drug.

♦ Wow, going over the Tecfidera poll results took a lot longer than I was expecting, so I guess rather than this post being a “Bits and Pieces”, it’s going to be more of “A Bit and A Piece”. I blame the length of the poll results section on the fact that I use voice recognition software to “write” my posts, which makes it far too easy to turn verbal diarrhea into written diarrhea. My apologies.

This last item, though, is quite important. The following video was produced by the Myelin Repair Foundation, one of my favorite MS research nonprofits. It features the family members of my buddy George, known on the Internet as The Greek From Detroit, who passed away last March (click here). The video recounts his struggle with MS, and the impact it had on him and all of those around him, while also telling the story of the MRF and the incredibly important work the organization is doing.

Please watch the video, which is both extraordinarily touching and very informative. The research being done by the Myelin Repair Foundation represents one of our best chances at finding a way to repair the nervous system damage done by multiple sclerosis, and their efforts deserve the support of all MS patients and the people who love them.

And to my friend The Greek, I miss you, pal…



Thanks again for all of the get well wishes, they were appreciated beyond words…

$150,000 in My Fridge, and I Feel like Crap

Okay, to be completely accurate, about four weeks ago I did have $150,000 in my fridge, but now I

only have about $100,000 sitting next to my butter dish. And that small fortune has done nothing but make me feel, at times, worse than I’ve ever felt in my life.

So, how did I acquire such a windfall? Did I find a bag of money in Central Park? No. Did I win the lottery (again)? Nyet. Did I slam my wheelchair into the shins of a Wall Street demonoid (the streets of NYC are lousy with them) and steal his pocket change? Nope. All I did was take delivery of five tiny vials of a medication called Acthar Gel. Each of those vials costs about $30,000, and the stuff in those vials did nothing but make me feel vile. Harrumph. To make the story even more interesting – and disgusting – just about 15 years or so ago those same five vials could be had for about $250. So, this is not only a story of my medical misadventures, but also one of pharmaceutical company shenanigans, which I’ll get into later.

Before I start my tale of woe, let me first state that my reaction to Acthar Gel is entirely atypical, so strange that my physicians are completely confounded as to what went down. The stuff is generally considered quite benign, and the root of my problems with it is my ever baffling, completely fracked up physiology, which the best minds in the business have not been able to figure out. So please don’t use my experience as any kind of example. If you are currently using Acthar Gel, or at some time in the future may be prescribed the stuff, I can just about guarantee that your experience will bear no resemblance whatsoever to mine. The substance is almost universally well-tolerated, and has been used by thousands of patients suffering from a variety of maladies with very few complications. This essay shouldn’t be seen as an indictment of Acthar Gel, but rather a testament to the depth of the medical mystery that is me.

Acthar Gel is simply an injectable, time released form of ACTH, a hormone naturally secreted by the pituitary gland that signals the adrenal glands to produce an increased amount of the body’s natural steroids. When used to treat MS, it’s usually given to RRMS patients who are experiencing an exacerbation, just as intravenous steroids are used to treat MS relapses. In both cases, the steroids – whether generated by the body itself or given intravenously – work in a variety of ways, primarily by suppressing the immune system and reducing inflammation in the central nervous system.

I definitely do not have RRMS, but my illness does look a lot like PPMS (Primary Progressive Multiple Sclerosis), a form of MS that afflicts about 10% of the MS population and is defined by its complete lack of relapses and remissions. Its sufferers instead experience a steady decline in neurologic function over the entire course of their illness. Though my symptoms and disease history do at first appear somewhat typical of PPMS, my diagnostic test results and certain other anomalous features of my disease don’t fit the PPMS mold. In addition to my neurologic problems, I have signs of some kind of systemic autoimmune activity, including widespread endocrine dysfunction. In other words, my hormones are a mess. If you’re interested, you can read more about my medical eccentricities in an older WK essay (click here).

Since people with PPMS generally don’t exhibit much inflammation in their central nervous system, steroids typically have little if any positive effect on them. This is one of the aspects in which my disease seems to differ from PPMS. Back in 2006, about three years after my diagnosis, my disease started to spiral out of control. In an attempt to put the brakes on my deteriorating condition, my neurologist ordered a 10 day course of IV Solu-Medrol, along with a weeklong stay in a highly regarded rehab hospital.

Remarkably, the steroids had an almost miraculous effect on me, restoring physical function that had been lost for months or years. I could suddenly use my right hand again, lift my right arm over my head, and my walking improved dramatically. Unfortunately, these improvements proved to be only temporary, fading within four months of my infusions. Even more unfortunately, I developed a very rare side effect of intravenous steroids called Avascular Necrosis (AVN), a condition which causes the bones in some of the major joints to literally die and then crumble. The AVN attacked both of my shoulders and hips. These days I’m living with the equivalent of two broken hips, and shoulders that are cranky at best. If I were healthier, I’d have had my hips replaced years ago, but in my current state it’s doubtful I’d be able to withstand the surgeries or do the necessary rehab afterwards.

As a result of the AVN disaster, doing intravenous steroids is obviously no longer an option for me, which is unfortunate because steroids are the only treatment I’ve tried which has significantly impacted my disease in any positive manner. That’s where the Acthar Gel comes in. By stimulating my body to produce more of its own natural steroids, it was expected that I would get at least some of the benefits of intravenous steroid treatment without incurring the risk of furthering my Avascular Necrosis. The solution seemed perfect, except for the exorbitant price of the drug, $30,000 for a tiny 5 mL vial, of which I’d need five. Surprisingly, my insurance company didn’t balk at the hefty price tag, and a few weeks after my doctor first mentioned my trying Acthar Gel, I received five little very valuable vials of Acthar Gel via Fed Ex.

Acthar Gel is an injectable drug, and the day after we received the stuff, a nurse came to instruct my wife Karen how to administer the injections. The plan was to do three weeks of injections, with the doses descending each week. The nurse herself gave me the first injection, and things seemed to go smoothly enough. Karen then gave me an injection each evening, and for the first few days I felt similar to the way I had felt on intravenous steroids. Kind of speedy, a little bit agitated, trouble sleeping, but nothing too terrible. I didn’t experience any obvious benefit, but we were just starting on the three-week course prescribed by my doctor.

After the fourth or fifth day, though, I started feeling pretty crummy. I developed a fever (I seem to always run a fever, another strange feature of my disease, but now the fevers were higher), my vision became blurry, my neurologic symptoms worsened, and I generally started feeling like the proverbial wet dishrag. I consulted with my doctors, and the general consensus was that my body was just adjusting to the Acthar Gel, albeit a bit strangely, and there was nothing to worry about. A few days later, much to my chagrin, I found myself feeling just about worse than I’ve ever felt in my life, barely able to get out of bed. I was told to stop taking the Acthar Gel after one more injection at a reduced dose, so as not to shock my body by stopping the stuff completely cold.

I hoped that after coming off the drug, my body would bounce back, and I’d get back to my good old, bad old normal. Instead, a couple of days later I was feeling worse than ever, and began experiencing a most inconvenient symptom. To put it bluntly, I started peeing more than I thought a human being could ever possibly pee. Really, it was incredible. For a few days, I became a living fountain of urine, a perpetual passer of piss, the mysterious source of my own Yellow River. This soggy experience only increased the misery of the situation, and lent an air of the surreal to the whole affair. I managed to stop urinating long enough to make it to my endocrinologist, who did a series of blood tests that came back, surprisingly, fairly normal. Unfortunately, I felt decidedly not normal, but expected that I would soon start feeling better. My expectations, however, were not met.

Now, about four weeks later, I’m still feeling like I kissed the “A” Train between stops (a very New York centric reference, I know. Just use your imagination). As part of my overall endocrine dysfunction, my cortisol levels generally run low, but now they are really low. I’m weak, get dizzy whenever I try to stand up, have absolutely no appetite or energy, and I’m spending the vast majority of my time in bed, and I hate spending non-sleeping time in bed. Suffice it to say, I’ve definitely seen better days.

To think, this dreadful experience only cost my insurance company $150,000, and I have about $100,000 worth of Acthar Gel sitting in my fridge, totally useless to me or anybody else, since it’s illegal to transfer a prescription drug, once delivered, to another patient. I don’t think the stuff has any street value, or I’d send Karen out in a trench coa to t try to sell the stuff to some desperate Acthar addicts. With the profits we could put a 10% down payment on a really small one-bedroom condominium here in NYC.

So, that’s the story behind the lack of new posts on Wheelchair Kamikaze for the last month. I’d like to thank all of my loyal readers for sticking with me. I’m desperately hoping that I’ll be feeling better soon enough, and be able to get back to my fairly loose schedule of regaling the Internet with riveting tales of life with a chronic progressive disabling illness, my take on latest in MS research news, and snippets of the random crap that regularly pops into my noggin.

Before I sign off, a few words about the outrageous price of Acthar Gel. As I mentioned numerous times above, a single small vial of the stuff now sells for about $30,000. One might imagine, then, that it must be some new high-tech compound that cost the drug company that manufactures and sells it, Questcor Pharmaceuticals, millions and millions of dollars to invent, develop, and bring to market. Wrong. The history of Acthar Gel dates back to the 1950s, when the Armour meat packing company was trying to find uses for the animal parts that didn’t make it into the food chain. They discovered that hormones extracted from pig pituitary glands could be used in humans to treat a variety of illnesses. Thus, Acthar Gel was born about 60 years ago, and approved by the FDA in 1952, before the agency required clinical trials to prove a drug’s effectiveness.

For much of its long history, Acthar Gel was used to treat a variety of arthritic, autoimmune, and rheumatologic conditions, and the price of the drug was relatively inexpensive, about $50 for a 5 mL vial by the time the mid-1990s rolled around. By then science had learned to synthesize many of the steroids that Acthar gel stimulates the human body to produce, which severely limited demand for the drug. For the last few decades, Acthar Gel's primary use was to treat a condition known as infantile spasms, a rare epileptic disorder that usually strikes children before the age of one, which can sometimes be fatal. Since infantile spasms afflicts only about 800 patients a year, and Acthar Gel is relatively expensive to produce, making the drug became a losing proposition. Questcor bought the rights to the drug in 2001 for a mere $100,000. Almost immediately, they raised the price of each vial to about $1200, and then in 2007 hiked the price astronomically, to $28,000 for a single 5 mL vial of the stuff. They didn’t change the formula and didn’t refine the compound, but in six years took a cheap, rarely used, 50-year-old drug and turned it into a blockbuster generating hundreds of millions of dollars in profit per year.

How was this financial magic act achieved? All through the graces of a law called the Orphan Drug Act. In the United States, this law grants incentives and privileges to companies that manufacture drugs that treat diseases that affect 200,000 people or less. The intent of the act is to give pharmaceutical companies reason to pursue treatments for diseases so rare that research, manufacture, and marketing of drugs intended to treat them would not otherwise be profitable. Since infantile spasms afflict far less than 200,000 patients, Questcor received orphan drug status for Acthar Gel, and indeed the drug is a godsend for the little victims of that epileptic disorder.

Once a drug receives orphan drug status, though, there’s nothing to prevent the company that sells it from finding other uses for it, and that’s just what Questcor has done with Acthar Gel. Along with raising the price to $28,000 per vial in 2007, Questcor embarked in a massive marketing campaign, in an effort to convince physicians to prescribe Acthar Gel for a wide variety of conditions, including MS, nephrotic syndrome, and rheumatologic conditions. Today, the treatment of infantile spasms only makes up about 10% of the drug’s sales, and Acthar Gel produces hundreds of millions of dollars in sales per year for Questcor pharmaceuticals (in the first nine months of 2012 alone, Acthar Gel sales amounted to about $350 million). Since dramatically increasing the price of the drug in 2007, Questcor’s stock price has skyrocketed, from $.60 to about $58 today. Pretty good investment, right?

For more info on Questcor pharmaceuticals and Acthar Gel, you can read an excellent article in the New York Times (click here), from which much of the above information was derived.

In short, Questcor pharmaceuticals managed to take a rarely used and almost forgotten drug, and in the course of less than a decade turned their initial $100,000 investment into billions of dollars in profit. All this without doing any research and development, attaining incredible returns almost strictly through the magic of marketing, along with a little help from a well-meaning law meant to allow patients suffering from rare diseases at least a glimmer of hope that a treatment for their disease might be developed.

Well, after recounting the above story, I feel even sicker than I did when I started this essay. It’d be bad enough if I was made this ill by the drug at its original price, five vials of which would’ve cost $250. Knowing that the stuff cost my insurance company $150,000, well, all I can say is that I’m going to crawl back into bed as soon as I can muster the strength to leave my computer desk.

PS, all well wishes and healing thoughts will be extremely welcomed…

At What Price Health? (Repost)

Well, I'm still in recovery mode from my last failed attempt at trying a new MS med. For all of those who have inquired, no, the medication was not Tecfidera, so if you have recently started the drug, no cause for alarm. The med I tried is not used all that often to treat MS, and I had a very atypical reaction to it (naturally). I'll provide full details in my next post, which I will put up as soon as I'm feeling better. Just a teaser to keep you interested: the drug costs $150,000 for a three-week supply, and I was only able to use about one week's worth. So, I have about $100,000 sitting in my refrigerator, just taking up space and doing nothing to help anybody. Way to go, Medical Industrial Complex!

In the meantime, I'm reposting the following piece, which first appeared on Wheelchair Kamikaze way back in 2009. It generated lots of comments back then, and I hope current WK readers find it just as interesting. Please stay tuned for a new post, coming just as soon as I'm feeling up to it…

For the last few days, I've been pondering a thought exercise that recently popped into my mind. Imagine, for a moment, that an almost miraculous cure for MS has been discovered, one that can alleviate all MS symptoms with a single injection. A patient simply has to go to their doctor's office, get the shot, and, voilà, 24 hours later they are completely symptom-free, their nervous systems restored to pristine condition and their general state of well-being suddenly better than even before they were diagnosed with Multiple Sclerosis.

Great, right? Sign me up!

Only, there's a catch. This "cure" comes with a terrible cost: after a certain amount of time, every patient treated with this injection dies painlessly in their sleep. In the "X" amount of time before they die, the patient remains in the full bloom of health, right up until the night they go to bed for the final time. The question, then, is what would be the minimum duration of guaranteed health that would entice you to take the shot? In other words, would being restored to perfect health be worth it to you if you knew with utter certainty that you would die in six months? One year? Five years? 10 years?

Would you be willing to trade a full life of chronic illness for a blissful time during which you would be completely unshackled by the chains of Multiple Sclerosis? For a time free of fatigue and cognitive dysfunction, of muscle spasms, spasticity and paralysis , of bladder and bowel issues, of the constant daily struggle of dealing with the rigors of this miserable disease? An interval during which you'd have no reason to even think about braces and canes and walkers and wheelchairs and MRIs and neurologists and lesions and a medicine chest full of pills that hardly even seem to matter? When you could walk and run and dance (dance!), drive and play and travel, and finally, finally, once again be that fully functional man or woman that you used to be, that you've dreamed of being since the day MS started taking its dreadful toll?

How many months or years of restored health would be enough to entice you to undergo a simple but profoundly effective treatment that carried with it the ultimate price? Of course, the answer must differ for each of us, based on our own current state of disability, our rate of progression, the level of our misery, and the amount of hope we have that a cure, or even a truly effective treatment, can be found in time to help us. The flavor of the disease a patient suffers from also enters into the calculus . Folks with relapsing remitting disease that is currently being managed effectively by disease modifying drugs might reject such a proposition out right, while patients with progressive disease, especially advanced progressive disease, would probably be much more open to trading longevity for a period of perfect health.

Certainly, marital status and family situations factor greatly into the equation. Single people, or those without children, might be more willing to sacrifice longevity for a chance, though brief, to be healthy once again. For those who are married, and especially those with children, the calculus gets infinitely more complicated. How much time with a healthy parent would it be worth for a child to then lose that parent? Difficult questions all, and ones I think reach to the very core of our beings.

Personally, after much thought, I think I'd put my "X" at somewhere around a year or a year and a half. If a physician approached me with a syringe, and told me that the injection would guarantee me 12 months of perfect health, but at the end of that 12 months, I would die painlessly in my sleep, I would give the offer serious consideration. Of course, I'd want more time, all the time in the universe, but this thought exercise requires that we consider the absolute minimum amount of time we would settle for.

One year would give me time enough to experience all of those pleasures in life that I now miss so terribly, to travel to the places my wife and I have always wanted to see together, and to spend time with those who I hold closest to my heart. I don't have children, so that's not a consideration. I do have hope that stem cells offer real promise as a treatment, but I'm unsure that this promise will be fulfilled in time to help me. I have my doubts about many of the avenues currently being explored by MS researchers, and though strides are being made, I'm uncertain that the mysteries of MS will be fully unraveled anytime soon, and given my rate of progression, soon is the only timeframe that really matters to me. In addition, there is now question as to whether what I have is even really MS, and what chance is there that some mystery illness will be solved before it puts me into a state I deem to be simply unbearable?

So what about you, dear reader? What's the minimum amount of time for which you'd be willing to trade your life for perfect health? What's your "X"?

Remembering Stella (Repost)

(Some good news and some bad news. First, the bad news. After trying a new drug (for me) to calm my mysterious  beast, I am instead caught in the throes of a struggle with it. Weaning off the drug now, so hopefully I'll be back to my "old normal" sooner rather than later. The good news is that all this should make for a pretty interesting blog post, including some outrageous pharmaceutical company shenanigans, the mysteries of my illness, and general adventures in medicine.. In the meantime, for your hopeful enjoyment, I'm reposting the below essay, written in January, 2010 about one of the best friends I've ever had. Thanks for reading, and a new essay will hopefully be up soon…)

My best buddy Stella passed away just a little over three years ago. She was a faithful friend with a huge heart who always knew just how to make me smile and often had me laughing riotously out loud. Stella was compassionate and sweet, and knew exactly how to live in the moment and seize every day. She was faithful, devoted and I knew that I could trust her entirely with my deepest darkest secrets. About the worst thing I can say about her is that she had the unshakable habit of loudly and vigorously chewing her paws in the middle of the night, while emitting strange noises that were impossible to sleep through.

Stella was, of course, my furry best friend, a yellow Labrador Retriever who came into my life in 1998, while I was still living in Fort Lauderdale. A coworker had just given birth to a baby girl and no longer had the time to care for Stella. I'd recently moved into a charming little 1940s Florida cottage with my then girlfriend, and was jonesing for a dog. So, the timing was perfect, and after two "meet and greets", during which Stella gave me the thumbs up, I was a happy new doggy daddy. Stella had just turned three years old when she came to me, and I was five years away from developing MS.

I hadn't had my own dog since I was a kid, but I had bonded with the canine companions of several friends and lovers that I'd met along the journey of my adult life. I was particularly close with a Dalmatian named Briar, whose owner unfortunately turned out to be a pathologically lying serial cheater who delighted in using my heart like a roll of Charmin. Quick life lesson: if you find out that your lover has cheated on every person they've ever been with, don't fool yourself into thinking you can somehow change them. Simply open your eyes to the truth, realize that once a person accepts such behaviors in themselves they will never change, and get as far away as possible, even if they have an adorable spotted four-legged creature with the most haunting eyes you've ever seen...

But, I digress. Stella and I quickly bonded, even as my girlfriend and I quickly unbonded. Turned out my Labrador friend enjoyed spending Sundays sprawled on the couch watching NFL games as much as I did, as long she could watch them while laying between my legs with her head nestled on my belly. We took long walks around the neighborhood together, although she wasn't much for jogging. The one time I took her out for a run, she made it about a block before squatting in the middle of the road and doing what dogs generally do when they squat. After completing that most natural of acts, Stella let me know that jogging just wasn't her thing. No harm, really, because jogging wasn't really my thing, either.

About six months after Stella joined me, the girlfriend and I decided to call it quits, and I decided to get the hell out of Florida, a place I never much cared for, even though I spent 10 years there. I think the tropical sun beating on your noggin causes some kind of dementia, because even though I felt like a stranger in a strange land the entire time I lived down there, for some reason I could never formulate actionable plans to leave. It was like, "gee, this place royally sucks, ooh, I think I'll go for a swim..."

Anyway, Stella and I were soon back in my hometown of New York City, living in a section of the city known affectionately as "Hells Kitchen". For a dog that was born and raised in Florida, Stella took to city life like a socialite. For some reason, she naturally curbed herself (if only the same could be said for socialites), and she loved the wonderful sniffing opportunities that the city streets offered up in droves. She also loved the take-out Chinese joint around the corner from our apartment, which always had partially eaten chicken wings discarded on the sidewalk in front of it. One of the few arguments Stella and I ever had were over her insistence on insanely gobbling down as many of those gnawed on chicken bones as quickly as she possibly could, but a few rounds of very stern "bad girls" helped her kick the habit. You see, she really was a "good girl", and my disapproval trumped the irresistible gristly remains of chicken wings, true testament of her feelings for me.

For about a year, Stella and I were strictly a duo, spending lots of time at neighborhood dog runs and in Central Park, where she'd occasionally take an ecstatic jump in the lake. She absolutely lost her mind during that winter's first snowfall, which was the first snow the native Floridian had ever seen. If pure joy could be embodied in flesh and blood, it would be Stella burying herself in mounds of freshly fallen snow and then wriggling on her back to make canine snow angels. Her glee was infectious, and soon I too was a snow-covered whirling dervish, joining Stella in her carefree frolicking, covered head to toe in the powdery white stuff blanketing the fields of Central Park.

After about a year back in the city, late one night in a neighborhood bar I met a girl named Karen, who, despite my best efforts, didn't seem very interested until I mentioned the fact that I had to get home to walk my Labrador Retriever. Turned out that Karen had grown up with Labradors, and, figuring that a single guy with a Labrador couldn't be all that bad, she gave me her number. Just about two years later, we were married. At first, Stella didn't exactly welcome Karen with open paws; after all, Karen had supplanted her place on the couch. But the two soon became buddies, and Karen even succeeded in getting Stella to lose a little weight (for a while, we referred to her as "Jabba the Pup"), much to the veterinarians delight.

For a year, everything was hunky dory, until one very cold day in March 2003, when I took Stella for a very long walk along the Hudson River. About 2 miles into our trek, I noticed that I'd started limping. I didn't think much of it, but in the following weeks, the limp in my right leg grew worse, and I felt my right arm starting to weaken. Several doctors visits and an MRI later, and I found myself sitting in a doctor's office listening to words like "multiple sclerosis" and "progression" and "spinal tap" somehow become associated with the words "me" and "holy shit".

Strangely enough, just about the same time, Stella also started having all kinds of health problems. I honestly believe that she was so empathic that she somehow shared my distress and manifested physical illnesses of her own. Between 2003 and 2006, Stella developed mast cell cancer and autoimmune hepatitis. She had multiple surgeries to get rid of the cancer, and was put on a variety of medications and a special diet to address the hepatitis. For a while, we were actually both on the same immunosuppressants, bought from the same pharmacist. On several occasions, it appeared that Stella was on death's door, but she always managed to somehow pull through, often to the veterinarian's surprise. He'd smile, shrug his shoulders, and offer the only explanation he could, "She's Stella..."

Through it all, Stella stayed Stella. Though she would suffer a while from her painful surgeries, and the hepatitis would sometimes rob her of strength and appetite, as soon as she felt a little bit better, her tail was wagging, her eyes were bright, and she was ready to embrace whatever joy that the day had to offer. In so many ways, she taught me how to deal with my own illness, which progressed continuously through the ensuing years.

Stella didn't waste any time bemoaning her fate, or thinking about what might have been, because she was blessed to simply not have the capacity to do so. As my condition has continued to worsen, I've often thought of Stella, and have realized just how right she had it. Feeling sorry for yourself or worrying about future calamity only serve to poison the present, and the present, the now, and our place in it, is the only thing in the entire universe that we have any real control over. Endeavor to live your life like a Labrador, attack each day like it's a great big rawhide bone sent from the heavens.

Eventually, Stella's illnesses and advancing years got the upper hand; the cancer returned, and my sweet little girl started slipping away. Over the Thanksgiving weekend of 2006 we boarded her at the veterinarians while we visited my mom in Florida, and when we returned the vet told us that Stella's condition had worsened, and he recommended we put her down. He brought her out to us with an IV already inserted into her leg, but upon seeing us I could see that familiar spark in her eye, and she started eating the treats I tried to hand feed her. We decided to bring Stella home, to give her the chance to make one more rally.

By this time I was no longer able to walk Stella, and most of her caregiving fell to Karen. Stella actually did rebound a bit for the first few weeks, but I guess the power of love can only go so far. A few days after Christmas, we brought Stella back to the vet one last time, held her, and said goodbye. Those weeks between Thanksgiving and Christmas became one extra month of bonus life for Stella, during which Karen took Stella to Central Park almost every day, and Stella ate all of the chicken and turkey she wanted.

Stella saw me through many transitions; from Fort Lauderdale to New York, from single to married, from well to Multiple Sclerosis. Aside from my wife, there is no being I have ever felt closer to, or more intimate with. I miss her still, and will for the rest of my days. Karen and I now live in a building that is wheelchair friendly, but doesn't allow dogs. If I somehow beat this thing, first thing we're doing is moving out of this place and getting ourselves a great big pooch, who will take Stella’s space, but surely not her place.

Here's my favorite photo of my pal Stella...