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, and 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 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, didn’t refine 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 make sure that patients suffering from rare diseases get 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…