CCSVI and MS: Cause, Effect, or Neither?

Image by Getty Images via @daylife

It's now been over 18 months since the news of Dr. Zamboni's vascular theory of MS, the CCSVI hypothesis, first made its way into the consciousness of the greater Multiple Sclerosis patient population. Touched off by news reports on Canadian television channel CTV, an inferno of hope raced through MS patients worldwide, and a firestorm of controversy regarding almost every aspect of the hypothesis was ignited, a conflagration that seems to only burn more intensely with each passing week. The relatively simple CCSVI hypothesis (which postulates that blockages in the veins draining the central nervous system lead to or contribute to the MS disease process) has managed to pit physician against physician, physician against patient, and patient against patient. While the various factions duke it out, an estimated 15,000-20,000 MS sufferers have undergone CCSVI venoplasty, with a wide variety of resulting outcomes, ranging from dramatic benefit to no benefit whatsoever to, in rare cases, a worsening of disease symptoms.

Several key questions have emerged regarding CCSVI during the last year and a half, primary among them whether or not CCSVI and MS have any link whatsoever, and if so, whether the venous anomalies collectively known as CCSVI are the cause or an effect of the disease. We've seen a steadily increasing flow of CCSVI research results, providing enough conflicting data to fuel all sides of the argument. As with all things Multiple Sclerosis, CCSVI presents a complex picture, and despite evangelical believers/nonbelievers on all sides of the squabble, at present the ultimate outcome of the CCSVI conundrum is as clear as mud. Here then is a brief look at some of the issues currently being batted about, with some small attempt on my part to make some sense of it all.

My personal belief is that CCSVI and MS do indeed have a relationship, at least in some MS patients. However, I acknowledge that my opinion is based primarily on anecdotal evidence, and anecdotal evidence alone is not enough to state anything with scientific certainty. Despite the insistence of many in the CCSVI advocacy community, the link between CCSVI and MS has not yet been established with evidence that measures up to the scientific standard. On its face, the basic premise of CCSVI, that restricted blood flow through the central nervous system, caused by vascular abnormalities that are very likely congenital, slowly cause damage to the CNS over the course of decades, eventually becoming significant enough to result in a clinical diagnosis of Multiple Sclerosis, seems simple and makes perfect sense. Yet upon closer inspection, the picture is not quite so clear-cut.

There have been several very convincing studies demonstrating that venous abnormalities now known as CCSVI occur more often in MS patients, and even in patients with other neurologic diseases, than in healthy control subjects. Conversely, there have also been quite a few studies disputing this. Almost all of these studies, pro and con, have relied on noninvasive imaging techniques (Doppler ultrasound or MRV) to ascertain the presence of these abnormalities. Unfortunately, neither of these noninvasive imaging techniques has proven to be entirely accurate, though Doppler ultrasound, in the hands of a skilled and well trained technician, does appear to be the more reliable of the two techniques. Still, we have research groups reporting widely divergent findings, and some of that divergence could possibly be attributed to the relative inaccuracy of the diagnostic methods being utilized.

Complicating matters further is the fact that human venous anatomy, with a few exceptions, has been very little studied. So little, in fact, that no clear-cut definition of "normal" exists when it comes to the anatomy of the veins that drain the central nervous system. It had previously been assumed that since the veins in question had so many built-in redundancies, any blockages encountered would be easily compensated for. While the Interventional Radiologists performing the CCSVI treatment procedure are reporting that the overwhelming majority of MS patients are indeed displaying a large number of venous abnormalities, we cannot state with any certainty that a significant portion of the healthy population does not also present with such abnormalities.

What are desperately needed are trials using catheter venography to ascertain the prevalence of CNS venous abnormalities in healthy control subjects. However, there are some ethical questions involved in performing this minimally invasive procedure simply for research purposes. Although the risk is low, catheter venography, like any invasive procedure, does carry with it the potential for dangerous complications, and the prospect of exposing healthy subjects to these risks has inhibited such studies from taking place. Until it can be established beyond dispute that vascular abnormalities in the jugulars, azygos, and other veins that drain the CNS are more prevalent in MS patients than in the general population, the question of the CCSVI/MS relationship will not be put to bed.

Let's assume, though, based on the anecdotal reports, that there is a connection between CCSVI and MS. The big question then becomes whether CCSVI is the cause of the disease, or an effect of the Multiple Sclerosis disease process. Again, there is enough conflicting data to support both sides of the argument.

Those who support CCSVI as the cause of MS site several compelling reasons for their belief. One of these is that many of the abnormalities being seen in the veins of MS patients, such as anomalous membranes and fused valves, appear to be congenital in nature, that is, patients have had them since birth (click here). If these defects are congenital, and occur in greater preponderance in MS patients than the healthy population, it would seem reasonable to assume that they play a causative role in MS disease etiology. Another argument in favor of CCSVI as the cause of MS is the growing body of evidence that suggests that nervous system tissues in MS patients are damaged before the immune system comes into play (click here for one such study), findings that would seem to contradict the prevailing theory of MS, the "autoimmune theory". The autoimmune theory states that, for reasons unknown, the immune systems of MS patients go rogue and start attacking the patients' own central nervous system tissues. If some studies done within the last decade are correct, and CNS damage occurs before immune system involvement, this would apparently discredit many of the basic assumptions of the autoimmune theory, and CCSVI provides an explanation as to how this damage occurs.

While I've long held the autoimmune theory in contempt, and I'm convinced that the aberrant immune response seen in MS patients is a symptom of some larger underlying and as yet undiscovered cause, I'm not sure that cause is CCSVI. While the CCSVI hypothesis does in some ways elegantly account for some of the mysteries surrounding MS (the venocentric nature of MS lesions, the reduced volume of blood flow through MS brains, etc.), it does not account for several of the more confounding aspects of the disease. It's difficult for CCSVI to explain the geographical distribution of the MS population, which sees a far greater prevalence of MS the further away one gets from the equator (click here). Related to this geographic distribution, CCSVI also can't explain some of the migratory observations made in regard to disease prevalence (click here). When a person below the age of 15 migrates from an area of higher disease prevalence to one of lower prevalence, they take on the characteristics of their new home. When the migrant is over the age of 15, though, they retain the propensity for the disease of the area they migrated from. In other words, a person under the age of 15 migrating from Maine (high prevalence) to Florida (low prevalence) has the same low chance of getting the disease as a Florida native. However, older migrants retain the higher chance of getting the disease seen in Maine. Furthermore, children of these older migrants, born after the move south, take on the same lower chance of developing MS as children born to the native population.

Similarly, CCSVI cannot account for the existence of "MS clusters", which are small concentrations of population in which MS appears to be epidemic (click here). The most famous of these clusters is in the Faroe Islands, an island group situated between the Norwegian Sea and the North Atlantic Ocean. Prior to World War II, MS was virtually unknown among the native islanders. During World War II, the British, who have a high incidence of MS, occupied the island, and subsequent to this occupation, MS has become epidemic among the native population. Another such cluster was recently identified in a small town in Ohio, where over two dozen MS cases were discovered within a six block radius.

The geographic and migratory components of MS epidemeology, as well as the existence of MS clusters, are heavily suggestive of an environmental (infectious or toxic) element to the MS disease process, and indeed, recent studies have linked several viruses, most of them in the herpes family, to MS. Just within the last week or two, a study out of Taiwan, which looked at hundreds of thousands of subjects, found that people suffering an outbreak of shingles, a painful skin condition caused by the varicella zoster virus (which also causes chickenpox), are three times as likely to develop MS within the year as those who didn't suffer from shingles (click here). Likewise, the Epstein-Barr virus has also been cited as a possible infectious trigger of the disease, with some scientists stating that if a patient isn't infected with EBV, they won't get MS (click here).

Another potential problem with CCSVI as the cause of MS is the inflammatory patterns seen in patients afflicted with the disease. According to CCSVI theory, disrupted blood flow through the CNS creates damage and inflammation to the cells contained within, through a variety of possible mechanisms. This would lead one to expect that the longer the condition persisted, a patient's levels of inflammation would slowly increase over time, in a steady upward slope. However, in reality, RRMS patients see their greatest amount of inflammation early in the disease, during its relapsing remitting stage. Once the disease moves into the progressive stage, and RRMS turns into SPMS (normally within 10-15 years when left untreated) inflammation levels decrease dramatically. Patients with PPMS, who start out with progressive disease, very often show very little signs of CNS inflammation. As a matter of fact, this lack of inflammation, seen as enhancing lesions on MRI images, is a hallmark of progressive disease. This is why anti-inflammatory therapies such as steroids generally have little effect on patients with progressive illness.

Of course, none of this directly contradicts the idea that CCSVI may play some causative role in the disease of some MS patients, but it strongly argues against the idea that CCSVI is the primary cause of the disease.

Some compelling evidence that CCSVI may be an effect of MS has been presented by several researchers, most notably Dr. Robert Zivadinov and the researchers at the Buffalo Neuroimaging Analysis Center. Although Dr. Zivadinov's findings have been savaged by some of the most fervent "CCSVI as cause" proponents, the totality of the research done under his direction does point to the possibility that at least some of the venous abnormalities now called CCSVI are a result of the MS disease process. Although Dr. Zivadinov's opinions only recently made headlines (click here), he in fact implied them in research presented in October, 2010 at the annual ECTRIMS (European Committee on Treatment and Research in Multiple Sclerosis) conference. One paper presented at that time demonstrated that the severity of CCSVI increases with the severity of Multiple Sclerosis symptoms experienced by patients, and with a more advanced disease course (click here). These findings were backed up by papers presented by researchers from Beirut (click here) and Italy (click here). Another study presented by Dr. Zivadinov found that subjects who presented with CCSVI had significantly more lesions and brain atrophy as measured by MRI than those MS patients without vascular abnormalities (click here). Yet another investigation presented by Dr. Zivadinov looked at the correlation between a gene implicated with MS, and CCSVI, and found that the data supported an association between MS disease progression and CCSVI separate from the suspect gene. The implications of these findings are that CCSVI could be a risk factor in developing the disease, or a result of the progression of MS (click here).

Additionally, it would seem to me that the high rate of restenosis in patients who have undergone CCSVI treatment venoplasty could also hint that CCSVI is more an effect rather than the cause of MS. Despite the wide range of treatment methodologies being employed, patients are still experiencing a re-narrowing of their previously unblocked veins far too frequently. This has been seen even in patients who have had stents placed in their veins, only to see their veins stenosing in areas not stented. If CCSVI were an effect of the MS disease process, one would expect to see repeated restenosis of the veins as that disease process continued to impact a patient's vasculature.

So then, what conclusion can be drawn? Is CCSVI the cause of MS, an effect of the disease, or does it have no relation to Multiple Sclerosis at all? My honest belief is that the answer could be all three, depending on the individual patient.

Multiple Sclerosis is a remarkably heterogeneous disease, meaning that it impacts different patients in vastly different ways. Across the wide spectrum of MS patients, the primary symptom of the disease may be fatigue, cognitive dysfunction, muscle weakness, spasticity, eye trouble, nerve pain, or any combination thereof. Some patients can have the disease for decades and show very little physical disability, while others find themselves in a wheelchair (or worse) in less than 10 years (sometimes much less). Some patients have a great many lesions and very little disability, others have few lesions but devastating disability. Confounding the issue even more, MS comes in several different flavors, from Relapsing Remitting to Primary Progressive, and evidence suggests that the disease process at work in progressive disease may be quite different than that underlying Relapsing Remitting MS.

Given such a wide array of disease presentations, and thus the likelihood that a variety of mechanisms may be at play, it could very well turn out that CCSVI plays no role at all in the disease of some patients, a more causative role in the disease of others, and could be an effect of MS in yet another patient population. Very likely the line between cause and effect may be quite blurred, with CCSVI playing an exacerbating role in a disease that almost certainly has, in addition to a vascular component, very strong genetic and infectious components as well. The mix may be dramatically different from patient to patient, and indeed, CCSVI may be THE major factor in the disease of some patients, but play absolutely no role in the disease of others.

This may be reflected in the breakdown of outcomes reported by some of the Interventional Radiologists doing the CCSVI treatment procedure. The most widely quoted is Dr. Gary Siskin, of Albany New York, whose group has done over 700 procedures. Dr. Siskin has found that one third of his patients experience dramatic improvements, another one third experience mild improvements, and a final one third experience no improvement whatsoever. Further complicating this equation is the definition of just what constitutes a dramatic improvement. For somebody who's most disabling symptom is fatigue, a lifting of that fatigue would undoubtedly be called dramatic. For somebody more disabled, like I am, a lifting of fatigue, while certainly welcome, would hardly be defined as a dramatic improvement.

As I stated earlier, clear as mud…

Related articles

• The Rorschach test of CCSVI research (macleans.ca)
• Higher CCSVI rates with MS, but findings still unclear (ctv.ca)

Bits and Pieces: Sex Sells Edition

Image by Nick Sherman via Flickr

Note: For the many readers who receive my posts via e-mail, please be aware that embedded videos in these posts do not show up in the e-mailed version. To view the embedded videos, please visit the blog page itself by clicking on the title of the post. Thanks.

Well, I wonder if my Internet hit count will go up because the word sex appears in the title of this post. Such is the power of titillation, long known by advertisers. Funny, the word titillating is itself kind of titillating. Come to think of it, so are the words "Bits and Pieces". Anyway, on with some titillating tidbits (yet another word with titillating tendencies)…

Don't worry, I'm not about to descend into the tawdry. Not that I'm above tawdry, mind you. Back in my healthy, single days, I always believed that a little occasional debauchery did a soul some good, as long as all parties involved took part in the monkeyshines of their own free will, and no harm, emotional or physical, was done to any living thing. Eat, drink, and be merry, and all that. Life is full of uncertainties, sometimes you've gotta eat dessert first.

Unfortunately, the effects of MS and many other chronic diseases can dramatically impact the ability to partake of such shenanigans, and a few recent news items got me thinking about how sexuality is quite often a silent casualty to such illnesses, much to the detriment of those suffering from them. Sexuality is an important part of the human experience, and of all the losses meted out by disabling diseases, the effect on the sexual self may be one of the most keenly felt, yet least often talked about.

So, I submit to you the following news items, which I think shine some light on the issue …

· A new documentary film, "Scarlett Road", details the efforts of Rachel Wotton, an Australian sex worker who specializes in catering to the needs of a very specialized clientele, people with severe physical disability (click here). Among her clients is John, a severely disabled multiple sclerosis patient who relies on a chin controlled wheelchair. According to the film's website (click here), the benefits John has received have not only been emotional, but physical as well. In addition to increased self-esteem, he's regained some physical functionality that he thought was lost forever (and not just in his nether regions).

Human beings are incredibly social creatures, and the power of touch and a warm embrace are very real indeed. Loneliness is difficult enough to deal with for those lucky enough to be healthy, but for those poor souls locked in dramatically unresponsive bodies, the feeling of enforced solitude must be crushing. Though their bodies may be broken, their minds and spirits certainly are not, and for those so profoundly stricken the need for physical affection, for the exhilarating warmth of the intimate touch of another human being, for the feeling of somebody gently lying beside them, sharing a sensual embrace, must certainly be incredibly precious, its fulfillment tremendously and perhaps infinitely enriching.

Despite the supposed sophistication and enlightenment of modern societies, the severely disabled are still stigmatized and marginalized, and although lip service to their humanity is often paid, in reality far too many suffer the anguish of being a personality trapped in a useless prison of flesh and bone, their psychological and emotional needs barely even acknowledged. Some may question the morality of sex workers being paid to satisfy the needs of the severely disabled; I would question the morality of a society that forbids it, that denies the fulfillment of these most basic human wants and desires to those who need them most.

Thank you, Rachel Wotton, if for nothing else than simply caring. I wonder if there are similar services available to disabled females suffering from the same lack of physical attention?

"Scarlett Road " will be premiering at the Sydney Film Festival on June 11. Hopefully, it will soon be available for viewing outside of Australia.

Scarlet Road Video from Paradigm Pictures on Vimeo.

· A 66-year-old wheelchair dependent man with multiple sclerosis, Mr. Jim Keskeny, was kicked off a nudist cruise through the Caribbean after he injured himself while trying to use the toilet in his "accessible" cabin (click here). The man was traveling alone, and considered himself a "nudist at heart", although he hadn't previously participated in the nudist lifestyle, and decided at some point during the cruise that he didn't want to take his clothes off after all. Following his injury, the cruise line decided that he was in too debilitated a state to be traveling alone, although Mr. Keskeny was a seasoned traveler, and unceremoniously dropped him off in Mexico, leaving him to make his way back home to the states. The passenger claimed that he was perfectly able to take care of himself, and that the cruise line had simply used his accident as an excuse to get him off of the ship.

I'm embarrassed to admit that at first glance this story made me snicker. After all, the whole scenario seemed a bit absurd, a very disabled man signing up for a nudist cruise, sure to be populated with some extremely able-bodied naked people, a situation that seemed rife with all kinds of potentially (pun alert!) prickly situations and scenarios. One could easily question the man's motivations, and ridicule the almost predictable state of affairs he found himself in, but I quickly realized I was casting judgment on the man when perhaps all he was trying to do was be "normal", and satisfy some lifelong curiosities.

Putting aside all questions of infringements on the rights of the disabled and the legality of cruise line's actions, who among the afflicted doesn't yearn for some normalcy, to just once act on our wants and desires without having to account for the physical and emotional burdens wrought by bodily disability? Though taking a nudist cruise might not be everybody's cup of tea, it obviously was Mr. Keskeny's, and by God he went for it, torpedoes be damned. Certainly, his motivations for wanting to take the trip were no more or less prurient than those of his able-bodied fellow passengers, so why should the fact of his disability make any difference whatsoever? If he wanted to explore his sexuality in this manner, or simply just wanted to feel the freedom he perceived in the nudist lifestyle, more power to him. Rather than be subverted by his disability and assume the role of social outcast, Mr. Keskeny asserted his humanity and followed his heart's desire, certainly displaying some bravery in the process. Good for him.

· On another sexually related tangent, the drug sildenafil, better known as Viagra, has been shown in animal studies to reverse the course of multiple sclerosis symptoms (click here).

Upon seeing this headline, I reasoned that since Viagra works as a vasodilator, opening veins, this could play into the CCSVI scenario. Upon further investigation, though, it appears that the mechanism of action in regards to MS is the reduction of infiltration by inflammatory cells into the central nervous system. The mouse model of MS, called EAE, was used in the studies, and mice with EAE don't have blocked veins.

The fact of the matter is that EAE is a terrible model for human multiple sclerosis, and is induced by injecting the unfortunately targeted rodents with myelin proteins, provoking an allergic reaction within the animal that results in central nervous system damage. This bears little if any resemblance to the disease mechanism of the human illness, which is why so many loudly trumpeted "breakthroughs" in MS treatment on mice fail to translate into similar success stories when tried on humans. The simple fact of the matter is that mice don't get MS.

However, if Viagra does eventually prove to be beneficial in the treatment of multiple sclerosis, how ironic that a drug renowned for increasing stiffness in a certain body part might relieve a disease one of whose hallmarks is severe muscle stiffness. All I can say is that if the famous little blue pill is effective in treating multiple sclerosis, a lot of newly spry men are going to need to get their trousers altered, needing a little more room just below the waist…

Related articles

• Disabled man says he was kicked off cruise (msnbc.msn.com)

Remembering Bobby Kennedy

Image via Wikipedia

I am a man with very few heroes.

It disturbs me to see the word hero tossed around almost indiscriminately these days, as it belittles the few individuals truly deserving of the honor. Though I respect many people, some deeply, there are only a few whose words and deeds have led me to attempt, usually with pathetic results, to emulate the examples set by them. One such person is Robert F Kennedy, who was felled by an assassin's bullets late in the night of June 4, 1968, 43 years ago today.

Bobby Kennedy was by no means a perfect man, as has been well-documented by numerous tell-all books and our insatiably gossip hungry media. He was a complex individual, intelligent, introspective, and headstrong, possessed of ego and at times known to be ruthless. But he was also an idealist, a man whose thoughts, and the actions driven by them, evolved through a life that saw devastating personal tragedy. After the assassination of his brother, President John F. Kennedy, RFK went through a long dark night of the soul, only to emerge more resolute than ever to devote himself to public service and fight for his deeply held moral convictions, against societal injustice and for the weak and disenfranchised.

Robert Kennedy started his political career working in the office of Sen. Joseph McCarthy, who at the time was in the midst of his vile early 1950s anti-Communist witchhunt, which resulted in the destruction of the reputations and livelihoods of dozens of innocent victims. From those ignominious beginnings sprang a career that saw Robert Kennedy champion civil rights, advocate for the poor and marginalized, fight organized crime, and help pull the world back from the very brink of nuclear Armageddon during the Cuban Missile Crisis.

After his belated entry into the 1968 presidential race, his campaign to win the Democratic nomination gained increasing momentum, culminating with his victory in the California primary on June 4, 1968. Minutes after delivering his victory speech at the Ambassador Hotel in Los Angeles, he was shot while attempting to exit the building with his entourage. Though an assassin, Sirhan Sirhan, was named and convicted, controversy still rages over the tragic sequence of events that transpired that night. Robert Kennedy lingered for two days, and died on June 6, 1968.

Had Kennedy won the nomination, and eventually the presidency, the historical timeline would certainly have been significantly altered, probably beyond all recognition. There would have been no President Nixon, no Watergate scandal, a quicker end to the Vietnam War, and no massacre at Kent State. Without these traumas inflicted on the psyche of the United States, one can only imagine that the arc of history could very well have been much more benign than that which did ultimately become reality. The promise represented by Robert Kennedy cannot be overstated, nor can the tragedy of his loss.

Perhaps the best way to illustrate the merits of Sen. Kennedy is to let the man speak for himself. On April 4, 1968, just two months before his own assassination, Dr. Martin Luther King Jr. was assassinated in Memphis, Tennessee. On the evening of the King assassination, Bobby Kennedy was scheduled to address an inner-city audience in the heart of Indianapolis, Indiana. Knowing that his audience would be largely black, and almost certainly unaware of Dr. King's assassination, Kennedy had little time to formulate his thoughts, much less write a polished speech. Without the help of aides or speechwriters, he jotted a few notes to himself on the ride to the site of the rally, and then delivered, almost completely extemporaneously, an eloquent and profoundly emotional speech. No teleprompters, no calculations of political consequences, just intelligent and respectful words delivered from the soul. He didn't speak down to his audience, but addressed them as peers, sharing with them the anguish of having suffered the murder of a loved one. As a result, Indianapolis was one of the few American cities spared vicious riots in the wake of Dr. King's assassination.

Here is the speech Robert Kennedy delivered that night…

Rest in peace, Bobby Kennedy.

On Friends, Old and New

Image via Wikipedia

"People come and go, and forget to close the door, and leave their stains and cigarette butts trampled on the floor, and when they do… Remember me, remember me"

-Brian Eno

There are several phrases that when heard or uttered can forever change the course of a life. "You're hired", "I'm in love with you", and "I do" are a few of the more common, all imbued with the power to positively alter one's destiny. On the flipside, there's a multitude of words that, when strung together, can have a negative, even dreadful impact. Those of us dealing with serious illness have all heard variations on the same devastating words streaming from a doctor's mouth, something along the lines of, "I'm afraid you have (insert name of illness here)". From the moment those syllables are comprehended, we are suddenly singled out, set apart from the world we inhabited just moments before, the land of the well.

No matter how loved or popular the recipient of such a diagnosis may be, they've now been forced into a new and alien social strata, that of the chronically ill, an exclusive club to which nobody wants to be a member. Though friends and family offer heartfelt and genuine gestures of comfort and sympathy, there is simply no way for them to truly understand the disorientation, fear, and alienation wrought by the verdict recently rendered. The newly minted patient, even if surrounded by a crowd, is left to navigate a frightening new reality in large part alone.

Make no mistake, the support of friends and family is vital to the mental and physical well-being of a newly diagnosed patient, but that moment of diagnosis does serve as a line of demarcation between an old reality that was very likely taken for granted and a new one fraught with uncertainty. This crisis point in a person's life can test old relationships, and unexpectedly offer opportunities to build new ones.

Over the course of a lifetime, there is a natural ebb and flow of individuals entering and exiting a person's world, an ever shifting population of friends and acquaintances that inevitably changes with the passage of time. A precious few of these people take up permanent residence in the timeline of life, and transcend friend to become family. True family is defined more by love than by blood, and I consider myself blessed to count among the innumerable persons I've encountered perhaps half a dozen who I know will be constants for the whole of my life. We may not be in perpetual contact, in fact we may not talk for months at a time, and perhaps not meet in person over stretches lasting years, but I am secure in the knowledge that when push comes to shove, no matter the situation, we'll always be able to pick up just where we left off, our bonds too strong to be broken by the strains of time, distance, or circumstance.

These rare relationships, some of which have spanned decades, are cherished and acknowledged as precious, for I realize they link me not only to people that I hold dear, but also represent a tangible connection to my own past, confirmation that what came before was not merely a dream, but a series of very real experiences that I was lucky enough to share with some special individuals. Though I've only been diagnosed for eight years, at times it's hard to remember a life without illness, a time when I was blissfully ignorant of most things medical. These friends turned family, who are of course deeply sympathetic to and aware of my difficulties, look straight past the fact of my illness to the essence of who I am. To them I will always be the same old Marc, and for that I am forever grateful.

Other friendships from my healthy life have been diminished by my illness, really at no fault of the people involved. Many friendships thrive primarily on a continuing series of shared social experiences, and as my disabilities have mounted and my ability to socialize has become curtailed, the spigot that fueled many of these relationships has been turned off. Phone calls and e-mails are still occasionally exchanged, and halfhearted motions are made at making plans to get together, but they almost never actually come to fruition. That's okay, really, as without an ongoing narrative, some relationships are bound to simply stall, and eventually wither. It's all part of the rhythm of life, the natural order of things.

In this age of the Internet and social networking, it's now quite common to reestablish connections with folks who once populated your world, but were long ago got lost to the ever shifting tides of time. I've found that most of these renewed friendships at first burn brightly with shared memories and updates on current circumstances, but once that initial flame is exhausted, they again recede into a state of benign neglect. A few, though, have turned out to be very happy and lasting reunions, with people for whom I never really lost affection, but only lost touch. It is a real joy to rediscover a misplaced but valued friend, like unearthing a buried treasure.

In all honesty, though, my illness has made me somewhat reticent to pursue some of these Facebook friendships, as I sometimes think I'd rather be remembered as the youthful and healthy me locked in my old friend's memories, and recounting the details of life since my diagnosis hardly makes for a lighthearted exchange of pleasantries. Perhaps this accounts for my almost phobic relationship with Facebook, which I suppose it's time I should just get over. I'm completely open about my illness with the world at large, after all, but somehow the prospect of detailing my saga to certain individuals remains daunting. Though I feel I've handled the emotional upheaval attached to illness quite well, I suppose the pain of my reality lies not too deeply beneath my Wheelchair Kamikaze persona.

Despite the love and support of family and trusted old friends, it's been incredibly important for me to develop relationships with fellow patients, others who simply "get it". As kind and sympathetic as the well people close to us might be, they just haven't lived the situation, and thus don't have the capacity to fully gauge the true measure of being chronically and progressively ill. It's like trying to get a blind man to understand the difference between blue and orange, the complexities are beyond words.

I can't imagine how difficult it must've been for patients dealing with chronic illnesses before the advent of the Internet, how isolated and alone so many must have felt. Soon after my diagnosis I discovered some of the Internet MS forums, and in them found a haven where was able to interact with others dealing with many of the same questions, fears, and emotions that were roiling inside of me. I derived incredible comfort and relief in these virtual worlds, populated by so many who had gone through situations similar to those that now confronted me.

I initially ventured onto these forums seeking only information, never imagining that I might find real friendship in the coldness of the words of strangers on my computer screen. But soon the anonymous screen names of those participating took on the characteristics of the living, breathing human beings behind them, and the catharsis of being able to commune with fellow patients was incredibly therapeutic. Before long it felt like some of my best friends were people I'd never actually met or even talked to. When I did eventually have the opportunity to meet some forum members in person it often felt as if we'd known each other for years, unencumbered by the awkwardness of unfamiliarity that usually accompanies first encounters.

Of course, not every MS patient is going to become a close friend, but I found that the shared emotions of dealing with a serious diagnosis can often strip away much of the artifice and posturing that goes on in every day social interactions, and patient to patient we can often cut right to the chase and dispense with most of the meaningless trivialities that get in the way of establishing the bonds of true friendship. I've developed heartfelt relationships with people I might not otherwise have interacted with, folks in locations and with backgrounds that I might never have crossed paths with or given a chance to without the unfortunate commonality of a shared disease.

When eventually we take our leave of this earth, as we all must, the friends we've made and the people we've touched are perhaps the truest measure of the lives we've led. Despite the hardships imposed by disease, and the impersonal nature of the medical world we've been forced to inhabit, we must never lose sight of our essential humanity, and relish and take joy in our close friends, old and new. Even in this high-tech world of instant messaging, Facebook, and tweets, a pat on the back, some gentle words of kindness, and an earnest gesture of support still convey that most important of human sentiments, genuine affection for those we are lucky enough to call friends.

Some Comments on Comments, and Also on CCSVI

Earlier today I received the following comment from a reader, in response to my last post which dealt with the Merck Serono "kickback" lawsuit (click here):

"I find it ironic that you can talk about big pharmaceutical companies acting badly when you yourself are acting badly. Twice I have posted about my ccsvi experience, and because it was not positive you have not posted it. It has not been the answer for me and it will not be the answer for others. This site in part talked me into going for this procedure. This cost me well over $20,000 and did nothing. You are pushing this procedure and not telling the full story of its failures. I know that this will not be published as you are biased and selling this procedure just as a pharmaceutical company was selling rebif. I really thought you were different."

To be honest, at first I was somewhat taken aback by this comment, as I feel that I've always tried to present CCSVI in a rational and evenhanded manner, and have neither dissuaded or persuaded patients to undergo CCSVI treatment. If anything, I've urged caution, often to the chagrin of the fiercest CCSVI advocates, as I feel the science of CCSVI and its treatment are both very much works in progress. I also felt very sorry for the commenter, whose disappointment and rancor is obvious (please, dear readers, don't leave comments disparaging the person who wrote the above comment. Although I completely disagree with the gist of the statement, everyone is perfectly entitled to their opinion).

I then realized that the above statement affords me the opportunity to address just how comments on this blog are handled, and to state clearly and succinctly my feelings on the current state of CCSVI research and treatment.

The blogging platform I use (Google's Blogger) provides a variety of ways with which to deal with comments made on any individual post. The blogger (me) has the choice of moderating all comments (in other words, no comments can be posted without my seeing them first), moderating only comments made on older posts (such as posts more than seven days old), or not moderating comments at all (all comments are posted without my approval on all posts, regardless of when they were initially published).

When I started Wheelchair Kamikaze, I chose to not moderate any comments, as I believe strongly in free speech and welcome any and all opinions that my scribbles might elicit, both positive and negative. The only way we can truly learn and expand our minds is to listen to opposing viewpoints, and indeed many of the comments left on previous posts that did not agree with what I had written have given me much food for thought. As the popularity of this blog has grown, the amount of comments left after each post has grown with it, to the point where I am sometimes unable to address each and every comment left by my readers. I truly feel bad about this, but I do read every comment, and deeply appreciate all who take the time to contribute.

Unfortunately, about six months ago, several spammers started leaving irrelevant comments on dozens upon dozens of blog entries, hawking everything from wheelchairs to CCSVI treatment centers. As I am of the opinion that spammers are among the scum of the earth (not quite as bad as some health insurance or pharmaceutical company executives, but close), and don't want to subject my readers to such garbage, I felt compelled to start moderating all comments left on posts more than one week old. Readers are free to comment on new posts, completely unmoderated, for the first week they are published. After that, I get an e-mail notification of each pending comment, and have the option of publishing or deleting it. As a rule, I publish all comments, as long as they aren't vulgar or abusive, unless they come from spammers. The spam is quickly deleted, an action which delights me to no end.

The only drawback to this system is that occasionally the notification of a pending comment gets caught in my e-mail spam filter, and winds up in my "junk e-mail" folder. Since the junk e-mail folder usually contains nothing but (surprise!) junk, I usually don't bother checking it. Therefore, if any of you try to comment on an older post, and your comment doesn't show up after a day or so, please e-mail me at WheelchairKamikaze@gmail.com and I'll do my best to locate your wayward post. Of course, you can also try submitting the comment again, but if your first attempt got caught in my spam eater, there's a good chance your second attempt will also.

Now, on to CCSVI, a topic which only seems to be getting more controversial by the day. In recent weeks, we've seen a variety of studies released, some supporting the CCSVI hypothesis, and others refuting it. Amongst those supporting it, there have been some that argue for CCSVI as the cause of MS, and others concluding that the vascular abnormalities collectively known as CCSVI are more the result of MS, or perhaps exists as a comorbidity with the disease. Internet forums and Facebook pages devoted to CCSVI have been riven by arguments both for and against, often pitting patient against patient. Unfortunately, hyperbole often rules the day, with each side regularly making claims that simply can't be substantiated by the available science.

Meanwhile, as the scientists and patient advocates duke it out, thousands of desperate MSers are getting CCSVI treatment here in the United States and at various locations around the world. The technique used to treat CCSVI, venoplasty, is a minimally invasive procedure that involves snaking a catheter through a patient's veins to the point of blockage or narrowing, and then ballooning it open (in some increasingly rare cases a stent is used for this purpose). Although the procedure sounds simple enough, there is in fact a steep learning curve involved for the doctors performing it, and there has yet to be standards of practice agreed upon by all specialists. Some interventional radiologists use larger balloons than others, some check more veins, some use stents more frequently than others, and the "aggressiveness" of treatment varies from physician to physician.

Despite the overwhelmingly positive patient reports that flood the Internet, the results of the CCSVI treatment procedure are actually quite mixed. Many patients do indeed find lasting benefit after going through venoplasty, but as is evinced by the reader comment that prompted this post, a sizable number of patients receive no relief at all after CCSVI treatment. Many others find temporary relief, only to face the crushing disappointment of a return of their symptoms when their veins revert back to their blocked or narrowed state (a situation dubbed "restenosis"). Some treated patients have developed blood clots or scarring in their treated veins, situations which are extremely difficult to resolve, and which can effectively shut down the affected vein for good. When reading patient reports on the Internet, or viewing them on YouTube, it's very important to keep in mind that most patients who are treatment "failures" don't make videos or write testimonials. It's simply human nature to shout our triumphs from the hilltops, but to keep quiet about disappointments.

The only thing that anybody can state for sure about CCSVI at this time is very little regarding the topic can be stated for sure. While it does look like there is a correlation between abnormalities in the veins that drain the central nervous system and multiple sclerosis, the question of cause or effect is far from decided. Furthermore, the prevalence of such abnormalities in the healthy population has yet to be properly quantified. Some studies suggest that as many as 25% of the population have venous abnormalities which would qualify for CCSVI, but show absolutely no ill effect. As shocking as it may seem, the human venous system associated with the central nervous system has been so little studied that no reliable definition of "normal" actually exists. Until now, the arteries, responsible for heart attacks and stroke, have received all the attention, leaving venous anatomy largely unexplored.

Further complicating the picture is the relative high cost of the procedure. Patients traveling abroad are typically paying about $10,000 for the procedure alone, without travel expenses figured in. Patients in the US, getting treated domestically, have in large part been finding their treatment covered by private health insurance. Unfortunately, that situation seems to be changing, as one of the major private insurers has started to reject CCSVI treatment claims, and there are reports that Medicare is now also refusing to pay for the procedure. Given the high rate of restenosis, and the wide disparity in treatment outcomes, patients need to seriously consider whether waiting for both the CCSVI science and treatment procedure to mature may be in their best interest, especially if they will be paying out-of-pocket.

My personal opinion is that MS is a very complicated beast, and in fact may not be one disease at all, but a collection of related maladies that share common symptoms and diagnostic criteria. The causative factors of MS very likely differ significantly from patient to patient, and almost certainly include genetic predisposition, infectious factors, toxins, and, in some cases, vascular abnormalities. Just as none of the existing MS drugs works on every MS patient, so too the impact of CCSVI will almost certainly vary from patient to patient. For some patients, CCSVI may be THE predominant factor in their disease. In others, the condition may play no role whatsoever, even if their veins do display abnormalities (remember the high likelihood that some healthy people display similar abnormalities).

As it now stands, each patient must educate themselves as fully as possible, and honestly assess the risk/benefit equation for their own particular situation, trying their best to understand the existing realities despite the fog created by conflicting media reports and Internet hyperbole. One of the best ways to do this is to listen to the researchers exploring CCSVI, and the doctors performing the CCSVI treatment procedure. Thanks to the magic of the Internet, patients can do just that. Recently, the scientists at the Buffalo Neuroimaging Analysis Center held a town hall meeting during which they detailed the findings of their ongoing CCSVI studies. Videos of the presentations given are now available on the BNAC website (click here). The Hubbard Foundation, another organization actively researching CCSVI and organizing CCSVI treatment trials, also recently put on a patient education forum, the videos of which can be accessed (here). Both sets of videos should be required viewing for anyone interested in CCSVI.

I'll leave you with a video interview, conducted by the CCSVI Alliance, with one of the most experienced CCSVI treatment practitioners, Dr. Gary Siskin. Dr. Siskin's insights and advice are invaluable, and his sober approach to CCSVI is necessary and refreshing. As Dr. Siskin says at the end of his interview, "Anybody who says they know everything about CCSVI is probably not telling you the truth, because the level of knowledge just hasn't gotten there yet…"

Big Pharma Behaving Badly (And Making Me Want to Vomit)

Image by BlueRobot via Flickr

Sorry about the nauseatingly graphic nature of the title of this post, but the following info makes my head explode.

Pharmaceutical industry giant Merck Serano this week paid $44.3 million to settle a lawsuit alleging that the company paid kickbacks to MS neurologists for prescribing its blockbuster MS drug, Rebif (click here). The scam allegedly included hundreds of doctors, and seems to have been centered on the Consortium of Multiple Sclerosis Clinics (CMSC), a nonprofit corporation that is supposed to help educate patients. Among its other activities, the CMSC administers the quarterly NARCOMS surveys, designed to build an ever growing database on MS and its effects on patients, which are diligently filled out by thousands of multiple sclerosis patients, including, until now, yours truly.

The lawsuit was brought by a former Merck Serano employee turned whistleblower, who was fired by the company after expressing disbelief that such underhanded and blatant misdeeds were going on with the company’s full knowledge and participation. The entire lawsuit document is available online (click here), and to save you the trouble, the good stuff starts on page 6.

We're not talking nickels and dimes here, but huge chunks of cash, on the order of $25,000 a pop. The CMSC allegedly funneled over $500,000 to various doctors, effectively operating as a money-laundering outfit for Merck Serano, and the fact that the company settled for $44 million indicates this disgusting scam went far deeper than what is apparent in the legal document. Unfortunately, by settling the lawsuit, Merck Serano has effectively prevented all of the gory details from coming to light, as would've been the case if the suit had been brought to trial, and thus become public record. The settlement covers fraud charges regarding Medicare and Medicaid, but what of the patients that may have been steered into injecting themselves with a drug that might not have been their best choice by scoundrels in white lab coats more intent on stuffing their pockets than following the Hippocratic oath? Where is their justice?

Coming at a time when trust between neurologists and their patients has become frayed due to the CCSVI controversy, the revelations provided by the lawsuit and subsequent settlement are especially revolting. It is simply outrageous that a drug company can get away with paying physicians kickbacks for prescribing drugs with what amounts to a slap on the wrist. The terms of the settlement, $44.3 million might sound like a sizable sum, but considering that sales of Rebif exceed $1 billion a year, the penalty is miniscule. Additionally, this is most likely only a quick peek under a very big rock, and it is just a glimpse at goings-on that would shock and dismay the legions of desperate patients who are the real victims of these disgusting, dishonest, and deceitful practices.

Before being forced out of work due to disability, I was employed by one of the huge worldwide entertainment conglomerates, which included in its portfolio some of the biggest record labels on the planet. In the music industry, what Merck Serano is guilty of is referred to as "payola". Simply put, payola is the practice of paying disc jockeys, radio station program managers, and other "hit makers" to play and promote the songs of a music company's artists. Back in the 50s, payola brought down the careers of some of America's top DJs, and to this day people go to jail when found guilty of the crime.

Now, what's more harmful to society, a music company paying radio stations to play the latest Beyoncé single (I'm just pulling an example out of thin air here, not accusing Beyoncé or her record company of anything) or a drug company paying doctors to prescribe their products to desperately ill patients? The rancid stench of greed and avarice has infected many of our most important societal institutions. After reading the above allegations, can any patient truly trust that their doctor has no ulterior motive as he scribbles the name of some drug on his prescription pad, very often in an office whose walls and shelves are filled with posters and promotional products handed out like shiny trinkets by pharmaceutical company representatives? That swirling sound you hear is the ethics of an entire society going straight down the toilet.

The fact that this case was settled, and not brought to court, ensures that the names of most of the players will not be revealed. I'm tempted to reveal those named in the lawsuit here, but as a settlement was reached, and no verdict of guilt ever handed down, they remain simply accused, not convicted. My personal ethics therefore prevent me from slandering those who might not be guilty as charged, however unlikely that may be. That's not to say that you shouldn't click the link (here it is again), read the lawsuit, and find those names for yourself. In fact, I wholeheartedly encourage you to do so.

Every single doctor and medical professional who received any of these tainted funds, or participated in distributing them, should be named, shamed, stripped of their licenses, and thrown in jail. They're a disgrace, each a pustule on the ass of humanity, and deserve nothing more than humiliation and degradation. Unfortunately, thanks to the niceties of a system that too often protects those in positions of power at the expense of the regular folks who rely on them, the scoundrels involved will remain anonymous, free to enjoy the fruits of their despicable actions. Despite our high-minded ideals of justice for all, far too often there is justice for none.

As for those involved in this medical and legal debacle, and the many other similar cons we will likely never hear about, may their eyes fall out. Bastards.

Related articles

• Merck Serono pays $44M to settle kickback charge (seattletimes.nwsource.com)
• PRESS RELEASE: Pharmaceutical Giant, Serono, Agrees to Pay $44.3 Million to Settle False Claims Act Case (fredericacade.wordpress.com)
• Georgia joins $44.3 million pharmaceutical settlement (ajc.com)

CCSVI As Punk Rock

Image via Wikipedia

I Want To Be Anarchy.

This was the battle cry of a generation of young rock ‘n rollers, who, in the late 1970s, donned black leather jackets, cut their hair short and spiky, picked up instruments they barely knew how to play, and declared war on a bloated music industry establishment. Sick of having music shoved down their throats by corporations that had become self-reverential and complacent, they decided to take matters into their own hands, and by doing so changed the course of rock 'n roll history.

After the incredibly vibrant and creative 1960s, which saw rock 'n roll evolve from a teenybopper fad into a multifaceted and sometimes profound art form, by the mid-1970s music seemed to be suffering from a particularly brutal hangover. Dominated by bands that had become behemoths, and musicians who had come to view themselves as royalty, rock 'n roll had changed from the soul of a youthful revolution to the sound of cash registers opening and closing. Sure, there were exceptions, but they were few and far between, followed by pockets of ardent fans but stuck in relative obscurity. Record companies barely supported the likes of David Bowie, Iggy Pop, The New York Dolls, and T Rex, preferring instead to throw their money and marketing might behind safer, less challenging acts, but in doing so stripped the passion out of an art form that had been born of it.

The Multiple Sclerosis establishment found itself in a vaguely similar position in 2010. Prior to the late 1980s, decade after decade of investigation had been spent making absolutely no headway against the disease, despite the best efforts of researchers. These were the dark ages, a gloomy time for patients diagnosed with MS, during which the disease was considered a "diagnose and adios" malady. There was precious little doctors could do for their desperate patients except wish them well. Researchers primarily concentrated on looking for an infectious cause to the disease, with a few suggesting that the vascular system may somehow be implicated. Despite the occasional report of a breakthrough, none ever panned out, and patients and their doctors were left with little option other than to simply watch the disease take its insidious toll.

In the 1980s, things changed. The theory of autoimmunity, which posited that for reasons unknown a patient's own immune system goes on a cannibalistic attack, destroying a body's own central nervous system tissues and resulting in the telltale signs of MS, took hold and started to grow roots. By the late 80s a substance known as beta interferon, thought to somehow modulate aberrant immune systems, was in full clinical trials on MS patients. These trials proved successful, even if only moderately so. A statistically significant percentage of patients with relapsing remitting disease were shown to suffer less relapses while on beta interferon drugs, and their MRIs displayed fewer areas of disease activity. It was hoped that this would translate into the delay or even cessation of disability progression, but at the very least these new drugs did increase the quality of life for some of the patients taking them, and physicians finally had a weapon in the fight against the disease.

As the autoimmune theory became more entrenched, MS research started focusing almost exclusively on finding better and more powerful methods of suppressing the human immune system. This led to the creation of new classes of drugs which suppress various parts of the very intricate and not fully understood immune system, substances which show more efficacy than the interferons in treating the symptoms of MS, but also carry with them the specter of serious and sometimes deadly side effects. While these drugs, too, have had a profound effect on the quality of life of some of the MS patients taking them, none address the still unknown root cause, or causes, of the disease. Problematically (for patients hoping for a cure, at least), MS drugs have become financial blockbusters, transforming multiple sclerosis into an $8 billion a year industry, a financial windfall which has further focused the attention of researchers on tinkering with the immune system rather than finding out just what causes it to suddenly turn upon the body’s own cells.

Back in the late 70s, restless kids fed up with the pablum being pumped out by the music industry (appropriately dubbed "corporate rock") started to create their own music. Though their output may have been technically less than masterful, the raw passion, emotion, and soul poured out by bands such as The Sex Pistols, The Clash, and The Ramones more than made up for their musical deficiencies. Combined with sarcasm, swagger, and a loud disdain for the mainstream (one of the seminal albums of the time, by The Dead Boys, was appropriately entitled "Young, Loud, and Snotty"), the brashness of these young punks was startling, and set the mainstream back on its heels.

Their popularity primarily still confined to the musical underground, the antics, in-your-face behavior, and outrageous attire of the early punks soon grabbed the attention of the media, who at first treated them with derision, as did the corporate giants the punks were attacking. Initially shrugged off as troublesome outcasts, many pundits predicted their quick demise. Instead, punk thrived and only gained in popularity, spawning a vibrant counterculture whose ethos spread from music into the visual and cinematic arts. Due to the popularity of a new communications entity, MTV, punk music and fashion spread quickly from the cities to the suburbs, and soon the major labels were scrambling to sign and market their own punk bands, now labeled under the more palatable moniker of "New Wave".

Late in the first decade of the 21st century, the Multiple Sclerosis establishment likewise found itself confronted with a radical new idea, called CCSVI. This hypothesis, first put forth by the Italian vascular surgeon Dr. Paolo Zamboni, states that blockages in the veins that drain the central nervous system either cause or contribute to the neurodegenerative disease we know as multiple sclerosis. Furthermore, Dr. Zamboni's initial trials showed that opening these blockages, using a relatively simple and minimally invasive procedure, seemed to dramatically relieve the symptoms of MS, suggesting an entire new avenue of investigation into the disease. At first, Dr. Zamboni's findings got very little attention, outside of a small group of patients keenly debating the relative merits and demerits of the idea on a small Internet MS forum. Soon enough, though, the Canadian media picked up on the story, and all hell broke loose.

Rather than approach the idea with open-minded scientific curiosity, the MS establishment initially tried to dismiss the idea of CCSVI as absurd, some going so far as to label it a "hoax". MS patients reacted with indignant fury, the pent-up frustrations of years of being treated with hyper expensive and nominally effective treatments that do nothing to address the essence of what's making them sick exploding like Krakatoa, creating a medical tsunami the likes of which had never before been seen. Carried along by new social networking tools such as Internet patient forums, blogs, and Facebook, the MS establishment could do nothing to harness the growing CCSVI movement, a patient driven initiative that forced the issue as patients took matters into their own hands and began traveling abroad in pursuit of CCSVI treatment. In the United States, Interventional Radiologists, the doctors who specialize in the catheter procedures used to treat CCSVI, soon started taking notice, treating MS patients for the blockages that were indeed been found in their veins. Though far from universally successful, anecdotal evidence does seem to indicate that treating CCSVI does, at the very least, relieve some of the symptoms experienced by some MS patients.

As the 1980s evolved, punk evolved with it. While mainstream rock acts still commanded a lion's share of attention, new bands, deeply influenced by the original punk rockers, burst onto the scene, and gained in popularity. Slowly, what once seemed cacophonous, outrageous, and even obscene began to be absorbed into the mainstream, and the lines that separated rock 'n roll genres began to blur. Though the revolutionary vitality felt by those in the center of the storm in the late 70s and early 80s was largely gone, and punk rock's fangs had been filed down, by 1993 punk reigned supreme as it flooded the airwaves and music stores under the new banner of "Grunge", a development that the leather jacketed, safety pinned, and spiky haired kids 15 years before could never have even imagined. These days, the children of the original punks listen to modern music that would not exist if it weren't for long gone bands that they've barely heard of, largely unaware of the history behind the music.

The future of CCSVI is likewise beyond prediction. There is much still to be learned, both about the condition itself, and how best to treat it. The significance of CCSVI in the big picture of MS still needs to be determined. Despite the hopes of the most fervent CCSVI supporters that it will prove to be THE cause of MS, and the doubts of the naysayers who claim it has no significance at all, the truth will likely fall somewhere in between, and could very well vary from patient to patient. For some, CCSVI may play a dominant role in their disease, for others it may play no part at all. Current treatment protocols need to be standardized and perfected, as we currently see too many problems with restenosis, clotting, and the occlusion of treated veins, but eventually these issues too will be worked out.

Perhaps what's most important, though, is the tremendous upsurge in patient self-advocacy, education, and empowerment that has been the product of the intensity of the CCSVI debate, which has quite possibly changed forever the dynamic of the patient-doctor relationship. No longer will we as patients be dictated to, but partnered with. Knowledge that was once the province of an anointed few is now open to the masses, a development with repercussions that may take many years to resolve. New generations of doctors will undoubtedly be more open to this changing dynamic than the old guard, but Pandora's box has been opened, and for a while, it seems that anarchy will have its day. Punk rock and CCSVI, Viva La Revolucion!

Related articles

• The Rorschach test of CCSVI research (macleans.ca)

Situation Normal, All Fracked Up…

Image by Mathieu Struck via Flickr

When I started this blog, I was determined that it wouldn't be a treatment diary, so I haven't detailed the daily, weekly, and monthly ins and outs of my medical meanderings. Still, I've recently written about the fact that my diagnosis has been called into question, and I've received several inquiries in that regard. I thought it might be helpful if I gave a few more details about my mystery diagnoses, and how it's affected some of the treatment choices I've made and continue to make.

After my diagnosis of MS in March 2003, I quickly became uneasy with my doctor's conclusion. Like most other newly diagnosed patients, I immediately hit the Internet, reading all I could about the disease and interacting with dozens of patients on MS forums. The more I investigated, the less convinced I became that I actually had multiple sclerosis. Yes, two lesions did show up on my MRI, one tiny spot in my brain, and a much larger and more troublesome lesion at the base of my brainstem. Tests on my cerebral spinal fluid, though, revealed no Oligoclonal-bands (click here), telltale signs of CNS immune activity that show up in a majority of MS patients. O-bands are a bit less common in patients with progressive MS, the flavor of the disease I was suspected of having, but still, their lack fueled my reason to doubt.

Additionally, I had a variety of peculiar physical symptoms and some health history that just didn't seem to mesh with a diagnosis of Multiple Sclerosis. About six years before my first MS symptom struck (a slight limp in my right leg, which has since progressed to severe weakness and spasticity in my entire right side, and progressing weakness on the left), I was suspected of having Discoid Lupus (click here), a form of Lupus that attacks the skin. Although that diagnosis was never quite nailed down, my doctors at the time were confident that I did have something very strange going on in my immune system. After approximately 3 years, my Discoid Lupus type symptoms (small skin blemishes that left tiny crater shaped scars, hence the word "Discoid") burned out, to my considerable relief. Soon after, though, I was diagnosed with Hashimoto's Thyroiditis, an autoimmune disease that destroys the thyroid gland. I started showing signs of other types of endocrine dysfunction as well, indicating a cascading failure of my pituitary gland. Although Hashimoto's Thyroiditis is often seen in patients with MS, pituitary problems are not. About three years after these endocrine problems surfaced, my telltale limp showed up, and I was soon given the MS label.

About a year into my MS saga, I switched MS doctors and started seeing my current neurologist. He immediately suspected that I might be suffering from some other disease (he suspected Neurosarcoidosis-click here), and ordered an extensive battery of tests. When none came back positive, he deduced that Primary Progressive Multiple Sclerosis was the most likely diagnosis, and so the initial determination stood.

Since there is no actual test for MS, determining whether a patient suffers from it is a diagnosis by exclusion. The physician explores other possibilities that might explain the patient's symptoms, and if none pans out, the diagnosis of MS is assumed. In many patients, multiple sclerosis makes itself readily apparent. A relapsing remitting course of disease, the occurrence of enhancing CNS lesions on MRI images, and the presence of O-bands in the cerebrospinal fluid are all highly indicative of a multiple sclerosis diagnosis. Cases of the disease that are progressive from the outset are harder to diagnose, as there are quite a few maladies that can mimic progressive MS (click here).

Despite the fact that progressive MS is notoriously hard to treat, and there are currently no approved treatments for PPMS, I am not the type to sit around and do nothing while some insidious enemy hacks away at my body, and my neurologist is known for his aggressiveness in fighting the disease. We embarked on a comprehensive battle to attack my disease, which has included a wide variety of treatments, some quite outside the box, which are literally almost too long to list. Suffice it to say, none has had any effect whatsoever on the course of my disease.

Along the way, my illness has shown itself time and again to be extremely atypical, most notably in the fact that my MRI images have never changed. The two lesions that showed up in 2003 are still there today, and have never altered in size or appearance, or been joined by any others. MRI images of my CNS taken eight years ago are indistinguishable from those taken six months ago. Because of all of the atypical features of my illness, I eventually decided to seek another expert opinion, and was seen at the Johns Hopkins MS center in the winter of 2006. After undergoing a rigorous series of tests, Johns Hopkins also concluded that although my disease was certainly strange, they could find no indication that it was anything other than atypical PPMS. During the following few years, I kept in contact with the doctors at Johns Hopkins, and in 2008, after I sent them some recent MRI images (which remained unchanged) and explained that my disease had progressed significantly, it was requested that I come back down to Baltimore for another examination. This time, the doctors concluded that I very likely did not have MS, but they could not come up with a suitable alternate diagnosis. Several were suggested (Sjogren's disease and mitochondrial disease among them), but further testing ruled out these other illnesses.

At this time, I was accepted into a study being conducted at the National Institute of Health's main campus in Bethesda, Maryland, which was seeking to identify patients with clinically definite multiple sclerosis for use in further MS research. The NIH was finding that many of the subjects they were using in their multiple sclerosis research studies were actually misdiagnosed, and these misdiagnosed patients were polluting their research data. Over the next 18 months, I made four visits to the NIH's tremendously impressive facility outside of Washington, DC, during which every conceivable test was conducted. At the end of the process, the NIH declared that my test results and disease presentation did not fit the definition of multiple sclerosis by any known diagnostic criteria, but they too could not come up with a reasonable alternative. Needless to say, the situation was, is, and I'm sure will continue to be incredibly frustrating.

My primary neurologist here in New York, who runs a state-of-the-art research laboratory in addition to his clinical practice, recently did an extensive analysis of my spinal fluid, which turned out to be so strange that he had the analysis, which took several weeks to complete, repeated to confirm the results. Unlike many other MS patients, my neurologist and I have a very comfortable and frank relationship, and he told me just how bizarre my spinal fluid scans were in very colloquial and colorful language (I'll let you use your imagination to fill in the blanks).

Since it now seems likely that I'm suffering from either a) one of the strangest cases of MS on record, or b) some other autoimmune process that is attacking my central nervous system in addition to various other bits of my anatomy, my neuro suggested that I try IVIG (click here), a treatment that has been shown to be effective in some cases of MS, and perhaps more importantly for me, a variety of other autoimmune conditions that can attack the CNS. IVIG is a blood product made up of the antibodies of over 1,000 blood donors, which has been shown to attenuate the aberrant immune response seen in a variety of diseases, and is typically given monthly. Since I have a history of experiencing unexpected and sometimes frightening side effects from new medications, we started out by doing half a dose of IVIG about a month ago (the treatment is usually given in two infusions over two days, but I only did one infusion the first time around). I didn't suffer any negative side effects, so later this week, on Tuesday and Thursday, I'll be doing my first full dose of IVIG.

Many of you are probably asking yourselves where CCSVI fits into my treatment picture. I had my first venoplasty about 13 months ago, which did reveal a blockage caused by a muscle pressing on my right internal jugular vein, but no other readily apparent venous abnormalities. Unfortunately, the muscle bundle is currently impossible to treat using the available treatment modalities, as ballooning would have no effect on the pressure being put on the vein by the muscle, and a stent would likely be bent out of shape, and possibly fracture, due to that same pressure. It is also uncertain as to whether the muscle bundle is causing significant disruption to the perfusion of blood through my central nervous system (Dr. Zamboni himself had a look at my images, and cast doubt as to the significance of the blockage). I had been planning to undergo another CCSVI treatment procedure sometime soon, because of the advances that have been made in the treatment protocol in the 13 months since my first attempt, but I've decided to put this off for at least a few months in order to properly ascertain whether or not the IVIG is having the desired effect. Doing a CCSVI treatment now would only muddy the waters, and the blood thinning regimen given after the procedure could interact with and/or interfere with the action of the IVIG.

I recently underwent a Doppler sonogram done to the Zamboni protocol, which did reveal signs of CCSVI (confusing, since my initial venoplasty did not reveal these abnormalities). This further blurs the issue, as it's become quite clear that I likely do not have MS, and CCSVI has been shown to have a high correlation with multiple sclerosis. However, recent studies also seem to indicate that the venous abnormalities known as CCSVI may also be prevalent in other neurologic diseases, so I very well could have both CCSVI and a disease other than multiple sclerosis. If the IVIG doesn't do it's stuff, I will definitely revisit CCSVI, very likely sooner rather than later. Even if I do experience benefit from IVIG, I'm inclined to undergo another venoplasty sometime in the not-too-distant future, to investigate the blood flow abnormalities indicated by the Doppler ultrasound. As I've written before in this space, waiting on getting a CCSVI venoplasty done is not a terrible option, as knowledge regarding CCSVI is constantly growing, and the treatments used to address it are evolving by the day.

As I'm sure you can imagine, this has all been extremely hard to sort out, but I'm now comfortable with my treatment decisions going forward. Despite the questions surrounding my diagnosis, I still consider myself an MS patient (since no other label really applies), and the experience of my disability progression is identical to that of a patient suffering from aggressive PPMS. In spite of the confusion and distress caused by all of this uncertainty, in a way not having a diagnosis gives me some reason for optimism, as it could turn out that whatever I have is more treatable than PPMS. I continue to hope that some brilliant doctor, upon examining me and going over my voluminous record of test results, will suddenly exclaim, "Eureka! You're suffering from the most advanced case of Cooties I've ever seen! Simply eat 10 red M&Ms a day for the next two weeks, and you'll soon be running marathons…"

Hey, if you don't have dreams, you have nightmares…