CCSVI: Some Words of Caution

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The subject of CCSVI (the vascular theory of MS) has proven to be incendiary, and has set the MS community ablaze. Initial studies into the hypothesis indicated substantial benefits could be gained by opening up the blocked veins in the neck and thorax of MS patients. These studies were soon backed up by an ever building wave of anecdotal patient reports of sometimes nearly miraculous improvements gained almost immediately after undergoing venoplasty, now known in the CCSVI universe as the Liberation Procedure.

In the wake of these reports, a tremendous amount of controversy has arisen, pitting MS societies and mainstream neurology against patients suddenly energized by hope, clamoring for access to a procedure they believe has a good chance to save them from the unrelenting ravages of the MS beast. Canada, which has one of the highest per capita MS rates in the world, has declined to even allow treatment studies of the liberation procedure to begin. Its single-payer health system refuses to recognize venoplasty as a potential treatment for MS, leaving Canadian MS patients with no options for treatment in their home country. The same situation holds true in most European countries. In the United States, the state of affairs is somewhat better, as an increasing number of physicians are offering the treatment to patients, although many health insurance companies won't cover it, and the cost of treatment is quite high. Additionally, waiting lists can extend for six months or more.

As a predictable result of this pent-up demand for treatment, a flourishing "medical tourism" industry has emerged around CCSVI, with clinics in Poland, Bulgaria, Costa Rica, Mexico, China, and India (and I'm sure I've forgotten a few) offering the procedure for a price, typically between $10,000-$20,000. It's been estimated that somewhere in the neighborhood of 2500 patients have visited these clinics, none of which have tracked the condition of their patients to any acceptable degree once the patients have departed for their home countries. Some of these patients have reported in at various sites on the Internet, but these patients probably represent less than 10% of the total patient population treated. Therefore, we have no good data on the effectiveness or safety of the treatments performed abroad.

The Liberation Procedure can take two forms: balloon angioplasty, in which tiny balloons are inserted into the veins and then expanded, thereby forcing open the veins, or stenting, a process involving the insertion of tiny mesh metal tubes into the veins, which when expanded prop the veins open. Often the two methods are used in conjunction, with patients receiving the balloon procedure in some veins, and stents in others. Both procedures carry the risk of clotting, although that risk is much amplified when stents are used. Because of this hazard, those who have undergone the Liberation Procedure are typically required to stay on a regimen of blood thinning anticoagulation medications for several weeks or months afterwards, necessitating the need for careful monitoring by qualified physicians to ensure the proper levels of medication are maintained. This aftercare can sometimes be hard to procure, as many physicians are reticent to treat patients for procedures that have been performed by foreign doctors and that they little understand. This problem has been especially prevalent in Canada, where the single payer health system has thus far refused to provide aftercare to patients that have gone overseas for "liberation".

In recent weeks, several troublesome (and in one case tragic) reports have begun to surface. Some patients returning from treatment in foreign clinics have experienced thrombosis (clotting) in their newly implanted stents, an extremely worrying condition that requires medical supervision (click here for article). In one truly horrifying episode, a young man who traveled to Costa Rica for treatment returned home to Canada, experienced thrombosis, and was turned away by local physicians when he sought their medical expertise. Ultimately, the patient returned to Costa Rica for treatment, and subsequently perished (click here for article). All of the patients in question had stents implanted in their jugular veins, which dramatically increases the chances of thrombosis when compared to balloon angioplasty, although that procedure too opens patients to potential problems with blood clots.

While the above incidents were transpiring, a conference on CCSVI was held at the annual ECTRIMS (European Committee for Treatment and Research in Multiple Sclerosis) in Gothenburg, Sweden. Many CCSVI research studies were presented, which are discussed in detail in the recently released article on Medscape.com (click here for article-you may be required to register at the site for access, a process which only takes a few minutes and is well worth the effort. Medscape is terrific resource for medical information). This article is quite long and offers an in-depth look at some very important research. It should be required reading for all patients interested in CCSVI.

The data presented at ECTRIMS was often in conflict, with some studies contradicting others, but the general consensus seems to be that while there is an identifiable correlation between CCSVI and MS, there is question as to whether CCSVI is the cause of MS. Rather, it may be a condition that is a result of the same disease process that causes the CNS damage seen in Multiple Sclerosis, or very possibly could contribute to the severity of the disease. It's quite possible that all of these scenarios may hold true, as MS differs so much from patient to patient that a variety of factors may result in its causation. In some patients CCSVI may play a major role in their MS, but in others it may play no role at all.

Given the above developments and wealth of new information, I feel compelled to offer the following words of caution. I know this message will not sit well with the most fervent CCSVI advocates, but I feel I would be remiss in not offering them.

While I am still a strong believer that CCSVI will prove to play a major role in unraveling the MS puzzle, I think that it is vital that patients use extreme discretion when choosing whether or not to undergo the Liberation Procedure, particularly if they must fly to far off destinations to procure treatment. According to one of the most experienced physicians performing the liberation procedure, Dr. Gary Siskin in Albany, New York, only about one third of patients treated receive dramatic improvements in their condition. Another third experienced minor benefit, and yet another third received no benefit at all. Furthermore, the rate of restenosis (veins closing back up) after balloon angioplasty is quite high, somewhere in the neighborhood of 50% within 12 months of treatment. These statistics alone should give patients some pause, as 66% of treated patients do not get the level of benefit they hoped for, and of those that do, 50% revert back to their previous condition, necessitating the need for additional procedures. This translates into 17% of patients who get liberated with the balloon method finding the lasting relief they sought.

The use of stents should be seriously questioned. In addition to the news reports above, Internet forums are revealing yet more patients suffering from stent thrombosis, and through this blog I've received numerous e-mails from other patients struggling with this problem. Stent thrombosis is only one of the potential hazards associated with the devices. The long-term failure rates of stents placed in the jugular veins is completely unknown. Most of the stents now being used were originally designed for use in thoracic arteries, where they are not subject to the nearly constant bending, twisting, and torque that they undergo when implanted in the extremely flexible human neck.

Data collected from two other patient populations that commonly receive venous stents (patients suffering from some cancers, and end-stage renal disease patients undergoing dialysis) is not especially encouraging. The failure rate of stents placed in dialysis patients has been found to be close to 50% after one year (click here for study). Although direct comparisons between disparate patient populations cannot be made, this data does provide reason for concern. Thus far, CCSVI patients treated with stents have not reported any instances of stent failure. However, the longest any of these patients have had stents implanted is only about 18 months, and the vast majority of patients fitted with stents have only received them in the last several months. I fervently hope that we do not start seeing a rash of stent failures in the months and years to come. The possibility can't be discounted, though, and only time will tell.

In conclusion, although CCSVI does hold tremendous hope for the future management of multiple sclerosis, we are presently in only the early infancy of investigation into the hypothesis and its relevance to the MS disease process, and of the practice of treating the condition with venoplasy. Beyond a doubt, future Liberation Procedures will bear little resemblance to those being done today, as new devices specifically designed for the task come on market, and the techniques and practices used to implement them are refined.

My heartfelt advice is that all those except the most desperate (and by that I mean patients with extremely aggressive disease who are quickly hurtling towards total disability) simply wait for 6 to 12 months before embarking on a quest for liberation. This will at least give some time for research to begin to catch up to patient expectations, and for physicians to better understand the best methods used to address the venous anomalies being found in MS patients.

I echo the warnings of virtually all of the most prominent physicians in the field, Dr. Zamboni included, that no patient resort to medical tourism in their quest for liberation. The risks of doing so are very real, particularly when the use of stents is involved. Balloon angioplasty is a much safer option, but the high incidence of restenosis means that many patients spending tens of thousands of dollars on treatment in foreign lands will find whatever gains they experienced lost, and will suffer not only from a return of their symptoms, but from broken hearts and broken bank accounts.

I fully understand the almost irresistible pull to get the disease taken care of NOW. I am close to being one of the desperate, if I'm not already there. This is why I chose to undergo a procedure this past March, which revealed a significant venous blockage but was unable to get it opened (mine is a very atypical case; the blockage is caused by a muscle outside of the vein pinching it almost closed). Hope is a powerful intoxicant, one that has been long absent for the vast majority of MSers. But we cannot and must not allow hope to eclipse reason. We all would like to see CCSVI advance as quickly as possible, but unfortunate events such as those recently reported will only provide fodder for those who would see the hypothesis relegated to the dustbin. Stay strong, friends, and act with extreme diligence.

Related articles

• Lack of MS treatment followup frustrates patient (cbc.ca)

Some Revised Thoughts on the Current State of CCSVI

Last week, I published a post on my thoughts regarding the current state of CCSVI research and treatment (click here). The post elicited much response, and many well thought out comments were left by readers who both agreed and disagreed with my points of view. As I've stated before, I welcome all readers to post their opinions, as a healthy exchange of ideas is the only way to draw informed conclusions, especially on subjects as new and controversial as CCSVI.

After reading and considering many of the responses arguing that research into whether or not venous blockages correlate with MS, and, if so, whether these abnormalities are cause or effect, were basically a waste of time and that treatment studies should take the forefront, I've changed my original point of view. While I still do think that academic research into the venous abnormalities we've come to call CCSVI is important, I now believe that treatment studies should be given the highest priority possible.

I've come to this conclusion for a variety of reasons. First, treatment studies themselves will show whether or not CCSVI abnormalities are indeed related to MS, and if they play a role in causing or exacerbating the disease. If, under rigorous trial conditions which include pre-and post-procedure neurologic evaluations, patients are found to respond favorably to the liberation procedure, then obviously CCSVI is not only related to MS, but plays an important role in the disease process. If patients fail to respond, then the opposite conclusions can be drawn.

Second, the noninvasive imaging techniques used to detect CCSVI have been shown to be less than reliable, and catheter venography has thus far proven to be the gold standard in finding blockages in the veins associated with the central nervous system. Based on the accounts of both the physicians who have performed the procedures, and patients themselves, Doppler sonography, MRV's, and CT venograms have all been shown to be prone to both false positives and false negatives when compared to the findings of catheter procedures, for a variety of reasons (technological limitations, operator deficiencies, etc.). Thus, any studies based strictly on noninvasive imaging will always have a cloud of doubt hovering over them. Better to cut to the chase, and perform catheter venography on test subjects. If that venography detects abnormalities, these abnormalities should be addressed by balloon angioplasty, which has been shown to be an extremely safe procedure when done by experienced physicians. Again, whether or not patients respond to angioplasty would answer questions of correlation and causation.

Third, if CCSVI does indeed prove to be a major piece of the MS puzzle, we need to refine the techniques and technology of the liberation procedure. Currently, many patients experience improvements soon after undergoing the procedure, only to see those improvements dissipate when their newly opened veins close up again. This phenomenon itself would suggest that CCSVI at least plays a role in the MS disease process, and also indicates that much learning needs to be done about the best practices, procedures, and equipment to be used when undertaking the liberation procedure. Virtually all of the medical equipment currently used in the treatment of CCSVI was developed primarily for use in arteries, which are vastly different in an anatomical sense to veins. Vein specific equipment, especially stents, needs to be developed if the liberation procedure does prove to be a major player in the treatment of Multiple Sclerosis.

Several other concerns have recently come to my attention. It seems to be becoming clearer that CCSVI is likely not just confined to the jugular and azygous veins, as has been thus far thought; the picture appears to be more complex. Dr. Zamboni has recently suggested that abnormalities in the lumbar veins could very well play a major role in progressive illness, and many venograms are finding abnormalities in the vertebral veins as well. The vertebrals are the vessels that return blood to the heart and lungs from the brain when patients are in an upright position. The jugulars take over this function when patients are lying flat. Thus, problems in the vertebrals could theoretically play just as important a role as jugular abnormalities. In my particular case, the catheter venogram done during my unsuccessful liberation procedure turned up blockages not only in my right internal jugular, but also abnormalities in my vertebral veins as well. Unfortunately, there is currently no accepted method for addressing stenosis in either the vertebral or lumbar vessels. Clearly, research into these concerns is necessary.

We must see collaboration between neurologists and vascular specialists if the myriad questions surrounding CCSVI are to be answered in any definitive fashion. I'm aware of several treatment studies that are currently awaiting approval, and of several doctors who are chomping on the bit waiting to get started conducting these trials. They'll have no problem finding patients willing to participate in these studies, but funding may be a dilemma. The MS patient population must get behind the studies, not only in body and spirit, but with their wallets too. When these studies are finally approved and announced, CCSVI advocates the world over, and those who love them, will need to step up to the plate and donate either directly to the institutions conducting the studies, or to nonprofit CCSVI advocacy groups that are currently readying to launch. Patient advocacy has pushed CCSVI out of the shadows and into the spotlight that currently shines on it, and that same advocacy will likely be necessary to take CCSVI from hypothesis to either positive or negative conclusion.

The CCSVI studies recently approved by the MS Societies of the United States and Canada will provide valuable data in assessing many of the questions that CCSVI theory has posed. I'm particularly intrigued by the study that will look at sets of twins in which one has has developed MS but the other has not. In fact, all of the approved studies will add greatly to the CCSVI knowledge base. But for a patient population desperate for answers, suffering with a disease in which time itself is an enemy, answering academic questions simply isn't enough. We must find out, as quickly and definitively as possible, whether or not the liberation procedure is as beneficial to some MS patients as anecdotal reports make it appear to be. Desperate times call for desperate measures, and for many MS patients, myself included, these are indeed desperate times...

Amazing Pre and Post CCSVI Liberation Procedure Videos (Updated)

The following videos were posted to YouTube by MS patient named Denise. I don't have much information about her, and I don't know where her procedure was done, or if balloon angioplasty or stents were used. Upon watching these videos, though, the results speak for themselves. If you are unfamiliar with CCSVI or the Liberation Procedure, please click here.

Here's the video taken a few days before the patient underwent the Liberation Procedure:

And here's the video taken less than 24 hours after her procedure:

Obviously, the improvements depicted are startling. I've been in touch with many patients who have undergone the procedure, both on the Internet and in person, and although many have reported improvements in their condition, this is one of the most dramatic cases I've seen/heard of.

It's hard to know what to make of these results. I don't think the patient in the videos above was "faking". Of course, I've no proof of that, but it would be a hell of an acting job if she was playing make-believe.

Naturally, it's thrilling to see such improvement, and I certainly wish that my procedure had gone as well (click here for info on my Liberation Procedure). From a scientific and medical standpoint, though, it's difficult to understand the mechanism behind the improvements depicted in the videos.

Certainly, less than 24 hours after the procedure, the reversal of nerve damage could not account for such a fast turnaround of symptoms. Nerve cells simply don't heal that quickly, and if damaged enough, they do not heal at all. I think it's safe to rule out nerve regeneration as being responsible for the improvements seen above.

The only other explanation I can come up with is that the opening of the patient's jugular vein had a remarkable anti-inflammatory effect on her CNS, which would mean that most of her problems were caused by inflammation, rather than actual nerve damage. Steroids are given to MS patients because of their powerful anti-inflammatory properties, and in some patients the beneficial action of steroids can be quite rapid, but not nearly as fast or as powerful as those that we see in the above videos. It generally takes cells at least a few days to recover from inflammation, so this solution too is problematic.

Dr. Sclafani, who did my procedure, reports that some of his other Liberation Procedure patients displayed surprising improvements soon after the procedure, but that in many cases these improvements were short-lived. Dr. Sclafani's sample size is small, though, so not much can really be read into them.

Whatever the reasons for the startling results seen in Denise's videos, I certainly hope she has lasting benefits, and continued improvement in her condition. As more and more research gets done on CCSVI, answers to the many questions surrounding the theory should start coming in.

Full-scale trials of the Liberation Procedure will be getting started this summer, and recently the government of Kuwait announced that all Kuwaiti MS patients will undergo the procedure, paid for by the state-funded medical system (click here for info). Kuwait has some 5000 MS patients, and it's expected that 10 patients per week will be getting "liberated".

Within several months, we should start getting some hard data regarding the effectiveness of treatment and the validity of CCSVI. For hundreds of thousands of MS patients, this data cannot come too soon...

IMPORTANT UPDATE: Wheelchair Kamikaze reader (and good friend) Bestadmom posted an alarming update on Denise's condition, which deteriorated about a week after her procedure. I'll include a brief summary of what happened here, and you can read a full account of the problems Denise encountered (in her own words) by clicking on the comments link, below, and reading Bestadmom's very informative post.

In a nutshell, Denise's condition reverted back to her "pre-liberation" days, with a sudden onset of her old symptoms. A return trip to the hospital where she had her liberation done showed that the stents that had originally been implanted during her procedure had restenosed and had to be removed (I was not previously aware that stents could be removed). A new set of stents was put in place (based on her account, it seems that four new stents in total were implanted), and the situation must have been dicey, since a neurosurgical team was put on standby.

The second procedure seems to have been pretty rough on Denise, but she is reporting that her symptoms do appear to be resolving once again.

Denise's experience is an enlightening example of both the promise and perils of the current CCSVI situation. Clearly, CCSVI offers the MS population real reason for hope, but the study of CCSVI, and its treatment, are still in their infancies. Techniques and materials need to be refined and developed specifically for the purpose of intervening in the array of venous blockages that have come to be known as CCSVI. This can only happen under the auspices of scientifically valid trials, whose progress can seem agonizingly slow for a patient suffering from progressive and debilitating neurologic disease.

Discretion is often the better part of valor, and it's very easy to allow hope to eclipse reason when one is staring at the dark at the end of the tunnel. Each of us needs to make their own determination of their tolerance for risk, but as more and more is learned about CCSVI, and its treatment, the risk profile associated with CCSVI should decline considerably. As previously stated, the next 6 to 12 months should be very telling.

One Week After Attempted CCSVI Liberation; Oh, What I'd Give to Be Boring...

Image by krazydad / jbum via Flickr

It seems I've spent my entire life being kind of unconventional, a little bit off-center, a person not wildly eccentric but filled with eccentricities. For the most part, my individuality has served me well, and over time I learned how to capitalize on my somewhat funky qualities to stand apart from the crowd, to separate myself from the writhing masses, and eventually to turn my quirks to my advantage.

Now, medically speaking at least, I find I'd like nothing more than to be a screaming bore, a real snooze, the dullest MS patient on the planet. Unfortunately, my tendency to be atypical has carried over to my disease presentation as well, and while it's garnered my case a lot of attention, that attention has not translated into medical solutions or relief from my illness.

From the get-go, my MS was labeled "atypical". As the years have gone by, more and more eyebrows have been raised by those considering my case, until finally this year the National Institutes of Health decided that I most likely don't have MS. Although my primary neurologist (a happily off-center guy himself) isn't as quick to dismiss MS as a likely culprit, he readily admits that I don't fit the profile of your typical PPMS patient. Unfortunately, the oddities of my case have not worked out to my advantage, as none of the traditional MS treatments have been able to lay a finger on my disease, and the myriad of unconventional remedies I've tried have been utterly useless.

So, I approached the radical CCSVI theory (click here to learn more about CCSVI) in a hopeful but skeptical manner. Even though I don't have classic MS, meaning the CCSVI theory very likely might not apply to me, a CT venogram done early last summer showed an area of stenosis very high up in my internal left jugular vein. Upon seeing this, the NIH opined that my disease may be primarily vascular in nature, but wouldn't commit to that judgment, at least not enough to warrant continued exploration of that possibility.

With no other options left to me, I investigated CCSVI and the idea of a vascular genesis to my illness with great interest, and after many months, with the help of a wily group of determined activist MS patients, found my way to Dr. Salvatore Sclafani, the Interventional Radiologist who performed the Liberation Procedure (a venogram and balloon angioplasty) on me last week. Knowing the peculiarities of my case, Dr. Sclafani and I really weren't expecting to find much in the way of vascular abnormalities during the procedure, but once again, I managed to defy expectations.

Upon injecting dye (via a catheter threaded through my vascular system) into my right internal jugular vein, Dr. Sclafani exclaimed, "Wow!", declared that my vein was significantly blocked, and that I did indeed have the condition now called CCSVI (Chronic Cerebrospinal Venous Insufficiency). Terrific, I thought, at long last a breakthrough!

Dr. Sclafani tried to open the blockage using balloon angioplasty. After four attempts, he had only limited success, the vein was only partially opened and most likely would close back down very soon. He also found some stenosis (narrowing) in my azygos vein, but because of its odd location was unable to fix that, either. Another venogram procedure, using different instruments, would likely be able to fix the azygos problem, but my primary problem appears to be my right jugular vein, which, when compared to the left, looks like something a very drunken Jackson Pollock might have painted on an extremely bad day.

So, the doctor and I are now left with the knowledge that I have some serious Central Nervous System blood flow issues, but are extremely limited in our options for rectifying them. The two most likely options would be traditional "cut the neck open" surgery to remove the malformed valve that is blocking my jugular, or to insert a stent into the vein to press that valve open.

Dr. Sclafani consulted about my case with the doctors and researchers in Italy who have been dealing with CCSVI for several years now, and they commented that they had never seen a valve like mine, that high up in the jugular. Once again, my individuality shines through. Hip hip freaking hooray...

The folks over in Italy suggested that whenever they'd encountered a similar jugular malformation, they'd recommend traditional surgery, or possibly a stent, although they generally try to avoid stents, which are exactly the same conclusions to which Dr. Sclafani had come. Dr. Sclafani talked about my case with some vascular surgeons, who didn't think "open neck" surgery would be a good choice, because of the distinct possibility of severe thrombosis (clotting issues).

This all finally leaves us with the stent option, which I am very uncomfortable with. Although several dozen CCSVI patients have been treated with stents, their use in jugular veins is somewhat controversial, for two primary reasons: stent migration, and eventual stent failure.

It's important to remember that the stents being used were all designed and approved for use in arteries, which are anatomically different from veins in extremely significant ways. A stent that gets loose in an artery generally gets pushed only deeper into the narrowing vessel, and doesn't inflict too much damage. A stent that becomes dislodged in a jugular vein, on the other hand, would inevitably find its way into the heart, with disastrous consequences.

My greater concern, though, is eventual stent failure. The stents now in use were almost all designed and approved to be placed in arteries located in the chest cavity, and these arteries are relatively rigid and not subject to much flexing. The stents now in use are constructed to withstand the tremendous pressures generated by the rhythmic pumping of the heart, not the torque, bending, and twisting they would be subject to when inserted into a vein in the incredibly flexible neck. Laboratory tests have shown that stents do indeed fail over time when subject to the kinds of stresses one might expect them to undergo when placed in very pliant jugular veins. The results of some studies suggest that stents placed under such stresses could likely fracture within 10 years. Needless to say, such a failure could be catastrophic.

Dr. Sclafani, ever the inventive guy, is trying to come up with some alternative solutions, and I'm confident that the good doctor will get it all figured out. In the meantime, though, the choice is between going for the stent, and the inherent dangers that entails, or waiting for the development of a unique procedure that would come with no guarantees, either. And all the while, the relentless drumbeat of my disease progression pounds inexorably onward.

Of course, I'll muster the forces and the gear up for the battles to come, but just this once, it sure would be nice to be scintillating dull...

Back Home, Safe and Sound

First of all I'd like to thank everybody who left comments and sent e-mails wishing me their best, offering their support and encouragement. I don't have the words to express how much I appreciate the comfort sent my way, and I am continually astounded that my silly little blog has touched the lives of so many.

I'm back home, safe and sound, after undergoing the CCSVI angioplasty procedure, which lasted longer than I expected, almost 5 1/2 hours, and discovered several abnormalities within my CNS vascular system. I'll detail these abnormalities in a later post, when my own understanding of what was found is more clear.

For now, I'll just say that the abnormalities that were discovered were not able to be fully treated at this time. Dr. Sclafani, who performed the procedure, is going to consult with a variety of experts to determine the best course of action to take moving forward.

As for the procedure itself, it really wasn't painful or even all that that unpleasant, just long and tiring. Not that I'd recommend it for a recreational activity, but the degree of anxiety I felt before the procedure was out of proportion to the actuality of the event.

Again, I'd just like to thank everybody for their wonderful show of support. I will post a detailed explanation of the findings after I consult further with my doctors, and can give an entirely accurate account. I expect I'll be able to do this within the next few days. There is surely enough misinformation on the Internet regarding CCSVI and the procedures used to address it without my adding to the confusion.

Again, thanks everybody...