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...