Update on "MS as Vascular Disease"

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A few months ago, I published a post about a radical new theory regarding Multiple Sclerosis, called the "Cerebrospinal Venous Insufficiency" theory, or CCSVI for short. I thought that now might be a good time to provide an update.

Just to review, several years ago researchers in Italy started looking at the vascular systems of MS patients, specifically by imaging the veins that are directly related to the central nervous system (the jugular and azygous veins). Incredibly, they found that in nearly all of the patients they studied there were blockages or abnormal narrowing of these very important vascular pathways. The researchers theorized that the vascular blockages they were finding could cause a reflux of blood back into the central nervous system, which in turn could lead to inflammation and edema, and eventually to a breakdown in the blood brain barrier, and thus be responsible for the damage seen in Multiple Sclerosis. About two years ago, the doctor leading this research, Dr. Paolo Zamboni, started performing modified balloon angioplasties on these areas of abnormality.

An interventional radiologist at Stanford University, Dr. Michael Dake, became interested in this research after it was brought to his attention by the activist wife of an MS patient. Dr. Dake traveled to Europe to meet Dr. Zamboni, and upon his return started doing some investigations of his own. He too found serious vascular abnormalities in nearly all of the MS patients he imaged. Based on these imaging studies, approximately 4 months ago Dr. Dake started performing endovascular surgery on these patients, placing stents in blocked veins to open up the areas of abnormality that he found.

To date, Dr. Dake has performed surgery on about 35 patients, most of whom have reported positive results as a result of the surgery. Although most of the patients who have undergone the procedure were not severely disabled due to MS, they all suffered from the usual array of MS symptoms, including cognitive dysfunction, heat intolerance, and severe fatigue. Many also suffered from varying degrees of motor and sensory dysfunction. Almost all have reported marked improvements in these areas.

Tragically, one of the patients who underwent the procedure died of a massive cerebral hemorrhage soon after, but the consensus is that the patient’s stroke was not directly related to the endovascular surgery that was performed. The blood thinning medication that was given postsurgery did contribute to the severity of this terrible event, but was not deemed to be a causative factor.

Dr. Zamboni recently published a summary outlining the results he's seen on the patients who were treated with his endovascular procedure, which he calls the "Liberation Procedure". In the 51 RRMS patients treated with the procedure, Dr. Zamboni claims a fourfold decrease in relapses. Furthermore, Dr. Zamboni performed the procedure on 18 patients who were suffering from acute relapses, and after surgery these patients recovered from their relapses in time periods ranging from four hours to four days. Dr. Zamboni cites this as "the best evidence that venous obstructions play a causative role in the complex pathogenesis of Multiple Sclerosis".

Dr. Zamboni and his team have organized a conference on "Venous Function and Multiple Sclerosis", to be held in Bologna, Italy on September 8 of this year. Several prominent US neurologists specializing in MS are expected to participate.

It's important to note that Dr. Zamboni's surgical results have been self-reported, and not subject to peer review. Additionally, no MRI imaging studies were done on his patients after they underwent the "Liberation Procedure", which would have been very valuable in assessing the impact the procedure had on the central nervous system lesions of those who underwent it.

Dr. Dake is planning on starting his own clinical study in September, 2009, which is expected to be limited to RRMS patients.

All of this information is extremely fascinating, and may offer a brand-new approach to understanding and treating Multiple Sclerosis. It's very important to keep in mind, though, that nothing has yet been proven, and that all of the results so far reported, as positive as they've been, have only come from two sources (Dr. Zamboni, and Dr. Marian Simka, who has done research on CCSVI in Poland). Scientific discipline requires that these results be replicated by independent researchers before anything definitive can be stated about this theory, and I expect that several groups will be attempting to do just that in the near future.

I personally find this research to be quite exciting, although I am maintaining a healthy skepticism regarding it. In my 6+ years as an MS patient, I've seen many such "theories du jour" generate tremendous enthusiasm, only to be disproven by the greater scientific community. In this case, though, my gut tells me that there might be something to this. I'm quite sure that this theory can't explain all cases of MS, but it does seem compelling enough to suggest that, at the very least, there is a subset of patients for whom the root cause of MS may not be neurologic or autoimmune, but vascular.

Research into the causes and treatments of Multiple Sclerosis has fallen almost exclusively into the realm of the neurology and neuroimmunology communities for the last 30 or so years. Though some impressive advances have been made, the disease has proven to be quite confounding when looked at from these perspectives. CCSVI offers a fresh approach at understanding the disease, and furthermore, offers the hope of some straightforward methods of treating it.

This research may turn out to be nothing but a flash in the pan, but there is the chance that it could revolutionize our understanding of Multiple Sclerosis. For this reason, I recommend that all MS patients pay close attention as the CCSVI story unfolds...

Update 11/21/09: Canadian television has done in a news report on CCSVI. Click here for the link.

Update 11/30/09: CCSVI: Separating Fact from Fiction. Click here.

Warning: Blind Curves Ahead

Receiving a diagnosis of MS, or any serious illness, is a reality shattering event. Despite the initial wave of fear, confusion, and consternation, there is one thing the receiver of such news knows innately; life as they know it will very likely never again be the same. Whether the disease eventually takes a mild course, leaving a person relatively intact for an extended length of time, or a more aggressive path, as in my case, landing its victim in a wheelchair within five years, life's future trajectory has been fundamentally altered. If not by the ravages of the disease itself, a patient's reality will be warped by the sudden realization of their absolute vulnerability.

How to deal with this sudden detour is up to the individual, and each person's reactions and coping mechanisms are as different as the ways in which MS can present itself. Some patients go into complete denial, and attempt to live life as if nothing has changed. Others shut down completely, unable to wrap their minds around their new, unfortunate, reality. Reading books, talking to doctors, and chatting on the Internet can only go so far. Ultimately, each individual must make his own peace with the disease. Truthfully, I'm not sure that peace is even the right word. Truce, maybe? Each person must come to a truce with their new reality, must reach an armistice with their illness.

Thing is, even without a diagnosis of MS, no person knows what the future holds. We live our lives with blinders on, blithely going about our routines as if those routines were written in stone, stable and secure and steadfast. Almost inevitably, something comes along to reveal that the underpinnings of our existence are not constructed of granite, but of gossamer. The stability of everyday life is an illusion for us all, healthy or not, and can be shattered in an instant. The loss of a job, a betrayal, an illness or accident, or even a simple lapse in judgment can forever consign all that was previously taken for granted to the scrap heap of what used to be.

When I was first diagnosed, I was working at one of the major TV/video/music production facilities here in New York City. The building was filled with folks who were among the world's best at their highly competitive jobs. Two of the people I was most friendly with there were named David and Bob. David was a flat-out genius, probably the smartest person I've ever met, who was paid a very nice living for simply being David. He was an innovator who helped bring about the digital age of recorded music, and was so well thought of that he was the go to guy when the industry's other big brains had problems that they couldn't solve. Bob was a gentle giant of a man, and as chief engineer of one of the world's foremost production facilities, he was no slouch in the brains department either. In an industry filled with tremendous egos, Bob was humble and grounded, an island of stability in an environment that could often turn into a maelstrom of emotional histrionics.

Upon hearing of my diagnosis, both of my friends were visibly saddened, and through the fusillade of "sorry to hear thats" that came at me from every direction, their compassion was palpable, and comforting. They genuinely felt sorry for me, but I knew from my own experience in dealing with other people's bad news that there was an element of "thank God it isn't me" mixed in with their compassion. It's only natural, as hearing of someone else's misfortune serves as an unwelcome reminder of our own vulnerability, a reminder that we quickly cast out like a container of soured milk. In the reality show of life, even the best of us feels thankful that it wasn't me who was voted off the program.

About three years after I was diagnosed, my friend David suffered a massive coronary after having dinner with his elderly mother. He simply got up from the dinner table, fell over, and that was that. Soon after, Bob was hit with a mysterious illness, and he wound up going on disability a month or two before I did. Through numerous phone conversations, we helped each other wind our way through the labyrinthine process and paperwork of applying for Social Security and long-term disability. Tragically, Bob's mysterious illness turned out to be a rare form of leukemia, and he passed away a little over a year ago. Even now, writing that sentence brings with it a sting of grief.

The point of all of this gloom and doom is that nobody knows what's coming. Life is a road made up of nothing but blind curves. Healthy or not, we must all constantly try to make the most of the present moment. The past is gone for good, and the future is unknowable. All we have is now, and even if this now is not what you once imagined it would be, it is still your most precious possession, because you will never have another one.

Those of us who have suffered loss after loss because of multiple sclerosis must concentrate on that which we still have, and not lose ourselves in all that's been taken from us. I can't use my right arm and leg , but I can use my left. I can choose to grimly lament the loss of what had been my dominant side , or I can try to do the best I can with the parts of me that still work. It's all a choice, and the power and grandeur of the human mind is that it can create its own reality. Being stuck with a miserable disease does not relegate one to being miserable. Without making an effort, the misery of MS is always there to sink down into. Avoiding it takes conscious action, the mental discipline to mindfully take control of your own emotional well-being.

This is not to suggest that one walk around brainlessly smiling like an overly medicated asylum inmate. A large part of the ability to cope with a disease like MS is acknowledging how much having the disease sucks, and then deciding that you will find a measure of contentment in spite of it. It's an effort that must be exerted each and every moment of each and every day, but as with all exercises, with time it becomes easier, and habitual.

My friends David and Bob both felt sorry for me, never expecting that within a handful of years I'd be the last of the three of us to still grace the planet. My life is farther from perfect than I ever expected it could be, but I still have life, and enough ability left to overcome my disabilities. This might not always be the case, but right now, at this moment, it is. Tomorrow, or next week, or next year? Who knows? I can try to plan for the future, but I can only live it when it becomes the now.

Despite multiple sclerosis, we are in the very same boat as our healthy friends and family, trying mightily to navigate uncharted waters with an imaginary map. Rest assured that there are rocks and icebergs and maybe even sea serpents ahead, but the only damage they can truly do to your essence is that which you let them.

Info on Wheelchairs

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Many people have asked me for information about wheelchairs, figuring that since I call myself the Wheelchair Kamikaze, I must know something. Truth is, the only wheelchair I really know much about is mine, a Quantum Q6000Z, and really, I mostly just know how to drive that chair like a lunatic.

The best resource I've found on the Internet for reliable information and opinions on all things wheelchair is the Wheelchair Junkie website, which is run by Mark E. Smith, one of the higher-ups at Pride Mobility, who is a power wheelchair user himself. Although he’s an employee of Pride, his site contains useful information that pertains to wheelchairs of any make and model. One of the most valuable features of the site is the message board, which is populated with lots of very friendly and very knowledgeable folks who are more than willing to offer their opinions and expertise to those seeking knowledge and camaraderie. The "Ask Mark" forum allows message board members to ask questions directly to Mark E. Smith, who knows just about everything about wheelchairs that there is to know.

Now, if you want to ask me about, say, what it's like getting into a head-on collision at full speed with a parking meter while driving a wheelchair, go right ahead. For all other inquiries, though, I recommend checking out Wheelchair Junkie.

Pleased to Meet Me

Image by melolou via Flickr

MS has wrought an almost incalculable number of extreme changes on my life. Physically, the changes are obvious. Right arm and right leg on the fritz, and I quite often have a wheelchair sprouting from my backside. Changes like that are hard to miss. More difficult to discern are the internal transformations, the changes in mind and spirit.

I'm quite lucky in that MS has not dramatically damaged my cognitive abilities. Many MS patients suffer terribly from the deterioration of their memory and their ability to think. So far, at least, my faculties seem to be relatively intact. My short-term memory isn't what it used to be, but that may be more a function of age than disease. If anything, dealing with multiple sclerosis may have actually heightened my senses of thoughtful perception, and has certainly enlarged my capacity to feel empathy for all of those who struggle to simply make it through the day, sick or not.

I've lately come to realize that MS has not only altered my perception of the world around me, but also of the world within me. It's changed the way I think about myself, in some unexpected ways.

When healthy, I suffered from the common delusion of believing in the limitless possibilities of the future. Although my rational mind understood that my youthful dreams of fame and fortune were not likely to be fulfilled, there was still a part of me that half expected some huge stroke of good luck to dramatically alter the course of my life, to suddenly elevate me into the stratosphere of society. Surely, there was still the chance that I might find myself sitting next to Jay Leno, chatting about my recent Oscar triumph. Nevermind that I hadn't acted in anything since my sixth grade production of "The Sound of Music", and I was much more likely to be directing traffic than a film anytime soon. In America, anything is possible.

Well, MS pretty much doused those flames. These days, my fantasies have less to do with winning Oscars than with taking a stroll around the block, though the chances of either are probably equally astronomical. Still, I find myself dealing with the world in a much more rational way. Rather than feeling resentful that my grand imaginary life was being thwarted by the realities of my everyday existence, I now find myself grateful for the simpler pleasures; lunch with a close friend, a nice day for taking photos, or even just a few hours when the pain in my hips mysteriously subsides. Here's a universal truth, brought to me courtesy of Multiple Sclerosis: The biggest blessing on earth is a quiet night at home spent with people that you love.

MS has stripped away the many trappings of life that had become central to my self identity. High profile job in a "glamour" industry? Gone with the wind... Sexy little sports car? Couldn't even get into one these days... Fashionable clothes and fancy shoes? Ha! Putting on my socks is now a painful exercise in acrobatics, and I could just as easily use buttons and shoelaces as I could split the atom... All of those externals that once so dominated my definition of self are now mere memories, and in their place I've gradually come to know a different me, a me that resembles one that I knew a long time ago, back when I was a child unencumbered by the accouterments of adulthood.

Despite the complexities of being disabled, life in some ways is now a much more simple affair than it was when I was healthy. Absent of the concerns of career and social climbing, I find myself free to pursue my whims and desires in a kind of newfound innocence. No longer confined by the boundaries of the workaday world, I can be as eccentric as I want to be.

I've always felt like something of a living anachronism, a man born in the wrong time. Well, if I want to spend my days in 1935, now there's nothing to stop me. So here I sit, listening to The Mills Brothers or The Ink Spots, and I never leave the house without wearing a fedora, preferably at a rakish angle. After all, what sets off a wheelchair in the summer better than a nice Panama hat? I can watch baseball to my hearts content, unencumbered by worries about that big project that is due, or tomorrow's budget meetings. I can spend my days making videos, taking photos, and writing, a situation that I literally used to dream of. Naturally, those dreams never included a wheelchair mounted camera, or writing about my experiences dealing with a dread disease, but, as my father used to tell me, if you want to dance you've got to pay the band.

Of course, there are eternal worries about my illness, but somehow, these are different than the ever-changing concerns I had during my healthy life. These new anxieties are immutable, unbending, and worn like a second skin. Unlike most of the problems I encountered before MS, there is really nothing tangible I can do about my illness, so the all-pervading anxiety it produces, while wearisome, doesn't usually overwhelm the mind. I do my due diligence, fastidiously keeping up with all the latest research, and aggressively pursuing my medical options, but beyond that, there is very little control I can possibly have over what MS is doing to my body on a day to day basis. As hard as it was to come to terms with that reality, the only thing left is to let it be.

I've found this new me to be much more honest with myself, much less likely to put up with dishonesty in others (especially if they're being dishonest with themselves), and completely disinclined to be convinced to do things out of social obligation. I've learned that saying no is not an act of selfishness when it's an act of self-preservation. Often times I simply don't feel well enough to live up to the expectations that some might have of me. I'm sorry to disappoint them, but if catering to others means that I'll spend the next three days in bed, it's just not going to happen. With the love and support of my very caring (and very indulgent) wife, I'm free to pursue interests and inclinations that had long lain dormant simply because life as a working adult had left no time for them.

Make no mistake about it, having MS sucks in every way it possibly can suck, and I will never be one of those patients who claims that "I have MS, but MS doesn't have me". MS most certainly does have me, by the balls (sorry, ladies). But, in a sense, MS has given me the freedom that most human beings lose upon entering grade school. The price for that freedom has been dear, and I would never have willingly paid it, but freedom, whatever the cost, bears with it an inherent sweetness. I've learned that it's okay to savor some of that sweetness, despite the horror and dismay of having progressive MS. The disease has allowed me the opportunity to rediscover myself, and, I must say, it's been an unexpected pleasure to meet me...

Great Webcast: Understanding Primary Progressive MS

Primary Progressive MS (PPMS) is an often poorly understood disease, even by those suffering from it. PPMS afflicts only about 10 to 15% of the MS population, and differs significantly from other forms of the disease. In visiting many of the Internet MS forums, I've found that it's quite common to see much confusion and misinformation about this form of the disease being passed from patient to patient, including such fallacies as "most people with PPMS are dead within five years of diagnosis", "PPMS inevitably leaves its victims bedbound vegetables", and "PPMS leads to the compulsion to drive wheelchairs crazy fast down crowded city streets". Okay, I made that last one up, but there really is a lot of confusion about PPMS.

The National Multiple Sclerosis Society has just released a series of three videos covering various aspects of PPMS, and I found them to be surprisingly honest and informative. Most importantly, the videos dispense with many of the old notions about the disease, and touch on some of the latest developments in the research and understanding of this very challenging form of MS. In my opinion, these videos are so useful that they should be viewed by anybody dealing with any form of MS, simply because they contain valuable information that crosses the boundaries of disease type.

The first video, "Understanding Primary Progressive MS", talks about the substantial differences between PPMS and the other forms of the disease (RRMS, and SPMS, and PRMS), and discusses some of the challenges involved with correctly diagnosing PPMS. This video features Dr. John Richert who is the Ex. VP, of Research & Clinical Programs at the NMSS, being interviewed by a woman who looks very much like somebody I dated about 14 years ago. I wonder whatever happened to Jill?

The second video, "Strategies, Research, and Hope for Primary Progressive MS", is to me the most valuable of the three videos. In it, Dr. Richert clearly makes the point that an "autoimmune" response is not the primary driver of the PPMS disease process, and thus none of the current crop of immunosuppressive or immuno modulating drugs are useful in treating it. He also states that this is also true for SPMS, and that there is more going on in the RRMS disease process than was previously thought, as well.

Hallelujah! I've been saying for years now that a misguided immune system is only a symptom of MS, and not the cause of the disease, and that focusing on suppressing or modulating the immune system is like treating a broken leg with painkillers. This disease damages far more than just the myelin coating of nerve cells, and it's great to know that research time and money is finally being spent on trying to comprehend the underlying cause of the disease, as well as searching for strategies to protect and repair damaged nerve tissue. This is a tremendous leap forward in the understanding of the Multiple Sclerosis disease process, and pertains not only to PPMS, but to all of the other forms of the disease as well. In my opinion, this video is must viewing for everyone afflicted with any form of MS.

The third video, "Moving Forward with PPMS", talks about the emotional and psychological impact of dealing with a progressive degenerative illness. It features Rosalind Kalb, PhD who is the Director of the Professional Resource Center at the NMSS, being interviewed by a very somber man who looks like somebody just defecated in his lap. Cheer up, dude, things can't be that bad...

At What Price Health?

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

Great, right? Sign me up!

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

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

How many months or years of restored health would be enough to entice you to undergo a simple but profoundly effective treatment that carried with it the ultimate price? Of course, the answer must differ for each of us, based on our own current state of disability, our rate of progression, the level of our misery, and the amount of hope we have that a cure, or even a truly effective treatment, can be found in time to help us.

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

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

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

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