Joe Kelley

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Hello, again, fellow ST’rs! Howard has again asked me to write an article for the newsmagazine updating my experience as a DBS surgery patient. For those interested in the first part of my saga, I refer you to the previous year’s April issue.

Many things have transpired since last April. Unfortunately, most of them not to my liking. As Howard mentioned in the last issue, there are to be seven articles in this month’s edition describing the DBS experience. Assuming mine is to be one of the seven, I am sure the other six stories will be inspiring portrayals of the positive and potentially life-altering aspects of this procedure. While I certainly don’t deny that DBS surgery has significantly improved my dystonia, DBS is not without its downside, a downside I rarely see discussed at length in any media presentation of DBS and dystonia. While I always remain hopeful, my intention is to present my experience as realistically as I can. What I think will emerge is a cautionary tale to those who have unrealistic or distorted impressions about what deep brain stimulation can do for dystonia. For the sake of brevity, I’ll focus in on two areas where I seemed to stumble the most: Expectations of surgery and commitment to the process.

To watch the typical television segment dealing with DBS and dystonia, it would seem quite reasonable to assume that, once the surgical apparatus comes off, the patient simply picks up his or her proverbial “bed” and goes home (with sincere apologies to the evangelist, Matthew). The results are often almost “miraculous,” and the media is certainly “in love” with miracle stories. A news teaser such as, “Medical procedure offers the promise of modest improvement for patients with dystonia!,” while perhaps being factually more accurate, certainly is not going to compel me to “tune in at 11!” What the reports universally fail to mention is that different types of dystonia respond differently to deep brain stimulation. The television segments almost always involve a person who is positive for the DYT1 gene mutation and has early-onset, generalized dystonia. Folks with this diagnosis typically do very well with DBS and can reasonably expect significant improvement. Those who are not DYT1 positive and whose dystonia, whether it be generalized or focal, is primary or idiopathic, have generally experienced moderate improvement in their dystonic symptoms. Finally, those with dystonia that is secondary to another condition (e.g., trauma, exposure to drugs) tend to respond less well to DBS, showing only modest gains. What is maddening is that there are exceptions to all of the above. I’ve heard of patients deemed as ideal candidates who have not responded to DBS at all. I’ve also heard of folks with secondary dystonia who have responded brilliantly. Since the doctors really don’t know how or why deep brain stimulation works in the first place, there is simply no way, beyond the general guidelines I’ve presented above (which have only recently emerged in the scientific literature), to predict how a person is going to respond to DBS. As they say in the commercials, “individual results may vary.” Needless to say, I think it’s vitally important to begin your DBS journey with realistic expectations. Discuss expectations at length with your neurologist and neurosurgeon to ensure you have an accurate picture of what is most likely to be your outcome.

My own struggle with unrealistic expectations came shortly after my first surgery in March of 2002. I say MY struggle because at no time did any of my caregivers foster any unrealistic beliefs. If anything, they “undersold” the potential benefits of the procedure. My difficulty was at least in part due to a “quirk” in the healing process known as the “micropallidotomy” effect. Any surgery, no matter how ultimately beneficial it may prove to be, is initially an assault upon the body. The assault to the brain by surgery causes the organ to swell. It seems that, in many patients, this swelling has the temporary ability to significantly suppress symptoms. The effect is temporary because, as the brain heals and the swelling goes down, symptoms, unfortunately, come back. After both of my surgeries, the “micropallidotomy” effect I experienced was profound. During those wonderful first few months after surgery, my symptoms were suppressed by 85 to 90%. Despite warnings that things “might get worse,” I found it very disheartening when, as the brain swelling went down, my symptoms returned. I mistakenly acquired the notion that correcting the problem was just a matter of finding the right settings for my DBS stimulators. Thus, I embarked on an almost three year search for the “magic settings” that would bring back that wonderful feeling of release.and relief. Despite frequent “reality checks” from my caregivers, I continued to persist in my mistaken notion that if we “twisted the dials” enough times, eventually we would find the synaptic “promised land.” While it is true that a skillful programmer can regain much of the benefit of the micropallidotomy effect, it may be an unrealistic burden to place upon yourself and your programmer to assume that he or she can “program” you back to that early, blissful state of relief. I have only recently accepted this fact. This state of acceptance has been the most significant step in my DBS journey to date. I would estimate my overall improvement in symptoms has stabilized in the 50 to 60% range. While this level of improvement has not allowed me to return to many of my pre-dystonia life roles (worker, musician, social butterfly, etc.), it has afforded me enough relief to regain the ability to enjoy each day. For that I am eternally grateful.

Another potential downside to DBS surgery is the commitment required on the part of both patients and caregivers. Unlike most surgeries where all you leave the hospital with is a cheap tooth brush and a little bottle of bad mouthwash, with DBS surgery you get the tooth brush, mouthwash…and several thousand dollars worth of delicate electronic hardware in your head and chest! It’s kinda like carrying around a Ferrari in your skull. And, like all finely tuned machines, your new “car” needs to be tuned and maintained to ensure top performance. This often means frequent trips to the “mechanic” which can be laborious and time-consuming. Also, like all machines, eventually parts wear out and have to be replaced. As I write this, I bear two brand new incisions over the new battery packs I just had “installed.” Sick of the car analogy yet? So am I. The bottom line is that you’ll likely be spending a lot of time in clinic waiting rooms reading four year old golf magazines (gosh, wonder where those came from?) and wondering if this is the visit you’ll actually be seen on time. It was a hard thing for me to accept but once you’ve had surgery, you are “married” to the hospital (or one like it) in which the procedure was done. Since DBS is something of a novelty, it’s likely that this new “bride/groom” is a large urban medical center. There were many times I wanted a divorce (I’m sure, at times, the feeling was mutual). Remember the old movie The Omen? Remember when that cute little spawn of Satan, Damien, made his first trip to church? He was not impressed. I must confess being able to relate to the poor little “demon seed” during several visits to the hospital. It is, however, one of the many things I’ve finally come to terms with. Again, if you are contemplating DBS surgery, this is an important consideration. Make sure your caregivers fully explain the level of commitment involved and be prepared to do some soul searching…am I up for this?

To say that the DBS journey has been an emotional roller coaster ride would be a gross understatement. I’ve been to the summit of almost complete relief to the lowest valley of despair. It’s been joyous and maddening; tremendously hopeful and unbelievably frustrating. Would I, if given the choice, do it again? Without hesitation, the answer is yes. The benefits have far outweighed the limitations. However, as significant an advancement as DBS surgery is in the management of dystonic symptoms, I cannot emphasize enough that it is most definitely an intervention of last resort. Any doctor worth his or her salt will exhaust ALL options before resulting to surgery. While the potential for significant benefit exist, there also exists the possibility for significant complication. Like any surgery, one must way the potential risk vs. benefits. Do your homework. Ask questions. Know, know, know before you decide. It’s likely one of the most important decisions you’ll ever make.

Joe Kelley

Deep Brain Stimulation Update

Hello again, fellow ST’rs! Howard has again asked me to jot down some of my musings regarding my life as a “bionic man” (everybody thinks they’re the first ones to tell me that joke)…aka as a Deep Brain Stimulation surgery patient. To give you a little background, I was diagnosed with pretty severe retrocollis, OMD (oromandibular dystonia), and a right arm intentional tremor back in August of 1999. After two and a half years of futility in terms of medical interventions (botox, oral meds, etc.), my neurologist suggested to me another option. He described a surgical procedure called Deep Brain Stimulation that had been used successfully for the treatment of Parkinson’s disease. The success surgeons were having in ameliorating Parkinson’s led them to explore more applications for DBS, in my case for dystonia. To make a long story short, my neurologist referred me to a neurosurgeon who agreed that it was at least worth a shot. I had my first electrode implanted in March of 2002. There was significant improvement, so much so in fact, that the neurologists were reluctant to do the other side of my brain. However, after much wailing and gnashing of teeth, the dystonia reared its ugly head again. We proceeded with a second surgery to implant an electrode in the other side of my brain in June of 2003. Again, the results were remarkable. I felt as though I had finally conquered dystonia.

In hindsight, I realize that June, 2003 was only the beginning of my DBS journey. After all of the dust had settled; after all of the brain swelling had receded; after all the wounds had healed, there began the arduous task of programming. For the first few months, it was a weekly or bi-weekly event. I was saving quarters at that time so as to have them at the ready to pay the tolls on my weekly journey up to Cleveland. I’d arrive at the hospital; we’d discuss any changes (positive or negative) in my symptoms, and re-adjust the stimulation parameters accordingly. I’d usually leave the office feeling some relief only to have it recede in days, sometimes hours. I was making progress, but it was two steps forward, one step back.

This went on for some months, perhaps a year. During this time, the hospital from which I was receiving care underwent an administrative and clinical restructuring. I received a new programmer and a new neurologist. Things were fine at first, but gradually, I began to notice that my phone calls to my programmer or doctor were either not being returned or were being returned by a secretary (gatekeeper) who invariably insisted that I “make an appointment.” I found this deterioration in communication unacceptable and sought the care of another team. I found that team in, of all places, Miami, Florida. It was a team of one…a force of nature going under the name of one Dr. Bruno Gallo. To say he is unconventional is an understatement. He was (and I’m sure still is) unconventional in the best way possible, however. He was responsive, caring, and above all…he listened. We were not at war with one another. I always felt we were equal partners in achieving one objective: improving my dystonic symptoms. He took me and my suggestions seriously. I correspondingly let down my defenses and remained much more open to his observations and recommendations. Guess what? I improved! In the intervening months, I made numerous trips to Florida for week long “intensive” programming sessions. I am forever grateful to Dr. Gallo. There are lots of “technicians” in medicine but sadly, not enough real doctors.

How am I today, November 8, 2007? I would have to say, “pretty darn good.” After three years of “relentless self-advocacy” (may be translated as being a pain in the butt) and finally finding a physician who would listen, I am now back in Cleveland under the care of an equally talented and caring team at University Hospitals of Cleveland. My new neurologist, Dr. David Riley, has been wonderfully open and responsive. My new programmer, Tina Whitney, has had little to do in terms of changing the settings. Dr. Gallo had me pretty much optimized. However, she did allow me the opportunity to suggest several changes to my programming, knowing full well I’d probably fall flat on my face. For that I am also forever grateful. There’s no better teacher than experience, and she allowed me to feel the effects of setting my stimulators outside of those parameters originally determined by Dr. Gallo. There is no doubt now…it’s as good as it’s going to get, and really knowing that brings me great comfort.

How good am I? Well, the tremor is, for the most part, history. My neck still pulls and my speech isn’t perfect but, all in all, I’d put my percentage of recovery at a consistent 75%. I’ve had my batteries replaced twice. This is not nearly as invasive as the original DBS. It’s more of an inconvenience than anything. I keep hearing rumors of “rechargeable” batteries, but I’ll believe it when I see it. Otherwise, my hardware has remained robust (no wires breaking, no accidental shut downs, etc.) and I hardly ever notice it anymore. I haven’t been to be programmed in close to a year. From a medical standpoint, other than a couple of prescription medications I am currently taking, my dystonia is no longer intruding on my life. From the standpoint of a person trying to make his way in the world, my dystonia is still something with which I contend. I had to abort an attempt at graduate school after I came to the sobering realization that I would not have the stamina to return to a 40 hour work week, let alone a stressful one. My symptoms can vary 5% points from that previously mentioned 75% recovery baseline…sometimes on an hourly basis. I have still not been able to play music and some days I feel that more acutely than others. I am not cured. I am, however, able to live a relatively normal and active life…something that was quite literally a fantasy before surgery. Like the rest of you, I hope and pray that someday in the not too distant future, a cure will be found for this soul stealing disease. However, in the meantime, DBS has afforded me the opportunity to remain relatively comfortable until that glorious time comes. I hope it comes soon for all of us…if for no other reason than to not have to hear that stupid “bionic man” joke ever again.

My mom and I wanted to thank you for hosting such a great symposium this year. This was our third year and we are looking forward to next years. E. Mathews