A Whole New Way To Be Wired

My decision to have deep brain stimulation (DBS) surgery was not easy, but there's nothing easy about any of the elements of this disease for the person with Parkinson's (PWP) or their care partners, families, and friends. There are always tough decisions, trade-offs, and hard choices between unpleasant options.

For me, there were two facts to consider that confused the issue: the fact that I'm still very young (48 is young, isn't it?), and the fact that I have been diagnosed for such a short period of time; it's only been about two years since my initial diagnosis. Those two facts come up repeatedly in conversations about my decision to have the surgery. “REALLY? You're having brain surgery? On purpose? You look fine to me–why would you want to do that?”

The last comment is one that most PWPs must repeatedly address, even with family members or close friends. On some days, we do look and feel better than on others, and I think we're all grateful for those days. I'm still new enough at this that responding to those well–meaning comments is difficult. It doesn't seem right to say, “thanks very much for saying that; however, let's not forget that I'm not better, I'm not likely to get better, and tomorrow I'm going to look like hell again.” There's a very fine line between reminding people that this disease is variable, capricious, and still here, and just accepting a nice compliment and the good wishes and concerns that lie behind it at face value. It reminds me of the Saturday Night Live skits that featured Debbie Downer–she never had anything good to say. It tends to stifle the conversation when you feel the need to say, “Yes, it is an absolutely beautiful day, and I've never seen the sky so blue and the sun so bright. I have Parkinson's disease, you know.”

So, those two facts, coupled with the fact that sometimes I appear to be relatively unaffected by the disease, plagued me as I weighed the pros and cons, sifted through the mountains of available information, and talked to dozens of patients, doctors, experts of all types, family members, and friends. I am very much on the young side for having DBS surgery–as a matter of fact, one of my neurosurgeon's nursing staff happened to mention that I was the youngest person she had ever seen to have the surgery. In addition, I've only been diagnosed for a short time; many people who have had this disease for years are managing their symptoms and treatments well, and have no interest in or need for DBS.

I've mentioned my initial belief that DBS is a choice of last resort; when everything else stops working, you have the surgery. This perception is fundamentally wrong, and part of my decision-making process was to understand that. My neurosurgeon explained that DBS is for people who still benefit from medical treatments, but with unacceptable side effects. “Unacceptable” is different for everyone, and there's no checklist-oriented cut-and-dried approach to deciding when it's time. He told me I would know, and he was right–I do.

Although I have problems with dyskinesia, the sleep disturbances are the main factor driving me toward DBS. It's true that I arrived at this point relatively quickly; much more quickly than most DBS candidates do. However, as I've started to say more and more often, “it is what it is.” The plan is that DBS will eventually help me to reduce the amount of medication that I'm taking, which will reduce some of the more significant side effects, which in turn will help me sleep (and not just in the car driving to work in the morning).

The process is now in motion, and I'm past the first milestone. It was somewhat anticlimactic–a full anesthesia MRI (the last that I'll ever have–MRI machines and metallic objects embedded in your brain and chest apparently don't go together). I wish I could tell you more about it, but all I saw was happy smiling faces in the MRI room, the IV in my arm, and a chemically-induced black curtain that came down momentarily and then came up again in the recovery room, with the nurse asking me, “well, welcome back. How are you feeling?” All I could think of to say was, "I think I was abducted by aliens for a moment."

I experienced no ill effects and no post-anesthesia nausea (hard to believe coming from me, I know; once again, go figure). Best of all, they collected good data–that's a real compliment coming from an engineer. It's particularly important to me, because the map of my brain that the neurosurgeon obtained using this procedure will allow him to plan a very precise implantation path for the electrodes in the DBS system, avoiding some of my favorite parts of my brain and finishing in EXACTLY the right place in my subthalamic nucleus. I didn't even know I had a subthalamic nucleus until recently.

According to my neurosurgeon, DBS is really not a very difficult procedure. In his view, the key to success is to perform the procedure exactly the same way every time, with the same equipment and the same processes, in the same order, with the same team, carefully and without fanfare. He doesn't want to get famous, or to write a paper on a new process, or to try new equipment - he just wants every procedure he does to be done as well as he and his team can do it, with predictably excellent results. Coupled with his experience level (over 1000 successful implantations) this approach made my choice of surgeons easy.

Next up in my process is something called a neuropsychological exam. Some people are better candidates for DBS surgery than others, and one of the major contraindications for DBS is signs of dementia. The neuropsych exam will look for those signs, and also will give me a baseline of cognitive function, memory, coordination, and several other things. With this information in hand, we can compare the before with the after and see which symptoms improved and which did not.

The surgery is scheduled for June 9. It's a moderately lengthy procedure, perhaps 3 to 4 hours long, and I'll actually be awake for a significant portion of it. I've always heard the saying that the brain itself has no pain receptors; I think that's a fascinating concept. What concerns me more is that they have to get to the brain first–past experience with wildly pitched baseballs, tree branches, cave ceilings, and open kitchen cabinets makes me quite confident that my scalp and skull enjoy no such freedom from pain. I'm sure that the surgical team will have thought of that.

Here's what's going to happen, as I understand it. I'll arrive at the hospital bright and early on the 9th, and I'll receive a new look that I haven't had since I was a baby–I will have my head shaved. I can't recall ever having seen my own scalp before, except for brief periods in the Air Force when I was overenthusiastic at the barber shop. The surgical team will then install something called a “frame" - a metal ring that's actually screwed into the bone of my skull that will serve to keep my head completely still during the procedure. They'll then perform a CT scan to validate my alien-abduction MRI, as a double check that I haven't done something to change my brain since then, and to verify that the surgical plan is still correct.

I'll then proceed to the operating room, where the team will prepare me (liberal local anesthesia please), and then do those things that are needed to provide access to my brain (I'm being circumspect here so none of us passes out reading this, including me). At this point, all of the careful preparation and exact planning becomes very important–they'll implant two electrical leads in my brain, following a predetermined path and ending at a predetermined termination point. I'll be awake during this process, perhaps so I can provide color commentary and tell jokes. Feel free to send me jokes that are appropriate for this particular situation–I got nothin'.

To make sure that the electrodes are in the right place, the surgical team will measure electrical signals at various places, and do some stimulation to test the response. Once they determine that all is well and everything is where it should be, I get to go to sleep. I'm really looking forward to that part of this procedure.

When I wake up, they will have anchored the electrodes to plastic plugs in my skull connected to long electrical leads. Those leads will have been placed under the skin of my scalp, neck, and upper chest, and will terminate in a new battery pack and stimulation computer that will be implanted under the skin of my upper chest below my collar bone. The system is entirely internally contained - when my hair grows back and the incisions heal, the most you will be able to see is a slight bulge in my upper chest. I'll spend perhaps two weeks or a little more recovering from the procedure, and then after 6 to 8 weeks my neurologist will begin to program the device. When we get the programming right, I'll start experiencing the beneficial effects.

As someone who works with engineering and science, and as a self-proclaimed "geeky guy," this procedure fascinates me. I am amazed at how much DBS has improved in the 10 years since approval by the FDA for Parkinson's disease, and I'm optimistic that it's only going to get better. One of the significant benefits is that the system can be upgraded, and can even be removed if necessary.

My colleagues think this is a significant opportunity to push the boundaries of science and experiment a little with the man-machine interface. One of the types of work we do is to look at how complex technical systems can be made to do things their designers didn't intend (we don't use the term "hacker" - it's just rude, and inaccurate as well). We take our work seriously, and I have the sense some of my team may take it too seriously. I've learned to smile and nod at the comments about the "Six Million Dollar Man," questions like "so, will you be Borg after this? Will we be assimilated?" and even my new nickname: "One of One" (a play on "Seven of Nine," a half-robot/half-human character from one of the Star Trek series' - I think I mentioned we're all geeks, some more than others). However, I draw the line at questions like, "what did you say the control system signal looked like? Do you know anything about the effective range of control? What kind of processor is in the stimulation system? Do you mind dropping by the lab for a few minutes when the system is installed? We'd like to test a theory." Engineers are just hilarious, in an incomprehensible kind of way.

If you're interested in more information about deep brain stimulation, a good place to start is the Medtronic website at:

http://www.medtronic.com/our-therapies/neurostimulators-movement-disorders/index.htm

Medtronic manufactures the DBS system that I will receive, and they provide a comprehensive description of how it's implanted and how it works. The Mayo Clinic website is also an excellent resource at http://www.mayoclinic.org/deep-brain-stimulation/. Finally, the AAPSG website at http://www.aapsg.org includes a list of links to a variety of information sources. Those links also have information about deep brain stimulation.

On a different note, I'd like to remind everyone of the 5K/1K Walk/Run for the Cure for Parkinson's taking place on May 14, 2011 in McAllister Park, San Antonio, Texas. Proceeds from this fundraiser will benefit Parkinson's research and the search for a cure. I'd love to see you all there, but if you can't be there in person, you can still have an influence by donating to the cause. Go to http://www.aapsg.org and click on the “Donate Now!" link on the bottom left.

Next time I'll describe the neuropsych exam, and talk about the changes and challenges that those who are "wired" have to face. I welcome your feedback, either publicly as a comment to the blog or privately to my email address at seeNOSPAMking@aapsg.org. Replace the first word with the right letter, get rid of the spammy stuff, and there you have it. Bad guys everywhere - you gotta fight back :-).

Next Steps

My long journey to a Parkinson's disease diagnosis was confusing and frustrating, but there was some comfort in having an answer. After years of wondering what was happening to me, I finally had a label and could develop a plan of attack. I was briefly relieved, until reality began to set in.

Dr. Elisabeth Kubler-Ross describes grieving as a five-stage process: denial, anger, bargaining, depression, and acceptance. I've been in all five of those places, sometimes all on the same day. It's not a steady progression, and I'm certainly not finished yet - I've really only just begun. Although Parkinson's disease tends to shorten expected lifespan slightly, it's not a fatal disease - many people with Parkinson's live long lives after diagnosis, with slow progression and years of good function. Unfortunately, although the speed of progression and the details are different for everyone, the direction and result is always the same.

With what I now know about this disease, I am amazed and awed by PWPs and their care partners, families, and friends who have been fighting back for decades against this slow thief of health and vitality. Parkinson's steals not only the ability to move and communicate effectively, but it also damages self-esteem, destroys relationships, halts promising careers in mid-stride, and sentences both PWPs and their ecosystems to a seemingly eternal cycle of medication, daily struggles with the basic activities of living, and a slow decline in spite of heroic efforts. A friend and fellow PWP once told me that Parkinson's is like, "being stuck on the railroad tracks in front of a slow-moving train - you know it's coming, but even though it takes years to get there, you can't get out of the way and you can't make it stop."

Since my diagnosis, I've worked to get the medications right and fought my way through debilitating side-effects just as every other PWP has at some point. I've lost friends I've known for years who just stopped responding to attempts at communication, and I've made many new friends in many places, including our AAPSG support groups (if you want to meet some really fascinating people who don't quit and who usually have a joke or story to tell, come to a support group. Sometimes the jokes are agonizingly bad, but we're all forgiving people). My family and I have also worked (and are still working) through the fear, uncertainty, and anxiety that comes with a diagnosis of a progressive, incurable illness. How long will I be able to continue working? What happens when I have to stop? How fast will the disease progress? How will our family change? Will we be OK?

The AAPSG, APDA, and other organizations can provide excellent resources, guidance, and referrals to help families answer these questions, but like the disease itself, the answers are different for everyone. In my family, we don't have these answers yet, and we're struggling to find them. However, what we do have is mutual love, commitment, and a dedication to finding the answers together. In this, I'm very fortunate.

It's become apparent over the last two years that I am particularly susceptible to side-effects from PD medications. I've described my problems with nausea (perhaps to the point that you're saying, "I GET it - stop, already."). I also have significant nighttime insomnia and daytime sleepiness, including sleep attacks from the dopamine agonist. Recently, I've begun experiencing other effects from the agonist (when you see a squirrel run across the living room floor and your cat on the couch doesn't move, that should tell you something). I also have the most well-equipped home theater and home automation system in the neighborhood, and I can't really tell if that's just because I like electronics and gadgets, or if Requip XL's notorious "compulsive behavior" side-effect is rearing its head. And, although it's a little unusual, I've begun having on-off problems and beginning-dose/end-dose dyskinesia from the levodopa.

With this spectrum of side-effects, increasing the dosage of my medications isn't an option. At this point, I can't go up in medication without more severe side-effects; I can't go down without being off most of the time. According to the advice of my neurologist, my neurosurgeon, and multiple alternate opinions, it's time to consider deep brain stimulation surgery (DBS).

I've only had a PD diagnosis for about two years, and I'm on the young side for having the surgery, so I've been resisting the idea for some time. When my neurologist first recommended it, I said, "You've got to be kidding - that's for late-stage Parkinson's, after everything else stops working, isn't it?" I apparently have a limitless capacity for being wrong, and I was this time, too - DBS actually works best for people who are still getting some benefit from medication, but perhaps with unacceptable side effects. It also seems to work best for people who are relatively early in the disease, who don't have other complications or significant cognitive impacts, and who are not falling often. Me, in other words.

So, after weighing the risk factors and evaluating the data (did I mention I'm an engineer?), I've decided to have DBS surgery in early June. Between now and then, I have a neuropsychological exam and a full-anesthesia MRI to do, and a few other things to get organized and put in order. We're talking brain surgery here - no matter how you look at it, there are risks. Even though the likely outcome will be very positive, my family and I have to prepare for all possibilities. This situation gives my typical approach of hoping for the best and preparing for the worst a whole new intensity.

Hope is good - it keeps you going when you might not otherwise. I'm hoping for the type of outcome I heard about from a new friend I met in Istanbul this month (thanks, Shelly and Warren :-), from a friend I met in DC last month (thanks, Greg:-), and from the experiences of people like Davis Phinney, the Tour de France cyclist who was brave enough to have his DBS surgery filmed for the benefit of our community. I have a great health care team, people I trust to give me advice, one of the most experienced neurosurgeons in the country, an outstanding neurologist who always tells me the truth (even if it stings a bit), and friends old and new that regularly bring tears to my eyes with their kindness and caring. I'm also surrounded by a loving family who are willing to do whatever it takes, even if we don't know what that means yet. With all that going for me, what's a little brain surgery?

So, onward through the fog. Next time - the lead-up and a description of the procedure itself.