Many people who contract an incurable disease devote themselves to finding a cure. I wasn't one of those people. I was a 27-year old registered nurse who worked in the critical care division at a local upstate New York medical facility when I was diagnosed with dystonia in 1993. Dystonia is an involuntary disorder characterized by overactivity of a specific muscle and/or group of muscles that results in abnormal movements, tremors, and bizarre postures. In my case, the cause of my dystonia has never been identified. This is true for most patients with dystonia.
I simply woke up one morning with a stiff or wry neck. I recall that my neck felt funny and that there was something wrong with it. It was as though some invisible force was pulling my neck and I was resisting. Yet, the more I tried to resist the pulling sensation, the greater the sensation became. I quickly developed spatial disorientation, began having falls, and developed an intoxicated gait. Through it all, I continued working in one of the busiest medical centers in upstate New York.
Within months, my neck was not getting any better, and my head hung to one side, feeling like a 2-ton rock. It was frozen in position and caused intense discomfort. Life was getting difficult, almost unbearable, and not a single physician was able to diagnose my symptoms. Eventually I diagnosed myself by finding a picture of a woman with a similar condition in a classic neurology textbook. The doctors and the specialists whom I encountered all seemed puzzled when I entered their offices to announce that I had found a diagnosis. It seemed to me as if they wanted to say something to me, but couldn't find the right words. A stress-related disorder? A "woman's disease?" By now, my hands had begun to fly up into the air gripping my hair in an effort to stop the violent twisting and turning of my neck. While this had the effect of stopping my neck from flailing around, it was only a very short-term solution to what was to become a lifelong problem.
When I walked, I looked like someone who had been in a bar for too long and had too much to drink-I knew people thought that I was drunk; perhaps they thought I was an alcoholic. When using public transportation, I learned to run past people so that I could get an unoccupied seat while at the same time avoiding those seats reserved on the public transit system for the "disabled"-after all I wasn't one of them! At that time, little did I know that my symptoms were classified into a group of disorders known in neurological circles as "chronic disabilities." I tried wearing a soft cervical collar at work to control the twisting and spasms. Patients and friends wondered why I was wearing such a thing. I always gave the simple answer-"stiff neck."
Then began a long, tedious 15-year ordeal of being treated with oral medications typically used for other neurological conditions-tics, Gilles de la Tourette syndrome, Parkinson disease, seizures-rather than for dystonia. Dystonia was, and remains, as cureless as it was in the early 19th century. Driving became impossible, and I relocated to Manhattan in 1998 where Botox was available to patients. When injected into muscles, it would temporarily stop my spasms for a period of 10 to 12 weeks. As far as I was concerned, I was facing retirement in my mid-30s. Nursing was my livelihood and I loved it. I hated looking in a mirror or at a photograph because I was distressed and upset at the person looking back at me. I didn't like that person. The posture that I saw was grotesque and horrible. There were moments when I felt caged in by the disease; it caused me to live a life that was lonely and isolated as well as a life of social embarrassment. In public, I began wearing a variety of scarves and sweaters with collars; they hid the physical distortion I saw each morning. I stopped wearing dresses. The mental anguish persisted, often in silence and unbeknownst to family, friends, and colleagues. Over time, immunity to Botox developed, and I began clinical trials with a newer form of botulinum toxin called MyoBloc. I derived benefits from this toxin for 8 years, even being able to ride my bicycle again.
Then in 2004, tolerance to MyoBloc developed, and the drug no longer worked. Oral medications were losing what little effect they had on my dystonia, and I began investigating the neurosurgical procedure called deep brain stimulation (DBS)-placement of a brain "pacemaker" that could override misfiring neurons and diminish the signs and symptoms of dystonia. DBS presented the possibility of alleviating not only muscle spasms but discomfort and despair as well. I didn't want to live in darkness when there was light. I also was tired of alternative or complementary care. I wanted a treatment that was more permanent, and DBS seemed to be it.
An online search brought me to Weill Medical College of Cornell University. With the procedure, I would be able to just live. To simply "be still," yet live a life in motion. Once I had signed the consent form for the procedure, I sought out a photographer to help me document my journey (see http://www.parasphotography.com/beka.html). I underwent DBS placement about 8 weeks later in a room filled with anesthesiologists, residents, and nurses. Within 24 hours of the lengthy operation, I was walking back to my apartment from the hospital and was eating and laughing. I had not experienced any of the associated complication risks: seizures, stroke, hemorrhage, or headaches.
I admit that the surgery is not "the run of the mill" surgery. The treatment uses a surgically implanted device, similar to a cardiac pacemaker, to deliver carefully controlled electrical stimulation to precisely targeted areas within the brain. Stimulation of these areas appears to block the brain signals that cause the motor symptoms associated with dystonia. I returned for programming of my pacemaker 2 weeks later with a new outlook on life. I began my own journey toward functionality and normalcy First, let me make it absolutely clear that DBS is not a cure for any type of dystonia, Second, each type of dystonia responds differently to DBS settings. Third, it is foolish to assume that what another person experiences with DBS programming is what you will experience. Finally, there are no set clinical guidelines for DBS or definitive patient selection criteria in dystonia.
I returned to work 5 weeks after DBS placement, which proved to be immensely tiring; not only were the patients very ill, but my colleagues asked many questions about my health. Within a month, I felt like a sinking ship. My gait became ataxic again, and I couldn't keep my hands out of my hair. I was leaning against walls again and no longer felt fluid in my movements. They had no beginning, middle, or end. Was this how programming was supposed to work? Was this so-called DBS success? Adjusting to DBS was not just adjusting to new settings, but it also involved adjusting to a new set of life circumstances and experiences. I began to recognize that DBS was a life-changing event-emotionally, physically, socially, and mentally. I am not too sure if I was as well prepared as I should have been at the start of DBS. There was so much to learn. I had the stimulator reprogrammed, and things were better again for a set time period.
I still did not understand how frequently adjustments or reprogramming would be needed. I felt confused about programming. Voltages, amplitudes, pulse rates, and widths. It all seemed like a foreign language in me. I was beginning to feel frustrated and distressed. I also began noticing a pattern. My settings were clearly lasting only 6 to 7 weeks at a time. Was I emotionally undone at this point? Life was new, and right now I was a real human being with too many chaotic emotions. Least of all, I didn't know what was coming next for me.
One day, for some unknown reason, I decided to check the device's battery-the second time ever-to see if things were working properly. A yellow light appeared, indicating that my stimulator was in the OFF mode. I became frantic, rereading the patient education materials provided with the stimulator 100 times, hoping that I had missed something. Increasing pain and twisting had been problematic, but I had taken no notice. Even though logic had said that something was wrong. I decided to turn myself back on and needless to say ended up with a power surge erupting in my head followed by my usual overstimulation side effects of headaches and nausea. At one point, I thought I had gone into a cardiac arrhythmia. That was the last thing I needed. My neurosurgeon concluded that antitheft detectors at a retail store had simply turned the stimulator off.
Unfortunately, I landed myself in another unexpected unintentional "wash-out" period of 11 days or so with ever-increasing symptoms. This time my symptoms again felt 10 times worse than they had before DBS implantation. My frustration level rose. I felt at a loss of how to troubleshoot the system independently without the help of anyone. As an intensive care unit nurse, I had learned the skills to problem solve acute patient issues and equipment failures in a matter of minutes. Now I couldn't even problem solve my own device. I was learning that stress, lack of sleep, and fatigue played a role in how well one coped with the hardware. These were three factors that I had dealt with rather confidently over the years. Having them chopped like fine tuna over time was scary, demoralizing, and unsettling to me.
I had to make some lifestyle changes: the most important one was redefining myself as person without dystonia and DBS. I had felt trapped by the device for months. The programming was not going in the right direction. Things seemed to be working fairly well until that magic 6- to 7-week mark arrived. I began wondering how I had come to my 1-year post-DBS anniversary filled with too many expectations that had not been explained to me before the procedure. As Roger, a friend who also had undergone DBS for essential tremor, had told me, I felt raw, awkward, and misplaced. I felt dreadfully lonely. On a cool, windy fall day, I realized that I was the only tenant who had been implanted with the device in my entire eight-story apartment building; I was the only person in the neighborhood strolling around with the device. There was no one riding the M2, M3, and M4 bus routes with the same device. There was absolutely no one at work with the implanted pacemaker. I was seen as "famous" at work for having an implanted device and would receive accolades such as "Bek, you look great!" Yet, I felt like I was the only person on the planet with the device.
My first-year anniversary date for DBS came and went. It had been a year since the device had been placed, and I was still learning about the system. It had its successes, yet pitfalls and failures also existed. One of the most important points to make is that those considering DBS should examine themselves as individuals, persons with dystonia, and their emotional levels, DBS expectations, and coping mechanisms. Today, there seems to be no consensus or universally agreed-on guidelines for the following:
1. The selection of patients who will most likely benefit from the procedure, specifically for dystonia
2. Training of personnel or staff who program the stimulators
3. How, when, or where programming should take place
4. How to educate patients and physicians about stimulator complications
5. How these complications should be managed
There seemed to be a criss-crossing of patients from one medical center to another across the United States, at least, attempting to find the "best" possible neurosurgeons who perform DBS for Parkinson disease, essential tremor, and dystonia. Good programmers are few and far between and are often not recognized for their special skills and talents in helping patients to manage the stimulator.
With proper programming, often no one could tell that I had a movement disorder. Yet, as time went by, programming became more difficult and time-consuming. Maintaining the right or optimal settings became tricky and complex and would cause many side effects including leg dragging, loss of speech, hoarseness, loss of verbal understanding similar to the aphasia that can be evident in individuals who have had a stroke, gait difficulties, and paresthesias. With time, I eventually found the best settings, but it was a strenuous, exhausting, wearing, laborious, and arduous time.
Feelings of darkness, hopelessness, doom, gloom, and despair continued. My mother called me practically every day for months. My younger sister had moved to California, so family support was all long distance. I felt disappointed that programming was now an ongoing issue. Then BOOM! We found the right settings, and for 9 months in 2007, I was able to work at full capacity in a local cardiovascular surgical unit without any further reprogramming.
After nearly 3 years, I really had no complaints; it had been a long, rocky road. However, from nursing colleagues, I began hearing comments that DBS had changed me, tweaked my brain in the process, altering my personality. What could I do? Had my personality really changed? I really didn't know if this was true. Physically, the feelings of doom had left me with immense weight loss-110 pounds at 5′8″. Some said that I was now perfect for a high fashion runway model. Foolish!
With time, slowly, difficulties walking again became routine, crossing New York City streets without being hit by a cab became a risk. Planning life became a routine again. Sleeping on ice packs to diminish the pain became a norm. Heat did nothing for me. The soft cervical collar was brought out of the closet. In reality, dystonia had been waxing and waning for the past year. But I had passed Years 2 and 3 with DBS success fully.
As I read The Ivy Briefs: Neurotic Adventures of a Law Student, a book by Martha Kimes (2007), I was forced to face the possibility that DBS itself might not last as long as my battery would. I had faced the "neurotic adventures of DBS," but failure had never been in the picture, nor had I even been told that was a possibility. So, what now?
There was also something else that my treating physician and I had observed over the past year, which had to do with electromagnetic forces. I had worked in settings that contained huge amounts of electrical equipment, especially the cardiovascular surgical unit. The electrical forces in each of the four intensive care units that I worked in were completely different, and each affected me and altered my settings, thus bringing about an acute return of symptoms. This phenomenon too was unexpected, but it is something to take notice of when teaching patients who hope to return to work about the device. Employers will have to learn about DBS themselves-about employees' needs and possible accommodation requests as defined by the Americans with Disabilities Act.
As I finish this portion of my journey, I want to reiterate that each person's DBS road is different. There is no cookie-cutter recipe for DBS or dystonia today. Yet, nurses need to be aware that programming is the greatest problem that accompanies the procedure for these patients. Anyone telling you otherwise is minimizing this problem. There will be "highs and lows." DBS will not be a cure. Also, be aware that patients may require new and recurrent medications to manage specific cognitive, physical, and emotional effects. Socially, life can improve to a certain extent, but overly high expectations can be detrimental, especially when dystonic symptom breakthroughs can occur at any time. Physical adjustments include simply feeling comfortable, an inner sense of peace with an implantable device. It took more than 3 years for me to feel safe and fearless with the device and with hardware in my brain that could go awry at any time. I also have learned what to avoid in relation to the strength of electromagnetic fields on my individual settings. I'm not so sure what lies ahead-a rechargeable battery? Programming improvements? Or simply a whole new life again? A new career in dystonia patient advocacy including disability law?
Beka Serdans, the author of this Perspectives feature, was the recipient of the 2008 New York Times Tribute to Nurses Award. This award recognizes nurses who inspire others to join the nursing profession. Beka received the award on December 2, 2008, during a ceremony that described her as an extraordinary nurse, patient, and human being. Beka's narrative here in JNN highlights her journey as a patient and her growth as a professional. This very personal journey allows her to communicate with and relate to patients with chronic illnesses in a way that many other nurses cannot. She continues to work at the bedside but orchestrates a grassroots, nonprofit advocacy group for patients with dystonia and provides a voice for their unique needs. The Journal of Neuroscience Nursing extends our congratulations to Beka for a job very well done!
V. Susan Carroll, Editor