WE MOVE
204 West 84th Street
New York, NY 10024
E-mail: wemove@wemove.org
wemove.org • mdvu.org
No One Like Me
I am fortunate not to have to carry the burden of my disease alone. My wife is my caregiver and sweetheart. Together we believe in living each day doing the best we can. We do not worry about tomorrow, or things out of our control. This has helped us stay positive and for me to be able to maintain my abilities to the best level possible.
– Kirby Nielsen
On Sept. 5, 2003, I went to bed feeling tired, but otherwise OK. As I was about to drop off to sleep, I had a 20 minute period of jerking. These jerks stopped as suddenly as they began and I soon drifted off to sleep feeling no ill effects from the jerking. The next day I felt fine but that night the jerking lasted a period of 1.5 hours. On the third day, the jerking began to appear whenever I sat still. They disappeared when I walked, ran, worked on the computer, or did other physical activities. The preliminary diagnosis was Myoclonus. Despite hundreds of tests and consultations with several leading neurologists, there is still no definitive understanding of why things have gone so wrong with me.
Along the way, nature has given us false leads such as two positive tests indicating the presence of the Achytlecholine Receptor Antibody (which causes Myasthenia Gravis) but anyone could see at that time, I was far from having that condition, I became known as the “Weird” one. We pursued the Paraneoplastic Syndrome idea because in addition to my movements, I also had a large cloudy area in my lungs and the lymph nodes in my lungs were slightly enlarged. We expected that I would develop small cell lung cancer. But thankfully, it never came as we pursued immune suppression therapy to try to stop the ever worsening and constant jerking. To make sure we stopped things in their tracks, I initially took 100 mg/day of Prednisone (oral). That stopped the movements and we began to suspect my immune system caused the jerking.
With a gradual reduction in Prednisone and the inclusion of Cytoxin into my treatment plan, we achieved a moderately successful job of controlling the jerking. By the fall of 2005, my white blood count decreased to the danger level and we had to stop the treatment. By this time, my movements had evolved into “choreaform”. One specialist gave me the distinction of having an “immune mediated, hyperkinetic movement disorder-choreaform”. It signaled the end of our Paraneoplastic theory since there are few if any cases where a Paraneoplastic patient went more than two years from the start of movements to the onset of cancer. No cancer-no Paraneoplastic diagnosis.
By this time, I began to notice that I had some slight memory impairments and I had also developed a problem with aspirating food and water resulting in severe choking. Fortunately, in addition to the top notch Movement Disorders Center, Ohio State University has a wonderful Voice and Swallowing therapist who recognized my difficulties immediately and put me on a series of exercises designed to strengthen my tongue and related swallowing mechanisms. The therapy worked and I got the choking under control. Along with the swallowing problems, we began to consider the possibility that I was suffering from some sort of dementia. It was thought that my memory would worsen quickly. As it turns out, a Cognitive Neurologist at Ohio State did a long term evaluation of my cognitive abilities and she has been able to rule out dementia. Instead, she identified a mild cognitive impairment, a problem with my short term memory. I can easily forget what I just did or heard or said, but can remember news events from long ago. Mild yes, but it was still disturbing to the pattern of my life.
2006 and 2007 were years primarily devoted to just trying to work and maintain my level of abilities. I had my voice and swallowing, plus my memory exercises to do, and I was still trying to work my way through the demands of running an insurance agency. This effort did not go well and by May 2008, I had sold all of my business and applied for full disability from my private insurance companies.
One of the dark periods of my journey came at the end of July 2008 when I had a recurrence of the “going to bed” episode of 2003 but this time with jerks that were so severe they literally moved me off the bed. Plus there was a new severity to the Chorea. After a brief hospital stay we began a new regime of Immune Suppression using CellCept and Prednisone. As of this writing (05/09), I have been able to discontinue the Prednisone, and my movements are nearly 100% controlled with CellCept 2,000 mg. per day. CellCept has given me a chance to at least appear normal, although I continue to have problems and issues in several areas such as choking, memory, shortness of breath, and balance. Now that things have once again stabilized, my physicians are re-evaluating my MRI’s and other data and looking more closely at whether or not strokes may have caused damage in the area of my Basil Ganglia.
At the earliest stages of this disease, my wife and I decided we needed to be straight with each other about our expectations and how we would live with all the fears and uncertainties in our lives. We have harbored no hopes of recovery since we have never been given any. Neither has anyone given us a timetable for the end. We agreed that we would aggressively pursue every option for treatment that was reasonable and we would not give in to the disease, whatever the name. But THE most important thing we decided was to simply do the best we could each day. We are thankful for each day and we do not worry about tomorrow. We will deal with whatever comes. Our approach has served us well. We have been able to parlay our attitude into 5.5 years of dealing with many ups and downs without any major dent to our spirits.
