When I decided to make the video of myself talking and post it on this blog for Dystonia Awareness Week it was already a few days into the week. During that week I spent some time on other Dystonia message boards and learned that I really only know about Spasmodic Dysphonia. There are so many other areas affected by Dystonia. I asked if anyone would be willing to share their stories on my blog. I've gotten a very good response and am going to feature on person a week for as long as I can. The following was sent to me by Lynn.
Benign Essential Blepharospasm (BEB) is a dystonia that affects the eyelids, causing excessively forceful blinking that renders the patient functionally blind for short periods of time; just long enough to drive a car into an accident or fall down a flight of stairs. As it is quite rare (about 1 in 20,000 have the condition), very few medical people are adequately trained or experienced enough to deal with it. I personally don't know of a single General Practice physician who has ever recognized or treated a single case of BEB. A Neurologist, Ophthalmologist, or Movement Disorder Specialist is most likely to provide a viable diagnosis. Even they do not know of a cure for BEB, although a patient can get considerable relief.
I have had BEB since about 1960. At first I was completely puzzled about what it was and how I came to have it (I still don't really know that) and I got no help from any of the people whom I asked for information. It showed up initially while I was bowling: I would pick up my bowling ball, take my position at the head of the bowling alley, and wait several minutes for my eyes to open far enough to see the pins. Have you ever tried to open your eyes with your fingers while holding a 16-pound bowling ball? It doesn't work well at all.
My doctor gave me an initial, tentative diagnosis of some sort of psychological aberration, for which I should go to a shrink. I did, and nothing changed. I tried that avenue a second and yet a third time, with no change in the state of anyone's knowledge. This went on for 30 years -- no help. No one had ever seen it before, nor knew what to do about it. I even got an opinion from my dentist – no worse than any of the others I got.
Looking back on my experience, I realize that I had met another man in much the same condition -- stumbling along the edges of walls to and from his office, blinking and mumbling to himself -- and I didn't then recognize him as someone with a variant of my own condition. I had no idea what I looked like to him or anyone else.
However, I was still working as a Computer Scientist/Software Engineer, for several companies, even though I soon gave up driving as too risky and stressful.
Finally, my fourth psychiatrist noticed my constant blinking and spasms and had, by chance, run across an article in a Psych journal about BEB. He suggested that we explore this idea and started me on treatments with a series of prescription drugs -- everything that had yet been suggested might combat this weird dystonia. Finally able to hang a name on my disease, I gladly went along with the endless trials of new prescriptions. But none of them did anything for me -- some had really objectionable side effects, most simply didn't do what I needed. Finally we turned to what we believed was our last resort, a toxin (of all things!), Botox, that had just been approved by the FDA for a related condition, cross-eyedness.
So we set up an appointment at Mass Eye & Ear, the famous hospital in the Boston/Cambridge area, to confirm my diagnosis and find a doctor to administer the Botox shots. The shooter was Dr. Gary Borodic. While he had far more experience with Botox than anyone else in the Boston area, he was still in experimentation mode. (I think my first dose was 90 MU, total, over 18 sites.) Even so, the results were far beyond any expectations I had, and I felt sure that, in 1990, we had the right solution at last. After the second set of shots, I said a grateful Goodbye to the Pysch and moved on.
We did have the right solution, although there was still a lot to learn. Over the next three years, we continued to learn about how much Botox to use and where to put the shots and how frequently I would need treatment. (By then I had moved to the DC area and was being treated by Dr. Neil Miller in Baltimore.) Since I believe every patient's answers to these questions are different, I give you this data not for comparison's sake but as a suggestion that anyone's opinions about YOUR treatment parameters may be wrong. Or Very right.
I was injected last week. My doctor and I agree that I regularly get, every few months:
1) Five shots per eye; three above the browline, one at each end of the upper lid.
2) 5 MU per browline shot, 2.5 MU for each upper lid shot. (40 MU total)
3) The pattern repeated when needed (every 6 months, which is exceptionally long.)
Am I nervous about getting these shots? Not at all. By now I welcome them. Are they painful? Two of them on each side are very painful (inner end of upper lid, center of browline, in the fatty tissue). But that is a small price to pay for another six month's relief from the spasms that I know will come if I don't get the shots. Especially since I know from experience that the pain will be gone by the time I leave the Dr's office.
Over time, I have learned what I look like to others during my spasms, and more important, what others' spasms typically look like, and I am no wallflower when I have the opportunity to help someone else understand what they are going through and feeling. I have run into perhaps 4-5 new sufferers every year, plus many more who stumble across the BEB Research Foundation's website, Blepharospasm.org and its Bulletin Board, and always talk or write to them about their condition. Almost always they start with no idea what's going on, and welcome the new information, especially when I can give them an expert's contact information (which by the way, is available through that website.)
Thank you Lynn for sharing your story!!! Please leave Lynn a comment!