Something is Wrong
One Woman’s Personal Experience with Dystonia
By Beverley Smith
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I do not play the clarinet. However I have something in common with some who do, something none of us wanted. For me it started with involuntary facial grimaces that opened my mouth bizarrely, making me look like I hated the world. As clarinetists play, it can be air leaks at the mouth corners, tremor, odd contractions of face muscles, involuntary puckering, involuntary mouth closing.
For me the condition moved to the neck, with a vice-like pressure to tilt my head left and down. For clarinetists, problems playing a few passages may spread to more passages, with spasms, unusual postures, and pain. There may be involuntary movements of the lip and tongue.
For me it became difficult to look straight ahead, to see where I was walking, or look at a computer. For clarinetists the trouble may increase, such as difficulty forming an embouchure or controlling the vibration of the reed and air flow.
And it is maddening. I wondered at first if mine was just a cramp from sleeping or from being “uptight”. One doctor also thought it was from anxiety. Clarinetists also at first may be told they just have poor posture. Some clarinetists think that they’ll recover if they just practice more. So they do, and it gets worse. Then they often are so sad, they also get treated for anxiety.
In my case, at the end of a teaching career, it was not fully tragic. I just retired early. However it often hits clarinetists younger, at the peak of amazing careers. It threatens a livelihood and a way to express deep love of music. The lifestyle of the professional musician, its electric energy, joy of performance, orchestra camaraderie, and fun professional rivalry is put at risk.
I did what most people do when diagnosed. I had never heard of dystonia so I scoured the Internet. I read biographies, got textbooks and read websites. The condition, because it affects muscles and levels of neurotransmitters in the brain, does not show up on X-rays or CAT scans or blood tests. It is only in the past few months that researchers have even found evidence of it on MRI.
Detecting dystonia: https://hms.harvard.edu/news/detecting-dystonia
Research has also found genetic links to some forms of dystonia: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7274927/
That means we know a bit more about how to recognize it and one of several routes it might come from. We are not yet clear on how it affects the brain or muscles and certainly not precise on treatments or cure.
I learned mine is called cervical dystonia. The “dystonia” is lack of muscle tone but the “cervical” confused me. When I told a friend, I could hear her blushing over the phone, and I reassured her no, not the womb, but the cervix – neck. In the case of clarinetists, it is called embouchure dystonia, or musicians’ dystonia.
Mine is in the most common location for dystonia, apparently. But it turns out musicians’ dystonia is very high incidence and way more diverse. Embouchure dystonia can happen for players of any woodwind or brass instrument. An estimate by William Upton in 2013 was that among professional musicians dystonia occurs in 2 of every 100.
For brass players, it affects ability to pucker and smile as they play. But it can affect any musician’s muscles used to produce music. It can affect the fingers of pianists, guitar players, and violinists. It can affect how percussionists hold their sticks. It can affect the vocal cords of singers so they can barely speak above a whisper. Dystonia can even interrupt the music careers of banjo players and fiddlers if it strikes the eyelids. A man in Nova Scotia, Canada had involuntary clamping shut of the eyelids and could no longer open his eyes to see what he was doing playing fiddle.
I was relieved to read of others with my condition. Clarinetists similarly have inspiring role models. Most musicians are amazingly creative at trying many answers, doing exercises, changing embouchures, or changing musical instruments. Pianists with problems in one hand try piano selections for the other hand only. Tim Redpath, former professional French horn player formed the group Musicians with Dystonia in 2000. In Australia, concert bassoonist Margot Chiverton had a documentary made about her journey. Trumpeter Robert Eshelby, harpist Anabel Gutierrez, guitarist Billy McLaughlin, pianists Carl Luepker, Gary Graffman, and Gohei Nishikawa have admitted online in YouTube videos or to media that they have been dealing with the condition. Violinists Clayton Haslop and Reinhard Goebel have admitted it too.
We all cope.
I respect the medical community for its standards, its tight research. They are protecting patients so treatments they offer are well documented. I was relieved that dystonia generally does not affect lungs, heart, digestion or lower life expectancy. However, from the clinical studies it was clear researchers themselves also are mystified. I read things like:
– At this time there is no test to confirm diagnosis of dystonia
– The causes of focal dystonia have yet to be determined
– It is not clear why some individuals who inherit a specific gene develop a severe form of dystonia while others who have inherited the same gene do not
– The role of environmental factors causing or contributing to dystonia remains uncertain
– With hand dystonia it is still unclear where the pain is coming from
– What goes wrong in the basal ganglia is unknown
– The prognosis of dystonia is difficult to predict
– Multiple surgical interventions have been performed, many of which have fallen out of favor
– The mechanism of action of deep brain stimulation in dystonia is poorly understood
I had an idea. It came partly from research studies. Scientists do want to know more about the patient experience. I read things like:
-Doctors have to rely on information from the patient’s history
– Any long term assessment of patients with dystonia must take into account the natural history of the disease
– Studies require accurate and detailed evaluations of the past histories of large number of patients
– It is critical not only to use appropriate rating scales, but also to take into account the intervention’s effects on activities of daily living and quality of life
Why was it that people did not know more about this condition? I learned it has been around for centuries. Hippocrates wrote of stiff neck. Alexander the Great’s statues show him in dystonic-like posture. A medieval scribe wrote of hand cramping in his profession. In the 1500s French writer Rabelais depicted a character with a twisted neck condition he labelled tortycolly. (The current term is torticollis). In the 1800s Charles Dickens portrayed two characters who may have had dystonia – one with head tilted to one side and one who could barely speak above a whisper. In 1900 painter Modigliani painted portraits of his common law wife, in what seems to be not just dystonic head tilt but also using a common gesture people often use to deal with the pain – a finger to the cheek.
Why is medical research so slow on this? I discovered some possible reasons. Besides earlier misunderstanding that dystonia was due to anxiety, even today there are hurdles. Clinical studies take time, need funding and require medical grants. Researchers propose a study, apply for a grant and wait. Since dystonia is rare, it is often last to get funding.
There is also the problem of conducting research. It is hard to find patients who admit they have it because of its career threat. They may not want to admit it, even to themselves and certainly not to the public.
There are support groups for dystonia, associations, Facebook groups, and I joined several. They are a hand up in a troubled time, giving and offering support. But they are not themselves clinical researchers.
There are other challenges to research. It would take time to pose all the questions that need to be asked. There are so many theories about dystonia’s cause for instance – virus? environmental toxin? past infection? traumatic injury? past surgery? genetic predisposition? diet? There are so many strategies to try to help – hot baths, cold packs, massage, or pillows. There is variation in treatments doctors give and in patient experience of injections, pills, surgeries. The sheer number of questions makes it impractical to ask them in a clinical setting. Doctors don’t have the time, because they are also treating people with Parkinson’s, essential tremor, brain tumors, traumatic injury. Patients rarely have time to come back over and over to be interviewed at the doctor’s office, paying for the commute, the parking, the time away from paid work.
So I had an idea. Why not put together all the experiences patients deal with and the mysteries researchers wonder about and bridge the information gap? Why not have a series of detailed surveys about all those little details without taking up so much doctor time? Why not have an online survey from the patient’s own home at their own convenience and make sure it is completely anonymous?
Patients alone can’t see patterns but the surveys might. Patients can’t understand the technical meaning of some the oddities but researchers would know what they meant.
Given the aggregated results, researchers might discover amazing things, create vital clinical studies that might lead to better treatments, even one day to a cure.
It is an idea. It is actually something already happening for other medical conditions. The official medical community now considers patient-lived experience a vital part of input, “natural history”. It does not replace clinical studies. It just supplements them.
The Michael J. Fox Foundation is already doing a similar study for Parkinson’s Disease.
I approached a few associations to do such a study about dystonia and was sad to hear that it was such a big project they were not interested right now. Well, what if we patients did it ourselves and just gave the data to the researchers?
So I set up a website with information about dystonia and an account with an online survey platform. I made sure the surveys were at the highest possible privacy settings – anonymous. I made sure it was clearly not a medical advice site and just asked questions. I made sure it was not commercial, costs nothing to the respondent, and promotes no product or service. This was as close as I could get to pure science.
And then I let others know of it: www.dystoniasurveys.org
In December 2020, we are at the 7 month point of the 12 month project. So far the website has had 11,567 page views from 76 countries, and 1,387 surveys have been completed.
I am getting great feedback from patients who seem grateful to be asked. I am getting encouragement from researchers who applaud the project, many of whom want to hear its results.
The website seems to cheer people up. I put on it pages from research about the history of the condition, incidence, types, medical terms used, technology, and even inspiring YouTube links to people with the condition. There is a page of brave and often funny quotes from people who cope with it daily.
I am deeply moved by the courage of people facing any hurdle. It is hard to get up every morning facing yet another day of this painful condition and yet there are millions of people doing just that, and people facing other challenges, often worse. The human spirit is amazing. Here some quotes that keep me going:
Alone we can do little. Together we can do much. – Helen Keller
None of us is as smart as all of us. – Ken Blanchard
The only source of knowledge is experience. – Albert Einstein
No one can whistle a symphony. It takes a whole orchestra – Halford E. Luccock