Dystonia Is Not a Country in Eastern Europe

There is so much confusion about “dystonia” and people always joke about how it sounds like a country. There are many countries in eastern Europe – Latvia, Slovakia, Lithuania — but dystonia isn’t part of that region. Don’t get me wrong, I love eastern Europe because my family immigrated from Lithuania in the early 1900s. Unfortunately, those who have this condition know it isn’t a country and not a laughing matter. Dystonia is an extremely painful condition that can be a stand-alone disease, or it can be secondary to another condition like Parkinson’s. Dystonia is my primary condition. I have what is known as dopa-responsive dystonia, which causes Parkinsonism for me (Parkinson’s like symptoms secondary to another condition).

What exactly is dystonia? The short explanation is that it is intense and unintentional muscle spasms that can occur throughout the body, or it can be isolated to one area. There are multiple forms of this condition. Dystonia’s muscle contractions can even result in postural abnormalities. Dystonia can be caused by Parkinson’s disease, among others. It can be brought on by genetic abnormalities, brain injuries, heavy metal poisoning and oxygen deprivation.

Unfortunately, this condition can be a possible side effect from antidepressants, antipsychotics and antiemetics (anti-nausea medications). When dystonia is localized to one part of the body it is called focal dystonia. Writer’s cramp is a form of focal dystonia and often for many the first sign of this condition (like myself as a child). Blepharospasm is a focal dystonia of the eyelid area and can cause uncontrollable blinking and light sensitivity. Cervical dystonia (spasmodic torticollis) is of the neck area and may cause the individual to tilt their head awkwardly and is extremely painful. Individuals who experience symptoms in more than one area and in adjacent body parts have segmental dystonia, and individuals affected across multiple areas of the body have generalized dystonia. Sometimes, like my dystonia, you can start with mild dystonia as a child in one area and then as the years go on you might develop generalized dystonia.

Dystonia has been quite challenging for me. I didn’t even realize I had this condition, nor did the medical community, up until about four years ago after a hospitalization (with acute rehab) that lasted 27 days. This was one of many hospitalizations from severe dystonic storms (status dystonicus). What is a dystonic storm? It is generalized dystonia on steroids. My body starts to spasms in my legs, feet, arms, hands, back, neck and even my diaphragm is affected making it hard to breathe. With all of these spasms, my lactic acid levels can skyrocket and this is why it is important that I am treated quickly at an emergency room to control the storms and to monitor my blood for acute spikes. Often after a severe storm, I will get extremely sick and then I need medication to help with the severe vomiting that can occur in-between episodes.

This condition started when I was a child and I noticed something wrong when I would play softball with my friends. I remember being only 9 years old and wearing my smelly rawhide mitt while playing ball in my neighbor’s yard. We were playing only a short time and I remember taking my mitt off because we were so hot and sweaty. I will never forget this, but when I took it off, I couldn’t bend my fingers. They were so stiff and rigid. I remember my grandmother threatening arthritis if I didn’t wear my winter gloves — but this was summer and we were glistening from the heat. I remember thinking that I may have given myself arthritis and folding down each stiff finger one by one. It was so painful. I still get rigid fingers today and even as I type this, my fingers get stuck and it is hard to type fluidly. Then I started to twist my ankles chronically and there was no rhyme or reason. This became such a huge issue for me and by the time I was 15, I was on crutches every other month from rolling my ankles on flat ground in my suede Exersoles (they were the rage in the 1970s – flat rocker bottom shoes also known as “earth shoes”). My parents didn’t seem to think this was an issue and just thought I was a “bull in a china shop” at the height of 5 feet 10 inches and the large frame inherited from my Lithuanian grandmother. Looking back, I shake my head in disbelief that no one saw this as an issue. Then in my late teens, I would have terrible neck cramps, back spasms and my eyes would force themselves shut and that was the worst. I would panic when that would happen because I couldn’t see. I would tear profusely. It was so painful and debilitating but it would happen very sporadically and I never knew when. Even though I was very fit in my 20s and 30s, I always struggled with writer’s cramp, leg and foot cramps, back and neck spasms and the eyelid spasms never knowing they were all linked to one disease. Then I hit my 40s and as I entered my late 40s and early menopause, the wheels came off my bus. I literally fell apart and my dystonia became a really big, chronic problem. My neck was the first to become severe and I remember hardly being able to turn my head for over a year after going on a roller coaster on the Jersey Shore. I knew something was very wrong. I would go for a relaxing pedicure and when the massage part came, my feet would contort in spasm if they pressed too hard or moved my toes in an awkward position. Relaxing pedicures became filled with anxiety because I never knew if my feet with ball up with intense spasms. The pain was excruciating.

As I got to my 50s, my health declined markedly. I went from a busy, working mom with a very successful psychotherapy practice to a disabled, depressed, lost soul trying desperately to figure out what was wrong with me. See the thing with dystonia is that is very rare and often misdiagnosed. Many people with real dystonia are often told their symptoms are functional (symptoms that can’t be explained by a neurological disease) or psychological (conversion disorder) and dismissed — especially women.  The female hysteria model is still pervasive in the medical field, thank you, Dr. Freud! Women are often sent home from emergency rooms told they are anxious and depressed. Unfortunately, this is often not the case and many women are suffering severe medical and psychological fallout as a result. I was one of those women because so many people in the medical field have no idea what dystonia is. I remember being wheeled into an ER where they were supposed to have a treatment plan in place for me (the same hospital I spent two weeks in neuro-intensive care for dystonia) and the EMT told the nurses I had “dystonia” and three nurses in unison said, “what’s dystonia?”

Needless to say my head wanted to explode because I was promised by the medical director that the ER staff would be given an in-service on dystonia, after a different incident with a physician there who told me I need a “spiritual advisor” when I had an episode while being treating for asthma (I was sent over to the ER by a physician at an urgent care that said I needed a higher level of care). Yes, this ER physician said I didn’t have asthma (I was hospitalized at least four times for multiple days and had multiple pulmonologists confirm this) and that my dystonia was really anxiety and I was seeking drugs! Which was “insanity” to me because I had a full bottle of almost 90 benzodiazepines in my purse (that is what they use to treat my storms). You can’t make this stuff up!

The reason why I bring this all up is that the medical field also needs to be better educated about this, and if you do not know what something is, that doesn’t mean it doesn’t exist. Dystonia is a real problem for so many people. For the Parkinson’s community, it can be one of the hardest symptoms to manage. It is a very painful condition that is poorly understood and inadequately managed. Unfortunately, the treatments we have help (Botox, muscle relaxants, Sinemet and deep brain stimulation surgery) but they do not seem to fully control this disease. I was prescribed Sinemet (carbidopa-levodopa) after that long hospitalization. That drug was just like the levodopa Dr. William Sayer discovered in the movie, “Awakenings” (great movie if you haven’t seen it). I was the bumbling Leonard and like him, I was broken free from the cement that encased me. I went from dragging my legs and contorting in spasms to being fluid again — like the Tin Man finally getting his oil after seizing up in the woods surrounding Oz! I will never forget the day after I was given the medication, my roommate was shocked at how well I was able to walk. I knew that this was a miracle drug for me. My dose needed to be increased, but now thanks to this medication, I have my life back. I cannot help but think when I do have breakthrough spasms that I might wind up like Leonard, a prisoner in my body but I shake that off and find gratitude. I have deep gratitude for the doctor who was smart enough to prescribe this and gratitude for being able to embrace my life fully again. I am different, I am not the same athlete I was before and I just started working again very part-time, but I am moving and living and for that, I am over the moon thankful! And ironically, I have found a spiritual advisor who has helped me get past the trauma of not being taken seriously at first by those who should have recognized dystonia in the first place.