This article appeared in the Dystonia Dialogue.
“In my family, we were all aware that my father had a problem with his hands,” explained Carole Rawson, “and my parents’ generation was one in which, if there was something bad going on, you didn’t talk about it. He was very closed-lipped about it.” Carole’s father went most of his life without a name for his problem until Carole began having problems too.
“I was born a lefty and when I was in the fifth grade, I no longer could write with my left hand. We realized I must have what my father had, so I taught myself to write with my right hand.” The solution was temporary. Before the end of fifth grade, Carole could no longer write with either hand.
In 1972, Carole and her father were diagnosed with writer’s cramp, a focal hand dystonia. She was 15 years old. He was 53. At the time, treatment options were few. The medication offered did not help. Carole persevered without treatment through high school, college, and a career in retail during a time when writing by hand was pervasive in everyday life. “It was painful,” she said. “There were no special accommodations back then. I was always coming up with new, inventive ways to hold my pen. At work, people would ask me why do you hold the pen so funny? And as soon as I started telling them, it was like they wanted to crawl under the desk and were sorry they asked.”
Carole and her husband Doug met as undergrads at George Washington University in Washington, DC. Both native New Yorkers, they settled in Santa Monica, California. The couple have four children. “I was always very forthcoming about the dystonia,” said Carole. “All the kids knew about it.” She joked that between her writer’s cramp and Doug’s “terrible handwriting,” she did not have high expectations for her children’s penmanship: “My feeling was as long as they don’t have dystonia, I don’t care how they write.”
When Carole and Doug’s third child Liz was around age 11, a familiar pattern began to emerge. “My handwriting was really poor and I remember sitting in class, in either fifth or sixth grade, and my friend would massage my hands because I complained that they hurt so much,” said Liz. By the time Liz was 14, it clicked for Carole that Liz’s problem was the same as her own. Carole contacted the Dystonia Medical Research Foundation (DMRF) for assistance locating a movement disorder specialist, and Liz was diagnosed with dystonia by a leading expert in Los Angeles. She promptly began teaching herself to write with her opposite hand, but, just like her mother, Liz soon lost the ability to write at all.
Hand dystonia is among the most challenging types of focal dystonia to treat medically, especially when the symptoms are present only during a specific activity, such as writing. Botulinum neurotoxin injections can be helpful, depending on the muscles affected, but individuals must often permanently adapt to losing fine motor control of the hands.
Carole taught Liz early to advocate for herself, especially regarding accommodations at school mandated by the Americans with Disabilities Act. “At the start of every school year I’d meet with my teachers and explain dystonia,” said Liz. “I also made the very wise decision of buddying up with my guidance counselor and people in the office, so I always had them on my side.” Carole accompanied Liz to meetings with educators at first, but by sophomore year Liz was handling it on her own. “If it got to a point where I needed reinforcements, she was there in a second,” said Liz, “but my mom also gave me the space to attempt it on my own, to go talk to teachers and ask for what I needed. It’s something I’m always very grateful for.”
Liz graduated high school and, following in her parents’ footsteps, attended George Washington University in Washington, DC. Liz had managed well in high school despite the challenges of dystonia, and there was every expectation that her success would continue.
Carole recalled, “When she left for school, it was like anybody else leaving for school. I mean, she had dystonia in her hands and she was going to deal with it like she always did.”
Liz agreed: “This is what we understood dystonia to look like. We didn’t understand at that point that there were so many different manifestations of dystonia and that it could spread to my legs. That wasn’t a thought in anyone’s mind.”
When Liz’s doctor mentioned deep brain stimulation (DBS) for the first time, the idea seemed extreme relative to her symptoms. DBS is a neurosurgical procedure to treat dystonia that involves implanting electrodes in the brain and a battery-powered stimulator in the chest wall. Liz was not interested: “I’ve been needle phobic and doctor phobic my whole life and when my doctor offered [DBS] to me as an option, I remember laughing and telling him he was crazy and that I would never get it.”
While away at college, thousands of miles from home, Liz experienced a slow creep of troubling difficulties. “I was having trouble walking. I ended up fracturing my ankle because I tripped over myself. I would take a cab two blocks because I just couldn’t stand it.” One day toward the end of her freshman year, she was so exhausted after completing an assignment and walking a short distance from the library, she crashed at her dorm and slept for 22 hours.
Despite a close relationship with her parents, Liz did not readily talk about her deteriorating condition. “It was like pulling teeth to get information from her as to how she was doing,” Carole said. “Liz never complained.” A check-up with Liz’s movement disorder specialist confirmed that the dystonia was spreading to her legs and feet, and could explain the pain and loss of control.
“I wasn’t always the most forthright about the state I was in,” admitted Liz, “but my parents were always there when I needed them and they were always supportive in a way that allowed me to maintain my independence. I felt like I had a safety net if things went bad.”
Liz continued: “It can be really hard to establish your independence as a young adult if you are never given the opportunity to fall—and to fall enough until you feel like you need to ask for help. Asking for help is a really important lesson to learn.”
At the start of Liz’s spring semester of sophomore year, her dystonia symptoms became overwhelming. “It was bad. My body wasn’t functioning anymore,” she said. She could not walk, and barely left her dorm room for a week—even to attend class. She texted her mom late one night, having reached a breaking point. Liz decided to take a medical leave of absence from school and return to California to have the DBS procedure her doctor had been recommending for years.
Carole immediately suggested she would fly out to help Liz move out of her dorm. Liz refused. “I think that could be hard as a parent,” reflected Liz. “I said, no, I don’t need help. I think many parents would say, I don’t care, I’m coming anyway. But my parents trusted me to know what I needed.”
In March of that year, at age 19, Liz underwent DBS surgery. Carole said, “The hardest time for me with Liz was when she went in to have the DBS surgery. I felt so responsible for the whole thing. She would not have been going through any of this if not for me.” Fortunately, the surgery went smoothly, without complication. Her recovery was slow at first, but within a few weeks she regained her strength.
Just two months after the DBS device was implanted, Liz returned to Washington, DC for a summer internship with a presidential campaign. That September she continued working full-time for the campaign while enrolled in a full schedule of classes. She was traveling home to California every four or five months for appointments to adjust her DBS settings. Ideally, the appointments would have been scheduled weeks apart, not months, to determine the optimal settings required to control the dystonia, but Liz was determined to keep up with school. “I was about 20% better,” recalled Liz. “I was still definitely disabled. But better was better.” She was grateful to have had the surgery.
After graduation, Liz spent the summer at home in California, working with her neurologist to fine-tune her DBS settings. In the fall, she relocated to New York City to begin her career. “Within six months of moving, I was basically asymptomatic,” she said. She has since taught herself to write again, can walk with little difficulty, and works full-time for a marketing technology company. “I have tried to make every decision in my life based on things that I want and not based on what might be easiest for me. There are so many things in New York that make it hard for a disabled person to exist, but I’ve always wanted to live in New York and I wasn’t going to let any of that stop me.”
Liz and Carole joined the DMRF Board of Directors in 2018. Both are registered brain donors. Carole has volunteered for numerous research studies at the National Institutes of Health. The Rawson family has generously supported the DMRF since the 1990s, including recent Dystonia Zoo Walks on both east and west coasts. Carole has yet to find an effective medical therapy to treat her writer’s cramp. She recently began experiencing mild dystonia symptoms in her legs and feet.
“Up until Liz [developed dystonia], I looked at my dystonia as a gift because I felt it was a great equalizer,” said Carole. “It makes me appreciate life more and has given me the ability to better empathize with others.”
As a DMRF Board Member, Liz is especially sensitive to the experiences of young people with dystonia, particularly those who are navigating the transition into adulthood. “Don’t rule out jobs or opportunities ahead of yourself – apply for the job, apply for the internship,” she would suggest. “Give people a chance to welcome you. Give people the chance to make accommodations for you to be able to do the job. Everyone’s dystonia presents differently, and for many of us there are jobs that our bodies aren’t able to do, but don’t rule things out because you make assumptions about the people who are doing the hiring. Go for it.”
Carole added: “Liz has so much grit and perseverance. Learning to be an advocate for herself at an early age has been an amazing tool for her throughout her life. Don’t let your disability define you—let it make you stronger. It will always be a part of you, but don’t let it be who you are.”
Early-onset isolated dystonia is a disorder characterized by progressive problems with movement, typically beginning in childhood. Symptoms include involuntary muscle contractions, twisting postures in specific body parts such as an arm or a leg, tremors, and other uncontrolled movements. Signs and symptoms of early-onset isolated dystonia typically appear around age 12. Abnormal movements or postures in an arm or leg are a common first sign. The involuntary movements may initially occur while a person is doing a specific action such as writing or walking. In some individuals, dystonia spreads to other parts of the body. The signs and symptoms of early-onset isolated dystonia can vary dramatically, even among family members. Several gene variants have been associated with early-onset isolated dystonia, most notably the DYT1/TOR1A gene discovered in 1997.
The Dystonia Medical Research Foundation is a 501(c)(3) non-profit organization dedicated to advancing research for improved dystonia treatments and ultimately a cure, promoting awareness, and supporting the well-being of affected individuals and families.