"It's slowed me down. It hasn't stopped me." Former Sacramento poet laureate Julia Connor tries new treatment for essential tremor
The cup in her right hand jiggles, threatening to splash coffee across her artist’s table. Steadying the cup, much less sipping from it, is virtually impossible.
For Julia Connor, a painter, sculptor and Sacramento’s former poet laureate, it’s one of the tiny, innumerable hurdles she faces daily dealing with uncontrollable shaking in her hands, known as essential tremor.
Often misdiagnosed as Parkinson’s disease, essential tremor causes involuntary trembling of the hands, arms or head that often leaves patients unable to tie their shoelaces, zip their pants, brush their teeth or lift a fork to their mouths. It may add a distinctive warble to their voices. Some cannot legibly write their own names; others have been forced to quit work. Many are embarrassed to be seen in public.
It’s not fatal, but there is no cure.
Within three months of her 2012 diagnosis, Connor lost the ability to write anything legible with her right hand.
“It’s closed the poetry, the writing part of my life,” said Connor, who headed the city’s poet laureate program for five years, until 2009. “It slows me down a great deal, but I persevere.”
An artist who works with her hands to write, paint and teach, Connor has had to largely abandon those pursuits due to the common but little-known brain disorder, which afflicts an estimated 7 million or more Americans.
“It used to be referred to as ‘benign tremor,’ but just because it does not result in death does not make it benign,” said Dr. Codrin Lungu, program director of clinical research for the National Institutes of Neurological Disorders and Stroke in Bethesda, Md. “When it stops you from being able to eat, there’s nothing benign about that.”
A new ultrasound treatment approved last summer by the U.S. Food and Drug Administration is now offering hope to millions of essential tremor patients. Called a breakthrough treatment by some neurologists, it uses MRI-guided ultrasound beams that heat up and destroy a tiny part of the brain’s thalamus that controls movement. Based on clinical trials at six U.S. medical centers that found a 47 percent reduction in tremor severity, it’s seen as an alternative for those who don’t respond to drug therapies or don’t want invasive brain surgery.
Connor is one of 30 patients on a waiting list to undergo the procedure at Stanford Medical Center, one of seven U.S. centers using the novel approach following clinical trials.
“The demand is beyond what we expected,” said Dr. Casey Halpern, a Stanford neurologist, with patients coming from throughout California, as well as Nevada, Arizona, Washington, Oregon and Hawaii.
Halpern, who oversaw Stanford’s clinical trial, said in an email that trial participants experienced a 60 percent decrease in the severity of their tremors over 12 months. “We expect our results for patients to be even superior to the findings in the (clinical) trial, as the learning curve was steep,” he said.
For years, the typical therapies for easing tremor symptoms were anti-seizure or blood-pressure medications, with uneven results and often unwanted side effects. In some cases, botox is injected into hands to weaken muscles and slow the shaking. A more invasive treatment is “deep brain stimulation” surgery in which an electrode is implanted in the patient’s thalamus and linked to a chest pacemaker to help calm the shaking.
The new MRI-guided ultrasound treatment is “a breakthrough for people with essential tremor,” said Dr. Ehsan Hadi, a movement disorder neurologist with Dignity Health in Sacramento, who said Julia Connor is his first patient to try the new treatment. “It’s unique in that it’s noninvasive surgery.”
Similar targeted ultrasound treatments have received recent FDA approval to treat uterine fibroids in women and prostate cancer tissue in men, as well as to alleviate pain in bone cancer patients.
Essential tremor is often an inherited neurological condition; those with a family history have a 50 percent higher chance of developing it. Although it primarily affects those over 40, it has shown up in young adults and children. In Connor’s case, her older sister developed tremors at age 19.
“I’d thought I was scot-free,” said Connor, 75, who sports soft wisps of turquoise in her short-coiffed gray hair. “There’s no warning they’re coming. They just arrive.”
Unlike Parkinson’s, essential tremor usually affects people only when they’re focused on a task or activity, whether it’s lifting a plate or holding a hairbrush. (Parkinson’s tremors, by comparison, typically occur when hands or limbs are at rest.)
To cope with everyday tasks, patients are often advised to try workarounds: Drink with a straw, shave with an electric razor, use address labels to fill out their name on forms. Weighted dishes and utensils, designed to be easier to grasp, are recommended.
Connor has made many of those adjustments since she was diagnosed in 2012. Her husband, theater entrepreneur Jim Anderson, has to button her buttons and put in her dangly earrings. She buys pre-chopped onions rather than risk slicing a finger while preparing vegetables. She clutches her coffee cup with two hands to quell the shaking. When using her ceramics tools, Connor tucks her hands and elbows into her torso, trying to steady the trembling. A lifelong right-hander, she taught herself to print using her left hand.
On a recent morning, Connor demonstrated the difficulty of seemingly easy tasks. In her self-described “second-grader” scrawl, she painstakingly printed a note with her left hand: “Today is Tuesday.” She then attempted to draw a spiraling circle, one of the evaluation tools used by doctors to diagnose essential tremor. Instead of a smooth spiral, Connor’s shaky hand rendered it as a jagged, chainsaw-like disc.
Paula Lavin, a retired county government manager in Roseville, first realized something was wrong while eating at a Chinese restaurant, where she kept fumbling with the oval soup spoon. She was diagnosed with essential tremor in 2000, not long after relocating from the Bay Area. Today, her voice has a distinctive warble. She can no longer write legibly, so she uses her iPad to slowly type out notes or phone messages. When eating a meal, she often wears a smock or towel over her chest to catch the inevitable spills.
“My entire calendar has to be on my iPad because if I write it down, it’s almost impossible to read,” said Lavin, 76, whose father had undiagnosed tremors. “If I was cutting up vegetables for soup, I can easily cut myself. It’s very hard to control a knife.”
About five years ago, Lavin started a support group that meets monthly at Kaiser Permanente Roseville. In many cases, it’s a matter of encouraging individuals with essential tremor to feel confident while out in public, she said. “For me, the idea of being stuck at home overweighs the idea of being embarrassed. But a lot of people (with essential tremor) haven’t been out to eat in years. They’re embarrassed to be around other people.”
Essential tremor is difficult to diagnose, say movement disorder experts.
“We don’t have imaging or a blood test that gives us a clear answer. It’s a clinical diagnosis, so it’s subject to human error,” said Lungu, of the National Institute of Neurological Disorders and Stroke. He said researchers are looking for genetic markers and other clues that could lead to easier diagnoses or possible genetic interventions.
The new guided-ultrasound treatment, which only recently became available in U.S. hospitals, isn’t cheap, costing an estimated $40,000 to $50,000. In November, Medicare announced it will cover about $10,000 of the cost. (Stanford declined to state the procedure’s price tag, saying it is “still being finalized.”)
Connor, who had to give up most of her teaching and poetry workshops, now relies on sculpture sales and her husband’s work as a teacher and founder of the Short Repertory Company to help cover the couple’s household bills and medical costs. A GoFundMe site has been set up to cover the estimated $50,000 cost of her Stanford treatment and hospital visits.
If her Stanford treatment works, Connor hopes she’ll be able to publish another book of poetry. Beyond that, she has one simple desire: “To pick up a pen or pencil and write a sentence. And make a list. … People have no idea how beautiful it is to make a list. ”
Essential tremor at a glance
What it is: Affecting an estimated 7 million or more Americans, essential tremor is a neurological condition that causes uncontrollable shaking, usually in the hands, arms, head or throat. Frequently misdiagnosed as Parkinson’s disease, it is not fatal but can make everyday tasks difficult if not impossible, such as drinking from a glass, tying shoelaces, using a fork, brushing teeth or writing a word. Unlike Parkinson’s, the tremors typically do not occur when hands are at rest, but when focused on a task or activity.
Who it affects: Often an inherited condition in families, it typically appears in those over 40 but can be found in young adults and even children. Among famous people affected are actress Katharine Hepburn, World War II Gen. Douglas MacArthur and rocker Ozzy Osbourne, according to the International Essential Tremor Foundation.
How it’s treated: Drugs, such as some anti-seizure and blood pressure medications, can help control shaking. Some physicians use botox injections. There’s also “deep brain stimulation” where an electrode is implanted in the thalamus of the patient’s brain, then linked to a pacemaker in the patient’s chest to help subdue shaking.
New approach: Under a new treatment approved last summer by the FDA, patients are placed in a MRI scanner that targets a precise spot in the brain, then high-intensity ultrasound beams are used to heat and kill tissue in a tiny area believed to cause tremors. Requiring no anesthesia, patients are immediately tested to see if there’s a reduction in tremors. The procedure continues until satisfactory results are achieved. It can only be used on one side of the body, so patients usually choose the side most affected. In U.S. clinical trials, patients showed nearly a 50 percent improvement in tremors three months after the procedure.
Support groups: The International Essential Tremor Foundation lists support groups, such as one in Sacramento that meets monthly at Kaiser Permanente Roseville Medical Center. For details, contact organizer Paula Lavin at email@example.com.