Doni Jones was the "mean old English teacher" who would make her students do plenty of homework to hone their writing, communications, and critical thinking skills.
She first noticed a slight tremor in her hands while grading papers or writing on the board in the early 2000s and knew it would eventually get bad enough to affect her teaching. She had watched both her mother and father struggle with tremors. They never got a diagnosis—her mom called it "palsy," and Doni assumed it was old age or Parkinson's that was causing her dad's tremors.
When her tremors started to progress around the 2008-2009 school year, Doni decided it was time to retire. It would be five or six more years before the tremors took a turn for the worse. She got online and found the official diagnosis: essential tremor. As a person who always wants to find the solution to a problem, she also started looking for a way to fix it.
Doctors don't know exactly what causes essential tremor. It can happen to anyone, and it tends to run in families. Someone with a tremor has a specific area of the brain where a pathway in the thalamus is disrupted. Researchers discovered that interrupting or destroying these tissues improves the tremor for many patients.
People often mistake essential tremor for Parkinson's disease, like Doni did with her dad. But there is one key difference: Parkinson's causes tremors while a person is at rest. Someone with essential tremor doesn't shake unless they are trying to do something, like eating or writing. However, much like Parkinson's disease, essential tremor can be very debilitating.
In retirement, Doni took up several hobbies, including family history studies, humanitarian work, and gardening. She also wanted to make quilts to give to her five children, 23 grandchildren, two great-grandchildren and to donate to others in her humanitarian work. But there was one problem. "You can't thread a needle with a tremor," she said.
It was disrupting other parts of her life too. She had a hard time eating and drinking and couldn't cook anymore. For a proud and independent woman who had always done that for her family, it was devastating. Her husband and children were patient and stepped in to help, but her new disability was taking away the life she loved.
"I felt like I was missing out on a lot of things," Doni said. "Like my life was just gone."
Initially, all she could find was information about deep brain stimulation (DBS). It's a common procedure that doctors use to treat essential tremor. DBS is safe and effective, according to John Rolston, PhD, a functional neurosurgeon who formerly worked at University of Utah Health. He works closely with Matthew Alexander, MD, a neurointerventional radiologist in the Departments of Radiology and Imaging Sciences and Neurosurgery at U of U Health, to help patients with neurological challenges like essential tremor.
"[With DBS] we create holes, then use imaging guidance to place probes in the right spot in their brain," Dr. Rolston said. "Then we connect the probes to a generator implanted in the chest, a lot like a pacemaker. It sends pulses to the spot and changes how the tissues behave."
This procedure is different from focused ultrasound because the tissues in the brain are not destroyed. You can increase or decrease the pulses as needed to get the right level of stimulation. You can also stop them at any time.
In a focused ultrasound procedure, surgeons find the tissues that are causing the tremor and focus the energy of about 1,000 beams right to that spot. Like a magnifying glass that concentrates sunlight and burns one small spot, that section of tissue is destroyed.
While any brain surgery has risks, focused ultrasound risks are low, Dr. Rolston says. "If someone has side effects, they will most likely be numbness, weakness, or balance and coordination problems. The area we're targeting is close to the part of the brain for sensation in your face and hands and for balance and coordination." That's why surgeons do this procedure with someone awake—they can see the change in the brain as it happens and check if side effects are occurring.
For Doni, the idea of DBS probes in her brain wasn't ideal. She kept looking for other options while taking medications that could improve her tremor and slow the progression of the disease.
Then she saw a news story about a man in England who had a focused ultrasound procedure— and his tremor went away completely. It was a brand-new treatment in the U.S., and at the time all she could find were hospitals in Florida and Nebraska offering it. As she was trying to find a way to get to Omaha to have it done, she found out it was also available at U of U Health—only a few hours' drive from her Cedar City home.
But there was another problem: her insurance wouldn't cover procedures at U of U Health. She went back and forth with her insurance, but they kept insisting that she could only get DBS done by doctors that were in their network.
"It was just day-to-day anxiety," Doni said. "I was so discouraged and so angry at the insurance company's lack of compassion. Aren't they supposed to help people? I'd get a little bit of hope that it was finally going to happen and that it would really change my life. Then I would get deflated when the insurance company would send me a letter denying it."
She even started thinking about paying for the procedure out of pocket, no matter the cost. Dr. Alexander and Dr. Rolston both advocated with the insurance company to get it covered and eventually they approved it.
Doni scheduled the surgery for March 2020, which happened to be right around the time that the COVID-19 pandemic started. Doni had waited so long and now a solution was in reach, but she would have to wait a couple more months to get it done.
In May, the surgery finally moved forward. Doni wrote her name and drew a wiggly spiral as best as she could with her tremor. Doni would repeat this assessment several times throughout her treatment. It helps doctors test how well the procedure is working in real-time. Then, nurses shaved her head while Doni joked that she and her husband (who is also bald) would be twins for a while. They numbed her skull and placed the helmet with four screws to keep her head completely still throughout the procedure.
Next, they sent her into the MRI tube and found the spot in her brain where they wanted to target the ultrasound beams. They did a short "test" beam and brought her out of the tube to write her name and draw the spiral again. If the surgeons see improvement, they know they're targeting the right area.
"I didn't feel any pain," Doni said. "I just laid there thinking, 'Could this really work?' And I knew it would."
After several times going in and out of the MRI tube and reassessing Doni's writing, the procedure was over. They asked her to sign her name one final time and draw a spiral. Both were perfect. She was able to go home the same day.
"It's pretty awesome to see the difference for the patient right away," Dr. Alexander said. "It almost seems too good to be true."
Immediately, Doni could cook, garden, write in her journal, and thread her quilting needle. She started telling everyone she knew about the procedure in the hopes that it could help others. Now, she gets calls from friends and even complete strangers who want to know about the miracle cure for essential tremor. She points them to the U of U Health website about focused ultrasound.
"Nobody should have to suffer with essential tremor when a cure is so close and available," Doni said. "That was the only thing holding me back from living my life. I just thank the university for giving my life back to me."