Her husband calls Kathleen Ruth-Tepper a hard-headed woman.
And he means that figuratively and literally, as her determination finally led her to a solution for the tremors that caused her hands to shake so severely, she could no longer cook, sew, play the piano, button a blouse or put on earrings.
The fact that her skull was a specific thickness made it possible for the innovative treatment she received at UCHealth which used high-intensity focused ultrasounds (HiFU) targeted at specific parts of her brain causing her tremors.
“Fortunately for me, the test I had to undergo before the procedure showed I have a very hard skull. Yay!” Kathleen said with a laugh and then looked at her hands
“This is so important! I can sew buttons; I even threaded the needle. I can garden. I can do crafts with my grandchild, and I’ve got more energy to spend with her.”
Bill, her husband of 20 years and a retired home builder and developer, agreed: “She’s almost a different person now.”
A Denver native, Kathleen, now 73, has dealt with tremors since she was a teen.
“I knew I had it. My mother had it, my grandfather and great-grandfather had it. They used to call it ‘familial tremors.’”
What Kathleen and her family members suffered from was essential tremors, a neurological condition that causes hands to shake, usually one worse than the other. It can also affect other body parts such as the head and voice. For more than half of the people who have it, the cause is genetic and is inherited from a parent. For the other half, the cause is unknown, though stress and anxiety can increase tremor frequency.
Kathleen’s adult son also experiences minor essential tremors – another crucial factor that pushed her to find a cure so she could give him hope for his own condition. She has another reason to get her tremors under control: Bill has been diagnosed with Parkinson’s disease, and it is important she is able to assist him if his condition deteriorates. She also has three grandchildren, including a very active 6-year-old, who likes to play a lot.
Essential tremors begin to affect her quality of life
As she got older, Kathleen grew accustomed to her shaking hands and kept them out of sight when possible: in her lap or by her side. One hand, her left, shook more than her right.
“She was good at hiding it,” Bill said.
She gradually realized the tremors were becoming more noticeable. For instance, while living in Oklahoma and working in a job that required public presentations, the notes in her hand would shake during her speeches.
“I would think, ‘This isn’t good, they think I’m nervous, but I’m not.’’’
As the years passed, the shaking got worse and harder to conceal. She compensated with elaborate techniques to complete daily routines, such as holding a nail polish bottle with both hands and carefully bringing the brush to each finger with two hands.
When one nail polish episode ended with her inadvertently throwing the nail brush across the room, she faced up to how serious her condition had become.
“I thought, ‘That’s it. I’m done with this.’’’
More activities started to fall by the wayside as the effort to complete them became too difficult. Kathleen and Bill recalled that a trip to a restaurant might involve her food flying onto someone else’s plate – or face – as she attempted to bring the fork to her mouth.
“She’s always kept her sense of humor about this,” Bill said with a smile, as they both laughed.
But her optimistic and can-do attitude belied the despair she was beginning to feel. She had always been outgoing and an extrovert, but that was changing.
Her emotional and psychological health suffered, with the tremors taking a greater physical toll as she could feel them throughout her entire body.
“I was exhausted,” she said.
They were beginning to affect her vocal cords, altering the sound of her voice, and making it more difficult for her to be understood. The tremors were also causing spasms in her throat, and she was choking on food. Bill even had to perform the Heimlich maneuver when a piece of apple got stuck in her throat.
“Bill and I are happy together. I have a good life. But there was a part of me that was miserable. I was withdrawn. I never wanted to leave home. When I went to bed at night, I was so tired. The tremors were exhausting, in both hands and in my jaw. I was totally embarrassed. I think I was depressed.”
The latest treatment for essential tremors: learning about ground-breaking HiFU
Determination is one of Kathleen’s traits. Strength is another. In addition to dealing with her tremors, she has overcome traumatic brain injuries from two auto accidents, one dating back 28 years ago.
She also battled back from a severe case of sepsis caused by a kidney stone infection in 2017. The ordeal left her in a hospital intensive care unit for 40 days where she was on a respirator, followed by a month in an acute rehab facility where she had to re-learn how to swallow, sit, stand and walk. The tracheotomy and necessity for intubation during her illness exacerbated her hand and speech tremors.
“I’ve been tested. I’ve been challenged,” she said.
Before seeking help at UCHealth this spring, she was on various medications to control her tremors and had seen numerous providers as she searched for answers. Eventually, she was told she had Parkinson’s disease, (a very different disease as opposed to essential tremors), characterized by insidious onset of rest tremor, slowness of movement and impairment of gait. It is not uncommon for patients like Kathleen to be misdiagnosed.
Further investigation led her to the diagnosis of essential tremors. Medications were not resulting in an improvement in her everyday life, and she was referred to the University of Colorado Advanced Therapies in Movement Disorders on the Anschutz Medical Campus.
The specialized team there consists of many experts, all working together to provide cutting-edge treatment for movement disorders, including tremors.
After much discussion, Kathleen and the team determined that HiFU was a great option for her. With HiFU, a small area deep inside the brain that regulates movement is targeted through high-frequency ultrasound while the patient is awake in an MRI scanner.
The surgery involves an incision and an overnight stay, and results in at least a 70 percent immediate tremor improvement for patients.
UCHealth began treating patients with HiFU in 2020, but the procedure went on hiatus as medical experts prioritized treating patients during the COVID-19 pandemic. Surgeries began again last year when 23 patients successfully underwent the treatment, and the department has the capacity to annually help about 50 patients.
“The image-guided treatment we are using is fascinating,” said Mari Kelley, a UCHealth registered nurse and movement disorders navigator. “The patients come in with tremors at their all-time worst, as we ask them to not take their medications before the procedure, and they leave with a near-steady hand. It’s remarkable.”
Patients seeking help have tremors in their voice and head, but the majority have tremors in both hands, Kelley said.
The first step toward HiFU eligibility is a full neurological workup to determine an essential tremor diagnosis. If that is established, a patient is asked whether they have optimized other options, such as medication and physical and occupational therapies. If patients still feel that their tremors are not under control after exhausting these methods, they can continue with treatment consideration.
Tough decisions as Kathleen opted for treatment for essential tremors
For Kathleen and others, that means a CT brain scan to calculate skull density ratio (SDR). This is critical, as a patient must have an SDR of 0.4 or higher. Skull thickness plays an important role in how successful the ultrasound will be in destroying targeted brain cells causing the tremors, and creating lesions in their place so they don’t grow back. Kelley explained that the denser the skull, the more effective ultrasound waves can go through it. The result is a permanent lesion in the region of the brain causing the tremors.
About 80 percent of essential tremor patients meet this criterion, she said. Once a UCHealth care team determines they are good candidates, they must get a high-intensity MRI a few weeks ahead of the surgery to map the targeted site in the brain.
UCHealth is one of only five institutions in the country that take the extra step of using MRI tractography to specifically identify the affected brain cells, Kelley said. This includes 3D-modeling and imaging to visually represent the area of the brain that will be targeted with the ultrasound beams.
“We map out where we want to put the lesions and where we want to stay away from. That is why we are so successful at what we do,” Kelley said. “We are getting our targets exactly where we want to get them.”
With the brain mapping complete and the patient ready, the half-day procedure can commence. What does that mean for patients like Kathleen immediately before the procedure?
- They complete an additional neurological examination. A set of “spiral” drawings with a pen and paper is performed. Most can’t keep their hand steady when they draw shapes, since they have stopped taking their tremor medication. The neurology team wants their tremors to be at an all-time worst during the procedure.
- They undergo another brain mapping MRI to compare to the previous one.
- They are asked to complete a second set of spirals.
- And last, and certainly the toughest: Their hair is cut and then their head is completely shaved. Even the smallest piece of hair risks fire danger, as well as obstructing HiFU sound waves.
“This is very difficult,” Kelley said. “It’s particularly hard for women. Hair is part of our identity, it has power, and specifically for different cultures, that is even more true. Having your hair shaved is a big deal. With women, hair represents so much.”
For Kathleen, who had long, lovely hair she had grown out during the past few years, the thought of having her head shaved was a terrible one.
With Kelley’s help, Kathleen discussed the HiFU treatment with two female patients who had undergone it, and their experience bolstered her courage to schedule it for herself in April.
“After talking with them, I decided to do it. I could sense they were so happy. Of course, I was nervous because the procedure is newer and it’s my brain,” she said. “But I want to be able to help Bill in the coming years. I want my son to see me do this so he knows he could do it if he has to, and my grandchildren as well if they are faced with this.”
A few minutes before the procedure commenced, her hair was cut. She tied it in a pink ribbon and placed it in a plastic bag and plans to donate it to an organization that makes wigs for cancer patients.
“It was emotional for all of us,” Kelley said.
Then, Kathleen’s head was secured into a frame that locked into the MRI table to prevent it from moving for the next couple of hours. A cap, or halo, was placed around her head in which ice-cold water circulated.
An added challenge: She is claustrophobic, and the thought of going into a narrow MRI machine for several hours was daunting. She tried to meditate to help her relax, and thought of her father, who died three years ago.
“I could feel him with me there, telling me it was going to be OK,” she said with tears in her eyes. “You take the people who love you, and who you love, into battle because you are also battling for them. And that’s what I kept reminding myself. There were a couple times I thought I’ve had enough. I wanted out. But you can’t do that – you have to pull yourself together.”
Providing crucial encouragement was Kelley, who had been by her side since the very first visit and was with her during the entire procedure. “She truly cares and makes everything easier, Kathleen said.
Kelley offered encouragement, rubbing her feet, holding her hand and keeping her informed of the treatment progress.
“Having someone there makes a big difference,” Kelley said. “She did amazing. She’s like a rock star, and a role model for women advocating for themselves.”
Most procedures require about one or two brain lesions to greatly reduce tremors. The procedure begins with the HiFU beams on a low-heat setting lasting 12 seconds to ensure proper alignment, with one or two additional deliveries of HiFU waves performed to improve tremor suppression, as well as to avoid unwanted adverse effects. In general, a total of five to eight deliveries of HiFU waves are performed.
Finally, the temperature is increased to a level that destroys the cells contributing to the tremor. These are called, “treatment sonications.” The goal for Kathleen was two treatment sonications, one for her left-hand tremor and one for her voice.
The procedure is lengthy because after each HiFU wave is performed, the patient is brought out of the MRI machine for a brief neurological examination to check on strength, sensation, coordination and tremor evaluation. For instance, can they bring a spoon or bottle to their mouth? Can they make spirals on a piece of paper? How does their voice sound?
When the HiFU was complete, Kathleen was in disbelief. She accomplished something she hadn’t been able to do for decades: hold her left hand still.
A future without tremors
“I couldn’t believe it. I could not believe it,” she tearfully recounted. “I just kept looking at my hand. “
When she spoke to her son later that day, he was amazed at how easy it was to understand her. And more importantly, he told her how proud he was, a trait her husband attests to as well.
“It takes an incredible amount of courage to go through everything she has,” Bill said. “She’s very brave.”
Kathleen no longer chokes, and the tremor in her voice is gone. And while some patients experience follow-up problems with gait and balance, and may need physical or occupational therapies, Kathleen has had no negative side effects.
As federal guidelines require at least a nine-month time period between treating both sides of the body, she plans to return to UCHealth in January for her right hand.
“I’m a different person. I am not the old Type-A personality Kathleen. But I am a new person, and it’s exciting. I have told everyone at UCHealth that I would do anything to help them with this program because of what it has done for me.”