Dianne Zabel

Special education teacher gets help when tremor threatens to keep her from the classroom.

For 30 years, the first day of school in Mrs. Zabel's elementary special education class in Melbourne, Fla., always started the same way. She stood in front of her apprehensive new charges and extended her arms before her.

"You'll notice that my hands shake and my head shakes. Like you, when I was born, something went wrong in my brain," she would tell them. "Like you, I didn't do anything to deserve it or to cause it. And like you, I have to make the best of it. I'm here to show you how to do that."

School can be a harrowing ordeal for children in special education classes. "Special" hardly describes the treatment they can receive from other students. Dianne Zabel, now 60, knows it all — the looks, the taunts, the cruel copying of imperfect physical motions. Her frank embrace of differences and difficulties, and her pledge to see her students through it all, functioned like a class mission statement.

But as her condition progressed, the tremor threatened to get in the way of her mission. Dramatic action would be required for her to continue her teaching.

In her 31st year of teaching, a new brain surgery procedure performed at Mayo Clinic Jacksonville stopped Dianne's disabling shaking caused by an inherited disorder known as essential tremor. And, for the first time since fifth grade, her hands no longer shake when she performs a task. But it doesn't stop her opening day embrace of differences.

"I call these my horns," she now tells her students, patting the two small knobs on the top of her head just behind her hairline, where the scalp was replaced after electrodes were surgically implanted into her brain. Though her hair is artfully arranged to conceal the small bumps, there's never been any hiding in Dianne Zabel's class, and she isn't about to start.

Then she tells her students about her different brain; about the wires a surgeon implanted; about the battery the size of a playing card under her skin, just below her collar bone, that the wire connects to; about the way it reorganizes the electrical circuits in her brain to stop the shaking.

"I can be of most use to these children by showing them I know — really know — what it's like to be different. Then they can trust me. And that's where true learning begins, where there's trust," she says.

For Dianne Zabel, trust also is where true healing begins. "I never imagined I could live the life I'm living today. I thought I'd always shake," she says. "But when the shaking got so bad it looked like I'd have to give up teaching, I decided to trust my care to three people — Dr. Robert Wharen Jr., Dr. Ryan Uitti and Nurse Margaret Turk — and Mayo Clinic, and it changed my life."

The change begins

On Dec. 30, 1999, Dianne Zabel lay on an operating table at Mayo Clinic Jacksonville, her head immobilized by a halo restraint. Eyes wide open behind the blue surgical drape, she waited for surgery to begin. There was no feeling, no pain.

Mayo Clinic neurosurgeon Robert Wharen Jr., M.D., worked on her skull while neurologist Ryan Uitti, M.D., held Dianne's right hand. Nurse Margaret Turk held her left.

"I couldn't have done it without those three people right there, right where they needed to be, holding my hands and doing the surgery," Dianne recalls.

Drs. Uitti and Wharen were among the first physicians in the United States to perform a procedure called deep brain stimulation, which had recently been approved by the U.S. Food and Drug Administration.

The procedure requires the implantation of a tiny device called a thalamic stimulator deep within the brain's message-relay center, the thalamus. This corrects the debilitating shaking of hands and head caused by essential tremor, an inherited movement disorder. In addition to brain surgery, patients have a pacemaker-like unit implanted under the skin on the chest. The pacemaker transmits electrical pulses through a wire threaded under the skin to a lead connected to the thalamus stimulator. The electrical pulses it delivers correct irregular brain circuits that cause the shaking.

If the surgery worked, Dianne Zabel could finally revel in the ordinary things most people take for granted — things like going alone to the grocery store, confident that she would be able to sign a check legibly, and eating in public without worrying about stabbing herself with a fork.

And if the ordinary efficiencies and pleasures of life weren't enough motivation for her to face this daunting surgery, there were the extraordinary children she teaches. They gave her courage. She had a mission to fulfill. If she didn't try deep brain stimulation, the progressive nature of essential tremor would end her teaching career.

About essential tremor

Essential tremor is an inherited movement disorder that affects an estimated 5 million people in the United States, most of them age 60 or older. It can affect almost any part of the body. But it most often affects the hands — especially during focused use of the hands during simple tasks such as writing, cutting with a scissors or shaving. In some cases, the head, voice and arms are also affected and tremble.

Although the trembling symptoms can be confused with Parkinson's disease, essential tremor is fundamentally different. Unlike Parkinson's disease, essential tremor has a strong tendency to run in families. In essential tremor, the trembling is most pronounced during use of the hands. In Parkinson's disease, the trembling occurs when the hands are resting or fully supported. With essential tremor, the shaking gets progressively worse over time but does not lead to other serious complications. In Parkinson's disease, the shaking is but one symptom. Others develop over time, such as stiffness, balance problems and loss of facial expressions. Essential tremor can occur at any point in a person's life and progress slowly. Parkinson's disease is strongly associated with advanced age. Essential tremor is five to 10 times as common as Parkinson's disease.

What goes wrong in essential tremor? Dr. Wharen explains it this way: "We know that there are some circuits in the brain that are firing abnormally. And if we interrupt this abnormal firing the tremor stops."

There is no known treatment for early cases of essential tremor that could blunt its progressive nature. While several medications are available that may reduce tremor, they often have side effects, such as grogginess, sedation, depression and low blood pressure, that can be as disabling as the shaking.

Certainly that was so in Dianne Zabel's case. "I felt like I was drunk," she recalls. "Between the shaking and the treatment I'd gotten for it, life was getting really difficult."

When a doctor mentioned to her in 1997 that a new procedure called deep brain stimulation was available, and that nearby Mayo Clinic Jacksonville was one of the first places in the world to perform it, she jumped at the chance. She and her husband, Al, drove to Jacksonville, and met Drs. Uitti and Wharen. She booked a hotel room, preparing for a full evaluation.

Then she froze. She thought of dying during surgery while her two children, Shane and Jill, were still in college. "There was no way I could risk it: they needed me."

She canceled the surgery and returned home. She continued teaching her special students with the same deep and resourceful spirit that had always gotten her through her challenges. She held scissors against her stomach to steady her hands as she cut. She placed her left hand on her right to guide it as she wrote report cards. Though parents complained of her handwriting, she got through it.

Finally, when Shane graduated from college in 1999 and Jill began a teaching career, Dianne was ready to face her fears — and pursue her hopes of regaining hand function so she could continue teaching.

The surgical turning point

During surgery, Dr. Wharen carefully checked for the misfiring circuits in Dianne's brain. She felt no pain or pressure. She looked into Dr. Uitti's eyes, then into Nurse Margaret Turk's, for calm and courage. She thought of her family, her students. Then she spoke.

"Is Dr. Wharen still here?"

Dr. Uitti smiled and squeezed her hand to reassure her. "Yes, he's still working. He didn't leave us all here."

Then came the turning point. The site to be corrected had been identified. To be sure that Dianne's cognition and language centers were not affected by the implant, the surgical team needed to talk to her, which is why the surgery is performed with the patient awake, though sedated. Proper responses to physicians' questions are proof that no vital communication circuits have been disturbed.

"Please tell me your name," Dr. Uitti asked her.

Dianne hesitated. Again Dr. Uitti gently prodded, "Please tell me your name."

Again Dianne hesitated. Finally, at a third prodding, she replied to Dr. Uitti in her best classroom storytelling voice: "What is my name!? Why, Rumplestiltskin is my name!" quoting the Brothers Grimm fairy tale that she has for decades read to students.

This might have been cause for alarm in another patient. Not so with Dianne Zabel. The medical team was relieved to hear her pluck and charm and humor were as active as ever.

Moments later, Dr. Uitti asked her to hold up her right arm. This time she complied at the first request, slowly raising the quavering limb. Dr. Uitti was adjusting the stimulator in her brain. As he did, Dianne Zabel's right hand stopped shaking for the first time since she was 10 years old. It simply extended out before her, straight and calm and steady, where seconds before, it was shaking.

Said Dr. Uitti: "Ladies and gentlemen, it doesn't get any better than this."

"I was almost speechless with joy, it was so incredible," Dianne says.

Dianne left the hospital 24 hours later. She drove with her husband the 200 miles to her home in south Florida. There, she entered the new millennium feeling like a new woman, with new, steady hands.

"It was so wonderful!" she says. "I drank hot coffee from a mug! I ate soup from a spoon without spilling! And the biggest thing of all, my husband and I went on a cruise and I ate at a table with 10 strangers.

"When you shake, you stay in because it's too embarrassing and you're always self-conscious. But now I could do things I'd never even imagined, things I had always been too afraid to do because I knew I would fail, my hands were shaking so badly. But finally they work.

"I sometimes think God put me here this way to do this work with special children," Dianne says. "I can really help these children. And when my hands aren't shaking, I can really go to town."

(This story first appeared in the April 2004 issue of The Mayo Clinic Checkup, a complimentary newsletter available to anyone interested in the latest news from Mayo Clinic in Jacksonville, Fla.)