From the Yakima Herald-Republic Online News.


Posted on Saturday, November 13, 2010

A quest for equilibrium
By David Lester
Yakima Herald-Republic

 

YAKIMA -- Gene Pugnetti climbed mountains, big mountains in Tajikstan and Alaska.

He ascended Mount Rainier 18 times and climbed others in the Pacific Northwest's volcanic ring of fire: Mount St. Helens and Mount Hood.

He gave up the sport when he became a husband and instant father 15 years ago after reconnecting with a high school sweetheart at a class reunion.

Little did the 56-year-old computer expert for Yakima County know that he would have to climb yet another mountain, one he couldn't see or touch.

Pugnetti has Meniere's disease, an illness of the inner ear that causes debilitating vertigo -- the violent, carnival-ride spinning kind -- and nausea that lasts for hours. Meniere's results when a membrane in the inner ear ruptures, upsetting the brain's perception of balance. Other symptoms include ringing in the ears and hearing loss.

Nobody knows what causes it, and there is no cure.

Pugnetti's diagnosis about eight years ago began what would become a ground-breaking, indeed history-making medical journey.

Last month, physicians and researchers at the University of Washington Medical Center in Seattle implanted a device into Pugnetti's head that's supposed to control the attacks, making him the first such patient in the world.

It's not certain yet how well the device -- a modified cochlear implant -- will work. Initial tests more than a week ago proved promising, but doctors say more are needed.

But the procedure offers a big promise for permanent relief from the chaos that is Meniere's disease. Other balance disorders may also be helped by the device.

 

Pugnetti is one of an estimated 615,000 people in the United States with the disease, and another 45,000 are diagnosed each year, according to the National Institutes of Health.

In his pioneering journey as a patient, it helps that Pugnetti is a big believer in technology. He runs the county's security and network operations and enjoyed a successful career at IBM before taking a buyout in a reorganization.

"If it works, it is an answer for those suffering vertigo. That is a pretty cool thing," he said, sitting in the living room of the family home that offers a panoramic view of Yakima and the mountains beyond that he once climbed.

His doctor, Jay Rubinstein, a UW ear surgeon and auditory scientist, is also optimistic but cautious about declaring victory. Still, he said Pugnetti's status as the first recipient of the modified implant classifies him as a trailblazer.

"What we have learned from him is we can safely place such a device and have the suspected effects when you activate it. What remains to be seen is whether we can treat Meniere's," he said. "If we can't, we will learn critical information to treat other balance disorders."

Pugnetti, the son of a longtime newspaper editor at the Tri-City Herald and Tacoma News-Tribune, first experienced Meniere's about nine years ago during an attack that left him helpless.

"It was quite severe," he said of the vertigo and nausea. "It lasted two to three days and went away. With hindsight, you can look back and say that was probably the first attack of Meniere's."

He thought it was an ear infection and so did his doctor. But the attacks continued, albeit infrequently.

"It is such an unpredictable disease. It is difficult to diagnose and the fact that there is no known cause makes it insidious," Pugnetti said.

By then, he and his wife, Phyllis, had moved to Yakima. The attacks, meanwhile, became more regular. Another visit to his doctor led to the diagnosis by an ear, nose and throat specialist in 2006.

 

To cope with the attacks, Pugnetti learned to control the nausea by sitting quietly, his hand placed firmly over his eyes, which would twitch furiously.

He recalled one attack while he was in the basement of the county courthouse. After making it up the stairs, he sat down on the steps outside the Second Street entrance, covering his eyes.

Sensing he had had a bad day in court, a female passer-by stopped and said, "God still loves you."

Valium helped with the vertigo, but the life he and Phyllis had built was changing. Phyllis, who worked for 20 years as an optician before earning a degree in sociology at UW, became a partner in dealing with the disease.

She prepares his meals from scratch, avoiding packaged and processed foods to limit sodium -- a trigger for attacks -- to about half a teaspoon. He had to give up red wine, one of his enjoyments. And he rarely drives because of the chance of an attack.

"Because of the restrictions on what he can eat, we no longer travel," said Phyllis. "It is difficult to visit family because I have to take food or take people out of their kitchens. A dinner with family can be a little bit difficult."

 

Even with the dietary changes and coping methods, the disease continues to torment Pugnetti, not least because it poses the threat of a serious injury in a fall.

"People don't understand the severity of this illness," Phyllis said. "This lasts for hours. It is not uncommon for vertigo to last eight to 10 hours or longer."

Looking for answers, Phyllis found Rubinstein through a Google search for the best physicians for balance disorders. A phone call led to a consultation.

The first step was to install a shunt in the inner ear, but that provided only temporary relief.

Another possible route was surgery to shut off the ear, cutting hearing in his right ear from 45 percent to nothing.

But Pugnetti latched onto something Rubinstein had told him about implant research on monkeys at the UW primate research center. They decided to try it.

Rubinstein said Pugnetti is a perfect candidate for the implant because of his analytical background and ability to articulate his symptoms.

With Dr. James Phillips of UW, Rubinstein developed the modified implant device, adding software and electrodes. A National Institutes of Health grant aided development and the initial testing on monkeys. The U.S. Food and Drug Administration approved the device earlier this year.

Pugnetti likens the device to a pacemaker for the inner ear.

The implant is made up of three electrodes inserted into the semicircular canals of his inner ear, a separate processor he will wear over his ear, and a handheld device that will let him send an electrical charge through the electrodes.

 

When an attack begins, Pugnetti will put on the processor and operate the remote, which he must carry at all times. The electrical charge to the electrodes is designed to counteract the vertigo symptoms. It works like this:

During a Meniere's attack, the balance center in the inner ear tells the brain that the body is spinning. The electrical currents will interfere with that message.

"This is all in theory," Pugnetti said. "The theory is you can send signals that will cancel out the signals sent to the brain."

Pugnetti says he's been able to deal with the disease with help from his wife and an understanding boss, George Helton, director of technology services for the county.

Theirs has been a long journey, but one the couple views more as a lifestyle change than a sentence to a limited future.

Pugnetti is glad he can blaze a new trail, even though it's not literally to a summit this time.

"It will be a route for someone who wants to climb the mountain. They will have a trail," he said. "I have marked it out for them."

 

* David Lester can be reached at 509-577-7674 or dlester@yakimaherald.com.

Gene Pugnetti suffers from Meniere's Disease which causes severe dizziness, nausea and hearing loss. He may, however, be able to control the disease with the help of a device implanted behind his right ear. He's first person ever to receive such a device.
GORDON KING/Yakima Herald-Republic
Gene Pugnetti suffers from Meniere's Disease which causes severe dizziness, nausea and hearing loss. He may, however, be able to control the disease with the help of a device implanted behind his right ear. He's first person ever to receive such a device.
A device implanted behind Gene Pugnetti's right ear may control his bouts with Meniere's Disease, which leaves him dizzy and nauseous. He's the first person in the world to receive such a device.
GORDON KING/Yakima Herald-Republic
A device implanted behind Gene Pugnetti's right ear may control his bouts with Meniere's Disease, which leaves him dizzy and nauseous. He's the first person in the world to receive such a device.