A life reclaimed

The latest chapter in the life of Brian Terhorst, the 49-year-old general manager of Chico’s KCHO Northstate Public Radio, could be described as one of miraculous redemption following decades of physical challenge.

In 1987, while attending Somoma State, he received devastating news. He’d gone to a neurologist to find the cause of his lingering troubles with basic physical movements such as running and walking, which had started when he was just 7 years old and living in northern Virginia. The neurologist performed 10 minutes of simple resistance tests and pronounced Terhorst had muscular dystrophy, or MD, an incurable disease that progressively weakens the muscles, including the heart, resulting in slow paralysis and early death.

“That night at the foot of my bed I lost it,” Terhorst said in a recent interview at KCHO. “I just wept like a baby.”

He underwent years of testing to find which of the nine major types of MD he had. The two most familiar, Duchennes and Becker, were shared by “Jerry’s Kids” on the Jerry Lewis muscular dystrophy telethons that aired from 1966-2010.

Many of the tests, he said, were painful, including the time doctors extracted a piece of his leg muscle for analysis. In the 1990s doctors told him they couldn’t find his exact type of MD, but that it would slowly worsen and eventually his heart would fail. There was no possible cure. “It was like a death sentence,” Terhorst said.

Even so, Terhorst went on to manage Nevada City’s iconic KVMR community radio station from 1996 to 2006. There he hosted a folk and acoustic show called Harmony Ridge that he continued when he became general manager at KCHO in 2007. (The show ended this past June.)

Simple tasks like walking became major ordeals and resulted in frequent falls. Just standing up after sitting was so excruciating that Terhorst avoided trips to the bathroom as much as possible. In 2004 he was forced to rely on a wheelchair to get around, which made life much easier physically but harder emotionally.

“I wondered if the staff at KVMR would see me as vulnerable,” he said.

It was about this time that he also began having trouble breathing. With MD the diaphragm slowly gives out, as do all the muscles closest to the body core. Terhorst wiggles his fingers and moves his hands and arms to show the muscles in his extremities are still flexible.

In June 2011, still searching for his particular type of MD, he was referred to a doctor at the University of California, San Francisco (UCSF). He traveled there with his then-fiancée, Gail Peters, a Butte College instructor and administrative assistant. Mysteriously, all the tests came back negative. Terhorst said he felt excited by the possibility that his death sentence might not be valid.

“I could see a little gap in the fence,” he said.

Terhorst was given further encouragement when a consulting San Francisco neurologist suggested he might have an entirely different disease: Pompe (pronounced like the ancient buried Roman city of Pompeii), also known as acid maltase deficiency, or glycogen storage disease type II. It is similar to MD, with the major differences being that it is treatable and many of its symptoms are reversible.

A movie about the real-life search for a treatment for Pompe called Extraordinary Measures and starring Harrison Ford had come out in 2010. Encouraged, Terhorst researched the condition on the Web.

“I thought, ‘Wow,’ all the symptoms fit. This might be what I have!” he said. Equally encouraging was the fact that Pompe does not affect the heart and Terhorst’s heart had always been fine.

On Dec. 30, 2011 Terhorst and Peters were married.

“I never saw Brian as someone with disabilities,” she said, “just someone who used a chair to get around.”

While still holding out hope of a new diagnosis, Terhorst said, he assumed his vows of “till death do us part” meant that he would be the first to die. Upon returning to UCSF in January 2012, less than a month after his marriage, doctors gave him demoralizing news: They did not believe he had Pompe.

Terhorst refused to accept the doctors’ conclusions and pleaded for them to dig deeper. He surmised the doctors had been looking for “early infantile” Pompe, which shows itself at birth. Terhorst believed he had the type that manifests itself in childhood or later, called “late onset” Pompe.

“I felt bad insisting the doctors check for the second type of Pompe, and didn’t want to tell them how to do their jobs,” Terhorst said. “But this was vital for me.”

The doctors relented and in a week they conducted a genetic test.

“The lab results took forever,” Terhorst recalled. Four months later, in May of this year, Terhorst was notified of the results: He did indeed have late-onset Pompe.

“That day Brian left a frantic message on my work phone saying, ‘Call me! Call me!’” Peters said. “I was worried something was wrong, but when I called he told me, ‘I don’t have MD; it’s Pompe!’”

They were both overjoyed and celebrated with champagne and a special dinner at Chico’s Cocodine Thai restaurant. A second follow-up test confirmed the diagnosis.

“I was so stoked after having lived with the MD diagnosis for 26 years,” Terhorst said. “I still wake up every morning and can’t believe I don’t have it.”

Both Terhorst’s and Peters’ families were also ecstatic and thought it was a miracle.

Terhorst’s treatment for Pompe begins later this month and will involve twice monthly transfusions of the liquid medication Lumizyme. Terhorst said the drug has four major benefits, three of which are that: it halts the progression of Pompe; it often improves the pulmonary muscles, which means he is looking forward to breathing normally again; and it improves the muscles that facilitate standing and walking.

“I’m hoping one day I’ll see his 6-foot, 4-inch body standing,” Gail says.

The fourth advantage is that Lumizyme allows users to live a normal lifespan. Terhorst is overjoyed.

“Every day we hear tragic stories of cancer victims, car crashes, etc.” he said. “Now I’m in the middle of a story that’s just the opposite. What an awesome thing.”

In the past, as his ability to lift objects and then even walk slowly faded, Terhorst said, he tried to remain positive and see the glass as half full.

“I decided to live a full life, and I feel the universe has rewarded me for it,” he said. “This is beyond a dream come true, and we are now both looking forward to a long life together.”