Roger Hollenbaugh of Bandera suffers from Stiff-Person's Syndrome, a puzzling disease with no known cure and an uncertain long-term prognosis despite undeniably real symptoms. Photo by Jessica Hawley

Bandera resident learns to live with rare, debilitating disease

By Jessica Hawley - Staff Writer

Only a few years ago, Roger Hollenbaugh drove an 18-wheeler all over the United States and beyond the country's border into Canada. He enjoyed taking photographs of the vast and varied landscapes to capture the visions he rolled by at 70 mph.

"People thought I was nuts that I would take pictures through the windshield," he said.

Today, the 37-year-old carefully thumbs through those pictures with rigid fingers, his trucking days a memory. In September 2001, after suffering for more than two years from symptoms that doctors labeled psychosomatic, Hollenbaugh was diagnosed by Dr. Michael Winkelmann, in Jackson, Miss., with stiff-person's syndrome, an extremely rare and, as of yet, scarcely studied neurological disease.

Although the cause of stiff-person's syndrome is unknown in most cases, Hollenbaugh believes his case developed while he was receiving treatment for a migraine headache in a Kerrville hospital on July 10, 1998.

He stated that the hospital staff did not lock the rails on the cardiac bed they placed him on and it collapsed. He said that he hit his spine on the base and split open the back of his head on a cabinet he flipped onto.

According to Hollenbaugh's account, doctors shaved the back of his head, stapled the laceration closed and sent him home the same day.

"They didn't give him any antibiotics or even clean the wound," said Hollenbaugh's aunt, with whom he lives in Bandera.

For several months after the accident, he experienced post-concussion pain and possibly a bacterial infection that moved into his spinal column. Hollenbaugh soon noticed that the left side of his body began to immobilize. He couldn't pick his leg up to bend his knee and the nose of his shoes wore off before the sole showed signs of wear.

Hollenbaugh also experienced an increased sensitivity to loud noises. The sound of an ambulance or police siren sent shocks throughout his system and the left side of his body completely locked up.

Despite repeated and consistent complaints to doctors, he was told that his symptoms were imaginary. He was treated for pain management and nothing more.

Dr. Art Leis, a neurologist and senior scientist at Methodist's Center for Neuroscience and Neurological Recovery, said that Hollenbaugh's condition went undiagnosed because he had a disease that many doctors have never encountered.

According to information from the National Organization for Rare Disorders, doctors from Yale University have theorized that stiff-person syndrome may be an autoimmune disorder in which the body's natural defenses against invading organisms attack connections in the brain and spinal cord where gammaaminobutyric acid (GABA) flows between nerve ends, helping to modulate the central nervous system.

Leis explained that when the body has too little GABA, there is nothing shutting down the nervous system.

"When startled, your body freezes and becomes firm like a rock and you fall like a piece of lumber," Leis said.

Hollenbaugh has undergone a battery of treatments, including plasmapheresis, a procedure that separates blood cells from plasma to remove unwanted toxins and metabolic substances from the blood. The plasma is exchanged with other human blood and then retransfused back into the body. He also had between three and four inches of his spinal cord shaved off and replaced with a titanium capsule that feeds liquid medication to his body from a pump surgically implanted in his lower abdomen.

All of these measures are meant to treat and maintain his symptoms. There is no known cure.

Late last year, Hollenbaugh lost complete use of his legs. He needs a customized motorized wheelchair to fit his six-foot-four-inch frame because he does not have enough upper body strength or coordination to move a manual wheelchair.

He wears an eyepatch to eliminate the double vision that onset last Christmas and his speech has become slow and slurred.

"Some days it's really hard to understand me and other days it's okay," he said. "It frustrates me. People treat me like I'm stupid because I sound like this, but I'm not."

Because neither Medicare nor Medicaid will cover the costs of a disability van, he and his aunt have improvised with an elaborate homemade pulley system to get him in and out of her pick-up truck. But after the 10 minutes it takes just to get him seated, Hollenbaugh is left exhausted and out of breath.

Last week, Guaranty Bank set up a donation fund account for a van.

"A new van costs $40,000. All I need is something used," Hollenbaugh said.

No matter how bleak life may seem at times for Hollenbaugh, his spirit lifts when he talks about his two-year-old black chow Shadow.

"He's my baby," Hollenbaugh said. "When I'm sleeping, if my hand falls off the bed, Shadow licks it until I pick it back up. He's very protective."

Hollenbaugh JAH