Conquering a life of immobility
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
What is Stiff-Person Syndrome?
Stiff-person syndrome is a rare progressive neurological
disorder characterized by constant painful contractions and
spasms of voluntary muscles, particularly the muscles of the
back and upper legs. Symptoms may occur gradually, spreading
from the back and legs to involve the arms and neck. Symptoms
may worsen when the affected individual is anxious or exposed to
sudden motion or noise. Affected muscles may become twisted and
contracted, resulting in bone fractures in the most severe
cases. Individuals with stiff-person syndrome may have
difficulty making sudden movements and may have a stiff-legged,
unsteady gait. Sleep usually suppresses frequency of
contractions. Stiffness may increase and patients may develop a
hunched posture (kyphosis) or a swayback (lordosis). Researchers
theorize that stiff-person syndrome may be an autoimmune
disorder. Other autoimmune disorders such as diabetes,
pernicious anemia (a chronic, progressive blood disorder), and
thyroiditis (inflammation of the thyroid gland) may occur more
frequently in patients with stiff-person syndrome.
Is there any treatment?
What causes SPS? Well, it's been known for over 20 years that
most SPS patients have antibodies against the enzyme GAD65,
which is required for the production of GABA, the main
inhibitory neurotransmitter in the brain. The body shouldn't be
producing antibodies against its own proteins, but unfortunately
this does happen quite often, for various reasons, and the
result is autoimmune
diseases.
The drug diazepam, which relaxes the muscles, provides
improvement in most cases. Baclofen, phenytoin, clonidine,
or tizanidine may provide additional benefit. In some
patients, immunomodulatory treatments such as intravenous
immunoglobulin may be beneficial. Physical and
rehabilitation therapy may also be needed.
What is the prognosis?
There is no cure for stiff-person syndrome. The long-term
prognosis for individuals with stiff-person syndrome is
uncertain. Management of the disorder with drug therapy may
provide significant improvements and relief of symptoms.
What research is being done?
Research on stiff-person syndrome is aimed at enhancing
scientific understanding of the disorder and evaluating new
therapeutic interventions
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