Oregonian: Exploring a Medical Mystery
March 4, 2005
by Dee Anne Finken
(Editor’s Note: This story originally appeared in the Oregonian)
Emanuel Tanne toils away on Oregon Health & Science University’s Pill Hill in the shadows of the big hitters, the scientists and physicians whose discoveries are changing the medical world.
Tanne, by comparison, is no big gun. A retired ophthalmologist from Vancouver, he doesn’t regularly win hefty research grants or make national headlines. His efforts, however, are aimed at raising money for research and setting up a registry that could change the lives of thousands of people around the world who have a little-understood disease, a neurological mystery that has stumped physicians for ages.
The puzzle is intracranial hypertension, an agonizing disorder. It occurs when a person has too much cerebrospinal fluid—the liquid that cushions the brain inside of the skull and that transports nutrients to brain tissue and carries away waste. The additional pressure from the extra fluid can result in vision loss, blindness and neurological deficits that can cause a person to stumble or fall. Most often, IH manifests itself with severe and debilitating headaches.
“Picture a migraine headache times 10—and sometimes plus that,” said Fran Stuart of Salem. “Sometimes they get so bad I can’t get up.”
Suffering like that is what drew Tanne out of retirement and prompted him and his wife, Amy Lois Tanne, to become involved. The Tannes’ adult daughter suffers from IH.
“She was very sick, and there was just very little research and very little public awareness about the disease, and so we felt we needed to do something,” Emanuel Tanne said.
So in 2001, the couple founded the Intracranial Hypertension Research Foundation, the only nonprofit organization in the world devoted to supporting medical research of IH. In conjunction with the Casey Eye Institute at OHSU, the Tannes also set up a medical registry to collect the records of people with IH. The Intracranial Hypertension Registry is housed in an office affiliated with the Casey Institute on Portland’s Marquam Hill. Tanne said one of the main problems for an IH sufferer is loss of vision because of swollen optic nerves, so hooking up with the institute was logical.
“They have an interest in IH,” he said. “Because of vision complaints, as well as severe headaches, often a patient will present first to an ophthalmologist.” Those doctors specialize in eye disease.
The registry—the only one of its kind and containing medical records of more than 400 patients around the world—supports researchers working for a cure by providing a growing database of information on sufferers.
“The foundation is a tremendous resource, and the registry is a tremendous idea,” said Dr. Steven E. Katz, an Ohio State University neuro-ophthalmologist who is the leader in IH research. “By registering patients on a national level, it really broadens our ability to do clinical research studies.”
The registry also is involved in research at the University of Arkansas to find an animal model for IH study. Ultimately, Tanne said, physicians hope to establish a comprehensive patient care and research center in the Portland area, perhaps next to OHSU. Researcher access to the kind of information the registry provides, Katz added, is particularly important given that “We haven’t made a significant advancement of our understanding or treatment of this disease in 100 years.”
Various researchers’ estimates vary about the number of IH sufferers, from one in 100,000 to 20 in 100,000. Overweight women of child-bearing age tend to have higher numbers, but it can affect infants.
Over the years, Katz has observed greater numbers of IH patients, a development he attributes at least in part to the nation’s increase in obesity.
“It’s probably not the only factor, but it’s one of the factors,” he said.
Stanley Fishman, a retired anesthesiologist who serves as the registry’s medical director, said it’s unclear how many people have IH, but certainly “IH is a condition that is underrecognized, underappreciated, underreported and underfunded.”
Most commonly, IH is idiopathic, meaning there is no known cause, and that form generally affects the child-bearing-age women. IH may occur secondary to systemic disease, such as lupus, or to a drug reaction, most often tetracycline, Katz said.
Often a patient with IH can go years without a proper diagnosis. And even once it is pinpointed, a patient can get lost in the shuffle of medical specialists.
“Nobody really focuses on the needs of the IH patient,” Fishman said. “Often the patient will see quite a variety of specialists and tend to have to go from one to another.”
Stuart, a 47-year-old office specialist, is well aware that there is no known cure for IH. Last year, she had her worst IH-related experience. A shunt her physician had inserted from her skull into her stomach to carry off excess fluid got infected, and she developed pneumonia. Because of complications, doctors had to induce a coma to keep her body stable.
When she awoke 13 days later, “I had forgotten how to stand and how to walk. I had to go through rehab,” she said.
During the past 12 years, she has had 88 spinal taps—an often-painful procedure in which a long needle is inserted into the spine—to ease her IH symptoms. She now undergoes acupuncture to help relieve her body of the aftereffects of last year’s incident.
“Basically, you get too much pressure in your head, and the only way you can relieve it is by surgery or spinal tap,” Stuart said. The 6- to 8-inch needle allows a physician to draw off excess fluid. Medication is another treatment option, but the drugs have drawbacks.
As researchers have begun to turn to the database for more information, Fishman said the registry has been able to collaborate with them to turn out research reports, which he hopes will garner more attention. That, he said, could prompt more research, which could lead to more reliable drug options and—the ultimate goal—a cure.