INTRACRANIAL HYPERTENSION RESEARCH FOUNDATION
WHAT: A nonprofit patient registry, research foundation and information clearinghouse.
WHERE: Based at Oregon Health & Science University.
WHO: Emanuel Tanne, M.D., and his wife, Amy, started the foundation after their daughter was diagnosed.
WHAT’S NEW: The foundation won a $25,000 grant from Chase Community Giving in a round of Facebook voting and wants your second-round vote to get $1 million for research and registry development.
RELATED STORY:The $25,000 prize and $1 million hope.
CONTACT/VOTING: Go to http://www.ihrfoundation.org and follow the Facebook link.
Portland, OR PORTLAND — Jessica Tanne’s headaches were beyond horrible. The Vancouver native was at college in New York City when she became debilitated by brain pain that knocked her life out from under her. She couldn’t study. She couldn’t walk. She lost weight. She wound up hospitalized.
That was in 1995. What followed was three years of doctor visits, medical tests and despair.
“Even though I’m a physician, I didn’t know what to tell her and neither did many other doctors who saw her,” said Jessica’s father, retired Vancouver ophthalmologist Dr. Emanuel Tanne, 73.
It took a spinal tap to arrive at a diagnosis. Experts watched Tanne’s cerebrospinal fluid climb higher and higher in a tube, driven by abnormally great pressure — just the way you’d watch a thermometer indicate extreme heat on a 100-degree day.
That abnormal pressure is called intracranial hypertension. Tanne now takes nearly two dozen tablets of medication per day to keep it in check. And her parents have formed a nonprofit foundation and patient registry at Oregon Health and Science University to study, publicize and combat this very rare disorder.
How rare? Emanuel Tanne estimates there are 25,000 Americans with the condition. That’s almost certainly a low-ball estimate, he adds, because the condition is not widely known, often misunderstood or misdiagnosed, and sometimes triggered by diverse underlying situations, from brain tumors and strokes to diseases like lupus or leukemia. In Jessica Tanne’s case, the spark was a prescription acne medication containing a super-high dose of vitamin A — a common culprit.
“There are a number of drugs you have to be careful with,” he said. The first known description of intracranial hypertension, he said, is embedded in the 16th-century journals of a group of Arctic explorers who were trapped with nothing to eat but polar bear meat — which is loaded with vitamin A.
Although there can be many triggers for IH, nobody knows the underlying cause. Since the brain is constantly producing fresh cerebrospinal fluid, the problem may be a malfunctioning natural drainage system that fails to keep the system in balance.
One of the toughest things about IH is that it’s invisible. It tends to strike obese women — leading to an unfortunate stereotype — but it can strike anyone at all, including the very slender Tanne, 36.
“It’s a rather cruel paradox,” Tanne said. “They lose their sight, they are completely debilitated by pain, their families don’t always understand.”
“Patients don’t look ill but they are suffering terribly,” said Tanne’s mother, Amy Tanne. Extreme pain has them landing in emergency rooms or running to different doctors in search of relief. And baffled doctors may eventually shrug those problem patients off, concluding they’re actually hooked on painkillers or otherwise can’t be helped.
“It is a very traumatic process,” said Amy Tanne. “As a parent going through this, it is terribly frustrating because doctors know so little about it.”
Also traumatic is the one sure-fire way to treat IH: draining the fluid to relieve the internal pressure. The relief can be immediate, Tanne said, but since the body is always generating new spinal fluid, repeated surgeries — spinal taps or installation and maintenance of failure-prone shunts — may be necessary.
And that can lead to a whole new set of painful problems, from scarring to astronomical medical costs.
The only medication that’s widely prescribed for IH is acetazolamide (trade name Diamox), which suppresses an enzyme crucial to cerebrospinal fluid production. But it was developed for other purposes decades ago and does not always work with IH. It can have serious side effects, from gastric irritation to kidney stones and potassium deficiency.
“There is no good medication and there is no cure,” he said.
Even if you accept the low-ball number of 25,000 people in the U.S. living with intracranial hypertension, Emanuel Tanne said, the medications, surgeries, doctor visits and other costs of the condition add up to an estimated $444 million per year. The IHRF Web site got hits from people in 99 countries in one recent week, he said.
Meanwhile, the main mission of the Intracranial Hypertension Research Foundation is the development of its patient registry: a growing database of detailed medical records of people who live with the condition. Those records are a gold mine of data just waiting to be tapped by IH researchers, Emanuel Tanne said.
Right now there are more than 1,400 people in the registry and a handful of studies under way — like analysis of shunt failures, drug safety for fetuses, new diagnostic tools and neuro-imaging, direct and indirect costs, management of IH patients in emergency rooms and patient perceptions of treatment outcomes.
To people who get a diagnosis and barely know what to make of it, the registry and the foundation at OHSU — a cramped little office on the seventh floor of a physical-plant building — represent something more: community. It’s comforting to know there are other IH patients out there. The annual conferences the foundation sponsors for IH patients and their families are “emotionally charged,” Tanne said.
“Talking to other patients, recognizing you’re not alone, is really powerful,” said office volunteer — and IH patient — Ralpha Staeger. “Everybody wants to know, ‘When’s the next conference?’”