Brigham and Women’s Starting Adult Hydrocephalus Program

Normal pressure hydrocephalus (NPH) is a chronic neurological disorder usually occurring in older adults characterized by walking difficulty, trouble with bladder control, and dementia. But because these symptoms often arise during aging and are seen in other disorders, NPH is often undiagnosed or misdiagnosed as Alzheimer’s or Parkinson’s Disease.

More than seven million people in the United States have dementia, but according to Brigham and Women’s Hospital, between five and 10 percent of them, more than 375,000 Americans, may have undiagnosed NPH. But the scary part is that the number of Americans living with NPH is expected to double and approach one million over the next 30 years as the Baby Boomers generation ages. The Brigham estimates that treating hydrocephalus in the elderly population would reduce U.S. health care costs by about $25,000 per patient over a five year period, mostly by avoiding placement in nursing homes and other care facilities.

Dr. Mark Johnson, a brain tumor neurosurgeon at Brigham and Women’s says that most patients with NPH live with debilitating symptoms for many years before they are properly diagnosed. “Frequently patients and their caregivers attribute the symptoms to some other disease, like Alzheimer’s, Parkinson’s or just old age,” he says. “Unlike these other disorders, however, a relatively simple surgical procedure to place a shunt which drains the excess brain fluid can effectively relieve the symptoms of NPH, sometimes dramatically.”

Susan Sontag had terminal brain cancer in the mid-1990s and underwent an experimental treatment and survived. She and her husband Rick started the Sontag Foundation as result of their experience with brain cancer, and so far the foundation has given more than $40 million dollars to support brain tumor research and other worthy causes. But even though Mrs. Sontag survived cancer, her cognitive skills declined dramatically, and she had difficulty walking, among other things. Her family, friends, and even some doctors though that these were just side effects of the brain tumor and nothing could be done. But Dr. Johnson suggested that they look into NPH, and he was right.

Mrs. Sontag’s improvement was immediately noticeable. In February, she underwent a test procedure to drain her brain fluid on a temporary basis. When she arrived at the hospital, she was in a wheelchair because she could only walk short distances. Within the first three days after the test procedure, she was able to stand from a chair and walk briskly down the hallway on her own. Her cognitive function also improved. “I could see the old Susan from seven or eight years ago re-emerging before my eyes,” Dr. Johnson says. “It was like a second medical miracle had been granted to her.” After the successful test Dr. Johnson performed the shunt surgery to make the changes permanent.

As a result of this experience, the Sontags gave $1 million in seed grant funds to Brigham and Women’s Hospital to establish the Adult Hydrocephalus Program, the only one of its kind in New England. The program will improve the understanding, diagnosis, and treatment of NPH through advances in clinical care, public education, and ground breaking research.

“Brigham and Women’s Hospital and Harvard Medical School provide an ideal environment to implement the Sontags’ vision of a comprehensive, cutting edge clinical and research center aimed at improving the recognition and treatment of NPH,” Dr. Johnson, says.  “Hundreds of thousands of undiagnosed NPH patients and their caregivers stand to benefit from the public outreach, expert care and cutting edge research that their gift has made possible.”

The Sontags have been long-time supporters of brain tumor research, and are now committed to tackling NPH as well. “We’ve now twice experienced first-hand the devastating impact of a disease that is not well understood,” Rick Sontag says in a press release. “I think there are real reasons why this all came together in the way it did.  I probably have another mission here, something else I was meant to do.  And I’m lucky enough to have the means and opportunity to do it.”