Meet Frank: Normal Pressure Hydrocephalus

Appeared in the foundation's newsletter, LIFE~LINE, Fall 1999

All his life, Frank was blessed with excellent health. He loved the outdoors. Together we skied, hiked, played tennis, and enjoyed our sailboat out on Long Island Sound. At age 70, Frank seemed ageless, he was full of vitality and we enjoyed life to the fullest.

Changes started to appear in Frank's early 70's, barely noticeable at first - a slight insecurity in his gait (walk), a shuffle with his right foot when he was tired, some occasional balance difficulties. By 1993, now age 75, he could no longer ignore his balance problems, although he denied the other changes that had become increasingly apparent to me: his gait had adopted a wider base, his steps at times were small and irregular, there were signs of forgetfulness, and his sense of orientation had started to suffer.

On New Year's Eve of that same year, Frank felt cold and tired - and suddenly his legs refused to carry him to bed. The next day at the emergency hospital, he was diagnosed with Normal Pressure Hydrocephalus (referred to as NPH), "made worse by a febrile (high body temperature) infection," probably just a cold. He was admitted to the hospital that same day.

The following morning, I found my husband in a terrible state - unable to stand up, walk, he was confused, and unable to even sign his name. The neurologist explained that the only help for Frank would be insertion of a shunt and, although he was not in favor of the operation (because of frequent complications and an uncertainty of success), it was probably the only chance for Frank to walk again. After consulting with several neurosurgeons, an emergency operation was scheduled - then suddenly cancelled, or rather postponed. Frank's condition had started to improve and the surgeon wanted to be sure that all signs of infection had cleared up before proceeding. Frank was sent home - and he made a remarkable recovery! Within days, he was able to walk without a walker or cane and he triumphantly declared he would never agree to the operation for a shunt. His neurologist, who had agreed to the emergency surgery only reluctantly, supported him strongly - and continued to do so after I started to see, and report, new signs of decline.

About a year after his first diagnosis, I was successful to get Frank's permission to seek a second opinion. We saw a professor of neurology at one of New York's major teaching hospitals. He questioned the diagnosis and put Frank through a series of tests without conclusion. More tests were proposed "to search" or "to fish", as he termed it. Our New Jersey doctors discouraged us to continue, at the same time offering no help. The (original) neurologist continued to advise against a shunt since it only worked "in about 20% of cases" and was "more trouble than help." The standard advice seemed to be: "Go home, do the best you can and be happy."

I was not happy! Deep inside I felt that we were following the wrong path that there must be some way to help Frank. We tried neurological physical therapy for four months but, although we had the most caring therapist, it did not help, just as our doctors had predicted.

Ultimately, in the Spring of '96, a kindhearted social worker, the leader of Frank's prostate cancer support group, opened a door for us. Under "orphan diseases" in her big reference book for support groups, she found a brief paragraph on hydrocephalus, which included some telephone numbers. That's how I connected with Debbi Fields. In that first call to Debbi, I kept her on the phone for over an hour - I was so relieved to be able to talk, to share, and to finally have found someone who understood my frustration and fears. Debbi put me in touch with Ann Marie Liakos, who was then the Executive Director of NHF.

Without knowing us at all, Ann Marie did a search for us on Normal Pressure 'Hydrocephalus and Aging. She directed me to several research stories, specifically a report on the results of a 1987 study by the National Institute of Health: "Plasticity in the Aging Brain - Reversibility of Anatomic, Metabolic and Cognitive Deficits in Normal Pressure Hydrocephalus Shunt Surgery: I must have read it a dozen times - in total disbelief. It described the case of a 78-year-old woman, and (it) sounded almost like a carbon copy of Frank's story - from the initial difficulties to the slow decline over a three and a half year period, "to the denial and total lack of concern about her condition. It was an incredible success story and described how the woman, after shunt surgery, recovered and was able to return to a normal life. The story concluded that Normal Pressure Hydrocephalus is one of the few forms of dementia that can be reversed. The problem is knowing who would be a successful candidate.

I could barely conceal my excitement. With the help of a friend, we started our research. We contacted the National Institute of Health, source of the research report, only to learn that all doctors involved had moved on to other parts of the country. The Institute suggested we look for a specialist at a major teaching hospital, and since we were in the process of moving from New Jersey to Maryland, Johns Hopkins was a natural. After several calls, we were referred to Dr. Michael Williams, a young and enthusiastic neurologist specializing in Normal Pressure Hydrocephalus.

At the end of August, we had our first meeting with Dr. Williams who took great patience and compassion to explain the nature of hydrocephalus to Frank and for the first time, Frank listened and seemed to understand the seriousness of his illness, if only a 50/50 chance. Dr. Williams told us of a relatively new test, the Lumbar Catheter Protocol, which would determine whether or not Frank would be a candidate for shunt. Frank agreed to go ahead with the test as early as possible.

At the end of September, we moved to Maryland. By that time, Frank's condition had deteriorated alarmingly. He was only able to walk with great difficulty, holding onto furniture for support and balance. Bladder control and mental function had also become an increasing problem! In mid-October, we were able to check into Johns Hopkins for the 5-day test. The lumbar catheter was inserted to monitor the cerebral spinal fluid (CSF) pressure, followed by controlled CSF drainage over 3 days.

After 2 days of CSF withdrawal, Dr. Williams invited Frank for a walk down the hospital corridor, just like he had done the previous day, when Frank's gait was "magnetic", with a wide, stiff base. Not so that (second) evening! Frank got up and suddenly his feet seemed to fly - loose, natural, and easy like I had not seen in years! I will never forget that broad smile on his face and on the face of his doctor! On day 3, hand-in-hand with Dr. Williams, they "jogged" down the corridor and all the nurses were applauding. We had the proof that Frank's problems were indeed caused by NPH, and that he was a good candidate for a shunt!

After the surgery, Frank did very well. His bladder control was restored almost instantly after the surgery. The fog that surrounded him for so many years was lifted. His balance was excellent. We have traveled to Alaska and Germany, to Eastern Europe to celebrate his 80th birthday in Prague (a dream of many years), and we cruised the Mediterranean Sea.

None of this would ever have happened if a kind-hearted woman back in New Jersey hadn't searched her big black book of support groups for a reference on hydrocephalus. If Debbi Fields hadn't given me new courage not to give up, and steered me to a caring woman by the name of Ann Marie Liakos, who searched her computer for us to find the National Institute's report on the reversibility of the syndromes of NPH. And, if a young, devoted doctor hadn't been able to open Frank's mind and make him understand that he had a chance! We are eternally grateful to all the wonderful people who helped us gain a second lease on life.