I am now 73 years old. On April 24, 2007, at age 67, I had a programmable ventricular peritoneal (VP) shunt inserted in my brain at St. Michael’s Hospital, Toronto, because I had adult onset idiopathic Normal Pressure Hydrocephalus, also known as iNPH. The diagnosis took at least 2 years to make and involved several medical specialists, some of whom declared my symptoms psychosomatic. Others felt I had hydrocephalus, but that I should wait to be shunted because of the dangers of the operation and the risk that the outcome would not result in significant improvement. My earliest symptoms had been a sensation like that of walking on gum, and progressed to an inability to walk without a walker, uncontrolled urination, difficulty concentrating and understanding complex ideas, and trouble remembering things.
Over a couple of years my symptoms gradually worsened and even after taking medication for my urinary incontinence, I soon had to wear adult diapers.
My husband is fond of saying: “During the Jewish High Holy days in September, 2006 before Gilda’s shunt was inserted, I had to take her to our synagogue, which was less than a third of a mile from our house, in a wheelchair. During the same holiday in 2007, about six months after the shunt was inserted, she walked there and back each day for the Jewish New Year, and three times on the Day of Atonement.” However, my memory, concentration and ability to figure out things were still weak and I was still urinating frequently and sometimes without much control.
I would like to share a little of what my life was like before my final decision to get shunted. My family doctor sent me from one specialist to another and I underwent many different tests to try and diagnose the cause of my early symptoms. All were negative. Finally, he sent me to a neurologist who could not detect anything amiss during his careful neuro-physical exam. It was only resolved after an unrelated visit to my endocrinologist, who had a keen diagnostic sense, and in response to my sad story carefully looked at my gait and general condition. He saw evidence of neurological problems and referred me back to the neurologist. By then a few months had passed and the neurologist also picked up the signs and sent me for a CT scan.
When the results of the scan came back, the neurologist said he found it difficult to believe but the results showed enlarged ventricles and the report from the radiologist said it seemed that I had hydrocephalus. The neurologist in turn referred me to a neurosurgeon saying, “Those guys know a lot more about this than we do.”
Neurosurgeon: “I looked at your CT scan results but your symptoms are not very pronounced. Come back and see me when you are in a wheel chair, wearing diapers and not sitting there taking notes.”
Indeed, I gradually got worse and my neurologist scheduled a cerebral spinal fluid (CSF) flow study, where a radio-opaque material was injected into my spine in the lumbar area and x-rays were taken every two hours to note the CSF movement. After several images, the final x-rays were taken the next day. The radiologist’s report noted I appeared to have communicating hydrocephalus. I returned to the neurosurgeon.
Neurosurgeon: “Yes, but you are not ready for surgery yet. The operation has significant risks and you may not benefit from the shunting.”
My husband and I wanted to find a specialist who had more clinical experience with adult hydrocephalus. We located a neurosurgeon at the Montreal Neurological Institute who had been seeing seven or eight adults a year and went for a consultation. He thought I likely had iNPH, but also thought I should wait before undergoing shunting. In the meantime I had been seen by two different neurologists who specialized in movement disorders. They were of the opinion that I did not have Parkinsonism, though one also told me that my symptoms likely were psychosomatic. As I worsened, my Toronto neurosurgeon, who had encouraged the examination by the movement disorder neurologists, told me he would operate if I was prepared to accept the risks.
Along the way, I had joined the American-based Hydrocephalus Association (HA) and I decided to go to their annual conference in the Spring of 2006, held in Baltimore, Maryland, in order to learn more about my condition. I went with my younger daughter (my husband could not come) and a member of the Spina Bifida and Hydrocephalus Association of Ontario‘s (SB&H) adult support group. My husband prepared a dossier of all my tests including a CD-Rom with my CT scan and an MRI. The idea was that our daughter would take the file and scans to the office of Dr. Michael Williams, then co-director of the The Hydrocephalus Center at Johns Hopkins University Hospital and now Medical Director of The Sandra and Malcolm Berman Brain & Spine Institute of LifeBridge Health, and leave it for him to read. From reading some of the medical literature on NPH we knew that Dr. Williams had a lot of experience and was involved in significant research on hydrocephalus. We planned to ask him for a consultation. Then it seemed fate took a hand.
I was at the conference, walking from one session to another when I lost control of my walker and walked into a wall. This attracted the attention of one of the speakers, Dr. Michael Williams himself, who was a member of the HA’s Medical Advisory Board.
Dr. Williams: “If you like, I will see you for a consultation in a month regarding your diagnosis and potential for benefiting from a shunt operation.”
My husband and I were quick to seize the opportunity. It was then about 5 years since my very first symptoms and, as I’ve said, my condition was deteriorating. Dr. Williams gave me the most intensive neurological physical examination I had ever had, after which he said he had no doubt whatsoever that I was suffering from iNPH. The question remaining, he noted, was whether I would benefit significantly from being shunted. “Because we’ve had a cancellation at the clinic I can see you in two months for a continual lumbar drainage test, under special conditions which require you to be in a neurosurgical bed for three nights.” Again we jumped at the chance, avoiding a much longer wait.
This test certainly was known in Ontario, but rarely done because there weren’t enough scarce neurosurgical beds to reserve one ‘merely’ for testing. I have since learned that now it is done in London, Ontario. This test must be carried out very carefully, as it involves continual drainage of cerebral spinal fluid via a lumbar catheter, which stays in place for three days. There is a potential for infection, which could then lead to encephalitis and worse. The test requires careful monitoring, avoiding over-drainage; repeated testing of one’s walking ability and daily cognitive testing are part of the regimen.
After the first full day of drainage, Dr. Williams took me out into the hallway outside my room. Pointing to my husband at the end of the corridor, and with a twinkle in his eye and a proud look on his face (or so I interpreted it), Dr. Williams said to me, “Walk over to your husband and give him a kiss.”
“Walk without a walker?” I thought. “I can’t!”
But I could and I did. I felt like one of the patients in the movie Awakenings, in which people who had been “frozen” in time and space due to their infection during the 1918 influenza pandemic became functional (for a time) after being given el dopa. I wondered whether a similar time-limited result might be my fate. So far it hasn’t been.
The testing at Hopkins indicated that I had an 85 to 90% chance of significant improvement with a shunt. This was an enormously important factor in my decision to have a shunt. I had been told repeatedly in Toronto that about twenty percent of patients suffer inter-cranial bleeding and possible strokes while on the operating table during the shunting procedure, and something like five percent actually die; while statistically only about half of those shunted experience significant improvement in their symptoms. We also knew from the literature that waiting too long reduces functional recovery. This was the dilemma I faced without being given much hope from most of the doctors, except for Dr. Williams and his colleague, Dr. Wilson.
I believe those tests saved my life. The results gave me confidence to have the surgery in Toronto (which was fully covered by the Ontario Health Insurance Plan), despite its risks. Drs. Williams and Wilson provided a full report to my new Toronto neurosurgeon, Dr. Michael Cusimano, whom I selected after Dr. Williams had asked a very distinguished Canadian pediatric neurosurgeon he knew (Dr. James Drake, head of Neurosurgery at Toronto’s Sick Children’s Hospital) for a recommendation of someone in Toronto who had a “special interest” in adult hydrocephalus. And thankfully Dr. Cusimano accepted me as his patient.
Now that I am at the six year post-op mark, I feel in many ways like my old self but it has taken a lot of hard work. After my first neurologist looked at my initial CT scan ten years ago and said I had hydrocephalus, I quickly found the Hydrocephalus Association and connected with the wonderful people there. They sent me literature about NPH and information on their activities, including their national conference. The Baltimore conference, aside from providing me with valuable information, linked me to Dr. Michael Williams and thus started me down a road that confirmed my diagnosis and gave me the confidence to undergo shunt surgery in Toronto with Dr. Cusimano. The key to my success was HA, with which I stay connected and continue to refer people to, in addition to the important work being done here by the Spina Bifida and Hydrocephalus Association of Ontario, the largest such organization in Canada. We have but a tenth the population of the United States and so many fewer sufferers of all forms of hydrocephalus, thus both organizations are very significant to those of us with hydrocephalus in Canada. My life has been impacted positively by HA and I hope and trust that the HA will be successful in raising the profile of hydrocephalus in the United States through its advocacy efforts, and also to continue to respond to the needs of patients and their families with literature, conferences and personal contact. These efforts surely will result in much greater awareness of hydrocephalus in both countries and will contribute to alleviating the hardships of those who suffer from it at all age levels.
Overall, this has been a journey covering some twelve years and I can say with complete conviction that each of us who suffers from this still incompletely understood condition has to have hope, help and enough self-confidence to make the journey yield as much as we can under the circumstances we each find ourselves facing. I hope my own story gives you some ideas and the conviction to move forward. I think writing about my post-op life will help others take some of the same steps.
Join Gilda for the continuation of her blog where she shares her post-operative rehabilitation, ideas for accessing community services and self-help activities, and the importance of involvement and finding the proper support.