Hydrocephalus: It Could Happen to You

I have gained a whole new awareness of Hydrocephalus in the last three years, and decided it was time to share after learning that September is Hydrocephalus Awareness Month.  I even had some awareness in childhood, remembering my mother telling me about a cousin’s baby that was born with “Water on the Brain,” as it was called back in the 1940s. There was no treatment, so the baby lived for a few years and died without learning to walk or talk.

There is still no cure for Hydrocephalus, but treatment is available. The symptoms of Hydrocephalus can mirror that of many other medical conditions, and this can lead to a misdiagnosis, or contribute to a slow diagnosis. Awareness, therefore, can be key to achieving a timely and accurate diagnosis.

The Hydrocephalus that I have been living with for three years is that of my husband, Paul, who was not a “typical” Hydrocephalus patient. Paul manifested some symptoms that went undiagnosed for at least 15 months. 

Middle aged at 53, Paul began shuffling his feet. “Pick up your feet when you walk,” I would tell him. The shuffling became especially pronounced when we visited his brother in Wisconsin for New Year’s weekend in 2007. Paul was sick on New Year’s Eve, and spent the entire day in bed, except for the occasional shuffle down the hallway. I drove him home on New Year’s Day, and he experienced bowel incontinence at the end of our trip. It never occurred to me that this might be caused by a disease of the brain. Paul’s holiday flu turned into pneumonia, which was treated with a short hospitalization and IV fluids.

Paul recovered from this January illness and returned to work. But his gait continued to deteriorate, with the dragging of his feet when he walked. Appointments with a neurologist finally led to him being scheduled to have a test to remove spinal fluid in late July.

“Your mother is non-responsive today,” the call from the nursing home where my 93-year-old mother resided informed me the day before Paul’s appointment for the spinal tap. I told them I would visit Mother after Paul’s appointment. I thought it was important for him to keep this appointment, hoping we could make progress in determining the cause of his abnormal gait.

After a University Med student, a resident MD, and the supervising MD were all unsuccessful in placing the needle in the correct space of Paul’s spine, we left the clinic and arrived at my mother’s bedside to find her lucid. Paul and I were with my mother when she passed from this world on July 28, 2007. 

Six weeks later, Paul had another spinal tap, this time guided with a fluoroscope, and this procedure was successful in removing some spinal fluid. But the MD performing the procedure failed to measure the amount of fluid, so the procedure did not result in any diagnosis.

Paul’s walking continued to deteriorate, and his doctors sent him to the therapy department, where he was issued a cane. He used this to help steady him when he walked. After his walking deteriorated further, he was given a walker. His legs grew weak and floppy like a rubber chicken. He fell frequently. We turned the calendar to a new year, 2008.

One snowy evening in the Winter of 2008, my phone rang. It was Paul, calling me on his cell phone, after he had fallen outside on the sidewalk, trying to get into the house when he came home from work. I did my best to help him get up, and also tried to help him whenever he needed to move from room to room in the house.

In March of 2008, Paul’s manager told him he could no longer be at work if he could not maneuver to the restroom by himself. He applied for short term disability, and I found a new doctor for him. I drove him to his appointments.

At a new clinic, we needed to start with a Primary Care Physician. We made an appointment with Dr. Cara Houle at the Hopkins Clinic of Park Nicollet, as this small clinic (now unfortunately closed) had parking very close to the building. Paul had his first appointment with Dr. Houle on a Thursday, and she ordered some blood tests immediately. On Monday, she called and invited Paul to her office to discuss the tests. “Your liver function is impaired,” she informed him. I was upset, as the prescription medicines he was taking were supposed to be monitored by the doctors prescribing the drugs. Blood tests are typically given at six month intervals, but the doctors did not order a blood test, even though it was quite visible that Paul was having difficulty walking. I learned that the liver metabolizes proteins, and if the function is impaired, the major muscles of the body can be starved of proteins, and the result can be impaired muscles, like legs that have difficulty holding the body up.

Dr. Houle advised us she wanted to admit Paul to the hospital as an inpatient. Immediately. I was both alarmed and relieved. Taking care of him at home was difficult since he fell down so frequently, and often disregarded my insisting that I help him when he needed to move from one room to another.

Paul was admitted to Methodist Hospital in Saint Louis Park on Monday, 17 March 2008. He was seen by several specialists during his first hours in his room. Progress. I went to Kip’s Pub and celebrated my Irish heritage, feeling the Luck of the Irish was beginning, finally, to bless us. Dr. Houle’s decision to hospitalize Paul seemed a good strategy to sort out the numerous symptoms he was displaying and look for causes.

One of the specialists that examined Paul was from ENT (Ears, Nose, Throat). “Your sore throat is caused by tonsils that are abscessed and should be removed,” he advised. The surgery was performed on Wednesday. This was a distraction from Paul’s neurological symptoms. Paul was discharged the next Monday with a diagnosis no more specific than “Abnormal gait disorder.” The medicines he had been taken off of because of liver malfunction were gradually reintroduced, and his legs were one again strong enough to hold him up. He continued to use the cane or walker since he still had some balance issues.

“How is Paul doing?” asked my friend Joyce when I saw her at a social event in March. She had seen Paul using a walker at an event in January. I informed her he had been hospitalized, and was no longer at work. We still did not have a diagnosis. “What do you think is wrong with Paul?” Joyce asked me. I confessed I was worried it might be Parkinson’s Disease, as the young resident MD had referred to his symptoms as “Parkinsonian-Like Symptoms.” I had used the internet to research Parkinson’s. I was troubled by the knowledge that Parkinson’s is not curable, and is a progressive disease. “So do you think Paul has Parkinson’s?” was Joyce’s next question. “No, I’m not sure,” was the best I could reply. Joyce then asked, “What kind of symptoms does Paul have?” When I mentioned the gait disorder, Joyce said “Do you think Paul might have Hydrocephalus?” I replied, “Yes, I think it’s possible.” This had been mentioned by several doctors, but we were still without a diagnosis. As an advocate for a mother with Parkinson's Disease, Joyce had learned much about neurological disorders.

Lacking a diagnosis for a medical problem is a kind of purgatory that can be difficult to live in. Not knowing what causes symptoms can lead to speculation, and one can go down a path that can be very dark. Facing a diagnosis of a disease like Parkinson’s can be very depressing, for both the patient and their family members.

Paul continued to have medical appointments following his week of hospitalization, and we finally arrived at the diagnosis of Normal Pressure Hydrocephalus (NPH) about a month after his discharge. Paul was referred to a Neurosurgeon, Dr. Andrew Smith. We researched Dr. Smith to learn he had been awarded an M.D. degree in 1968, so had 40 years of practice in medicine! We found a great deal of reassurance in his years of experience after the unsettling episode with the young Med student and the resident that were unsuccessful in performing the spinal tap. Dr. Smith could install a shunt in Paul’s brain to enable the excess spinal fluid to drain into his abdominal cavity. He had performed the procedure thousands of times. “In which ventricle of the brain do you install the shunt?” I asked Dr. Smith. “The one that does not control language,” was his reply.

Normal Pressure Hydrocephalus is found more often in men in their 70s and 80s than it is in middle-aged men, Dr. Smith told us. A brain injury may be a causative factor. Paul had fallen off a truck during his service in the U.S. Army. He was a company commander in Korea, and he was the leader for his unit. I don’t think he was treated or examined after this injury, which occurred in a remote location not close to an Army medical clinic.

Paul’s surgery to install the shunt was performed in May 2008, just following Mother’s Day. It had the usual risks of any surgery performed under anesthesia, along with a high risk for infection. Paul was placed in Intensive Care immediately following his surgery so he could be carefully monitored for the next 24 hours. After several days in the hospital, Paul came home with a partially shaved head and surgical staples in his scalp, neck, and abdomen. The shunt ran from his brain to his abdomen, and was inserted through several incisions. It’s quite an amazing device, a piece of plastic tubing with a valve that can be adjusted externally using magnets. The adjustments enable the proper pressure to be obtained, and can cause some headaches when changes are made. This shunt treatment can enable a patient with an incurable brain disease to lead a fairly normal life. 

Paul quickly convalesced and was soon able to resume activities like gardening and driving. He returned to his work as a systems engineer at the end of June. He went back part time the first week, since viewing a computer screen for more than several hours each day was not advised by his doctor. The return to work was stressful, as his department had moved to a different plant location while Paul was home and disabled, and his manager had transferred to a different location, and Paul was assigned to a different manager. Paul’s work projects had been distributed to other engineers, and he was assigned new and unfamiliar work on his return. 

Paul had an appointment with a neuropsychologist in late 2008 for testing on cognitive functioning. The first tests had been administered in April, following his Hydrocephalus diagnosis, and before the shunt surgery. The post-surgical testing showed a decline in cognitive functioning. Paul experienced frustration while trying to perform his duties as a systems engineer. Subsequent testing showed further decline in two additional years. Paul consulted with his care providers and followed their advice to apply for disability in April 2011. If the cognitive functioning was showing a decline during all three years following surgery, it was not considered likely to improve.

While Paul’s career as a systems engineer has been ended by Hydrocephalus, we remain grateful that medical technology enables him to live a fairly normal life. He is able to walk without assistance, to drive, and to speak coherently most of the time. Hydrocephalus is a treatable disease. But it must be properly diagnosed to be treated, and family members are often the advocates that are helpful in obtaining the diagnosis. Advocates can be helpful by observing symptoms, giving feedback, and encouraging the person suffering the symptoms to seek medical attention that will hopefully lead to a proper diagnosis and appropriate treatment. Advocates are often the children and grandchildren of the adult patient, since Hydrocephalus is often found in people in their 70s and 80s. Paul was the non-typical middle-aged patient with no children and no grandchildren. His primary advocates were his manager at work, and his wife. These were the people that encouraged him to seek treatment that finally lead to diagnosis and treatment. Symptoms can be researched by a competent physician and referred to specialists for diagnosis and treatment. Reporting all symptoms is important, as some that may not appear to be related might help the physician to make the diagnosis.