Issue #24: Summer 2009

One Man’s Journey with Parkinson’s

by Adrienne Watt

“Move it Smith, come on, move those legs.”

Former Reserve Army Colonel (and my stepfather-in-law) Ian Smith has barked those orders countless times over the years, but not to his troops. Smith is actually yelling at himself. He is trying to get his legs moving well enough so he can get into a car.

He also gets stuck walking through doorways. Literally immobilized. But he can move up and down stairs at an alarming speed. He says, “If my world were a series of stairwells I could get around much more easily.” When he’s on the street, he sometimes walks along the curb, up and down, because he can achieve the same effect as he can with stairs. For a period of time he would wake his wife in the middle of night with distressing screams, but was barely aware he was doing it. Twenty-five per cent of his day is often spent immobilized. Sometimes he has trouble moving from a chair. Another 25 per cent is spent dealing with uncontrollable movements. These are Ian’s struggles with both the symptoms of Parkinson’s disease and the bizarre side-effects of the medication.

The four main symptoms of the disease include involuntary, rhythmic shaking of a limb, head or entire body; stiffness or inflexibility of the limbs or joints; slowness of movement or absence of movement; and impaired balance and coordination. The uncontrollable movement (different from the tremors) often exhibited in Parkinson’s patients is called dyskinesia and is a side-effect of the medication.

Parkinson’s is a progressive, degenerative neurological movement disorder affecting about 100,000 Canadians. It typically develops in people over the age of 65, though a small percentage of people develop young-onset Parkinson’s before reaching 40.

“I was 48 when I was diagnosed with Parkinson’s by a neurologist,” he says. “I was 47 when another neurologist specifically ruled out Parkinson’s. I was 44 when I went to my GP with the first symptom. Now, with the benefit of 20/20 hindsight, I know that I actually had the first symptoms when I was 42.”

The first symptom he can recall was foot cramps and general fatigue when running (he used to log multi-kilometre runs daily). He noticed unusual stiffness in his knees when bowling one night, and a few years later he started having trouble doing up buttons and holding a fork in his left hand. Parkinson’s becomes progressively disabling, making daily activities like bathing or dressing increasingly difficult.

Our movement is normally controlled by dopamine, a chemical that carries signals between the nerves in the brain. When cells that normally produce dopamine die, the symptoms of Parkinson’s appear. There is no cure, but new treatments and research being conducted at UBC and elsewhere are providing hope. UBC researchers at the Pacific Parkinson’s Research Centre (PPRC) and the Brain Research Centre are addressing three overarching questions: what causes Parkinson’s; what factors contribute to the complications of Parkinson’s and its treatment; and can Parkinson’s be used as a model to understand how the brain works normally?

Researchers are involved from many disciplines, including physicists, chemists, radio-chemists, image analysis experts, geneticists, epidemiologists and psychiatrists. Dr. Jon Stoessl, director of the PPRC believes this type of work can’t be done in isolation. “You really need a team with varied
backgrounds,” he says. “We have basic scientists and basic neuroscientists involved, but our overall focus is clinical.”

For instance, studies are underway in collaboration with researchers in psychiatry to understand the psychological problems associated with the disease. Many people with Parkinson’s may have cognitive function and mood or depression problems. Under the influence of medication, some patients
can even develop severe behavioural side effects including excessive gambling and shopping habits.

From examining occupational risk factors with the school of Population and Public Health to working with geneticists at the US-based Mayo Clinic (world leaders in the genetics of Parkinson’s), the team approach has led to many advances. The collaboration with the Mayo Clinic resulted in the identification of a dominantly inherited gene that is the most common genetic cause of Parkinson’s.

A key methodological backbone and common thread for many of the Parkinson’s studies taking place at UBC is Positron Emission Tomography (PET). This imaging technique provides insight into the processes that lead to Parkinson’s, as well as the way in which the brain compensates for having the condition. Ian Smith has been involved in a PET research study since 1999. While he knows that the studies may not ultimately help him, he sees the potential for improved treatment for future Parkinson’s suffers or even prevention of the disease altogether.

“Gradually more and more things have been taken away from me because of this disease,” he says. “I have less time available in the day to contribute and do the things I have always enjoyed.” He has always been involved in his community and is an avid gardener and baker (he makes delicious Christmas cakes). Never one to let an obstacle stand in his way, Ian has found ways he can continue to contribute in his community. “One thing I can do is donate blood regularly. The PET study is another thing I can do that can help others.”

Using PET, researchers monitor the brain’s chemical processes, including the creation, storage, release and breakdown of dopamine, allowing them to assess how the brain compensates for its loss. Researchers are also using PET to understand the bewildering complications of Parkinson’s. Symptoms like slowness of movement and stiffness respond well to medication in the early stages, but over time these benefits drop off.

“Often people have a couple of good hours and then the medication effect washes out,” says Stoessl. “But when the medication is working, patients often suffer squirming, writhing or involuntary movements. We are using PET to understand the mechanisms that contribute to those complications and to understand what is happening to dopamine once it’s released.”

It’s this ping-pong effect that has been the most frustrating for Ian. Both Ian and Angie (my mother-in-law) express frustration with the inability to plan their days. Ian thinks he is “on” about 50 per cent of any given day, but when this “on” time will be or when he will not be functioning well due to the dyskinesia or rigidity is totally unpredictable.

“The only thing that is predictable is that I will have some of each (rigidity and dyskinesia) every day.” From simple tasks like making dinner to planning elaborate vacations, Ian and Angie face uncertainty on almost a daily basis.

“It’s really difficult to plan,” says Angie. “Sometimes Ian makes plans for lunch with a friend and then he is unable to go. When we travel we might want to go on a tour or for a walk, but if Ian gets stuck we may not get back in time. It’s a real worry.”

In the past he was bothered that he would be perceived as disabled, but not any more.

“I tried to avoid being seen as disabled but now it doesn’t bother me. I’d just as soon get out and do stuff.”

Ian hasn’t had any cognitive function or mood problems. In fact in my experience he never appears down or depressed. Finding a balance through medication with the symptoms of Parkinson’s and the side-effects of the medication is a common struggle for many Parkinson’s patients. He currently takes 15-20 pills a day, one of which alone costs $8. But because of unpredictable results, it often feels like a crapshoot. He has tried a number of alternative therapies to help manage his symptoms including acupuncture and shiatsu and he regularly uses meditation and Qigong exercises to help when he is really immobile. It was his acupuncturist who told him about the work being conducted at UBC.

He has been open to experimental and alternative therapies since his disease was diagnosed. This summer he will undergo one
of the most nerve-wracking to date. An electrode, similar to a pacemaker, will be implanted in his brain through a small opening in his skull and then attached to a power source implanted in his chest. The operation, called Deep Brain Stimulation (DBS), will be lead by Dr. Christopher Honey, UBC associate professor of Neurosurgery and director of research for the division of Neurosurgery. There are two stages to the DBS operation. First, with Ian conscious and under local anesthetic, Honey will drill two holes into Ian’s skull and then for the better part of the day will look to find the exact location in the brain that is the target for the implant. Ian will be an active and crucial participant in this part of the procedure. He will answer a variety of questions and perform certain tests to help confirm the target. The DBS electrode will then be implanted in the target site.

Is he scared? “Scared stiff.”

The second stage of the surgery is the insertion of the Implantable Neural Stimulator (INS). While Ian is under general anesthetic, a wire from the DBS electrode will be tunnelled under the skin on his neck and connected to the INS located under a pocket of skin on his chest. Approximately six weeks later, the stimulator will be turned on. By delivering an electrical stimulation to a precisely targeted area of the brain, the procedure and device aim to manage some of the most disabling motor symptoms of Parkinson’s.

DBS is not a cure or even a guarantee. The operation will help patients function as well as they ever have on medication, but for longer and with fewer dosages, giving Ian more “on” time in a day. According to Vancouver Coastal Health’s last survey of 50 consecutive patients, 90 per cent reported that the operation was a success.

But the fact is that Parkinson’s has no cure, it is progressive and degenerative. DBS patients normally feel the positive effects of the stimulation for two to five years, then it eventually becomes less effective. Just like medication.

“I’ve made my peace with the concerns I have about the surgery, but it’s at the point where I am so frustrated with the disease that if there’s something that can give me improved functionality for even a few years, it’d be worth it.”

Ian’s hope? To increase the percentage of “on” time during the day, allowing him to plan for something as simple as making his delicious stuffed chicken breasts.

Angie and Ian are practical people and have thought about their plans after the operation.

“We will look at our financial resources and how much time we likely have with improved functionality and come up with a realistic program,” he says. “Travel will definitely be a big part of it,” says Angie. “Now, we’re doing things that are safe. After the surgery, I’d feel more inclined to go to the Mediterranean and hope Ian can take full advantage of being in a different part of the world.”

“Smith” has every intention of continuing to order his legs to move, but hopes that after the surgery they will be quicker to jump to attention.

“If we can count on me being mobile eight hours a day, I can live with the other stuff,” says Ian. “Being able to move, walk around
and really see and appreciate everything is what it’s about.”

Adrienne Watt is a Marketing and Communications Officer for UBC Alumni Affairs.