Had Tom Graboys Been A Neurologist
After writing the Tribune column, I encountered two books that changed my understanding of PD. Each challenged me to serve fellow Parkies more vigorously and effectively.
One is the autobiography of the late Thomas Graboys, MD. The other is the provocative and authoritative masterwork of Oregon journalist and Parkie Jon Palfreman.
Graboys was a beloved Boston cardiologist who struggled for many years with dementia connected to his Parkinson’s.
He famously wrote a book,Life in the Balance: A Physician’s Memoir of Life, Love, and Loss with Parkinson’s Disease and Dementia(Sterling). It bared his innermost thoughts and emotions about the feel and look of advancing Parkinson’s and dementia.
I imagined how Graboys, a master at patient care, would break the news of a Parkinson’s diagnosis if he were a neurologist and not a cardiologist.
All of us end up in the neurologist’s office with a variety of seemingly unrelated problems. That’s one of the many things that make PD so frustrating.
When the doctor’s verdict is rendered – Parkinson’s disease -it is the day many will never forget. For some like me, there is a momentary sense of relief that the accumulating symptoms have a cause and a name. For others the reaction is terror, shock and confusion.
We ask: “What does this mean for us?” A brusque answer one person received was “your symptoms will only get worse.” In my case, the answer was “at your age of 75,something else will kill you first.” Few receive information beyond the diagnosis that day.
Commonly, we leave the doctor’s office with a pill prescription and instructions to return in three months. We are on our own. We get no literature explaining what Parkinson’s disease is, what we can do about it, and what our future holds. What if Tom Graboys had been the one delivering the news?
He would have taken the time to give us a short explanation of the disease, encouragement about the therapies available to treat it, and some information about the importance of exercise and diet.
He also would have given us a set of instructions about exactly where on the internet we could find reliable information, where exactly and what specific exercises we should seek and information about support groups in our area. He would tell us that patients who do well with the disease don’t let it own them – they own and control it. You don’t have to do this alone, he would say.
Graboys would not have done this from behind his desk but sitting close to us in a reassuring manner.He would have written down on a piece of his stationary his home phone number and told us to call anytime we needed help. He would not have said to return in three months, but in one month. At that second meeting, we would discuss what we had read and the questions we had.
Graboys also would explain at that second meeting that there was a Parkinson’s Center to join. It would provide educational seminars, special programs for caregivers and recommendations on finding physical and other therapists. He would work closely with the center to see that care provision was modified as needed.
The Graboys approach would reduce our anxieties and provide an organizational anchor.
Graboys would write out the medications he was recommending and what they were for. He would ask us what he or she thought was a reasonable exercise regimen. Dietary and other lifestyle changes would be discussed, to help us enhance our life. He would call those elements our plan.
It was the “contract” between Graboys and patient that, if adhered to, would help ensure a positive outcome. And because the plan was personal to each, it was more likely to be honored.
Just leaving the office with a plan inspired hope because implicit was the message that we could do things to take control of our illness.
Indeed, while 300 words may have been on that page, there really was just one: hope. The written plan inspired hope that by following instructions we could enhance our chances of living out a fairly normal life.
The second book is Jon Palfreman’s Brain Storms:The Race ToUnlock The Mysteries of Parkinson’s Disease(Macmillan).
Palfreman is an experienced journalist and educator who was diagnosed in 2011. He is best known for his documentary PBS work on Frontline and NOVA.
In his book, you meet a skilled dancer, Pamela Quinn, who after contracting PD found ways to retrain her brain functions so that she could still dance gracefully.
Writes Palfreman (3): “Quinn is certainly an outlier, with a slowly progressing form of the disease. But we can all learn from her. Her wisdom exhorts Parkies to keep active, to mindfully circumvent gait and balance issues.”
As Quinn puts it, ” ‘We must treat the mind as a muscle; it needs to be strengthened and made flexible just as much as our legs and core.’ ”
Palfreman argues persuasively that the classic symptoms of PD – rigidity slowness and balance problems – may be what he calls “the tip of a clinical iceberg. It now seems that Parkinson’s disease takes hold of an individual decades before any tremors appear and continues wreaking damage throughout the brain until the end of life.”
A patient’s symptoms can be far ranging, well beyond the classic neuromuscular ones. Palfreman writes (4):
“… in light of this evidence, many neuroscientists are lobbying to rebrand Parkinson’s disease from a motor disorder to a whole-body condition, involving an enormous number of signs, symptoms, and complaints.
“These include not only the classic features – such as bradykinesia, tremor, rigidity, postural instability, stooped posture, shuffling gait, freezing of gait, dystonia, facial masking, small handwriting, dysarthria (problems with articulation), dysphagia (trouble swallowing), oily skin, bladder problems, pain, constipation, and loss of smell – but a growing list of other problems as well.
“People with Parkinson’s experience neuropsychiatric symptoms such as depression, anxiety, hallucinations, cognitive impairment, and impulse control disorder (the last caused by dopamine agonists).
“They suffer from a whole host of sleep-related disorders – including REM sleep behavior disorder, excessive daytime sleepiness, restless legsyndrome, insomnia, and disordered breathing while sleeping.”
Palfreman pulls no punches in describing PD’s trajectory (5).
“… people with Parkinson’s progressively lose core pieces of themselves. We forget how to walk. Our arm muscles grow weaker. Our movements slow down. Our hands fumble. Simple fine-motor tasks like buttoning a shirt or balancing spaghetti on a fork become a challenge. Our faces no longer express emotions.
Our voices lose volume and clarity. Our minds, in time, may lose their sharpness . . . and more.
“The ‘more’ varies widely from patient to patient. Some lose the sense of smell. Some shuffle instead of stride, freeze in place as they try passing through a doorway. Some drool. Some have all the symptoms, others just a few. Some are dramatically helped by the drug regimen prescribed, others continue to struggle as the regimen gets tweaked.”
Palfreman fingers the alpha-synuclein protein as the major culprit, writing (6): “I find the notion that the disabling symptoms of Parkinson’s disease that I and other Parkies experience are caused by toxic species of alpha-synuclein spreading prion-like [think Mad Cow Disease] throughout the brain to be a very powerful one indeed.
“In a story with many setbacks, this body of research gives me genuine hope — it suggests that, in theory, chemical interventions to break up and destroy the misfolded protein aggregates might help slow, stop, or reverse Parkinson’s. If given early enough, such treatment might even prevent the disease from ever reaching clinical significance.”
Writing in the New York Times, Palfreman said (7):
“Here’s the theory scientists have come up with: Sometimes good proteins go bad. For multiple reasons (like genes, environment and age) proteins can ‘misfold’ and stick to other proteins. When proteins do this, they can become toxic, capable of jumping from cell to cell, causing other alpha-synuclein proteins to do the same and potentially killing neurons (especially dopamine-producing ones) in their wake. This process is not confined to Parkinson’s disease.”
An intriguing hypothesis Palfreman offers is that until recently human beings rarely lived beyond their middle years. Could it be, he asks, that aging cells lose their ability to produce healthy alpha-synuclein?
Cambridge University protein chemist Christopher Dobson says nature requires “that we live long enough to pass on our genes to our offspring, but it doesn’t really care after that. And so it’s evolved proteins that are stable enough and protected well enough by cellular defense mechanisms to last forty, fifty, or sixty years, but there’s not much margin of safety.” (8)
Palfreman concludes that he thinks four issues in particular are important for the Parkinson’s community.
The first is improved delivery of l-dopa to the brain. Less than 10% of a typical dosage now reaches the brain due to the competition with other proteins to cross the blood-brain barrier.
The second is the placebo effect. The placebo effect is where a patient is given a dummy medication in a clinical trial of some other medication yet feels symptomatic relief.
Medicine Net defines the placebo response this way: “A remarkable phenomenon in which a placebo — a fake treatment, an inactive substance like sugar, distilled water, or saline solution — can sometimes improve a patient’s condition simply because the person has the expectation that it will be helpful.” (9)
The third is recognizing the importance of non-motor symptoms such as cognition, sleep disorder, pain and depression.
The fourth is the need to develop personalized medicine. Palfreman defines this as a “collaborative culture of care where specialized professionals and engaged patients work together to try to achieve optimal outcomes.” (10)
Personalized medicine intrigues me. An example is Parkinson Place in Sarasota, FL. It is an integrated facility with education, social activity, exercise and counseling for people in different stages of their disease. It involves teams of providers such as a neurologist, therapist, psychologist and legal experts.
I will write about two other examples of personalized medicine in subsequent chapters: Palliative Care and PD SELF.
Parkinson’s can involve many bodily functions, making it in all likelihood a systemic disease. End stage can be stark but must be faced to plan properly your remaining life with PD. Models exist for engaging patients in improving their care and increasing their odds of a positive outcome.