Postcard from the Edge

Like ‘Morning at Campbello’ (see below), I wrote this for my ‘Road Show’ project — traveling US Route 1 from beginning to end. I thought it fit equally well here in ‘Postcard from the Edge,’ and am posting it here in honor of the new year, but more particularly for a friend and fellow Drew classmate, CLA ’76.

When I set my departure date for this trip, photographing the New England foliage had been one of my modest objectives. But earlier in the year I’d volunteered to participate in a clinical trial for a new drug-delivery system, and the team at Boston Medical Center had kept me busy with examinations and tests through September. Now I was worried I’d miss one of the best shows that New England has to offer.

But, of course, we worry about the small things because the big ones too often prove awkward and unwieldy.

In early 2012, I began to notice something wrong with my left hand. It was minor – an occasional loss of coordination, when touch-typing would become difficult, or when fastening shirt buttons became a struggle. It came and went, but began to grow more frequent and noticeable over the course of several months. Increasingly, I found that I had to tell my fingers how to make each move, step by step, in order to accomplish a simple task. The mental commands that once operated smoothly in the subconscious – push this button, pick up this coin, tie this bow – had to be moved squarely into conscious thought.

I wasn’t particularly concerned, but I made an appointment with my GP, who sent me to an orthopedic specialist, who ran some tests. He sent me to a spine specialist, who ran some tests. He sent me to another orthopedic specialist, who ran some more tests, including EMGs.

By this time, in addition to the loss of coordination in my left hand, my neck had started to get stiff, and I couldn’t turn my head to the left. This led to new challenges, particularly for other motorists entering from the left. But the orthopedists still hadn’t figured out what was going on.

So they sent me to a neurologist. He ran a battery of tests, too, and then ordered an MRI. The morning of the MRI, as I waited to be inserted into the machine, the techs stepped out of the room for a minute. While they were out, I made the mistake of sneaking a look at the doctor’s instructions.  A few words jumped out.  Big, ten-dollar words. Old-people words.  ‘Stroke.’  ‘Tumor.’

Now I was beginning to get concerned.

But the MRI showed nothing. The neurologist looked pensive and said, “I have someone in Boston who I want you to see. He’s one of the best in the field.” And so on February 28th, 2013 – nearly a year since I’d started out on this little expedition – I found myself looking out on Boston’s Longwood medical complex from the eighth floor of Beth Israel Hospital. That’s where Beth Israel’s Movement Disorder Center is housed.

Dr. Daniel Tarsy and his earnest young neurology residents had more tests for me. “Tap your right foot on the floor as fast as you can. Now, the left foot. Close your eyes, extend your right hand, and bring it back to touch your nose. Now, with your left hand. Let me see you walk up and down this hallway.  Tap your left index finger and thumb together as fast as you can.  Now, do the same with your right hand. Now subtract from 100 in increments of seven while you’re doing it.  I’m going to give you three words to remember; in five minutes, I’m going to ask you what those words are.  Ready?”

I was ready for the tests. What I wasn’t ready for was the answer.

“I’m afraid you have Parkinson’s disease, Mr. MacDonald,” Dr. Tarsy finally informed me.

“You mean, that’s all it is?  You had me worried for a minute.” Sarcasm is typically my go-to strategy when things get weird or stressful.

Actually, I didn’t say that at all. I was too surprised to say anything.  Althea Silver, the Center’s research coordinator, handed me a book, 100 Questions & Answers about Parkinson’s Disease.  “This will help you,” she said. She may have even patted my shoulder.

I had my doubts. Explain, maybe, but ‘help’? I asked for something to put it in, but on my walk to the T station it burned a hole in the bag, like a copy of Playboy. Dying to look, too embarrassed to do so.

Later, as I dug into the book, I realized that I knew almost nothing about Parkinson’s. I recognized the disease’s hallmark tremors, rigidity, and the involuntary dance-like movements that I subsequently learned are known as dyskinesia – trust me, new afflictions will improve your vocabulary immeasurably – but beyond those, I was clueless. What little I did know could be credited to the advocacy of the Michael J. Fox Foundation.

For instance, what is Parkinson’s?

Very briefly, it’s caused by a decrease in the body’s production of dopamine, a neurotransmitter manufactured in a deep region of the brain called the substantia nigra. Dopamine helps regulate movement and emotional responses – and its depletion produces the jerky, uncoordinated movements and flat emotional affect one sees in those in advanced stages of the disease. Interestingly, it also helps control the brain’s reward and pleasure centers, enabling us not only to see rewards, but also to take action toward them. Some 60,000 Americans are diagnosed with Parkinson’s each year; worldwide, the disease afflicts somewhere between five and seven million people.

Just why the brain stops manufacturing dopamine is unclear. It has been attributed to ageing, for one.  Most patients are diagnosed between the ages of 55 and 65, and Parkinson’s has been viewed as one more example of the body wearing out. But this fails to account for those who are afflicted with early onset of the disease, some of them reportedly in their teens.

Environment has also been considered as a possible cause, although no one is sure quite what toxins may be involved.

Meanwhile, the role of genetics in Parkinson’s, once dismissed by neuroscientists in their search for a cause, has taken on much greater importance thanks to research over the last two decades. Geneticists are now confident that at least six forms of the disease are classically inherited.

That genetic research has helped contribute to the identification of what scientists now believe is the underlying culprit in the disease: a common protein called alpha-synuclein. For reasons still to be determined, the alpha-synuclein protein found in the brain sometimes mis-folds, forming sticky toxic clumps that jump from cell to cell, killing neurons along the way.  The discovery of this rogue protein has invigorated the Parkinson’s community, leading to an outburst of enthusiasm, hope and research funding – but this is going to be a long trip, so we can unpack that and related matters down the road.

Symptoms differ depending upon the individual and the progression of the disease, and they can be both numerous and astonishingly diverse. They include the tremors, dyskinesia, and stiffness and rigidity (bradykinesia) noted, but go on, and on: weakness; muscle cramping (dystonia); tingling in the extremities (paresthesia); anxiety and depression; loss of appetite; loss of smell and taste; drooling; reduced verbal articulation and projection; increasingly tiny handwriting (micrographia); double vision; difficulty swallowing (dysphagia); choking; constipation; sleep disorders…

In fact, it’s now accepted that the pathology underlying Parkinson’s extends well beyond the substantia nigra, affecting other neural networks, and that this, in turn, leads to many of the disease’s non-motor symptoms. This implies that the disease actually begins much earlier than has typically been thought – years before the movement disorders that typically lead to a diagnosis. The ramifications are both profound and alarming.

Parkinson’s is progressively debilitating. As of yet, there is no cure, only drugs that can help alleviate the symptoms and, in so doing, improve quality of life. Levodopa, a dopamine replacement introduced 50 years ago, remains the gold standard for Parkinson’s treatment. With two levodopa tablets, nearly all of the symptoms I have magically disappear – at least for a few hours.  Although levodopa often brings with it its own problems when taken for extended periods, it offers Parkinson’s sufferers a degree of normalcy – and not just an improvement in the quality of life, but an extension of it.  Prior to the advent of levodopa, Parkinson’s sufferers might expect to live four or five years beyond a diagnosis.  Thanks to the drug, that timeframe can be as long as 15 to 20 years.

Ask a Parkie, and he or she can tell you the precise date of their diagnosis. That’s what we call ourselves: ‘Parkies.’ Like zombies, I sometimes think in my more cynical moments: recognizable, particularly among one’s own, but not a tribe of which I really want to be a member. As Jon Palfreman reveals in his superb book, Brain Storms – The Race to Unlock the Mysteries of Parkinson’s Disease, a diagnosis marks an irreversible change in identity: “…the moment,” he writes of himself, “that one version of me ended, and another version began.”  Yes, Jon, too, suffers from the disease.

Following my diagnosis, I struggled – indeed, struggle still – with that issue of identity. It took me a year to tell my children that I had the disease. And I continue to wrestle with identity’s various handmaidens, such as ambition and accomplishment, and its ugly step-sister, self-worth.

But I had a more immediate challenge. In its earlier stages, Parkinson’s can be practically undetectable – even by professionals, as my sojourn proved. I mean, I looked okay.  Right?  

Inside, though, I felt as if I’d become old almost overnight.  The gross and fine motor skills on my left side, especially in my arm and hand, would suddenly desert me. My arm didn’t swing properly when I walked (a tell-tale sign of the disease). My body felt stiff, and slow, and sometimes my sense of balance was off and I’d lean to the side. When the levodopa wears off, I feel… creaky, like my joints and muscles need lubricating.

For months I struggled to find a metaphor that would make it understandable to others. Finally, I found it in no less a place than The Wizard of Oz: Like the Tin Man, I needed regular oiling if I wasn’t to stiffen up and silently rust away.

Not long ago I had lunch with Jon Palfreman. As it turns out, we live quite close to one another. At first glance, Jon can be quite intimidating. Not only is he tall, but also British, with a public-school accent. Before he earned his doctorate and became a professor of journalism at the University of Oregon, he spent many years as a distinguished journalist and filmmaker, first with the BBC, and later with his own production team, making award-winning documentaries for PBS. He earned a raft of prizes and honors as long as your arm. And then he sat down and wrote one of the definitive books on Parkinson’s.

And yet his thoughtful and incisive questions, his patient ear, his curiosity and genuine interest in others – and, in return, his remarkable candor and approachability – quickly made me feel as if we’d known one another for years.

Over lunch, we asked each other a lot of questions about our respective experiences with Parkinson’s. Late in the meal he asked me what was on my bucket list. I stammered for a minute – the first time during the meal that I’d felt at a loss for words – and finally mumbled something about having been fortunate enough to have achieved many of my dreams. But I named a few plans regardless, lest he accuse me of a paucity of imagination.

As I drove away, I thought about my answer, and winced. The truth is, my list is still miles long, but I didn’t have the heart to tell Jon I wasn’t sure how many of them I’d be able to get to.But this journey on Route 1 – this was achievable, and immediate, and maybe a test for others to come. And so, it had taken on an importance beyond all appearances.