An Alert, Well-Hydrated Artist in No Acute Distress
The story of two artists with incurable neurological disease sharing fear, frustration and friendship as they push to complete the most rewarding work of their careers
When trying to make a diagnosis, a doctor is presented with the outcome of a story — the patient’s condition. She or he must bushwhack backward through that patient’s health history, focusing on elements that might be relevant in order to most expediently arrive at the “what” of an illness and a treatment plan. The “why” of it is a critical, but not urgent, piece of the narrative that is chased down by scientists or the curious patient.
It’s impossible for me to think about my friends’ and my own illnesses without wondering what details of our personal histories might point to causes. And, aside from the particulars of each case, I see our diseases as part of a larger narrative about the health of our generation. Or, more precisely, the ill health of our generation. My robust father, who died at 91, two months before my Parkinson’s diagnosis, often commented on the difficulty of growing old and watching friends die. I understood the pain he must have felt, watching the lights go out around him as the shadow of his own inevitable death moved closer. My equally vigorous father-in-law said to me, “You have Parkinson’s and here I am, 88 years old, with nothing ailing me.” He shook his head because it didn’t seem fair to him and because my disease makes him sad. It makes me sad, too. But my sadness has an edge to it because too many of my generation have been stricken with serious illnesses. My friends and family have dealt with five different kinds of cancer, heart disease, stroke, neurological disease, a rare blood disorder, diabetes, fibromyalgia, lupus, psoriatic arthritis and a host of other debilitating autoimmune diseases, all long before the age of 60.
Yet, journalists continue to optimistically present evidence of our longevity: “The median age of cancer death is 72. We live long enough for it to get us.” And, “Cancer mortality is decreasing.” Statements about cancer’s biologic inevitability seem especially heartless considering that many cancer patients won’t live nearly long enough to experience “inevitable.” Similarly misleading is the spin that’s often put on Parkinson’s disease: the reason it seems like more people have Parkinson’s is that we’re better at diagnosing it. My primary care doctor doesn’t buy this. She’s concerned about the increasing number of patients she sees with PD, many of them under 50. “Parkinson’s has clearly been on the rise,” she says.
As we boomers age, TV ads depicting retired folks wallowing in freedom, adventure (and Viagra), seem less and less relevant. Most of us are spending a lot of our free time caring for not only elderly parents, but also our very ill friends or ourselves. Can we bear to ponder why? Maybe not so much. Occasionally when I sing this solemn note, someone will exhale an exasperated, “I know!” But often, people will give me that, “Well hello, sunshine!” look.
Along with rejoicing in our greater longevity, people of my generation often wax nostalgic about the freedoms we had as kids that our own, “overprotected” kids haven’t enjoyed. Indeed, my childhood on the coast of Maine was full of delicious risk-taking. Before I was even ten, my friends and I would skate unsupervised on thin ice (literally), get caught in powerful tidal currents in sailboats, and lose our way in the woods. There were incidents and accidents galore, but they were considered part of life’s big adventure. Even the story my parents often recounted about my running down the hill when I was two and launching myself off our dock into the cove always elicited light-hearted laughter. “That’s why we called her ‘fleet-foot,’” my mother would say. (My grandfather, dressed in a wool tweed suit, had been in the yard and ran down the hill and jumped in to retrieve me.)
We were awash in freedom. Freedom to run wild through our neighborhoods or ride alone on a public bus, to row a boat without a lifejacket, to bike without a helmet, to wrestle and sprawl, unbelted, in the back of the cigarette smoke-choked family station wagon. Freedom to eat Twinkies, Velveeta, Fruit Loops, a smorgasbord of canned and frozen foods miraculously preserved by new chemicals. And that yummy school paste! Blissfully unaware of pollution, we were allowed to wade into anything liquid, bake ourselves jerky-brown on the beach and chase the DDT truck as it spread its cool fog through our fields. We were the aerosol generation; anything that could be sprayed would be sprayed: hair products, deodorant, mosquito repellant, paint…The inexpendable air and endless ocean could surely carry away our insults. The earth’s generous crops fed us well, thanks to powerful pesticides developed after World War Two. Compared to the era of our parents’ childhoods — wedged between two world wars and chastened by The Great Depression — ours was a lucky time to be a kid.
But then a few good earth stewards crashed our reckless party. In 1962, Rachel Carson, in her book The Silent Spring, laid out the impact on the earth and its creatures of widespread use of DDT and other chemicals, kicking off the environmental movement that would lead to the ban, ten years later, of DDT for agricultural use. In 1964, the U.S. Surgeon General released its first report on the dangers of cigarette smoking. (Fun fact: research shows that smokers are less at risk for Parkinson’s.) President Nixon created the Environmental Protection Agency in 1970, which wrote and enforced measures passed by Congress designed to protect the environment from human activity. But enforcement came too late for those of us who’d grown up with “better living through chemistry.”
Rarely, members of my Parkinson’s Facebook group will discuss possible causes of their PD. Some point to prolonged exposure to certain chemicals and many others mention the well water they grew up drinking, their proximity to land where pesticides like DDT were used, or their family’s own liberal use of insecticides. Research has shown that drinking well water located within 1/3 mile from crops treated with these chemicals is associated with a 66% — 90% increased risk of PD, and the brains of PD patients have been found to contain higher levels of them than brains from control subjects. Since the ban of DDT, two herbicides, Agent Orange, a defoliant used during the Vietnam War, and paraquat have been tied to Parkinson’s.
One of my most troubling discoveries soon after my diagnosis was that at least some of the organic fruits and vegetables I’d been buying for my family for many years were likely to have been treated with an insecticide shown to cause Parkinson’s in animal models. Because Rotenone is derived from the roots and seeds of tropical plants, it was considered “natural” and has been used ubiquitously in organic farming as well as in flea powder and lice treatment for humans. In 2004, following up on research linking it to PD, the EPA ordered a study to test inhalation neurotoxicity. Rather than submitting to the study, the makers of Rotenone opted to discontinue its use in food growing; in the U.S., it is now used only for piscicide (fish kill). But because the EPA has not banned Rotenone, organic produce treated with it in other countries can be imported by the U.S. and labeled organic.
Scientists know that environmental toxins alone — pesticides, solvents (like those used in dry cleaning), certain metals and tainted well water — can’t be blamed for Parkinson’s disease. Most people who’ve had even long-term exposure to pesticides won’t develop the disease. Rather, a combination of nature and nurture makes a person susceptible to PD, a case in which “genetics loads the gun and environment pulls the trigger.” Parkinson’s is heritable; people who have a family member with the disease have a 4%-10% higher risk of developing it themselves if they carry at least one of several gene mutations associated with PD. LRRK2 is the most common mutation known to cause familial PD and occurs in 1–2% of all people with Parkinson’s. Some with PD carry variants of the gene ABCB1, which restrict ABCB1’s role in helping the brain to flush out toxicants. Studies have shown that the relationship between exposure to pesticides like DDT and Parkinson’s disease is 3.5 times stronger in those carrying the ABCB1 gene variants than in non-carriers.
For the approximately 90% of people with Parkinson’s who, like me, don’t have the gene variants associated with PD, scientists believe there have been more than one cause for neuronal death in our brain’s substantia nigra. For example, in a literal case of adding insult to injury, the risk of Parkinson’s doubles when someone exposed to certain pesticides has previously sustained even a moderate head injury. A blow to the head interrupts the blood-brain barrier that protects the brain, allowing toxins to chronically seep in and destroy dopaminergic neurons. Head trauma also produces an increase in alpha-synuclein, a protein that can become pathogenic and clumps in the brains of people with PD (alpha synuclien plays a major role in PD and will be discussed in a future episode.) Add to the mix LRRK2 or other gene mutations implicated in Parkinson’s, and the risk is even higher.
Before learning about the one-two punch etiology of PD, I had hypothesized that the monstrous mosquito population in Maine and the prophylactic spraying of insecticides, including our family favorite, OFF!, were to blame for my PD. But then I discovered what might be another clue. At the recommendation of a friend, I went to see an osteopath for a nerve irritation in my lumbar spine. After gently manipulating my skeleton from my knees to my skull, the osteopath said, “I’m going to easily get a handle on the problem in your back, but what really concerns me is your head. Have you been in a bad accident?”
I was taken aback. I told him about two serious car accidents I’d been in as an adult, neither of which had caused me harm enough to be seen by a doctor.
“Anything else? A bad fall?” he persisted.
I thought about this. “Well,” I said. “I did fall eighteen feet from a tree when I was about ten.”
“Aha!” he said. “How badly were you hurt? Did you hit your head?”
I pictured the messy grove of sumac trees that grew on the steep, dirt slope next to our house in Maine. My parents had planted them to hide the neighbors’ new concrete ranch house, across the cove. They were tall and spindly, their trunks no more than three or four inches across, their narrow branches brittle. I was a monkey, forever pushing my luck, scrambling from limb to limb. One day when I was high in the trees, the limb I was sitting on broke. The fall was long enough — eighteen feet, we measured later — that I remember reaching out for branches I passed, hoping to interrupt my descent. I landed on my side, winded and stunned. My mother, who was enjoying what was probably iced tea in a lawn chair right next to the sumacs, heard the crack and the thud. I don’t remember anything about the time after the fall — my sister thinks she remembers I was knocked out for a bit — but I must have seen a doctor because I recall having to wear some kind of neck collar for a couple of weeks.
The osteopath explained to me that the head and spine are designed for constant movement; in a healthy adult, there’s an inherent rhythmic motion of the cranial bones that changes the shape of the dura mater, a membrane that encloses the brain and spinal cord. He felt that my cranium, and therefore my dura mater, had been immobilized — a condition he would typically find in a patient who’d experienced serious head trauma. My cranial rigidity, combined with hypercontracted spinal muscles caused by long hours at the computer and aggravated by Parkinson’s, he said, could be restricting the flow of blood, lymph and cerebral spinal fluids. By mobilizing my cranial sutures and applying gentle pressure to surrounding tissue, he felt he could release restrictions and restore more normal function.
The osteopath stopped short of suggesting that his finding could be a clue to my PD, and I had read nothing in the Parkinson’s literature that implicated a restricted flow of fluids to and from the brain in the development of the disease. And, because I understood that osteopathy stands to the side of the medical establishment, I didn’t share the osteopath’s ideas with my other doctors. But the connection made a lot of sense to me: my brain cells have been dying at an abnormal rate; couldn’t this be at least in part because they’ve been insufficiently supported by my body’s circulation system? I thought about Hadley, too: could her pulmonary stenosis at birth and her lifetime of extremely low blood pressure have impaired the bloodstream’s transport of oxygen and other nutrients to her brain cells, making them vulnerable to toxicants like the epoxy resin she used while sculpting?
Last year, a year after my first visit to the osteopath, a major discovery was made that strongly supports the idea that an impaired flow of fluids could be a contributing factor to my PD: a network of lymph vessels in the membrane surrounding the brain and spinal cord that connects the brain to the body’s immune system. Until this finding, scientists believed the human body had been thoroughly mapped and that the brain’s and body’s immune systems were separate. It was assumed that the blood-brain barrier largely protected the brain from the full assault of pathogens on the body as well as the immune response they triggered. The discovery that the central nervous system and immune system are connected not only provides new clues about neurological diseases like multiple sclerosis (MS) that are known to have an immune component, but also suggests that immune cells also might play a role in Parkinson’s. In PD, incorrectly formed alpha-synuclein proteins collect in the brain and become pathogenic, driving neuronal death. Since the recently identified lymphatic vessels are responsible for clearing away dead cells and other waste, scientists hypothesize that in Parkinson’s, a malfunction in the vessels makes them unable to properly dispose of the neurotoxic proteins.
In 2014, it was discovered that chronic activation of the immune system is the cause of many symptoms of neurodegenerative disease. Initially, our immune system gears up to protect our body from infections, toxins and injury, but if activation is prolonged, it can cause a heightened inflammatory response. In PD, this means that overactive immune cells in the brain attack dopamine neurons that help control the body’s movements. Learning this was an aha! moment for me: In Episode Six, I discussed how my integrative medicine doctor had explained my illness in my early 40’s as being caused by an overactive immune system, a diagnosis my primary care physician at the time had a good chuckle over. Looking at the research, it seems more and more likely that he was on target and that the immune activation could have hastened the onset of Parkinson’s. Now, the unearthing of vessels connecting the central nervous system to the immune system will shed light on exactly how the body’s immune response impacts the brain. This carries enormous implications for the future of PD treatments. In the meantime, I will probably continue to have the osteopath work on relieving the tissue and cranial restrictions he’s found, in an effort to ameliorate the lousy janitorial job being done by my newly discovered lymph vessels.
All of us begin life relentlessly asking why? why? why? To be human is to want to know. When I first joined our Parkinson’s Facebook group, I had a powerful desire to pursue the“why” of our shared disease. I imagined collecting data from all our members through an exhaustive survey that would ask about everything: health history, injuries, environment, work habits, emotional life — you name it. We had a ready-made young onset PD study group, I figured; all we had to do was harvest the information. But I soon realized that people who are sick generally are more inclined to look not backward, but forward, at developments that will bring new therapies, or even a cure, while staying as well as they can. I needn’t have worried that our data would go uncollected, though. Since my diagnosis, numerous scientific entities have begun administering online surveys that will inform PD research; I participate in two that are ongoing with the genetics company 23andMe and the Michael J. Fox Foundation.
A patient with a progressive neurological disease who asks “why?” is not likely to significantly change the course of her illness. But as we learn more about new factors that might contribute to the development of neurological conditions, we patients can broaden our approach to living with disease, and healthy people can take more preventative steps. Already, most of us have done what we can to avoid toxins and unhealthful environments. Now that we know that a stressed immune system creates excess inflammation that in turn can precipitate neuronal death, perhaps we will think twice about living a life of multitasking and more seriously pursue activities like sleep, meditation and yoga, which are known to calm the nervous system and relieve the immune system of its hypervigilance. Now that we know our precious brain, once considered “immunologically privileged,” is connected directly to the body’s immune system, perhaps we will be more vigilant about keeping our bodies healthy and treating anxiety and depression, which affect our immunity. The growing publicity about the potentially dire, delayed consequences of head trauma might make us more willing to don a helmet when skiing or riding a bike and less eager to encourage our children to play contact sports. With so many of us struggling with Alzheimer’s and Parkinson’s — in the U.S. alone, 5.4 million and over 1 million, respectively — it’s time to consider that we didn’t simply get dealt a bad hand (pun intended) and wind up with a devastating, mysterious brain disease.
Our bodies, our brains: they are our houses for life. When they start to fall apart, we don’t get to rip them down and start over. But if we ask enough questions, we can get to know them and care for them, room by messy room.
Note: For those who are worried I will never get back to Hadley’s story, fear not! And please bear with me through these digressions. Thank you!