Doug Lindsay knows a thing or two about adrenal glands. A few years back the University City native bought a few batches of pig adrenal glands. He has successfully persuaded a veterinarian to dissect the adrenals of a freshly deceased cat and looked into enticing an adventuresome surgeon to have a go at a human cadaver's pair.
The adrenal glands Lindsay fixates upon most, though, are his own.
Perched one apiece atop the kidneys, human adrenal glands are generally no bigger than the tip of a pinky finger. But the hormones they produce play a vital role in regulating metabolism and in triggering the body's "fight or flight" response in states of distress.
Lindsay suspects his adrenals produce too much epinephrine (known more commonly as adrenaline), and that he's hypersensitive to the oversupply. He wants to correct the problem by doing away with the region at the center of each gland, the medulla.
The conventional wisdom in surgical circles is that excising a person's adrenal medullae is a dicey proposition at best. Lindsay is convinced it's doable.
He demonstrates on a shelled egg.
"Apparently the surgeon will be cutting into the gland like this, and opening it to the degree he can," the 32-year-old explains, slicing through the hard-boiled orb's white exterior with a paring knife. "From what I'm told, the medulla is frail and crumbles — kind of like this yolk — so they'll go in there with something flat, maybe a little hard, and start scooping it out."
Here Lindsay switches to a teaspoon.
"Their goal is to get it all, so I think they'll use an electrosurgical tool and burn what's left to make sure any of the yolk — the medulla — left in there is dead. Then they'll literally sew this back up" — he guides the white egg halves back together — "and then eventually they'll do the other gland."
With that Lindsay strides from kitchen to living room in the Ladue ranch home where his Aunt Jeanne and Uncle Terry Carmack live and where he occasionally receives visitors. He projects a clean-cut visage, having long ago traded shaggy, tawny locks for a neat crop. He completes the look with wire-rimmed glasses, creased khakis and button-downs. His gait is slow and deliberate. The compactness of the floor plan here makes it easier for him to move about, if only for minutes at a time. And it's here that he keeps his reclining wheelchair, an elaborate, six-foot-long contraption that allows him to converse nearly eye to eye.
Ensconced in the chair, the De Smet Jesuit High School graduate launches into a lecture on de-medullation in animals, illustrating his points via a 363-page computer file that contains his medical records along with numerous scientific texts, including a lone article that chronicles the long-ago de-medullations of two Danes.
The document essentially comprises the sales pitch to surgeons Lindsay hopes might treat him with this innovative surgery.
It has been ten years since Lindsay came down with a mysterious illness. Of its numerous symptoms, the most debilitating is known as orthostatic intolerance. Translated into plain English: When he's in an upright position, Lindsay's blood pressure drops and his heart rate soars. He sweats and becomes lightheaded. If he sits or stands for too long, he risks a heart attack or stroke.
Healthy, Lindsay was known as a hell of a dancer. He liked to write and play banjo. He was a storyteller. He liked the rush of a speeding car.
Sick, the guy who never had trouble finding an audience couldn't get doctors to listen to him.
While his friends went about their twenties marrying, completing the seminary, hanging a shingle as J.D. and Ph.D., Lindsay hit the books from bed. He became a self-taught student of medicine, trying to puzzle out what ailed him.
His symptoms were consistent with Chronic Fatigue Syndrome (CFS), a frustrating condition with no known cause or cure, shared by as many as 4 million Americans. But Lindsay ultimately confirmed he suffers from something rarer: a dysautonomia, or breakdown in the autonomic nervous system, which regulates bodily basics such as blood pressure and metabolism. The spectrum of dysautonomic diseases is broad, and the cause for Lindsay's particular dysfunction remains unknown. It could be CFS, or an autoimmune virus, or any number of things.
Such as a problem with his adrenal glands.
Doctors say the earlier a person is given an accurate label for a malady, the better the chances of adequate treatment and ultimate recovery. Tell that to the estimated 25 million Americans who are afflicted with what the medical community calls "orphan diseases." Medical science has identified more than 6,000 such conditions, each of which affects fewer than 200,000 people. Many more still await discovery and a full workup in the medical literature.
Because the mysteries can be so confounding, the specialists few and the resources limited, it can take years to pin down and treat an undiagnosed ailment. Patients are expected to be — well, patient, while science slogs through the riddles.
As Mary Dunkle, spokeswoman for the Connecticut-based National Organization for Rare Disorders, puts it: "Many people will never get a diagnosis."
Doug Lindsay's quest for health has hurtled the Rockhurst University dropout into some improbable adventures, including making a presentation about his condition at a medical conference. But most of his life transpires in two rooms of his childhood home in U. City, where still lifes painted by his mother adorn the walls, a handwritten sheet of paper hanging above the fireplace admonishes "Pray, hope and don't worry" and his electronics — several computers, a workhorse printer/copier — fill the spaces between his three beds: one for sleeping, another for watching television (Burn Notice, Book TV and the NBA) and the third for pursuing his research.
His parents separated in 1999 (they later divorced), and his mother moved into a nursing home in 2006, leaving Lindsay the house to himself. A night owl, he turns in past 3 a.m. and rises by noon. Regular visits from a nurse and workers from a disability organization furnish the bulk of his social interaction outside the Internet and phone. Because Lindsay is unable to work, Medicaid pays the bills.
Mentors and friends needle Lindsay about what they call his "control freak" tendencies, but they admire his tenacity and his power of persuasion. He treads on terrain where professionals could be quick to dismiss him, yet he has managed to capture the attention of top-flight clinicians around the nation.
"His story is fascinating and illustrates the frustrations associated with chronic orthostatic intolerance — for patients, for physicians, for caretakers and for researchers," observes David Goldstein, an M.D., Ph.D. and senior investigator at the National Institutes of Health who has penned several tomes on the autonomic nervous system and who has corresponded with Lindsay.
"He's very, very sophisticated," adds David Robertson, a neurologist and professor of medicine at Vanderbilt University who has become a sounding board for Lindsay. "Very few people understand and think about their illness with the level of resolution that Doug thinks about his."
It was June of 1999 when Lindsay suddenly found himself sweating through several undershirts a night. He was twenty years old and just starting a summer job in a biochemistry lab at the University of Kansas in Lawrence. Feverish and achy, he couldn't complete a day's work without feeling exhausted.
He traveled back to St. Louis to see his pediatrician. The doctor suspected mononucleosis and told him to rest at home.
Once he was feeling better, Lindsay returned to KU. He made it through three days before he had to quit. "Walking to and from the lab made me so lightheaded and sick that I had to lie in a lump for an hour in the car before driving back to my room to go lie in a lump there," he recalls.
Lindsay came home again, hoping to sleep off the sickness before the start of his senior year at Rockhurst, where he was majoring in biology. In August he revved up his new Camaro and drove himself back to school. "I got in one day of my senior year, and I came home. That was it," Lindsay recounts. "Two weeks after that, it was really bad."
"It" kept piling on: constant pain in his forearms, back and legs. "I felt like somebody had run cheese graters over my tendons," he says. Spells of lightheadedness, sweating and racing pulse walloped him without warning: "You'd sit down to watch TV for ten minutes, you'd be fine, and all of a sudden it hit you."
After prolonged periods upright, Lindsay would find himself staggering back to bed, drenched with sweat and in pain. "I was like a doll without strings," he says. "The room would be spinning. My heart would be pounding. If you put a piece of paper on my chest, you could see it fluttering."
The simplest tasks — showering, climbing stairs — left him limp for hours.
Lindsay was skeptical of the mononucleosis diagnosis, and more so when a lab test for the virus came back inconclusive. Visits to two internists led him to a handful of specialists over a period of months. "Neurology, endocrinology, internal medicine. St. Mary's, St. John's, SLU," Lindsay ticks off the list. One physician suspected multiple sclerosis. Tests proved negative. Other doctors said the so-called post-fatigue from mono could persist for three to five years.
Lindsay grows indignant at the memory. "What was I going to do, go stare at a calendar? Tell my friends, 'I'll call you after the next Olympics?'"
He tried changing his diet. Gorging on vitamin B12. Acupuncture. Nothing helped.
He thought about the fact that he'd never had the stamina for team sports — or, for that matter, for any physical activity that demanded endurance. But it was his family's medical history that really gave him pause.
Jeanne Carmack, Lindsay's aunt, had fallen ill in her twenties. She first felt crippling muscle rigidity, then inexplicably gained almost 40 pounds. Carmack ended up spending two entire years in bed, immobilized by pain and fatigue.
Doug's mother, Barbara Lindsay, had developed similar symptoms at age 30. At first she couldn't lift her toddler son. Then she became exhausted by daily work, errands and chores. Before long she couldn't leave the couch.
The two women each consulted dozens and dozens of doctors over three decades. They pleaded with God for answers, visiting the Carmelite Monastery to pray at the statue of St. Jude when the hard-hewn pews at their own church became too painful.
But to this day neither sister has ever received a conclusive diagnosis to explain the root of her problems. Equally mysterious: Carmack has reclaimed a relatively normal life; Barbara Lindsay needs 24-hour nursing care.
As for Doug Lindsay, six months into his illness he was sure of one thing: He had what Mom and Aunt Jeanne had.
Desperate, he delved into physiology textbooks, and by the spring of 2000 he concluded that his autonomic nervous system was malfunctioning. Centered in the brain and spinal cord, the apparatus regulates involuntary physiological functions such as heart rate, blood pressure and body temperature. In states of distress, the so-called fight or flight conditions, the system gets assistance from the adrenal glands to restore the body's equilibrium.
Lindsay wrote up a summary of his hypothesis, complete with bibliography, and presented it to several of the physicians he'd seen previously.
"I had cut my hair, trying to look professional, and I bring the bibliography [to the appointments], right, because I see myself as this Jesuit-educated scientist. And they say, 'Problems like this don't exist,'" Lindsay says. "One person suggested I see a family member of theirs — who's a psychiatrist."
"I got sick, my mom is sick, my father left, I can't do anything. I can't go to a psychiatrist who will say, 'He's sad, and that's why his heart rate is up. He used to have a 3.9 [GPA] and now he doesn't go to school anymore. He says he's in pain, but we can't see pain.' Get out of town!"
One morning in late 2000, Lindsay found himself in the emergency room, fearing he was having a heart attack. He recalls telling a nurse he thought he had an autonomic nervous system disorder.
"They call my doctor, and a couple hours later they come to me and they say, 'He said you should have gone to see a psychiatrist and you didn't go.'"
He was discharged.
The one plausible explanation Lindsay's doctors had to offer was Chronic Fatigue Syndrome. But he was skeptical.
Medical lore suggests U.S. doctors saw precursors to CFS as early as the Civil War, though it wasn't until 1988 that the Centers for Disease Control coined the term. To be diagnosed with CFS, a patient has to have experienced more than six months of fatigue coupled with at least four other symptoms, including muscle or joint pain, sore throat, post-exertion exhaustion, short-term memory problems, headaches and fitful sleep.
Doctors say the condition is difficult to diagnose because pain and fatigue can't be measured, and because there's no diagnostic tool to test for the illness in the way that cell biopsies, for example, can reveal cancer. The process is typically one of elimination.
"Basically, if the doctor has excluded every medical or psychiatric explanation, then you have CFS," explains Suzanne Vernon, scientific director at the North Carolina-based CFIDS (Chronic Fatigue and Immune Dysfunction Syndrome) Association of America. "It's a bummer of a name," she adds, "and that really didn't help it get much scientific traction."
In fact, many physicians initially suspected that the disorder was actually a mental illness that involved fabricating symptoms as a ploy for attention. Psychiatry was commonly prescribed.
"When I started working in the field, the term 'yuppie flu' was often used in a pejorative way to describe these patients," recalls Peter Rowe, a CFS expert at Johns Hopkins University in Baltimore. "The science that has built up around it has helped change those views."
There's still no known cause or cure, but most physicians today acknowledge not only that CFS is real but also that it can be more debilitating than many other chronic illnesses. Says Kurt Kroenke, an internist and professor of medicine at Indiana University: "[CFS] doesn't kill people, but in other ways you could look at it as a slow death."
One interesting finding of recent years was the discovery of orthostatic intolerance in a subset of CFS patients. It is a symptom shared by patients with certain forms of dysautonomia.
Not every CFS patient has autonomic dysfunction, just as chronic fatigue is not universal among dysautonomia patients. But when it comes to research, both fields remain relatively unexplored, their pathology shot through with mysteries. In a handbook for dysautonomia patients, NIH researcher Goldstein writes that "[d]espite the fact that dysautonomias affect over a million Americans, you will probably find that very few people and even few doctors have heard of dysautonomias."
Paola Sandroni, a neurologist at the Mayo Clinic in Rochester, Minnesota, calls dysautonomia "a big spectrum of diseases. You can go from the mildest form, which may manifest itself with dryness of the eyes and mouth and a little sweating in the feet, to the Cadillac of the spectrum, which is Multiple System Atrophy, when the patient can't stand without passing out, the bowels don't work, guys have sexual dysfunction, there's a risk of heat stroke — everything fails."
Some dysautonomic diseases are acute and reversible, others chronic and degenerative. MSA is fatal. Many other dysautonomias are considered "manageable," though not curable. Rarely is a dysautonomia caused by a genetic mutation.
Perhaps the main element that distinguishes dysautonomias from other illnesses is the fact that they are "mind-body" disorders, writes Goldstein. "This is a difficult subject for both doctors and patients. The problem is the old notion that the body and mind are separate and distinct in a person, and so diseases must be either physical or mental. If the disorder were physical, it would be 'real,' something imposed on the individual, while if it were mental, and 'in your head,' it would not be real, but something created in and by the individual." (Goldstein notes that treatment of dysautonomias can involve psychiatry.)
Laurence Kinsella, chief of neurology and neurophysiology at Forest Park Hospital, says patients who suspect they have autonomic dysfunction have "come out of the woodwork" since he launched his lab a little over a year ago. "These people come in to see you who've been homebound for years without a diagnosis — you make one and you give them their life back," says Kinsella, who may be the only St. Louis doctor specializing in dysautonomias. He says he has about 50 such patients in his care.
Mary Dunkle says the National Organization for Rare Disorders hears from more than 10,000 people each month who are in search of answers to their ailments. "The number-one issue people contact us about is the difficulty of getting an accurate diagnosis," Dunkle says. Her group likes to upend an analogy taught in medical school: When you hear hoof beats, think horses. "We say that while it might be perfectly logical to think first of the most common thing, always keep in the back of your head the idea that it may be a zebra."
Linda Smith spent twenty years searching for a doctor to explain her racing heart, fatigue and frequent fainting. "When I was telling the doctors this must be genetic, I had an expert laugh at me," Smith says by phone from Red Wing, Minnesota, home of the National Dysautonomia Research Foundation (NDRF), the nonprofit organization she founded in 1997. "I said: 'I can prove it. I have an identical twin! I'll bring her in, and you can study her, too.'"
Robertson and his fellow scientists at Vanderbilt University took Smith up on the offer and proceeded to find a genetic defect that was causing her dysautonomia. The case was chronicled in the New England Journal of Medicine in 2000. "Five of my ten siblings had it, including my twin!" Smith exclaims. "I'd told them, and yet they didn't want to listen because I'm a simple everyday person. I don't have a doctorate in this stuff."
If Linda Smith was the zebra, Doug Lindsay is the self-described "guy with the unicorn in his back yard."
In the spring of 2004, Lindsay lay on a mattress in the back of a minivan piloted by a friend, en route to Alabama to undergo his first test for dysautonomia.
It had been three years since Linda Smith furnished him with documentation on autonomic-system disorders, and by now Lindsay was certain he had one.
The trip to Alabama was the culmination of an extensive research and lobbying effort on Lindsay's part, during which he'd cold-called physicians and biochemists all over the nation and mastered science-speak and the art of name-dropping, learning how to use a conversation with one distinguished doc to garner the attention of another.
In one twelve-month span, Lindsay had left St. Louis three times to attend science and medical conventions, purchasing multiple plane tickets for himself in order to recline en route and marshaling a squadron of friends to steer his wheelchair, open doors and stall doctors until he could be rolled in to the conversation.
"We were a circus show," recalls his friend, Zach Jones, now a film producer in Portland, Oregon. "Here he hasn't even graduated from college yet, and we roll him up to these Ph.D.s so he can challenge their ideas and have a discussion. These guys have been studying this stuff for a long time — they know what they're talking about — and he'd be able to go toe to toe with them."
For the doctors it was downtime. For "ol' Doug Lindsay," as he sometimes calls himself, it was a lobbying effort to win back his life.
He was tireless, courting M.D.s day and night, confirms his friend Matt Krentz, a Citizen Kane's waiter better known as the writer-director of the local basketball flick Streetballers, who accompanied Lindsay on several of his trips.
"You have to picture an after-party, and all these doctors are dancing — women are even coming over asking Zach and me to dance — and we're kind of smiling, trying to be nice, but really we're like, 'Um, we're here to get you to help this patient, and you're asking us to dance?' Luckily, everywhere you go you meet one person who wants to help."
In the end that person was Cecil Coghlan, a cardiologist and dysautonomia specialist who agreed to assess Lindsay's condition at his dysautonomia lab at the University of Alabama-Birmingham.
So it was that on March 16, 2004, Dr. Coghlan strapped Lindsay to a flat board at the ankles, hips and head, then watched his patient's blood pressure and heart rate as the board was raised gradually to an upright position. Known as the tilt-table test, the process provides a measure of orthostatic intolerance in dysautonomia patients.
Coghlan typically tested his subjects' vitals for 45 minutes upright. Lindsay's heart rate soared so high that he had to be lowered after only seven minutes so that he wouldn't pass out. He fared better than only 10 percent of Coghlan's similarly afflicted patients.
The results were a vindication: Lindsay was definitely dysautonomic.
The cause, be it CFS or something else, remained unclear, but Coghlan was on board for an experimental — and counterintuitive — treatment plan Lindsay proposed.
Epinephrine is delivered to the body's organs via norepinephrine, a related neurotransmitter also produced in the adrenal medullae. Norepinephrine exists in drug form, marketed under the trade name Levophed. It is primarily employed in intensive-care units as a last-ditch effort to revive patients in cardiac arrest.
Lindsay theorized that he might be able to use Levophed, delivered to his bloodstream in a small but constant quantity, to fool his adrenal glands into dialing back epinephrine production and delivery, thus literally leveling his equilibrium.
"Think of it like alcohol," Lindsay explains. "You drink it, and you get pretty tipsy in the short term. But if you keep on drinking, over the long term you build up a tolerance."
Coghlan wrote Lindsay a prescription for Levophed, administered via a pump in a continuous infusion through a surgically implanted port.
Coghlan is well respected among specialists in autonomic dysfunction. But those who were interviewed for this article expressed surprise that he endorsed the Levophed treatment, even in a low dosage. Several called it "highly unusual" and characterized it as potential malpractice.
"It never would have occurred to me to try it," allows a more measured David Robertson, the dysautonomia expert at Vanderbilt with whom Lindsay has corresponded over the years. "But, you know, the world is full of surprises. He believes he feels better on that."
Still, adds Robertson: "That's a surprise, I think."
Lindsay says he was well aware his suggestion seemed radical. "One big [doctor] in the field said, 'If you do it wrong, it'll kill you.'"
More commonly, doctors prescribe so-called beta blockers, a class of drugs that cancel the effects of epinephrine and that are most often used to treat ailments including high blood pressure, overactive thyroid and anxiety.
Lindsay has experimented with beta blockers over the years, but the drugs ratchet up his pain and fatigue. He says doctors have suggested potential long-term benefits if he were to remain on the drugs for months at a time, but he's not convinced he can withstand the short-term side effects. "That's a steep gamble to take," he says.
Even with Levophed, his stamina has deteriorated to the point that in order to stay plugged in to the world he now requires audio books, dictation software and a hands-free telephone. Yet he is convinced the norepinephrine infusion has helped keep his disorder at bay.
"It's hard to believe this is what 'good' looks like," he concedes, reclining one afternoon at home as the Levophed-delivery apparatus strapped to his hip clicks and hisses. "But I am so much better than I was. It's been a miracle."
Dr. Coghlan, who recently retired, did not respond to an interview request. Lindsay has since found a new doctor willing to prescribe him Levophed. He declines to identify that physician.
As a kid Lindsay refused to wear shorts. In high school he joined a fly-fishing club peopled with twenty- and thirtysomethings. During college he was the dude who rounded up five bucks from everybody in the dorm and proceeded to grill up a feast.
"I wouldn't call him the class clown, but he was very funny, very witty in a very smart way," says Brian Nolan, a St. Louis lawyer who attended De Smet in the mid-'90s with Lindsay. "When we wrote for the school newspaper, I was the guy doing the features, while Doug wanted to get all the dirt on the subjects of the day."
Zach Jones, the Oregon-based film producer, recalls wanting to be Lindsay's friend the moment he saw him cut a rug at a Rockhurst party. "In walks this character in a full-on '70s suit. I didn't know him, but I could tell that he was 100 percent into what he was doing. He was the guy wearing that suit, motherfucker. And he was dancing — man, he was dancing pretty much almost by himself. And he owned it."
Friends say the pre-illness Lindsay was beloved by girls and guys alike, if from a distance. Steven Ladd, an artist in New York acclaimed for the handbags he designs, recalls the day Lindsay didn't get an invitation to join Ladd's fraternity and came to him for an explanation. "It was like, 'Doug, you're kind of neither here nor there. You're not staking your loyalties. You're not claiming your territory,'" says Ladd. "He was very social, very fun, very active, he could do a little disco, and we loved him for it. But he was almost this self-proclaimed Fun Guy."
The independent streak created a handicap when Lindsay first got sick. Says Jones: "I think he definitely learned quickly who his real friends were."
Lindsay initially took advantage of his "terrible opportunity" to read widely: from Voltaire to Bruce Lee, James Joyce to F. Scott Fitzgerald, even Lord Byron's 700-page poetic satire on Don Juan. Then, a year into his illness, after the shock had worn off, he took a strategic tack toward cultivating friendships.
"We'd gone to the same college, but to be honest I didn't know exactly who he was when he called me [in 2000]," Krentz recalls. "I think he just wanted someone to talk to at first."
Krentz says he soon began to realize what made the friendship work.
"You look at Doug and Steven [Ladd] and me, we're all trying to accomplish things for which there's no road map, where we have no business being: me trying to make this movie, Steven getting his purses into places like the Louvre, and Doug trying to solve medical mysteries."
Ladd says he hadn't heard from Lindsay in two years when, out of the blue in 2000, his former buddy rang him and launched into a two-hour soliloquy about his medical dilemma — in a manner Ladd didn't find compelling. "He had this chip [on his shoulder] — he was so arrogant," says Ladd, who proceeded to tell Lindsay he'd be better off dead than acting like an asshole.
Adds Ladd: "Ever since then it's been a magnificent journey with Doug."
Krentz is Lindsay's crisis coordinator. He has driven his friend to the hospital in the middle of the night and moved Lindsay's mother into the nursing home. Nolan, who reconnected with Lindsay at a high school reunion three years ago, now lends a hand for the occasional trip to visit Barbara. Jones indulges Lindsay in two-hour telephone conversations.
As for Ladd, he says he takes it upon himself to remind Lindsay "that he has to have something good in his life. Small victories. I tell him I need to be able to hear about something interesting happening with him. I say, 'Doug, you may die of this, your mom may die of this or you may never get past this, and so you've got to have something else going on. Or you're just the Guy Fighting This Illness.'"
Lindsay returned from Birmingham with newfound optimism. There was the prescription for Levophed and the fact that benign, non-cancerous tumors are a common cause of adrenal malfunction and can be surgically removed. Dr. Coghlan, his physician in Alabama, had him undergo a series of radiological scans in St. Louis.
But the images turned up no tumors.
Upon closer inspection of the scans and through consultation with a handful of research M.D.s, Lindsay concluded that his adrenal glands are abnormally large. Convinced that excising the culprits might eradicate the dysautonomia, he got to work researching surgical options.
But finding a surgeon to scrape out one's medullae is no simple proposition.
Angelique Reitsma, a physician-bioethicist at the University of Pennsylvania who has authored national guidelines for innovative surgeries, cites numerous reasons for a surgeon to attempt a new procedure: "It's very cool for surgeons to be the first to have done something technically challenging. They like to show that off among their colleagues at conferences. They like to publish about it. For lack of a better word, it's a total turn-on."
Hospitals benefit from a marketing standpoint if they're able to advertise a specialty in a new area, Reitsma adds, and the entire medical community's body of knowledge expands. "It's great to help somebody, and it generates business."
At the same time, say ethicists, doctors must remember the cardinal rule of medicine: First, do no harm.
"No one can force us to do something we don't think is the right thing to do," notes Ira Kodner, a professor of surgery at Washington University and the former head of the school's erstwhile ethics institute.
Kodner says that means checking the science: Is there enough substantial research to show why the procedure might be worth it? It also means looking into the vulnerability of the sick: Is the patient so desperate that he can't adequately weigh a procedure's risks and sign off on them?
"Surgeons don't like to kill people," says Ronald Munson, a professor of philosophy at University of Missouri-St. Louis and the author of a widely used medical ethics textbook. "They are nearly all reluctant to operate on somebody who is not likely to benefit from a surgery."
The condition Lindsay believes he suffers from has a name: bilateral adrenal medullary hyperplasia. But nearly everything else about it — right down to its very existence — lacks consensus in the medical community.
"If the condition is real, it's rare," offers Quan-Yang Duh (the surname is pronounced due), chief of endocrine surgery at the University of California-San Francisco. "If it's rare, how do you get people to want to spend time and resources to work on it? You have trouble getting drug companies to spend money on drugs for orphan diseases. And you'll have trouble doing experimental operations to see if it works, because you don't have enough people to say, 'Hey, this works.' So it's one of these things where: How do you even get started?"
The biggest surgery-related risk clinicians say Lindsay would face is Addison's disease, a fatal condition in which the adrenal cortex shuts down.
The glands are small, and the medulla tends to be sticky. Surgeons say trying to scrape it from the gland would be comparable to removing peanut butter from a sandwich.
"There are significant ethical questions," concludes Duh, the surgeon at UCSF. "One can imagine a way to do it: You take out one side, scrape off the peanut butter, put the adrenal back in and see if it worked. If it didn't work, you haven't completely hurt the patient. At least the patient would not be Addisonian. Then you try the other side and see whether now the patient is better.
"But God, there are so many ifs in between."
A middle-aged crooner is singing Louis Armstrong's "What a Wonderful World" in the lounge of the Bethesda Meadow skilled-care nursing home in Ellisville when Doug Lindsay rolls in for an afternoon visit with his mother. Barbara Lindsay lives here, in a fourth-floor room she hasn't left since she moved in three years ago last November. She'll turn 60 in February.
Barbara and her only child exchange I-love-yous and a tender touch of hands. They talk twice a day by phone, but it has been months since Doug's last visit. He must make the twenty-mile ride from University City in a fully reclined passenger seat. The trip saps him too much to allow for more frequent get-togethers.
"Look at that hair," he says now, inspecting the thick tresses that splay across his mother's pillow.
"I've been asking for a haircut for a year," she confides. "I can't stand it. It's so heaaavy."
On the wall above Barbara's head hang photos of Doug, a string of Rosary beads and a typed prayer to St. Jude, the patron saint of lost causes. On the opposite wall is a map of the world.
Barbara Lindsay connects to civilization via radio. Typically NPR and KMOX, though a feather-light remote control lets her switch to KTRS for Cardinals games. "They get up every day, no matter what the weather is, and they do their job," she marvels of the ballplayers.
Barbara graduated from Fontbonne College in the 1970s and worked in a Washington University biochemistry lab before moving with her husband to Texas, where Doug was born. "He was walking by the age of ten months and he couldn't wait to get out," she remembers. "His first words were 'shoes on,' because he knew that meant he was leaving!"
The Lindsays moved from Texas to Tennessee and, in the mid-1980s, back to St. Louis. As her symptoms mounted, Barbara had to give up gardening, writing, painting and eventually drawing, too. Mothering became a challenge. "The last teacher's conference I went to, [Doug] was in the fourth grade," she says. "I couldn't find anyplace to sit that was comfortable, and there were stairs to climb."
By 1992 Barbara could no longer leave the house, and soon after she was confined to the first floor. "It was good to have him around," she says of her son's homecoming in 1999. "But then I felt terrible."
During a prolonged power outage that left the house without air conditioning in July 2006, Lindsay had his mother evacuated, fearing heatstroke. After brief stays at a local hospital and a different nursing home, she became a permanent resident at Bethesda.
The arrangement has proven difficult.
In 2007 Bethesda attempted to evict Barbara Lindsay for nonpayment. Doug and her sister Jeanne fought the eviction in court and won the following year when St. Louis County Circuit Court Judge David Lee Vincent determined the nursing home had erred in its billing statements.
In the meantime, Barbara Lindsay's condition continued to deteriorate. Today she can turn her head slightly and has limited use of both arms and hands but no core strength. For "exercise" once a day, two aides gather her up with towels and ease her into a chair, where she sits for an hour, if she can endure it.
Doug Lindsay says Bethesda shares blame for his mother's decline. He maintains that the nursing home has ignored the family's directions pertaining to dietary needs and daily toileting, causing Barbara to contract bedsores and infections and to lose so much weight that she now requires a feeding tube in order to receive adequate nutrition.
Lindsay has brought copious documentation to the Missouri Department of Health and Senior Services, to politicians and to federal regulators. Investigators at the state health agency have completed at least two official inquiries and have not substantiated any complaints.
DHSS spokeswoman Charisse Pappas declines to comment, explaining, "Whenever there's a substantiated investigation, that is public information, and we can release that. We can't just comment at all by law on closed, unsubstantiated investigations."
Debra Schuster, an elder-care attorney who pursued Barbara Lindsay's case for a time on behalf of the family, believes the health department "glossed over and minimized the issues" Doug Lindsay raised. Says Schuster: "I think because it is a very unusual condition, and because Doug is such an advocate, they were painting Mrs. Lindsay as having a psychosomatic, somewhat hypochondriacal condition, and basically marginalizing him as somebody who has too much time on his hands. I don't think they viewed [the problems] in proper perspective."
Barbara Lindsay's doctor, Elizabeth Laffey, agrees. "Barbara is unique, but that doesn't mean her symptoms aren't real. If I told them that she had diabetes or pancreatic cancer, they'd say, 'Oh my God, that's so bad, this is what we expect, here's what we are going to do about it.' And they would probably be fairly compassionate. Their perception is that she's making this up, because they can't prove that she's in pain."
Bethesda Health Group officials say they can't comment on Barbara Lindsay's condition or the level of attention she may require, owing to federal health and privacy laws. "We treat all of our residents alike. They all get the same kind of quality and care. Perceptions can be different, but that is our focus," says Pat Kapsar, an R.N. and senior vice president of corporate compliance for Bethesda.
"I do know Mrs. Lindsay's physician has played a close role in directing her care and the nurses have worked with her," Kapsar adds. "There is not a doubt in my mind that they are trying to meet her needs at the very best degree of quality that they can."
Doug Lindsay would like nothing more than to move his mother out of Bethesda, but Laffey says Barbara is unlikely to survive another transfer.
Doug's friends worry that the battle with Bethesda has robbed him of time to work on his own medical problems. "I think of it like the sand in the hourglass," says Matt Krentz. "At some point he won't have the energy to continue any of this."
At the same time, they understand why Lindsay won't relent.
Says Krentz: "He knows he's destined for that."
Last April a medical librarian at Oregon Health & Science University in Portland delivered what Lindsay took to be auspicious news: She'd found an article from the 1940s that describes surgeries on two Danish patients to remove their adrenal medullae. The patients had problems ostensibly different from Lindsay's, but it was the first scientific record of successful human medullary extraction he'd unearthed — and he was ecstatic.
Most of what Lindsay knows about the procedure comes from records of research done on animals — in particular a 1923 account of an experiment involving sixteen dogs.
"Who went into space first?" Lindsay posits. "The dog went first. The dog was the hero, and then you had John Glenn. That dog's a hero, and these dogs are heroes," he says, pointing to the medical text from the 1920s. "'Three died within 48 hours, one (No. 1) ran away after seven days in excellent health. Another (No. 10) did the same thing four days after [the] operation without having had symptoms.'"
Says Lindsay: "I want to be dog No. 1 or dog No. 10."
Several years ago Dr. Coghlan referred Lindsay to the Cleveland Clinic and the NIH for further evaluation. But Lindsay didn't end up visiting either facility.
"I was dying to go [to the NIH]," he says. But the testing would have required him to quit his Levophed infusion and undergo testing he says he wasn't sure his body could withstand. "If I stopped my meds for two months prior, number one, would I survive? I could have a heart attack. And two, how was I going to get Mom breakfast? Shower? Do anything?"
The search for a willing surgeon has so far been unfruitful. But just before Christmas Lindsay made his first face-to-face pitch for the surgery to doctors in Michigan. Strapped to a stretcher, he traveled by air courtesy of the Chesterfield-based nonprofit Wings of Hope; his friend Matt Krentz made the trip by car in order to meet Lindsay at the airport and accompany him to the appointment.
"Everything went well, everybody was very nice, and what happens from here remains to be seen," Lindsay says, though he will not name the hospital he visited — or, for that matter, any of the surgeons he has lobbied about the surgery — for fear, he says, of alienating future prospects and "discontinuing relationships."
The lack of success to date has not discouraged Lindsay's circle of advisers.
"Most people in his situation would just say, 'The heck with it! The doctors told me no, and that's it. My life is over,'" says Ken Blumer, a professor of cell biology and physiology at Washington University, who's been in Lindsay's corner for eight years. "Despite this significant but gradual deterioration of his condition, he still wants to find a solution for himself. Who can say no to a guy like that?"
David Robertson, the dysautonomia expert at Vanderbilt with whom Lindsay corresponds, suspects the cause of Lindsay's sickness "won't be clear to people anytime soon.
"It's hard to know what his illness would be like if he were a very different individual," adds Robertson. "A different individual would approach things in a very different way: He'd go to the doctor, and if the doctor didn't know what to do, they'd try something, and if that didn't work they'd try something else. They'd do the best they could and keep on going that route."
Lindsay, Robertson observes, "has elected to do the very opposite."
Ask Lindsay if he's ever tempted to scream, 'Why me?' and he counters with a soliloquy:
"Three million Bulgarians died of typhus in the First World War. Not from bullets, but from disease. If you could tell them we're worried about how much carbon dioxide we expend, they would die from laughter.
"If you are somebody who says, 'I feel bad about people three continents away with nothing to eat,' you don't see suffering the way I see it.
"I'm so proud of my friends. I'm not jealous. I'm happy for them because they're doing something.
"My mother is happy. She's in terrible straits, but her orientation is happy. How could you live sad for her? That's not what she would want for you.
"Now, there are fools who'd say, 'See, he wants to be sick.' That's idiocy. Finding what joy is available doesn't mean you're excited about being homebound for ten years and jealous about everything your friends are doing.
"I still have to live every day."
Editor's note: As originally published, this story may have implied that Doug Lindsay contemplated purchasing a human cadaver. Lindsay has clarified to Riverfront Times that he did not look into purchasing a cadaver, but rather that he "asked ethicists if it was reasonable to suggest to a surgeon that if they wanted to test whether the surgery was possible, they could try the surgery on a fresh cadaver, and asked ethicists what a surgeon at a hospital would face in terms of arranging this." Additionally, Lindsay did not purchase a bag of 50 pig adrenal glands as the original version of this story indicated. He purchased 4 formaldehyde-steeped hog kidneys from a batch of 50 offered by a science lab supply firm and procured several unpreserved glands from a Pennsylvania farmer. The above version of the story reflects the corrected text.
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