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
In my room at the Rochester, Minnesota TownePlace Suites after seeing “The Sessions” with Hadley, I grabbed my laptop and Googled “functional movement disorder.” I wanted to avoid issuing her more uninformed assurances.
I discovered that FMD is not uncommon. About one third of all new neurological outpatients present symptoms that can be only somewhat or not at all explained by an organic disease. About 5% of those experience sensory symptoms, weakness, seizures, or a movement disorder, and they can become as disabled as patients with Parkinson’s or other organic neurological diseases.
“All in your head” doesn’t honor the reality and complexity of this mysterious disorder. The term “functional” describes a condition caused by a change in the functioning of the nervous system, rather than by neurological damage or disease.
Although FMD was once labeled “psychiatric” and neurologists referred a patient with the condition to a psychiatrist, now they know that FMD neurological symptoms are not invented; they originate in the neural pathways connecting the brain and the mind. Unlike Munchausen syndrome, the mental health of a patient with FMD might or might not play a role in causing FMD. FMD can alter the way the brain sends or receives messages to or from the body, resulting in disabling symptoms like dystonia, in which an extremity adopts an unnatural, painful position, uncontrollable tremor, twitches, spasms and gait disturbance. All of these are also symptoms of Parkinson’s, which can make diagnosis difficult.
One way to distinguish FMD from neurological diseases like Parkinson’s is to think of it as a software problem as opposed to a hardware problem. An FMD patient’s “software” — his thoughts, emotions, behavior and sensations — has caused the nervous system to malfunction, so medications like levodopa that address the “hardware” damage responsible for Parkinson’s symptoms are not effective for FMD.
Unlike Parkinson’s or MSA, FMD can be cured over time with physical therapy, a patient’s determination, and strong emotional support. For this reason alone, I wanted to be persuaded that FMD was Hadley’s diagnosis. But the more I read, the more convinced I was that the condition didn’t mirror Hadley’s; she had autonomic symptoms that couldn’t be explained by FMD, and levodopa, at least for a while, had helped her.
Hadley’s fear that she would leave the Mayo with no clear diagnosis or be challenged to prove she had an organic disease was understandable. This scenario not only would mean more medical appointments, but also would impede her ability to get the help she needed to manage her illness. There’s a stigma attached to any condition like FMD that is believed to have a psychogenic rather than organic cause. The disability it causes can be perceived as less legitimate and therefore less deserving of help or compassion. As an example of this kind of bias, people often line up for months to supply dinners for a friend recovering from a stroke or dealing with cancer. But for those with less understood illnesses like debilitating chronic fatigue syndrome, say, or a major depressive episode? No casseroles for them. This bias not only cuts a patient off from critical support, but also feeds a patient’s fear that he is to blame for his illness.
In the morning, I awoke and inwardly shivered at the cool, maroon and grey color scheme of the hotel room, a corporate décor that coordinated with the landscape outside the window: chalky warehouses, chain link and patches of dead weeds rooted in asphalt under an overcast sky. The refrigerator motor grinded away. I imagined how difficult it must have been for Hadley that week, being alone in this austere place. The night before, she’d told me how she’d spent her time when not at her Mayo appointments. Since our hotel was outside town on a highway, there was nowhere to go without a car. One night, she walked to Target to buy sushi, crossword books and jigsaw puzzles depicting village scenes. In the room at night, she had Hot Pockets and Gatorade, then sat in bed with a tray, putting together the puzzles. She spent hours on medical websites. On Thanksgiving, she had gone to the home of a friend, a dermatologist with six children. It was a warm and welcoming evening, she said, but it somehow made her feel even lonelier.
Now, my phone chimed. “You won’t believe what I discovered last night,” Hadley had texted. “I can’t wait to tell you.”
We agreed to meet at the hotel elevator to make the drive to the Mayo for her nine-thirty appointment with the movement disorders specialist. She’d arranged to have this particular meeting today so that I could be with her, another set of ears. Outside, a light snow was falling, dusting the Chevy Impala. We marveled at the largest snowflakes I’d ever seen — their crystalline form was entirely visible; their clarity seemed like a good omen.
“To the Target,” I said when we climbed into the car.
We’d asked the woman at the TownePlace Suites front desk where we could buy yogurt and good coffee and she’d told us, “Starbucks. Inside the Target.”
“That woman, when she’s not working the desk here, she works in a medical lab,” Hadley told me in the car. Her mood was noticeably brighter than the night before. “She talked to me about…But let’s get some coffee in us before I tell you.”
“The” Target on route 62 seemed to contain an entire Main Street of merchandise and services and was overwhelming, exacerbating my sense of being lost with Hadley on a vast plain without landmarks. It was a relief when we were back in our car clutching our same-everywhere brand of coffee, and I had a Thelma and Louise fantasy of driving forever — past the two other Targets on 62, past the Mayo, across the continent to outrun this villainous diagnosis chasing Hadley.
“So,” Hadley said. She’d been up late, Googling. One of the diagnoses that the Mayo had on its list to look into was Wilson’s disease, a disorder in which copper accumulates in the liver and brain, often causing Parkinson’s-like neurological symptoms. Hadley’s copper level was normal, she explained to me, but her nocturnal research uncovered that an abnormal ratio of copper to ceruloplasmin, a copper-carrying protein in the blood, is also a marker for Wilson’s. Going over her blood tests, she discovered that indeed, her ratio was abnormal. She took her findings to the receptionist/lab technician at the TownePlace Suites who told her she thought the abnormal ratio was significant. Back in her room, Hadley called a lab technician friend in Montana who agreed.
As always, I was in awe of Hadley’s command of medical information; she seemed to be a walking, breathing Merck Manual. Her determination, rather than flagging after her six miserable days in Rochester, seemed to be intensifying. Of course it was — she was fighting for a diagnosis, for her life.
“Wouldn’t it be amazing if all this was just about too much copper?” Hadley said.
Inspired also by the caffeine, I felt my spirits rise at the thought. “It would be the best thing ever!”
Hadley was silent for a while. Then she said, “But I don’t want to bring this ceruloplasmin thing up to the MDS today because for sure, all my detective work will just make me look more like I’m nuts and have a functional movement disorder.”
“How about I bring it up to him then?” I said.
The Mayo’s 12th floor waiting area was huge, plush and comfortable. We chose a couple of upholstered chairs that faced the floor-to-ceiling glass windows that looked out at the other Mayo buildings and an attractive plaza. I had always associated medical appointments with bland, artificially lit spaces that worsen white-coat anxiety — that sweaty, slightly queasy feeling; now, I soaked in the natural light and the expansive space and views, hoping they’d soothe. If I’m nervous, I thought, how must Hadley feel? She told me that in fact, her stomach had been very upset since arriving at the Mayo over a week ago.
The MDS was ready to see Hadley and when we followed the assistant down a narrow hallway to his tiny, windowless exam room, I realized the patients, not the doctors, were the real beneficiaries of the Mayo’s luxury architecture. Hadley and I sat, both twisting our hands in our laps. I could tell she felt she was preparing for the Inquisition. “What if he decides it’s functional?” she said. I told her he wouldn’t.
The MDS came in and shook our hands. “She’s my friend,” Hadley explained to him.
As if to lend legitimacy to my presence, I added, “I have Parkinson’s myself.”
The doctor smiled; his manner was gentle, serious. He looked over the records on the desk in front of him.
“Hadley found some interesting information last night,” I blurted. I explained the bit about the copper-ceruloplasmin ratio and my cheeks flushed with heat; I suddenly felt like a student again, hoping my contribution wouldn’t end up humiliating me.
“Huh,” he said. “ I haven’t heard of that. I’m sure you don’t have Wilson’s, but I’m not opposed to checking your urine copper level. And we could also send you to the ophthalmologist to check your eyes for KF rings. We can certainly do that.” KF rings, Hadley had told me earlier, are rings of copper visible in the eyes of Wilson’s patients. The doctor’s offer sounded concessionary. He wrote on his pad, briefly checked his computer, then swiveled his chair to look at Hadley.
“I can sure see how you fooled all your doctors,” he said.
There was a slackness in the room now, like at the dinner table when guests have stayed too long. Hadley’s face was chalky.
“I think Hadley’s very worried,” I said as neutrally as possible, “that she’s going to return to Missoula without a solid explanation for her symptoms that are attributable to an organic disease process.” Since I was leaving tomorrow, this was my only shot at speaking up for Hadley at the Mayo. Knowing I had nothing to lose — he wasn’t my doctor, after all — I pressed on, naming Hadley’s symptoms. “And the fact that she has had not great, but at least some response to levodopa?”
“This is just not a clear case,” the doctor said. “But let’s run these couple of other tests. And it looks like you’ll be seeing the neuromuscular specialist next.”
Two hours later, Hadley and I looked at the MDS’s report online:
All of the patient’s symptoms could be explained by a disorder of excessive muscle stiffness, including her speech, her skeletal muscles, and possibly the urodynamic findings. I am not seeing any convincing evidence for an organic parkinsonism at all. I think the chances of this being Wilson’s disease is very low, but the patient is understandably concerned about that. We will get a 24-hour urine, and I will send her to Ophthalmology to look for Kayser-Fleischer rings, but I suspect they will be normal. I offered her blood tests for Wilson’s disease gene testing, but that has to be approved by her insurance company first so we will not get that now. She can always get that drawn at home.The patient will follow up on Thursday in the Neuromuscular Clinic for them to reassess whether they think she has primary lateral sclerosis to explain all this muscle stiffness. I think that is a reasonable possibility, but I think a functional etiology should also be considered. If no organic etiology can be found, the patient may benefit from our weeklong program in Physical Medicine and Rehab called the BeST Program, which helps people with functional problems to help retrain the brain and move normally. Of course, organic causes need to be ruled out first.
#1 Muscle stiffness
“Muscle stiffness”? With everything that was going on in Hadley’s body, with all the time and physical and emotional energy she’d spent, she and I couldn’t believe she could go back to Montana with a diagnosis of muscle stiffness, functional problems, and a prescription for retraining her brain.
I looked at Hadley, who sat expressionless and limp in the chair. It occurred to me that throughout this whole ordeal, I’d never seen her cry. She had three more days of appointments at the Mayo. In that time, I hoped with all my heart that the doctors would solve her mystery and pull her off the rollercoaster.
Until that time, I was pretty sure tears would not dare to fall.