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.
On our calendars for fall 2012, Hadley had several appointments with Dr. Youngman and I had my semi-annual checkup with Dr. Bright. Hadley was expecting more testing to confirm her MSA diagnosis while I was still stewing about how to follow up with Dr. Bright on our aborted phone conversation. Since he’d doubted Hadley had MSA even knowing Dr. Youngman disagreed, I began to worry that he might be a “good news doctor” who didn’t tell his patients what he didn’t think they needed to know. As his patient, could I trust him going forward to be totally honest with me about my health?
I convinced myself that if I asked him this question in an open, heartfelt manner, Dr. Bright would be receptive. But my emotions were running high; in addition to the trust issue, I was distressed about Hadley’s MSA and, I’ll admit, still angry that he’d told Hadley not to “obsess.” In other words, I was pretty sure that if I tried to talk to him in person, I’d cry. Horrors! Then what? I would risk his feeling blindsided and reacting in a way that could make things worse. So I wrote him an email. I left Hadley out of the conversation because I wanted to make it clear that my chief concern was our own doctor-patient dynamic.
Hi Dr. Bright,
Because in our phone conversation in July we were both caught off-guard, I want to follow up to be sure I convey what’s most important to me as your patient.
For two years while I was having PD symptoms, my various health practitioners told me I was anxious, that my symptoms were nothing serious. It felt demeaning to have them tell me not to “obsess” or “be so anxious” when I knew that something was wrong. I didn’t need their pats on the head or empty optimism; I needed them to take my concerns seriously, to delve in and investigate. As a reasonable patient who’s informed and in touch with her body, if I have concerns about symptoms or medications and they’re totally dismissed without clear, hard evidence, I’m neither consoled nor helped. For me, “I don’t know,” are better words to hear than, “It’s nothing,” when I know that it’s something.
It’s not what I know that frightens me; it’s what I don’t know. Knowledge allows me to be alert and prepared for what might come. When I have unexplained symptoms, I never assume the worst. I research all the possibilities just as any scientist would. I understand that for some patients, not being given information that’s in a grey area might be helpful. If given the choice, they might even be grateful not to hear the scary black and white stuff about their health. I’m here to say that is not me! My body is a mystery that I’m sentenced to live in and my doctors, with their expertise, help me to solve that mystery. In the nature of a true mystery-solvers collaboration, I want them to share all the clues with me — scary or not, when they pop up on the horizon. You are the voice of my nervous system; I’d much rather get news from you than from Facebook or Dr. Google.
I know your patients all have different expectations of you, and I know doctors have different styles. I know you care a lot about your patients and that you keep in mind their best interests. I figured since I opened this can of worms by talking honestly with you about Hadley, I might as well ask you: will you take my worries seriously, knowing I’m never just looking for the easy-to-hear answer, and will you tell me “the scary stuff,” even if it’s not the strategy you’ve typically used? I might be asking something of you that you aren’t comfortable with; if this is the case, I hope you’ll be honest with me.
All the Best,
I’m sure that whatever frustration I thought I was tempering by writing carefully and from the heart was blaringly evident from the sheer length of my email. While waiting for Dr. Bright to reply, I worried that I’d doomed our relationship. Would he be angry? I needed him. I felt helpless, childlike. A couple of days later, Dr. Bright wrote:
I try to help each patient with understanding their symptoms and condition. I answer questions with as much precision as I can provide and I do not try to “hide scary stuff.” On the contrary, with my patients with more advanced symptoms, I often let them know the specific concerns I have about their condition.
I found myself studying his words the way sometimes at lunchtime, I peer longingly into my refrigerator, looking for that one satisfying bite of nourishment.
At my appointment several weeks later, I waited until we were done with the exam and told Dr. Bright how sad I was about Hadley’s MSA diagnosis. Despite my best efforts, tears welled in my eyes.
“Is she still walking okay without a lot of falling?” he asked. I told him yes. He shook his head. “It’s very hard to diagnose MSA and I wouldn’t give up on it being Parkinson’s yet.”
Part of me, I realized, was still eager to abandon my ship of sorrow for his optimism and I had a brief fantasy that he’d been right all along. As I stood to leave, Dr. Bright gave me a hug. I understood that the embrace was his way of telling me that he cared and wanted to be the best doctor he could be for me. It also felt like a gesture that signaled closure, and since I sensed that I’d elicited the deepest response from him that he could give, I resolved to let the business with Hadley go. But from then on, I would be extra careful to always be clear about what I needed to know from him.
In September, Hadley underwent a brain MRI. The scan showed minimal atrophy in the cerebellum that correlated with the side of her body that was most affected by symptoms. While this finding could support an MSA diagnosis, technically, the results were considered to be within normal limits. She also did a urodynamic study, which assesses how urine is stored and released by the bladder and the urethra. The test confirmed Hadley had a neurogenic bladder, which explained her occasional inability to void. Like her orthostatic hypotension, this condition was an indication that her autonomic nervous system was impaired, which could be a sign of MSA.
Dr. Youngman met again with Hadley in early October and spent three hours with her doing more testing. He ordered a DaTscan, or Dopamine Transporter Scan, a new imaging test that had been approved by the FDA only the year before. A DaTscan can be used to look at the brain’s dopamine activity. When a patient’s illness is uncertain, a DaTscan can help to confirm a Parkinson’s or multiple system atrophy (MSA) diagnosis if the scan shows a clear depletion of dopamine in the brain. A patient’s dopamine activity can vary greatly depending on age or length of illness, however, and because dopamine loss is associated with both MSA and PD, the DaTscan cannot differentiate between the two diseases. Therefore, although imaging can sometimes support a diagnosis made by a clinician, it can’t be relied upon alone to make a diagnosis.
As she was filling out the forms for her health insurance company to justify the necessity for the very expensive DaTscan, Hadley was startled when she read that DaTscans were sometimes used to distinguish between an organic disease process and a psychological one. She felt an old fear take hold: if the scan was normal, might Dr. Youngman revert to thinking her symptoms were all in her head? She remembered how, at her very first appointment, he’d wanted to rule out Munchausen’s syndrome, a serious psychological disorder in which a patient pretends to be sick or injured to get attention. Now, she found herself almost wishing the DaTscan would be abnormal.
To Dr. Youngman’s surprise, the scan revealed a slight abnormality, but the results were considered to be within the normal range, possibly indicating that Hadley had neither Parkinson’s nor MSA. Dr. Youngman then had Hadley do a levodopa challenge test that involves taking a very high dose of levodopa. Generally, if a patient has Parkinson’s, their symptoms will respond well to the flood of levodopa. Hadley had always felt somewhat better on levodopa, but her response to the challenge was not nearly as strong as Dr. Youngman had expected, which would seem to support a diagnosis of MSA, rather than PD.
Dr. Youngman was flummoxed by the tests’ inconclusiveness. So much so, that at her third appointment with him at the end of October, he had Hadley examined by a neuromuscular specialist who administered an electromyography (EMG) to test her muscle nerve cells. As was the case with the EMG I was given more than a year before I was diagnosed with Parkinson’s, Hadley’s results were abnormal. Finally, an abnormal result! The specialist told Dr. Youngman that this could suggest an upper motor neuron disease, or primary lateral sclerosis (PLS). PLS is a devastating disease that causes muscle nerve cells to slowly break down, resulting in weakness, but unless it develops into ALS — Lou Gehrig’s disease — it’s not life-threatening, like MSA
Since Hadley hadn’t experienced muscle weakness, she was puzzled by the test results and scrambled to make sense of what appeared to be Dr. Youngman’s shift in focus from MSA to neuromuscular disease. What about all the symptoms she experienced that were typical of MSA? “Could I have had the same abnormal EMG results if I had MSA?” she asked Dr. Youngman. He conceded that this was possible. Yet, as Hadley sat in the exam room with her husband, John, Dr. Youngman, and the neuromuscular specialist, she watched as Dr. Youngman became won over by the diagnosis of PLS. The other doctor left the room. To Hadley, Dr. Youngman seemed flustered and had a hard time looking her in the eye. He explained that he would no longer be able to help her because he knew nothing about motor neuron disease and that she would now be in the care of the neuromuscular specialist. He told her to stop taking levodopa, asserting that since she didn’t have Parkinson’s or MSA, any benefit she had been getting from it had to be a placebo effect.
“Well,” he said to Hadley and John, “this is a much better diagnosis than MSA. Here I had diagnosed her with Parkinson’s and she made all these friends who have Parkinson’s, and now she’ll have to say goodbye to them.”
Hadley was stunned. He was dismissing her just like that? How could he have been confident in his previous diagnosis of Parkinson’s and then MSA, only to reject them both based on new findings of another doctor and a DaTscan and levodopa challenge test, both of which are known to sometimes be inconclusive? The sudden change in diagnosis intuitively seemed ill-considered to her. Most painful, though, was that Dr. Youngman — the doctor she’d thought of as a friend and advocate — had abandoned her.
After her appointment, Hadley and John flew to the city where they’d left their car and started the long drive back to Missoula. They rode for miles in a dazed silence. Every now and then John would ask, “If it’s not Parkinson’s or MSA, how does he explain…” and he would name a symptom of Hadley’s. Hadley thought to herself, well, at least John’s on the same page with me.
It was snowing when Hadley and John got to the mountain pass two miles from the Montana border and suddenly, they heard a loud pop — a tire blowout. In the dwindling daylight, John swerved but managed to pull over. They called AAA and waited vigilantly, fearful that an approaching car would skid into them. Hadley had had to stop taking the levodopa for her appointment with Dr. Youngman, so she was extremely stiff and exhausted. But she took comfort in John’s calmness as they discussed this new turn her diagnosis had taken. After two hours, the tow truck arrived, driven by a man in his fifties who looked haggard and told them he had poor eyesight. When he asked if he could smoke while driving, they felt they couldn’t say no. In the darkness and falling snow, they crawled the remaining 112 miles to Missoula.
Hadley told me it was the scariest drive of her life.