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 the spring of 2012, before my fall trip to the Mayo to visit Hadley, I sent my revision of Dream House to an agent who had been reading the manuscript in 2011 when the it–just-screams-film! agent made her preemptive grab for it. I’d considered sending it back to the first agent who’d offered me representation, but he had much less experience selling fiction. I liked what I’d heard and read about Nora, and the books she’d represented. Unlike many agents who, if they acknowledge your query at all, send, or have an assistant send, a two-sentence rejection wishing you the best of luck, Nora was a generous communicator, unafraid of leaving doors open — a quality I appreciated, at least in the beginning. Our email relationship began with an apology soon after she received my manuscript in March:
Sorry to be slow. Have been deluged with reading; then after I read I carry the ms back and the day’s work is intense so it prevents me from writing to the author. And then in the office it is always incredibly busy, so days can pass.
A couple of weeks later:
I do have a lot of little post-its with notes. And some general notes I want to type out but am racing the clock now. Let me see if I can type out tomorrow and also send the pages with notes to you so you can see what was on my mind. I actually don’t think it is impossible that we would see eye to eye! But I have to write and I have to send and I am out of here in ten minutes now, with another editorial letter to client in the computer…so let me do what I can to relay my thoughts in a few days….
Of course, my hopes velcroed themselves to the message, double negative notwithstanding, buried in the middle of her hectic email: She wasn’t saying no. As promised, the next day she wrote:
I well remember this book from the last time I read it. I think the writing is fabulous. Your scenes are fabulous. You spin out amazing story material. And I am still a fan. My problem is that it does not yet totally fully resonate for me. And in this market you need the agent to feel 150%.
Despite what came after — a foot-long explanation of what in the story worked and didn’t work, her opening praise flooded me with optimism that I could revise the manuscript in a way that would take her to that magic 150% place. She closed by saying:
So I am in this weird spot. I like this a lot but the pieces of the puzzle obviously don’t fit together for me just right yet. BUT you are a terrific writer and this is a truly engaging world you create. I bet you are absolutely exhausted from editing and revising. BUT if you were to work more on this I would happily read again.
Warmth! I wanted Nora to be my agent. If she would “happily read again,” I would most certainly, ecstatically revise again. From March to November, I revised feverishly and when Jay, my editor, and I felt I’d made the storyline as clear as possible without hammering the reader over the head, I sent the manuscript back to Nora. In December, one week after I returned from the Mayo, she wrote me another foot-long email outlining the ways in which she still felt uncertain about the story. Then she added:
So I could be your guy! But today I am not all there. I am so sorry — this leaves you in a bad spot but at least you have my thoughts, and maybe they will be helpful. You are TRULY a strong beautiful writer. That is not the problem. The problem is that in this awful market, your agent needs to be 150% and the editors are that much harder to convince. So I have become much tougher!!! BUT I so appreciate your patience and your letting me read this. Tell me what you think of all this, when you can.
I was briefly tempted to hang my hat on “I could be your guy!” But this time, I was perplexed by Nora’s notes and questions. Some of her critique made me wonder if she’d read the manuscript while preparing her taxes during a cocktail party (she confessed, for example, that she’d forgotten the name of the main character who appears on every page). I was forced to consider the possibility that it wasn’t clarity she was missing, but something else altogether that even she couldn’t identify. I’d been working toward landing her for eight months. Where was I, percentage-wise — 85%? 135%? What were the chances that further revisions would get me to 150%?
I needed to move on. I thanked Nora for spending time on Dream House and retreated from the agent search. Here is where a younger writer or one without Parkinson’s would have simply compiled a new list of agents to approach; in the tough world of publishing, I’d gotten only a small taste of rejection. Rejection I could handle. But I didn’t have the stamina for the painfully slow pace and the needle-in-a-haystack nature of the submission process. Every day, my bradykinetic body reminded me that time was precious. Finally, in late April, I decided to give Dream House to the agent who’d wanted it in 2011. He was thrilled — 150%? — and it felt great knowing he’d convey his unequivocal passion for the book to publishers.
Meanwhile, Hadley was in a state of agonizing discombobulation, trying, like me, to make the pieces of her story fit so that it would be accepted and understood. Her miraculous-seeming response to the 25/100 formulation of levodopa remained consistent for a few months, but by May the medication was wearing off more quickly and sometimes it seemed as if it hadn’t kicked in at all. Could this point back to an MSA diagnosis? she wondered. Not wanting to alarm her family, she kept her worries to herself. But she continued to write about them.
June 14, 2013
I feel lost with my situation and where to go or what to do from here. I have so many commitments and projects to complete. How will I get everything done? How will I tell people that I won’t be able to complete what I said I could do? I wish I could spend my days with Sarah and John and soak in every moment. I feel like I have to get these things done before I can do that and what if it is too late? What if I work and when the projects are done I have missed my opportunity and time with my family? What was I thinking to say yes to the projects on my plate?? I am digging myself into a deep hole and I am worried I won’t get out of it.
Dr. Reid was supportive and together, they made decisions about her medication regimen but neither of them knew what they were treating. By now, through her organization, Summit for Parkinson’s, and our Parkinson’s Facebook group, Hadley had a large network of people to whom she could reach out. She collected names of movement disorders specialists in Colorado, California, Washington, Pennsylvania, Texas and Canada, and began researching them. She called a friend who works with the Michael J. Fox Foundation, gave her the doctors’ names and asked to have the MJFF’s medical experts identify the top three. Then, she posted the three names on our Facebook page, asking members if they knew any of the doctors. Two of them responded that they were seeing one of the doctors on her list, Dr. Truitt, in Houston.
For more than thirty years, Dr. Truitt had been seeing patients with movement disorders. His two patients told Hadley Dr. Truitt had “seen it all” and that Hadley could therefore count on a very frank assessment. Hadley was able to make an appointment with Dr. Truitt. In July, she and her mother, Jana, flew to Austin, where one of Dr. Truitt’s patients, our Facebook friend Lucy, met them with a dinner-to-go and the three of them together drove the three hours to Houston.
The next morning at Hadley’s appointment, a resident spent an hour going over her health history. He examined her and left the room for a long consultation with Dr. Truitt, during which Hadley felt very anxious that she would experience a rerun of her time at the Mayo Clinic. Finally, Dr. Truitt, a small, compact man with a thick accent, entered the exam room and shook hands with Hadley and Jana. After a few questions for Hadley, he became somber and very focused.
“Have you ever heard of multiple system atrophy?” he asked.
When Hadley told him yes, Dr. Truitt expressed without hesitation that her symptoms and tests supported an MSA diagnosis. “I hope I’m wrong,” he said. “But this is what I think it is, and now that we know, this is how we’re going to treat it.” He went on to explain that he treats MSA very aggressively with levodopa and bumped up Hadley’s prescription for levodopa from 300 to 1000 mg/day, three times the amount that I take for Parkinson’s, almost seven years from diagnosis. Here is how Dr. Truitt saw Hadley:
This 36 year old right-handed freelance painter/acrylic artist from Montana presents for evaluation of a 4 year history of progressive fatigue, global bradykinesia, gait instability, falls and dysautonomias manifested chiefly by orthostatic lightheadedness, urinary retention, constipation and cold hands and cold feet sign. She has tried various medication regiments in the past, namely Azilect, Mirapex, levodopa, and Amantadine with variable response as detailed above. The constellation of symptoms and signs suggests the diagnosis of multiple system atrophy (MSA-P). Her rigidity and bradykinesia are fairly symmetric. Her dysautonomias including urinary retention, constipation, history of orthostatic hypotension are prominent and have occurred early in her disease course. Moreover, the relatively rapid progression of her symptoms along with her young age are atypical of Parkinson’s disease. Her MRI brain and DaTscan were reviewed and would further corroborate a diagnosis of probable MSA. She has had an inconsistent response to levodopa in the past, which would further go against Parkinson’s disease. In sum, the combination of an adult onset, sporadic progressive onset with Parkinsonism, dysautonomia and inconsistent response to levodopa, abnormal imaging and relatively rapid progressive course corroborate a clinical diagnosis of probable MSA-P.
The name—multiple system atrophy—chillingly describes this disease’s course and inevitable, grim outcome: as nerve cells die and areas of the brain atrophy, multiple parts and functions of the body shut down until even breathing and eating are not possible. There are two types of MSA: MSA-P, in which parkinsonian symptoms like stiffness, slowness, gait disturbance and balance problems present first, and cerebella type, or MSA-C, whose features include coordination, balance and swallowing difficulties as well as slurred speech. Often patients start out with the symptoms of one type of MSA and gradually develop symptoms of the other, but eventually, all patients succumb to an autonomic nervous system failure. Whereas in Parkinson’s it is primarily the substantia nigra area of the brain that atrophies as its dopamine-producing neurons die off, in MSA, the substantia nigra, cerebellum and brain stem all experience significant loss of nerve cells. The brain stem is the command center for the autonomic nervous system, regulating heart and respiratory rate, blood pressure, digestion, salivation and urination, among other functions. A brain MRI will sometimes confirm a diagnosis of MSA by showing areas of atrophy, but not always. As with Parkinson’s, it’s believed that nerve cell death in MSA is attributable to an abnormal accumulation of the protein alpha-synuclien in the brain.
Multiple system atrophy is rare, annually affecting about 3 people per 100,000, predominantly over the age of 50. There is no cure for MSA. It progresses very fast compared to PD; a patient’s maximum life expectancy from diagnosis is about ten years. As with any disease, of course, there are outliers who are able to live much longer.
Treatment for MSA involves managing symptoms to make a patient as comfortable as possible and to maintain her body functions. As in Hadley’s case, levodopa can relieve parkinsonian symptoms like slowness, stiffness and rigidity, but typically, it loses its effectiveness with time, and not all those with MSA respond. Physical therapy is always recommended to help maintain strength and balance as long as possible, and speech pathologists help to improve speech and voice volume. Low blood pressure, which people with MSA experience when standing, is treated with steroid medication that helps the body retain salt and water; later, a pacemaker might be needed to regulate heart rate. Medications can help with urinary retention at early stages of the disease, but a catheter eventually becomes necessary. Late in the disease, both breathing and feeding tubes might be required.
Leaving Dr. Truitt’s office with the MSA diagnosis, Hadley cried briefly and told her mother she was sorry. When Jana hugged her and asked her why, Hadley said she felt awful that Jana would have to endure her illness along with Charlie’s Parkinson’s. She called John and her father, and texted me the news: “I know it’s terrible and I’m scared, but it’s so good to know what it is and to have someone tell me they’re going to help me with it.” She sprinkled her message with emoticons that I knew were intended to reassure me she was handling it. But they only made this new reality more surreal.
A few days later, Hadley told me that all the noise in her head from years of trying to get a diagnosis had been sucked out and she was experiencing a “huge silence.” Finally, she could stop trying to explain herself to the world. Finally, she had been seen.