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.
If you are having a particularly hard day, save this episode for a more optimistic one. I’m going to share a story that might wrench your insides. I share it because the timing of its arrival in my mailbox was poignant and more importantly because it is a thing of rare, intimate beauty.
About the time Hadley started questioning her Parkinson’s diagnosis, I was struck by the difficulties of one of our other Facebook group members, a woman in her mid 40’s. Jane had a flair for black humor; once she got me laughing with her graphic description of the war she’d waged on cockroaches in her New York City apartment in her 20’s. She lamented the copious amount of Raid she’d deployed, which she suspected had caused her Parkinson’s. Like me, Jane was a researcher. When it seemed like our public posts were becoming too pedantic, she and I exchanged private messages about, for example, the neurotoxicity of certain herbicides and pesticides.
Jane had undergone deep brain stimulation surgery (DBS) for her PD, but within months of the procedure, she was falling again — “flat on my face,” she posted. How could this be? I wondered. Typically, DBS had more lasting benefit. Concerned, I sent her a private message. She wrote back and told me about the various falls over the past few years that had left her with six broken ribs and a fractured clavicle and vertebra. She’d also been treated with Botox for excruciating dystonia (involuntary muscle contractions typical in PD), and it had knocked out the muscles in her neck, necessitating a cervical fusion. This nightmare she described at length, with candor and remarkable humor.
I continued to worry about Jane over the next several months. She wasn’t posting on Facebook and hadn’t responded to a private message I’d sent her. Finally, six months after our first messages to each other, she wrote.
In 2003 I started experiencing strange symptoms — balance problems, fear in the car, an altered sense of space. I felt something was wrong, especially the driving phobia. After visits to a number of specialists and with worsening symptoms, I consulted my friend, a hospital nurse practitioner I really trust who knows all the doctors around. She recommended a neurologist. She told me people thought he was odd, and that his bedside manner was atrocious, but she had the utmost respect for him.
He diagnosed me. He walked into the exam room, looked at me a moment, and said, “You have Parkinson’s.” “How do you know?” I asked. “Look at your face! You look like a judge!” And that was it, I kid you not.
He explained that he could give me a prescription now or try some tests that ruled out other things. I opted for testing, which ruled out the other things. So two weeks later I was on levodopa. My body responded like magic. The stiffness disappeared, the mask with it and I practically felt as good as new. Except that it confirmed Parkinson’s.
Given that, I dived into research about PD and meds. I asked my doctor why he didn’t put me on Mirapex and he said adamantly, “You’re still driving!” He considered the agonists too big a risk for sudden sleep. (I had narcolepsy as a child, so I considered it a pretty significant risk too.) I started to talk to other doctors. I went to a movement disorders specialist, who confirmed my diagnosis, but asked me why I didn’t take an agonist. He thought they were safe and effective. He proceeded to tell me about two accidents in which his patients had sudden sleep attacks.
I went to yet another MDS. Discussing the diagnosis (Parkinson’s) at the end of the session, he also asked why I didn’t take an agonist. He thought I should, and that he also had heard of accidents. I was now definitely in the no-agonist camp.
I should say that all of these doctors also thought I had remarkably low-grade Parkinson’s, almost invisible to the untrained eye.
The first doctor even predicted a mild course for me long-term. I asked him how he knew and he said, “Because I went to Harvard!” Possibly this is one reason people thought he was odd.
Still, I felt I was getting worse and not getting enough answers. I asked my first doctor for a referral to another doctor and he made one also for a psychiatrist he knew who specializes in Parkinson’s care (he proved to be really important, another story). I started in with the other doctor, who had an even worse bedside manner and a more violent antipathy toward the dopamine agonists. She had taken all her patients off them, given the frequency of bad side effects.
We tried some of the other standard drugs. I was starting already to show fluctuations and the idea was to smooth them out. Comtan made me sick. Tasmar seemed to affect my balance — I described it as “making me zoom everywhere,” now I know it as festinating. That was the start of my falling career, one of the things I do best.
Another attempted smoother, levodopa CR (controlled release), made me crazy, suicidal around the clock. I’ve since learned that others have terrible reactions to it and find it both unpredictable and ineffective. That’s my experience, for sure.
So in all, I felt like I was getting worse. So was my doctor’s bedside manner. She told me to shut up, once (with my husband in the room) and was openly dismissive when I asked about a handicapped placard. After a second request, she sighed and said, “No skin off my nose,” then wrote the letter.
[Sidebar: I went back to (the University hospital) to see if another doctor there would be better. This doctor was similarly abrasive and when I mentioned my current doctor, she was shocked: “She’s a great doctor! Why would you see anyone else? Go back to Doctor M____.” I’ll leave you to guess who Dr. M’s med-school mentor was.]
About this time I began to consider deep brain stimulation as an option. When I asked Dr. M she said she thought it would be worth a shot and referred me to (a surgeon she highly respected) for both surgery and programming.
The neurosurgeon proved to be charming, attentive, good at discussing the disease, and willing to look hard into my medication, so I also asked if he would take me on as his regular patient. He did. Though experiments like Azilect didn’t work, my dosing was under better control and I felt good. The only problem was worsening dystonia in my back and neck, which was becoming cripplingly painful.
At this point, I was still working. I wanted to be together for my clients. I told them all about my young onset Parkinson’s as I wanted to be both authentic and ethical. That said, I really didn’t want my symptoms to be a distraction. I depended on levodopa to keep me going. But I was in a double bind: I was taking a lot of levodopa to stay on, but that gave me dyskinesia. If I didn’t take it, I was stiff, slow, and full of negative feelings. I was becoming obviously symptomatic either way and dosing around my appointments was a full-time job. I had to start considering retirement. But I wasn’t quite ready.
In February of 2009, I asked (my doctor) for a Botox shot to relieve the dystonia. He told me it was completely safe in his many years of experience. However, it knocked my neck muscles right out. The following day I could not lift my head — either I was too sensitive or had too much. That was the final blow to my work. And my driving. I retired and went on Social Security disability. And my head hung off my shoulders for six months before the Botox wore off and the muscles came back.
To make a long fucking story short, I changed doctors again. The doctor told me it was his belief that his role was to give his patients the best quality of life possible since there is no cure, and that doing DBS early before my symptoms were bad would help most. He had also wanted to try Amantadine (gave me scaly livido reticularis) and Mirapex, which at that point I still didn’t want. So I returned to levodopa.
My symptoms were obviously worse and worsening. I finally did get DBS (again, another story), and after a brief, wonderful period of autonomy, I started falling again. I spent the year in and out of the hospital, recovering from a variety of fractures and an operation to fuse my cervical spine (an effect of the Botox months). But by early this summer I was back where I started — really, seriously, obviously disabled.
This past June, my doctor re-diagnosed me. I have either “severe, advanced Parkinson’s” or “multiple system atrophy with Parkinsonism”(MSA-P), also known as one of the “atypical Parkinson’s” diseases. What the fuck! Obviously, I respond to levodopa, which points to Parkinson’s. But also had worsening autonomic symptoms all along — incontinence, difficulty breathing, swallowing, talking, bad body temperature regulation, profound lack of balance, all progressing really fast — the good stuff that goes with MSA. And to the doctor, it “looks like” MSA. There’s a lot of overlap between PD and MSA, granted. The doctor has said to me the diagnosis is really an academic argument — it won’t change the treatment. Unfortunately, the prognosis sucks either way.
Now, after DBS, my stabilized meds routine, and my is-what-it-is diagnosis, the main treatment is physical and occupational therapy. I’m pretty much wheelchair-bound at this point, but I depend on levodopa to be able to talk, eat, sit up. It takes me about two hours to eat a meal now.
So this is the end of my current fuckin’ story. Sorry it took forever but I’m pretty slow as you’ve guessed. Hope it’s of some use to you, go ahead and ask if not. Sending you my best!
I was shaking by the time I finished reading Jane’s message. It had been eight years since she experienced her first Parkinsonian symptoms, and she was now in a wheelchair. I had a dreadful feeling that she’d taken the time to write down a blow-by-blow of her experience because she realized she was facing an imminent demise. I felt honored that she’d told me her story but miserable that she was slipping away. I struggled with what to write back. I thanked her for sharing her story and told her I was stunned and saddened and that I hoped she’d keep writing. A month later, she wrote, “Promise me you won’t get sad. I want to thank you for listening.” A couple of months after that, she wrote:
My husband is helping me write a letter to my father. I have to talk to him about the fact that I’ll probably die within the next four years, and I think that letter will be the first of many I need to write to explain to my family and friends. I’ve cried a lot but I’ve also made it a goal to live well in whatever time I’ve got. I’ll let you know how the letter turns out.
Several weeks passed. I wrote to Jane asking if she’d written the letter to her father. I received no reply. For months, Jane had no presence in the Facebook group. Finally, eight months after her last message to me, her husband posted on our Facebook page that Jane had died of complications from MSA. He thanked us for our support. He sent me a private message, saying how important it was to Jane to have had the chance to chronicle her disease so that she could think it through. He added that it took her forever because by then, she could hardly type.
I was touched by his note and asked him if I could share Jane’s letter with someone I knew who might be dealing with MSA, and more recently if I could publish it here. Both times he wrote back, “Yes, Jane would want her story to be helpful to others.”
Hadley and I had worried together about Jane before, but now that Hadley might be facing MSA, I thought long and hard about whether to share Jane’s story with her. On the one hand, Jane’s was a diagnostic journey that Hadley could relate to and contained information that could be useful. And, Hadley was a give-it-to-me-straight kind of patient. On the other hand, even though I knew Hadley still felt vibrantly alive, I worried she would be overwhelmed by Jane’s account of her struggle with MSA. I asked if she wanted me to send her the letter, and she surprised me by saying yes. After I sent it, I remained conflicted enough that I never asked whether she’d read it.
She didn’t say.