Flowers for Miss Joan

Posted by on 07.15.09 | 1 Comment
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I met my new neighbor yesterday. I took her some flowers from the garden and sat with her for awhile while her son ran an errand. He was working on the house before she moved in, and I stopped to welcome him to the neighborhood. He told me his mother “has some weird, rare brain disease with some really long name that makes her fall…” 

“PSP?” I asked.

He said, “No, it’s not some sissy disease, it’s really awful.”

You can imagine that I took umbrage at him suggesting that PSP is a sissy disease, so I cleared that up for him, and as I threw out the major symptoms and told him the full name, Progressive Supranuclear Palsy, he said “Yeah, that sounds like it, I know it’s got ‘super’ in it.”

Later I met one of her caregivers, the wife of the team who lives with her. I told her my mother had died of PSP, and we talked about the manifestations. “Is it bad?” she asked. “Yes,” I nodded. “It’s bad.” We left the conversation there and turned back to the party, with my assurance that I would help however I could.

When I went in to meet Joan, I saw Elizabeth’s mother, and my own mother, a little gray-haired lady with frail limbs sitting in a chair, leaning stiffly forward, squinting up at me with one eye shut, her sluggish voice dragging past a drooping, swollen lower lip, her hand slowly coming up to shake mine. I trimmed the flowers and arranged them in two glass vases from her cabinet, put them in two places where she’d be able to see them, where she didn’t have to raise her eyes. 

“Supranuclear Palsy” refers to a diagnostic weakness in the muscles and nerves that move the eyeballs up and down. If you hold your head steady and look up into your forehead, or down toward your cheeks, and you can’t move your eyes, you’re in for a really rough time. The disease is a multi-system atrophy much like ALS, which initially manifests like, and is often misdiagnosed as, Parkinson’s. Miss Joan told me she was diagnosed a couple of years ago, and in the past year has been getting worse quickly. I know what this means. I wonder if she does.

When I asked if she had been told what to expect, she gave me a confused account of where and when she had been diagnosed, but she didn’t answer the question. I thought I saw a tear below her dark glasses a few minutes later. 

“I have three sons,” she told me, and added a little wistfully, “…no daughters.” Her husband is dead. I wonder if she has anyone to really talk with about her life, her feelings, her physical decline and approaching death. My heart breaks for her, and for her family, her sons and her caregivers. They don’t know what’s coming. If I can be a guide to this family that’s going through it now, let me. People did it for me, they just did it on a computer forum. I just happen to be across the canyon in real time from Miss Joan.

I left shortly thereafter to go to yoga class. Before I left I told her how my mother and I had come to embrace the concept of “the new normal,” how things go along for awhile, then some aspect gets worse or a new symptom arises, and that becomes the new normal, and you figure out how to live with the new normal, until there is another new normal. I took her hand again to say goodbye, and she placed her other thin hand on my arm as we looked long at each other. 

I am forever entangled in my mother’s disease. It is an integral thread of my life, whether or not I will it. It’s true that the everyday horrors of the new normal have receded into the general haze of memory. Is that because even at the time the everday horrors were mingled with the plentiful, unbearable daily moments of tenderness and connection? 

Yet I live with “a new normal” anxiety: I will never know if that disease will come and rob me first of one capacity, then another, until finally all. I cannot live my life in wondering, but I can be aware. I can practice rolling my eyes upwards daily, and notice if one day they won’t go, or if one feels weaker than the other. I can practice yoga and balance, monitoring my muscular coordination. But what can I do if I see a sign of PSP in me? All I can do for now is draw on my experience with the disease, and help a neighbor live and die with it.

One way, perhaps, I can help, is to finally sit down and finish the book I began to write the year after my mother died, about her struggle with PSP. Here is how it begins…

Killing Mother

The year I turned 45 I was forced to seriously consider killing my mother. In the end, despite my best intentions, I did not do it. I did move from the ragged interior of the country to the urban east coast that year to help her die. 

When I drove away from my sanctuary hidden in the high desert, I believed that if she asked me to I’d be able to end her life. I also believed, even more strongly, that if I had the disease she’d been diagnosed with, I would wait as long as I still could, drive to the edge of the Black Canyon, videotape a short goodbye including apologies to those who’d have to clean up my remains, and take a flying leap off the rim of the 2000 foot chasm. I’d fly. I’d soar as I plummeted to the bottom. Theoretically I’d be dead of a heart attack long before I reached the bottom, but I’d try to stay conscious as the jagged boulders holding the river rushed to meet me. I’d spread my arms, try some of the freefall spins and moves I’ve seen skydivers do on tv, and cry out to god all the while offering thanks for the splendor of the life I was ending.

However, after several months with my dear mother and her rapidly degenerating brain stem, I had no certainties left. None at all. Not about my ability to assist her suicide, not about my own conviction to end my life if I end up with the same disease. Only after she finally did die, did I realize I’d lost my faith as well, somewhere during the long, quick ordeal.

I never thought, when I was growing up, that I could live farther than an hour from a coast. I thought I’d die of claustrophobia stuck inside the middle of the continent. Who could have imagined then that I would end up finding peace of mind here in the rural West. I’m within a day’s drive of anything I could ever need in life: the deserts, red and pink, apricot, sand and rock, of Utah and Arizona; the ghostly hills of New Mexico and O’Keefe’s paintings of them in Santa Fe; any number of rushing untamed rivers, wild in all or in part, accessible by foot or raft or canoe; the wild terrain of puma and bear, elk and mule deer, badger, coyote, bobcat; the most cold and barren peaks stateside, alive with alpenglow, and lower, the miracle of tundra; all these places a reliable vehicle and my feet can carry me and my longing within half a day’s journey. I do not miss the sea.

I love the sea, but perhaps I do not need it in the way that I need the spare wildness of the desert, or the sight of mountains, or the embrace of slickrock canyons. My mother helped me purchase land here, near the edge of a cliff overlooking a small canyon. She encouraged and supported me while I built an adobe house on the land. I have a magical greeting card she sent me while I was building the house. Inside it she had recorded her voice so that when I opened the card I heard her say “We love you, and we’re so proud of you.”

I kept thinking, over the years, to record over it a new message to send back to her. Always something held me back. When I found the card yesterday, cleaning out a drawer, I opened it to hear my own mother’s voice offering priceless, eternal words of encouragement and esteem. I am lucky to have those words in her voice where I can hear them any time I want. For I have been wanting, wanting to hear her voice again, from beyond. I have wanted her to speak to me in my dreams, or to speak to me in the garden, or to show herself. She both has and hasn’t confirmed her presence with me, but I only heard her whole voice once, in a dream, in which she was outside a window and I was talking with my father; I caught a shred of something she was saying from afar. And then she was gone. Today I have again her whole voice, telling me she loves me and is proud. 

Elsewhere, I have hours and hours of her voice degenerating. They call this disease “progressive,” but there is no positive direction to its course. I recorded hours of testimony to the awful destructive power of the disease as it gradually, hastily consumed her voice, her swallow muscles, her eyesight, her mobility, but left intact her awareness. I recorded, perhaps, the astonishing struggle between her desire to die and her will to live. 

The intensity of her will to live is part of what kept me from being able to kill her. Despite egregious bodily discomfort, and a lifetime of rage at one circumstance after another, still she managed to laugh, and to dress, and to go through the motions day after day. Until, one day, her desire to die finally overcame her will to live. 


Discovering PSP

Three years before she was correctly diagnosed with PSP, my mother Ali was misdiagnosed with Parkinson’s Disease. That summer, we took a trip to Alaska, her long-held dream of a cruise down the inside passage. She’d already fallen once by then, but nobody thought anything of it. You get old, you fall. Our first night out we were walking back from cocktails on deck, staggering in the ocean air along the narrow corridor between the deck railing and the ship’s wall, giggling and laughing at how we staggered, swearing it was the ship and not the drink that made us weave like weathered drunks back to our cabin. Possibly, in her case, it was partly the PSP. I blame my staggers on being seasick.

She fell again on a shore walk in Ketchikan, though it seemed clear that time that she’d tripped. We were walking over driftwood and smooth boulders and seaweed to see some petroglyphs along the shore.  She had passed on several of the proffered shore tours but, ever the art appreciator, not this one. She was bruised and sore afterwards, yet undaunted. In the end, we were so glad we’d gone to Alaska when we did. She knew at the time it would be her last trip. She didn’t even come visit me again, but sh
e did begin to fall more often.

When she fell, she fell straight back. Just dropped, no blackout, no light-headed warning, she just fell back. Once she hit her hip on the corner of a granite table in the lobby of The Home where she lived with my father, the Colonel; twice she hit her head on the apartment floor and required stitches. While falling was her most dramatic symptom, the most insidious might have been her double vision. I’d visited three years earlier when the Colonel had appendicitis, to help her with his recovery. I flew in and met her at Walter Reed in DC, the army hospital where her grandfather the general had finished out his life. After I’d visited with the Colonel, she and I went down to the car in the parking garage. I offered to drive but she said she was fine and so she drove into the bright light of the ramp leading into the heart of downtown traffic. I glanced over and noticed she had one eye closed.

“You’re driving with one eye closed!” I shrieked. “Why are you driving with one eye closed??” 

“Double vision,” she replied. “I only see one if I close one eye. It comes and goes.” By then the light was green and let us out and I couldn’t insist that she pull over. The hotel was only a block or two away, but after we arrived I took the keys. Eventually her eye doctor had prescribed special glasses to try to help the double vision. Prisms, she said. It didn’t occur to me to ask any questions, as long as Dr. Schefkind was doing something to fix it; after all, he was the doctor.

She’d also been seeing Dr. Williams, a neurologist who’d diagnosed the Parkinson’s. Where did he come from? How did she end up in his hands? I think he was on some list The Home published for their residents. Dr. Williams recommended that Ali read a book about Parkinson’s, and so she recommended it to me. I ordered it from Amazon and a few days later began to read. On page 11, Chapter 1, What Is Parkinsonism? I found this description of one of several diseases often mistaken for Parkinson’s: 

Progressive Supranuclear Palsy is the most common of the ‘Parkinson Plus’ syndromes. The initial clinical presentations may be like Parkinson’s disease. For this reason, it is often first diagnosed as Parkinson’s disease. The correct diagnosis is usually made several years after the first symptoms appear or not until after the patient dies and the brain is examined at autopsy. Patients with PSP complain of falls, gait disorder, visual problems, speech difficulties and swallowing abnormalities… Tremor is usually absent, and there may be a rigidity of the neck muscles that keep the head in extension. Patients have a wide-eyed ‘astonished’ stare that sometimes can be differentiated from the masked face of typical Parkinson’s disease. The diagnostic criterion by which PSP may be recognized during life is the inability to look down voluntarily…. As the disease progresses, other eye movement abnormalities may occur.

I read a few more pages, but I kept coming back to that paragraph. It described Ali’s symptoms to the letter. The first symptoms I noticed, not knowing they were symptoms, were her slump and her slowness, years before Alaska. She was sitting at my table sipping a drink. Her shoulders were hunched forward and her head hung so she was looking at the table rather than at me. She held her glass in one hand and raised it in slow motion to her lips. I told her to sit up straight, open her heart, lift her eyes. When she was reminded, she was able to square her shoulders somewhat, and hold her head up, but very shortly she’d forget and droop again. She also had that astonished stare. There’s no other way to describe it.

I made it to page 33 before I determined that Ali had PSP so why bother reading about Parkinson’s. This was in July, 2003. I suggested she bring up the possibility to Dr. Williams. So she told him, at her next appointment, opening the book to page 11, “My daughter thinks I may have this disease.” Naturally, being a highly trained neurologist, Dr. Williams said “I’m the doctor!” and that was the end of that discussion. I suppose I wanted it to be Parkinson’s and so I let his opinion stand. By that time I’d read more about PSP, and definitely didn’t want it to be that. 

But we started then trying to get her an appointment at Johns Hopkins, and did not get one scheduled until January 14. That November I visited for a couple of weeks, and I knew it was bad. I returned from my travels just before Christmas, and after the holidays found myself at loose ends, wondering about my purpose and value in life. I felt so lost and down that I prayed fervently for some work more meaningful than the job I was doing. And I had thought I was doing what I wanted to do. But it felt empty. 

My brother called from his cell phone on the way back to the hotel after mom’s visit to Hopkins and said “You were right.” This deep sense I’d been having since November that I would have to leave home for a long time to go care for my mother was validated with his words. I had received the mission for which I’d prayed, and in a strange way I felt relieved. Though I did caution myself that getting caught up in a “good rush” is just as bad as getting caught up in any bad rush: pursuing a benevolent or heroic path motivated by Ego is as spiritually ignorant as a life addicted to fear.

An hour and a half in the hands of a stellar young neurologist who knew what to look for and knew, above all, that the disease existed, and my mother had her answer. It turns out that many neurologists don’t even know enough about PSP to diagnose it. That day I looked up Dr. Katherine Widnell at Hopkins, and found a photo of a woman young enough to be my daughter. I
also telephoned the Society for PSP, which I found listed in the back of that infamous book I stopped reading on page 33. I told the nice woman there about the diagnosis and the symptoms I’d noticed for years, and said “I guess I’ll finish up some work here and then move out there, maybe in the fall.” 

“That may be too late,” said this unknown woman on the other end of the phone line, and so I resolved to move in spring. It was that simple.

I told mom on the phone a few days later that I was going to start winding things up here so I could move out there in spring. “Oh honey, I don’t want you to do that,” said my mother, “I want you to come when I’m dying dying, you know ~ I don’t want you to give up your life and come out here now.” I thought ‘but you are dying dying now, don’t you know, don’t you see?’ So I wound up the projects I was working on, and prepared to move out there at the end of March. 

During the course of those two months I often visited the PSP Forum online. When I’d spoken with the Society in January, the woman had told me about the Forum and suggested I visit it, but “maybe not just yet,” as she feared its content would be too intense for me to handle right away. Within two weeks after diagnosis I visited the Forum and became a regular reader. Every night that I logged on and read the threads of accounts of various wives, daughters, husbands, sons, and a very few parents struggling with PSP’s ravages on a loved one, I ended up bawling before I logged off. 

PSP is fatal and usually kills between one and ten years after diagnosis. PSP gradually takes all a body’s motor functions, voluntary and involuntary, while leaving the mind’s awareness virtually intact. Most PSP patients die of aspiration pneumonia after inhaling their own saliva or their dinner. 

PSP is about 1% as common as Parkinson’s Disease. Estimates suggest that perhaps as many as 20,000 people in the US actually manifest symptoms of PSP, but only a couple of thousand are diagnosed correctly; most are misdiagnosed with Parkinson’s. However, about 25 percent of people diagnosed with PSP end up, upon autopsy, having had something else, either Parkinson’s or one of the other rare, weird brain diseases that kill these days. I have to wonder if the human brain simply has not evolved as fast as our human strategies to outrun death far into old age. 

On the island of Guam there has been an unusually high incidence of a disease nearly identical to PSP, which some experts suggest is the result of a pathogen derived from eating fruit bats. Nobody knows what causes PSP. Eating fruit bats might contribute on Guam, but elsewhere? What little research there is suggests that there is a genetic component to PSP and there is also some kind of environmental trigger or triggers. The genetic component has been identified as the H1H1 haplotype on a particular gene, but nobody can figure out what the environmental trigger or triggers is or are. On the PSP Forum family members are trying to link it to exposure to certain chemicals, or to some sort of physical trauma preceding the first symptom, or to any tangible source they can identify. Yet the cause of PSP remains a mystery while the effects ravage thousands yearly, one family at a time. 



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