For one man, taking control of his own health care decisions was a major step in learning to live with muscular dystrophy

by Allan H. Macurdy


Muscular dystrophy (MD) and I are lifelong companions, and though I know our mortal enmity will some day be tested (and I will lose), we've maintained a relationship of wary coexistence. The disease and I were first introduced in second grade, but we'd been intimately connected from the moment my parents conceived me. As a genetic disorder, muscular dystrophy is as much a part of my identity as my eye color or my height. It was never an invader: The disease and I have fought for control over territory to which we both felt entitled.

Our introduction took the form of a death sentence, albeit in clinical language. When I was 8 years old, my parents were told that I had the disease and that it would lead to my demise, probably by age 15. Oblivious to the positivist echoes attached to the word, the medical community describes MD as a "progressive" disorder, meaning that the disease continues to destroy muscle tissue until it kills you. However devastating to my parents, all this was beyond my understanding as an 8-year-old, but at least there was now an answer when I asked why I fell down a lot or why I walked funny.

During my childhood, I rarely saw my disease-companion sitting at my table, and even more rarely thought about the role the disease played, and was to play, in my life. Such dark thoughts weren't a feature of my happy childhood. Indeed, I lived the typical life of a small boy in suburbia. Friends were everything, did everything. We pretended we were superheroes or professional athletes. We delighted in mischief. We disliked girls intensely. But MD was involved in much of my life and determined that my experiences would be atypical.

At age 8 or 9, when my friends would play, I began to notice that I couldn't keep up with the other kids. I was always last in races and was frequently tagged out, as were many kids, but it seemed that I never had enough energy for play. My most hated activity was the long climb to the second floor for art class. Each step in the flight was a new battle to get both feet on the next step while hanging on to the banister for support. But I also had classmates who were terrible at sports or winded by that same flight of stairs.


It was my experiences with the medical system, however, that most clearly differed from anything in my friends' lives. Although I was examined by many doctors in my parents' effort to have my condition diagnosed, my earliest memory of hospitals and doctors came during second grade, when I spent a week in Riverview Hospital undergoing tests. Having been to school, I knew I didn't like tests, but these were so strange that I almost began to enjoy them. In one room, a man glued wires to my head, dimmed the lights and told me to think about a rabbit. In another room, a beautiful lady took a scary needle and put it in my leg, but it didn't hurt. Because these people were friendly and took the time to explain what was happening and why, this wasn't a terrifying experience.

After the doctors determined that I did indeed have MD, I encountered the health care world in brief visits with various professionals. I saw my orthopedist and the physical therapist on a monthly basis, with occasional trips to the brace maker or hospital X-ray department. But my battle with the disease at this stage of my life was fought largely apart from doctors and therapists. After all, they were focused upon slowing the inevitable march of deterioration, rather than the day-to-day realities of trying to be a kid. Not that our goals were in conflict: Inactivity would not only be intolerable for a child, but it would also accelerate the destruction of muscle tissue. So with the blessing of the medical world, the challenge of climbing the stairs to my bedroom or rising to my feet after a fall were the tangible victories I sought, and savored.

By adolescence, however, my weakened muscles could no longer support the efforts of my growing body. I could no longer climb those stairs, and if I fell, I was now required to wait until someone came along to help. To a teen-ager, reliance upon other people, particularly adults, is the perpetuation of childhood. Anything that restricts any desire that a teen might have is perceived as an injustice, an effort to prevent entrance into adult privilege. For most teens, the source of those restrictions, and therefore those injustices, is their parents. But for me, the disease played that role: In fact, it was the inevitability that MD would prevent me from achieving the independence I so desperately wanted that bothered me the most. I had never really thought about it before, but at 13, I realized that I'd probably be dead in two years. In the face of that reality, all my struggle against the disease seemed futile. And what could be more unjust?


For a time, my anger and frustration threatened to overwhelm me. That sense of powerlessness pervaded my inner self, and though time moved on, to me it appeared that I would never get beyond my rage. But I soon tired of the melodrama and began to get on with my life. For some reason, as I neared the end of junior high school, I was no longer so angry, no longer caught up in the futility of fighting the disease. I can't point to any crystallizing moment when I found a purpose for my life or came to value the people around me. All I know, and all my parents knew, is that by this point I had gained faith that my life had value and, for this reason, I was much easier to deal with. I was now able to concentrate on both being a kid and developing the social techniques to work around others' reactions to my disability.

Over the next decade, my life proceeded in much the same way as did the lives of my peers. I went to college, graduated, went on to law school and prepared to enter my profession. Because the disease created only orthopedic problems, I learned to compensate so effectively that the disease was nearly irrelevant.

But this seeming irrelevance was an illusion of my own making. The disease was still very much with me. In the midst of bar exams and job searches, my respiratory system began to fail. As my breathing capacity diminished, the carbon dioxide in my bloodstream reached toxic levels. This led to a significant loss of stamina and concentration. In addition, because I couldn't expand my lungs I had frequent respiratory infections and pneumonias. I was no longer able to compensate for all these physical problems. This marked the beginning of an 18-month decline that increasingly forced me to seek out and rely upon medical professionals and the medical system.

In February 1987, two days before I was to sit for the bar exam, the chest cold I had been fighting deteriorated into a full-blown lower-respiratory infection. This wasn't unprecedented since, at age 27, I'd certainly had my share of illness. But something about this infection was new and alarming. For the first time, I was unable to clear secretions from my airways by coughing and I was becoming increasingly short of breath. Chest X-rays confirmed Dr. Hill's suspicions of pneumonia. I was started on a course of antibiotics and chest physical therapy, but after two days in the hospital it became apparent that I'd need some form of mechanical breathing assistance, at least until I had fought off the infection.


What happened then is a parable on the failings of the medical model to consider the person behind the symptoms. In treating respiratory failure brought on by MD, Dr. Hill had begun using the iron lung, a device long associated with the polio epidemic of the 1950s. I had a vague memory of seeing an old photograph of an iron lung in a book, but I was totally unprepared for the realities of using one -- or the fear. Someone wheeled this huge steel cylinder with a mechanical bellows at one end into my room. Behind it, much like infantrymen advancing behind the cover of a Sherman tank, came a squad of residents and medical students chattering about test results and pressure settings. With little more than a passing acknowledgment that I existed, the resident in charge ordered the nurses to put me into the iron lung and began to quiz his squad on the technical minutiae of my case.

Pause for a moment to consider the situation I faced. There I was in this intensive care unit, fevered, my lungs full of secretions and unable to get enough air. Such circumstances are frightening enough by themselves. In the midst of all that activity, my anxiety was heightened by the fact that no one was listening to me, no one seemed to be aware that I existed beyond the data in my chart. But more terrifying still was the iron lung, and the procedure necessary to be placed inside. First, the cylinder was opened and I was laid down completely flat inside, a position that made breathing even more difficult for me. Next, I was slid up so that my head passed through a hole in the end of the cylinder and rested on a shelf outside. I lay face up with a 3-foot steel ring around my neck like some grotesque Elizabethan ruff, unable to move my head more than half an inch in any direction.

All of this alone was enough to induce claustrophobia, but add an inability to breathe, carbon dioxide toxicity and fever, and the result was abject terror. None of the residents seemed to notice, however. At a time when the right words could have allayed my fear, or at least pushed it back, instead I met words that trivialized and excluded. The residents chattered on among themselves with great enthusiasm about arterial blood gas levels and the comparative advantages of negative versus positive pressure ventilation. But direct interaction with me consisted of awkward, perfunctory monologues, delivered with impatience and never followed by listening. Because stress makes breathing more difficult, respiratory patients can't fully benefit from treatment without controlling their emotions. I needed both information and the assurance that I wouldn't be sacrificed to some other agenda. My questions, and forthright answers, were central to my ability to cope with this crisis.

That the residents were completely lacking in respect or empathy wasn't only clear to me but also angered the nurses. The nurse assigned to my case -- John -- had to get me inside while dealing with my anxiety. John tried to explain what was to occur, and to offer calm reassurance, but each time he finished readying me for the process, the residents would transform my room into a noisy chaotic disaster and he had to begin again. After the second false start, John asked the residents, quite nicely, if they would mind taking their discussion out of the room. They complied with his request, but their compliance was short-lived.

Finally John had had enough. Raising his voice above the din, he said, "If you aren't going to help, get the hell out of here! You're making this 10 times harder on Allan." The residents were instantly aroused to righteous indignation, but nonetheless they filed back out. Once they were gone for good, we succeeded in getting me from the bed into the lung. The cylinder was closed, a seal was made at my neck and the bellows went into action. I had never before experienced that sense of total helplessness and vulnerability, and it was primarily the result of the residents' failure to treat me as the decision maker, the most interested party and an individual whose feelings counted. I vowed never again to be marginalized by medical personnel.


In that hospital experience, I learned many things about the medical system that had nothing to do with treating disease or caring, but everything to do with power. This system, like any other human system, is made up of political phenomena: hierarchies of professionals, chains of command, patterns of authority, institutional agendas. Seen as such, it no longer seems alien. Indeed, I have a political mind, so the system plays directly to my strength. Legally, all legitimate power is vested in the patient, providing, of course, that he's mentally competent and conscious. I was clearly both, but power will not tolerate a vacuum, so if I failed to use my power, someone else would. The device best suited to enhance the power of health professionals is their virtual monopoly on medical knowledge. Lack of access to information, except through such a professional, is extremely intimidating and causes the patient to give up a great deal of power. The institutions also coerce the patient through the loss of both privacy and scheduling autonomy.

Even the everyday language is laden with authoritarian nuance. If I as a patient were to decide, for any reason, not to take a given medication I would be described as "refusing" treatment, not "making a decision." If I were asked if I were in pain and answered in the negative, I would be "denying" pain, as if the pain exists but I just will not admit it. And if I were to "deny" or "refuse" too often, I would be labeled "non-compliant" with the supposedly legitimate authority of the medical professional. These few examples are indicative of at least a habit of mind, if not a system of belief, that regards patients as, at best, children in need of parental figures to make decisions for them, and confers superior status on the practitioner.

To be sure, most health professionals don't view their relationships with patients in such terms. Quite the opposite: Individuals are often drawn to the medical field out of a desire to care for others; if asked what they like most about being doctors, nurses or therapists, they would cite the satisfactions of caring. But I submit that one can be a caring professional, endeavoring in good faith to help others, and still participate in a system that seeks to diminish and marginalize the patient. Indeed, the caring ethic masks the power inequity by convincing practitioners (and the rest of us) that because they are here to help others, no one is oppressed, no one could be victimized and power just isn't relevant. Such isn't reality, however.


With this in mind, then, I fashioned a set of principles that now govern my interactions with health-care professionals and the medical system and track to a great extent the manner in which I conduct my life as an individual with a disability outside of my medical needs. These standards evoke for me dozens of relevant stories, and I will share some of them by way of illustration.

First, as the person with the greatest stake in my health care, I make the decisions and no health professional can overrule my preferences. This seems, at first, a somewhat indisputable position. In practice, however, health professionals have difficulty when the decisions I make are in direct conflict with their advice or preferences. These need not even be conflicts over weighty health problems, and they quickly deteriorate into absurdity.

One spring afternoon, a month or two after I had begun using a portable ventilator, I was outside getting some fresh air, strolling with one of my nurses in the park that runs down the middle of the avenue where I live. Side streets cross the park at one-block intervals and traffic from the avenue often turns onto the side street. As I reached one of the side streets, I checked to see if cars were turning left from the avenue and approaching me from my right. Concluding that it was safe to proceed, and not consulting the nurse, I crossed the street.

When I reached the other side, it was abundantly clear that the nurse was unhappy with what had just occurred. She stopped and said, "You should wait until I say it's safe to cross because if something happens I'm responsible." I had been living in Boston for nearly 10 years, so my first inclination was to burst out laughing, but instead I asked, "Are you responsible because I'm a fellow human being or because you're a nurse?" She replied that it was because I'm a fellow human being, but neither of us believed her. I then said, "If you want to continue on this case, you'd better get this straight: You have no decision-making authority whatsoever. You're here for your medical advice and as an emergency backup. I'm not mentally incompetent, and therefore you can't make medical decisions on my behalf, and you certainly can't decide when I cross the street. If you can't abide by these conditions, then you shouldn't be working here." I haven't seen her since.

This problem is the result, at least in part, of the work experiences of nurses in intensive care units where patients are gravely ill and in need of surrogate decision makers. Some nurses can't comprehend that home care with me is very different. But even professionals that seem to understand, that appear to "get it," will catch themselves or be caught making decisions for me and acting unilaterally. The ideology is deeply embedded.


The second principle that governs my relationship with the medical system flows from the first. As I'm the only legitimate decision maker, no one else is permitted to speak for me. Again this principle seems unassailable, but in practice it's a position more often observed in the breach. Far too commonly, a doctor or nurse enters my hospital room and speaks to another professional or to a family member rather than to me. Medical staff have also tried to order for me in restaurants, make requests for me in department stores and take it upon themselves to reprimand the concierge in my apartment building because the elevator was not working -- all in the name of health care. Such actions aren't only corrosive to my self-esteem but undermine my personal interactions and professional integrity by advancing the perception that my needs are so extensive as to render me irrelevant.

So if I'm to speak for myself and make the decisions, it follows that I must have complete information, and that's the third principle. The system is remarkably prone to keep information about a patient's condition and treatment away from the patient, while the professionals blithely discuss patient information in crowded elevators and cafeterias. At one point, in the intensive care unit, I asked to see my chart. The head nurse for the unit first stated that hospital policy didn't allow her to show me the chart. When I informed her that such a policy was in violation of the state patient rights law, she then said that she needed the approval of my doctor before she could produce the chart. The doctor told her that I was to have access to the chart whenever I deemed it necessary, but clearly the presumption that patients may not see their charts was well entrenched.

The fourth principle of dealing with the medical system involves the role my health care is allowed to play in the rest of my life. Health care is a means to a full and meaningful life; it's not an end in itself. But because the professionals deal only with the medical aspects of my life, they often lose sight of the impact of their recommendations on my career, home life and relationships. If their efforts aren't resisted, the medical agenda will overwhelm the human agenda -- the tail will wag the dog.

After nearly 35 years, my relationship with MD, like any other long-term relationship, has grown exceedingly close and complex. We have forged a bond as fellow travelers, and traveling the road together has taught me to fight but also to cherish life and the people who give that life meaning. For a long time, I believed that the disease couldn't be real if I refused to acknowledge its existence -- if I kept it out of sight. But I've always known my disease-companion to be close by, waiting just beyond my vision. Now I find that I prefer to invite my companion into the light of the fire rather than search for his eyes in the dark. .

Allan H. Macurdy is an adjunct assistant professor at Boston University School of Law and a staff attorney at Pike Institute on Law and Disability, part of the law school. He's also director of the Office of Disability Services at Boston University. This article is excerpted from The Patient's Voice, edited by Jeanine Young-Mason, and reprinted with permission from F.A. Davis Company.