The lady who thought she was crazy.
"I went in to see her after rounds the next morning," Dr. Brewer says. "She was sitting on the edge of the bed--sitting there with the help of a nurse. She was tall, with a beautiful figure, a beautiful face and beautiful, wide-apart eyes. She was also pathetic. She didn't seem to have any strength at all. She couldn't walk. She could hardly sit up. She could hardly lift her arms. I introduced myself and made some getting-acquainted talk. I took her history and did a routine physical and checked her mental states. She was just as weak as she looked. She was well oriented and alert. There was some evidence of conversion hysteria. I finished up with a lot of history and a lot of problems, but no strong impressions in any direction. I was inclined to go functional, but not entirely. I did a residency in neurology, and I always try to keep the organic possibility in mind. I started thinking 'multiple sclerosis.' Her age, for one thing, was right. Multiple sclerosis is a young person's disease. But I'm a listener. I think that if you listen long enough your patient will usually tell you what the trouble is. So I asked her what she thought was her trouble. She said, 'I'm a kook.' I said, 'Maybe so, but before we go that road I'd like you to see a neurologist I know.' She almost blew up. I think she might have hit me if she'd had the strength. She said, 'I don't want to see a neurologist. I've seen a dozen neurologists. I'm a kook. I'm in the kook hospital. I want you to fix up my kookiness' But I wasn't convinced. And that afternoon I called Fred Baughman, the neurologist I had in mind."
I met Sheila Allen in the spring of 1983, some five years after that pitiful outburst. We met, by prearrangement, in San Diego, in the office of Dr. Brewer's neurological colleague Dr. Fred Baughman, Jr. She looked good--the picture of health, and beautiful, and entirely free (if she had ever been otherwise) of kookiness.
"Oh, I was," she told me. "I was a kook. I had to be, after what I'd been through. If a person can be driven crazy, that was me. But, of course, I wasn't really crazy. I was simply in despair. I had been sick for so long--for almost four years. I was getting sicker and sicker. I was almost helpless. I went to Alvarado Hospital because I couldn't think of anything else to do. I had finally given up.
"I don't know when my trouble actually started. I mean, it came on so innocently. I guess it began in Dallas sometime in 1974. No, I'm not from Dallas. I was born in the Chicago area--in Maywood, Illinois. But when I was 14 my parents moved to Los Angeles, and I went to high school there. I ran track, and I was a cheerleader. I've always liked sports. I've always loved to run and dance and bike and that sort of thing. I went to Cal State, at Northridge, for a year, and I studied physical education. I went to Dallas because I wanted a change. Tehre were no problems at home, or anything like that. I just wanted a change. I got a job at a Sears store, demonstrating cosmetics. Then a friend told me that Braniff was hiring flight attendants. I had an interview, and they hired me and sent me to their school--six weeks of emergency procedures, first aid, posture, grammar, how to prepare the meals. I was based in Dallas, and I flew every stop between there and Chicago and sometimes New York--all over. I think March of 1974 was when the trouble first began. I was out dancing one night, and my legs gave out on me. I mean, I fell down. But I was able to get right up, and I said it was my high heels. That's what I thought. So I went to lower heels, and then one night it happened again. It was just like that first time. I was able to get right up and go on. Nobody seemed to think anything of it. So I wasn't really worried. But when I had my regular Braniff physical in August I mentioned it to the doctor. He gave me an extra-careful checkup and passed me. He said I was in great shape. He said I was probably just tired--too much standing. Well, flying is all standing. You hardly ever sit down on a flight. So that made sense. But then my legs began to hurt. I wasn't just tired, I also hurt--at the end of a flight, or walking through the airports. Like O'Hare. Or, especially, Dallas. You know how big it is. We were always having to walk from Gate 1 to Gate 22. My legs would cramp. It was a real tight pain. I would have to sit down and rest for a couple of minutes. Then I'd be all right. But all the girls were always complaining about being tired, so I still didn't think too much about it. Until it began to affect my arms. Just lifting two trays, and my arms would begin to tremble. It seemed like they didn't want to hold. Even a coffeepot was almost too much for my strength. I tried to get more sleep. But I just got tireder and tireder. And I began to almost miss flights because I ahd to sit down so often along the concourses and rest. So what I did was just get up earlier. If there's one thing I've got, it's will power. I drove myself as hard as I could. But I was getting worried now--real worried. I went to a doctor one of my friends knew. He said to take it easy. Stop driving myself so hard. He gave me a prescription for Valium.
"That would have been around the end of 1974. I thought maybe I wasn't eating right. It's hard to eat right when you're flying. All too often, after we had served the passengers their lunches or dinners there wouldn't be any food left. Most of the girls carried candy bars for those emergencies. I carried what I called my survival package--tuna fish and peanut butter and crackers and raw carrots and lots of vitamins. A lot of the girls recommended a shot of bourbon at bedtime. I tried that, and it was all right, but I couldn't see that it helped me any. I had to realize that my tiredness was different from the other girls'. So finally--I think it was in October, or maybe November, of 1975--I tried again. I went to an orthopedic specialist. I told him about my legs, how weak they were--and my arms. He looked me over; he was very thorough. But he couldn't find anything wrong. He said it was probably my job--the standing and the stress, and all that. His advice was to quit, to take a leave or something. But I loved flying. I loved my job. And I loved the money. I had a little Opel GT sports car. I had a nice apartment. I had a wonderful life. So I kept on working--getting to the airport early enough so I could make it to the gate, and getting back home late--and resting every change I got. I stopped talking about my problems to the other girls. I didn't want them to say anything. I wanted to keep my job. But I was only fooling myself. It was just too much for me. In February of 1976, I asked for a vacation, and they gave me three weeks' leave. I went to my favorite place--to Hawaii. I relaxed and rested and tried to enjoy myself. The three weeks went by, and I was the same as always. I just didn't have any strength. I went back to Dallas and gave my two weeks' notice. I said I had personal problems. Which was true enough. My sister Enid flew in from L.A. and drove me home in my little Opel--back home to my parents.
"It's hard to explain just how I felt. I was worried about feeling so tired and weak. But I still really thought it would go away. I still really thought I'd get better. Anyway, I couldn't just sit around the house. That isn't my way. I went out and got a job--two jobs, as a matter of fact. I got a daytime job demonstrating cosmetics again. I worked in various stores doing make-up. And at night I worked as a cocktail waitress in a club. I suppose that was kind of foolish. Because I had the same old trouble--the same problem with trays, the same problem with all the standing and walking. I had to wear heels at the cocktail job, and, of course, that made things that much worse. Every now and then, I dropped a tray. I could tell when it was coming on--I'd think, There goes the tray. The other waitresses dropped things, too. But they only dropped a glass or something. I dropped the whole tray. But the boss was crazy about me. That made everything O.K. This was in the summer of 1976. But finally the boss got to be too much for me. I had to quit. I had already quit the cosmetics job. Two jobs were too big a load for me, and the cocktail job paid better. When I quit that, I got a daytime job as a hostess in a restaurant at the beach. I guess I knew by then that there was something really wrong, but I wanted to work. I missed that airline income. It's hard to change when you get used to a certain level of living.
"When I first came home to L.A., I had gone to the family doctor. He couldn't find anything wrong with me. Then I met a chiropractor at a party and told him my problem. He checked me out and couldn't find anything wrong, either. He referred me to a woman psychologist. She game me an IQ test. That only told her that I was bright. So she gave me another test. And then another. She never seemed to try to think anything out--she just kept giving me tests. I decided she was a waste of time, and quit. I also quit my hostess job. I had to--I was getting weaker every day. Everything I did hurt. It hurt to comb my hair. My arms were so heavy and weak. It was hard for me to drive my car. Once or twice, it was really frightening. I'd try to brake or something, and my foot wouldn't move. One Sunday afternoon, I took my little three-year-old niece to the park. When we were driving home, a traffic light up ahead turned red and I started to brake and I couldn't lift my foot. Oh, my God! I thought. I'm going to kill us both. I told my niece to get down on the floor. I hung on to the wheel and we sailed across the intersection. Thank God, there was nobody coming. I don't know how I ever made it home. That was the last time I drove. I was at home now all the time. But I had problems even there. I don't know how many times my legs gave out and I fell down the stairs. They were carpeted, thank God, so I wasn't hurt much. That was going down. Climbing the stairs, I practically had to crawl. My mom was a problem, too. She kept saying I was just trying to upset her. She didn't really mean that. The real reason was that she couldn't bear to think there was something wrong with me. And my sisters. We would be getting ready to go out somewhere, and it took me so long to get ready they'd go off without me. I got so I was crying a lot. I'd try to pick up a glass, and my arm would go limp. And I'd say, 'Oh, no!'--and fall into a chair and just cry.
"But I still hadn't given up. When I quit going to that woman psychologist, my chiropractor friend suggested I see a psychiatrist. I was going out with the chiropractor some, and he was really trying to help. He was convinced that my trouble was psychosomatic. I didn't know what to think. Some days I was better, and some days I was worse. Some days I could get dressed and go out and everything was almost like normal. Then the next morning I couldn't even fix breakfast. I had a drawer full of prescriptions--Valium, Elavil, Equanil, all those drugs. Every doctor I went to see prescribed something. I tried them all and I couldn't see any difference. I couldn't see that any of them helped. So I went to a psychiatrist. He was a white guy. Some of the doctors I'd seen were white. Some were black. Anyway, I went to this psychiatrist once a week. We talked. We went over everything I'd been told by all the others. He said I must face it--I had a psychosomatic problem. I liked him; he was a nice man. He made me feel better about myself, which helped. But I didn't really believe what he said. Then I had my family--my mother and my brother and my three sisters--on me. They made it perfectly plain. They said I was crazy. I said to myself that they were crazy.
"About that time, in December of 1976, I met a nice guy from San Diego. He was a big guy--6'6" and 270 pounds. His name was Ira Watley, and he had been playing offensive tackle for Miami. He'd just got cut. He was going back home to San Diego, and he suggested I come with him--get away from my family. He knew they were driving me nuts. I thought about it. Then one morning I woke up and I almost couldn't get out of bed. But I made it, and started down to breakfast, and fell. I fell all the way down the stairs. I didn't know whether I was hurt or not--I hurt all over anyway. I must have looked awful, because my mother helped me up and took me to the emergency room of a hospital a few blocks away. There was a young doctor on duty. He examined me, and everything--my arms and legs and all. He said to wait, and went away. He came back with a textbook and he stood there studying it. Then he said, 'I would think you might have myasthenia gravis, but the symptoms aren't right. Myasthenia gravis starts with the eyes and face, with drooping eyelids and trouble swallowing. You don't have that.' He said he thought I ought to see a psychiatrist. He gave me a prescripton for Elavil.
"That made up my mind. I went to San Diego with Ira. He was really understanding. He wanted to get married. I didn't think so--not right then. But I was feeling a little better and getting a little restless. I wanted to do something. Ira suggested I go to school--to a business college he knew about. So I enrolled. That was in February 1977. But it was the same old story. I wasn't really any better. The school was on the second floor--a walkup--and those stairs were too much. I would go late, so people wouldn't see me struggling. It was one step and rest, another step and rest. Some days, I could go three steps without resting. I was taking typing. And finally I couldn't do it. I couldn't hold out my arms. It was a three-month course, but I never finished it. After that, I just stayed home. Except to go to the doctor. I had already seen a couple of doctors in San Diego. One was a regular internist. The other was a neurologist. They were like all the rest: psychosomatic, Valium. But Ira kept pushing me to keep trying to get some help. I even went to a doctor in La Jolla who tried to hypnotize me. I wouldn't hypnotize, and that frustrated him. He told me I didn't want to get well. Then I tried an orthopedic surgeon. He asked me to lift my arms. I couldn't do it. He stumped out of the room. He said he couldn't help me because I wouldn't cooperate. Then I tried another psychiatrist. I had four or five visits. The psychiatrists and the psychologists were all alike: What did your last doctor say? I see. Well, I agree. Psychosomatic. I'm going to give you a prescription for an antidepressant. Some of them started with my childhood. Some started now and worked back. I told them I had a normal childhood--a normal middle-class childhood. I told them I didn't hate my parents, and they didn't hate me. They were loving parents. The psychiatrist I went to four or five times, he saw Ira when Ira brought me to his office. I told you Ira is a very big man. So this doctor had a new idea. He asked me if Ira beat me.
"I started getting completely worse in June--June of 1978, Ira was away. The Winnipeg Blue Bombers had signed him on, and he was training up there in Canada. He had arranged everything before he left. He had his father come over and look after me. He sent me his check every week, and one of his friends--either Frank or Drake--would take me to the bank and to the supermarket, or wherever. Frank was very sympathetic. He had been in Vietnam and had some therapy. He persuaded me to try the state mental-Health center. I joined their group-therapy class. I went for a while, But the classes were kindergarten stuff, and I wouldn't take the drugs they offered. Elavil was one of them, and it actually seemed to trigger more weakness. It seemed to actually loosen my muscles. I had some bad experiences even without drugs. I'd sit down in a chair, and when I wanted to get up I couldn't. I'd have to wait for Ira's dad or somebody to help me. It got so I was afraid to cook, I remember one afternoon I had some vegetables on the stove. I left the kitchen and fell, and I couldn't get up. I just lay there smelling them burn. The house was full of smoke when Ira's dad came in and turned off the stove. It was an awful feeling. I wasn't good for anything. I used to sit and meditate and pray. I'd fix my mind on, say, fishing or shopping, and try not to think of anything else. I did a lot of praying. I'd say the Lord's Prayer over and over and over. It helped me, I think, I prayed in bed in the morning, too. I kept a Bible by the bed. In the morning, when I woke up, I'd read or pray, and finally I'd have the strength to get up. One morning, I couldn't comb my hair. I didn't have the strength. I finally had to lay my head on the sink, and that way I could use my arms a little. There was another time. Drake and his brother Lee took me fishing with them out on the park dock. After a while, I went to the bathroom. I sat down on the toilet, and I couldn't get back up. Nobody came in. I yelled and yelled, and finally Lee came in and got me out.
"I think that was about the last time I went out anywhere. I knew I was reaching the end of the line. My muscles were getting weaker and weaker. I had to rest more and more. I knew that pretty soon I'd be helpless. I had to do something before it was too late. I decided my only hope was to get hospitalized. And I knew that the best way I could get hospitalized was to talk depression and suicide. That was one thing I'd learned from all those psychologists and psychiatrists. The hospital I picked was Alvarado Community Hospital. It was the nearest one to Ira's apartment. It got Frank to drive me there, and he had to practically carry me into the emergency room. I told my story, and they took me in. I remember that date. I remember it very well. It was Wednesday, October 11, 1978, late in the afternoon. But the date I really remember is the following Wednesday, October 18, around noon. That was the day I met Dr. Baughman. He came into my room and looked me over and told me what my trouble was. I had myasthenia gravis."
"Well, yes," Dr. Baughman said to me. "It was almost as quick and easy as that. I was pretty sure the minute I saw her that her trouble was myasthenia gravis. The only other possibility was amyotrophic lateral sclerosis, and she was much too young for that." Dr. Baughman is a slight, wiry man of 50, with a bang of sandy gray hair and a wide, warm, country-boy smile. He was smiling now. "But it wasn't magic. Myasthenia gravis is a special interest of mine. I trained at Mt. Sinai, in New York, and Mt. Sinai has a rather famous myasthenia-gravis research laboratory. Some important work has been done there. So I learned about myasthenia gravis early, and it is always on my mind. There is a saying about diagnosis--about why doctors often fail to recognize one of the less common diseases. It goes, 'When you hear hoofbeats, you don't necessarily think of a zebra.' I recognized the hoofbeats of a zebra. That was my only magic. I won't say myasthenia gravis is a common disease. But it isn't a rarity, either. The national occurrence rate is one in 20,000. I see my share, because I'm aware. I average three to five new cases a year. Women seem to be more susceptible than men--particularly young women. Myasthenia gravis is a good descriptive name. The 'my' refers to muscle, and 'asthenia' come from the Greek for weakness. It isn't as gravis--as serious--a disease as it once was. It was first described in the 17th century, and it was named around the end of the 19th century, and in those days and up until the middle 1950s it was very often fatal. There is a treatment now. But, of course, it can't be treated if it isn't diagnosed. That's the problem. In that respect, Sheila was a classic case.
"I remember my first look at Sheila. Bob Brewer had called me and said he had some doubts about a psychiatric patient at Alvarado, and would I take a look. So the next day I went up to Four South when I finished my regular rounds. The first order of business is the visual impression. And oh, Lord! She was pathetic--truly pathetic. She was lying in bed and not exactly crying--sort of whimpering. There was a feeling of just hopelessness. Her beauty made it all the worse. I knew the moment I saw her that there was a profound, generalized muscle weakness. I called a nurse, and we got her sitting up. She tried to help, but it was a genuine, real weakness. Nothing functional about it. Her face was normal, and so was her speech. There was a slight nasal character, but not much. I had the nurse get her to her feet and walk. It was a definitely abnormal gait--it was a waddle. that indicated a hip-girdle weakness. We have a scale of evaluation. It begins at zero, then trace, then poor, then fair, then good. Sheila's hip and shoulder girdles were poor to fair. Her body and feet were in the good range. The weakness was symmetrical, and there was just no question about it. I knew her history, and I didn't believe a word of all those psychiatric evaluations. This was a serious muscle disease.
"It's sad to think how close she came to an early diagnosis. I'm talking about that young doctor up in Los Angeles. He suspected myasthenia gravis, but he made the common mistake of going entirely by the book. The books all emphasize that the presenting symptoms are drooping eyelids, facial weakness and palatal weakness--difficulty in speaking. The classic teaching requires the presence of one or more of those symptoms for a diagnosis of myasthenia. I had one patient who was referred to me by an ophthalmologist. A man had come to him complaining that he was losing his eyesight. The ophthalmologist recognized that the cause of his failing sight was the drooping eyelids of myasthenia gravis. I wish the textbooks were a little less rigid. But the fact is that a mere suspicion of myasthenia is enough, because there's a quick and easy diagnostic test that is almost entirely reliable.
"Myasthenia gravis is a fundamentally mysterious disease. I mean the basic cause is unknown. It is generally thought to be one of the several auto-immune disease--the consequence of some disturbance in the body's immune system. Antibodies appear in the blood that interfere with the supply of a substance called acetylcholine, which mediates neuromuscular function. I'm putting a highly complicated process in very simple terms. The result of this inhibition is a weakness much like the weakness induced by the poison curare, and the treatment of myasthenia gravis has derived from an understanding of curare intoxication. The site of the physiological defect in both curare poisoning and myasthenia gravis is the neuromuscular junction. In the absence of acetylcholine, the muscular response very rapidly decreases. A period of rest will, at least for a time, restore sufficient acetylcholine to allow for normal function, but only for a limited time. Then the weakness returns. Drugs have been developed that are antagonistic, in varying degrees, to the substance that inhibits acetylcholine. One of these anticholinesterase agents is involved in the diagnostic test for myasthenia gravis. Tensilon--or, generically, edrophonium choride--has the power to restore almost normal muscular function almost instantly, in a matter of moments. Its effect lasts only moments, but a positive response to Tensilon is diagnostic of myasthenia gravis.
"Tensilon was developed at Mt. Sinai in 1952, and I've used it many times. The effect is always starting. I gave Sheila the usual dose--ten milligrams, injected intravenously. I waited a moment, and then checked her arm strength. There was resistance to pressure--maybe 80 to 85 per cent of normal. So I asked her to get up and walk. She said, 'You know I can't walk.' I told her to try. She gave me a doubtful look--and sat up. Then she stood up. Then she walked across the room. It was a miracle. It's always a miracle, but this was one of the most miraculous. I'll never forget the look on her face. She was dazed. She was stunned. The tears were running down her cheeks. I felt the way I always do when I see that miracle happen. I felt--I don't know--almost godlike. Then she began to give out. I helped her back to bed. She collapsed. It was all over."
Sheila Allen was discharged from Alvarado Community Hospital on the morning of Thursday, October 26, 1978. That was 15 days after the day of her admittance and 8 days after her dramatic noonday meeting with Dr. Baughman. In the cours eof those 8 days, she was introduced to another, and more durable, anticholinesterase agent, called, Mestinon, and an effective dosage (one 60-milligram tablet every four hours) was determined. Tensilon is of only diagnostic value. The discharge summary noted, "Discharged to outpatient treatment, to be followed by Dr. Patricia Marrow for supportive psychotherapy (in adjusting to the presence of a chronic debilitating illness) and by Dr. Fred Baughman for control of myasthenia gravis." Her condition, the summary added, was "markedly improved." It was indeed. The friend who had helped her into the hospital was there to meet her on her departure. She was pleased by his thoughtfulness, she was glad to have his company, but she didn't need his help. She walked out of the hospital with the easy gait of any normal 24-year-old woman. She was, for all practical purposes, a normal young woman, and two weeks later, in early November, she was leading a normal life. She had a job demonstrating cosmetics at a San Diego department store, and she was attending evening classes at a school for real-estate brokers.
Dr. Baughman saw Miss Allen at his office soon after her hospital discharge, and once a month thereafter. At their December meeting, on December 5, he noted that her response to Mestinon was entirely satisfactory. It was better than that of many other patients in his experience, and inferior to none. Nevertheless, he watched her closely and questioned her closely at their monthly meetings. There is always a chance that Mestinon, or any other anticholinesterase agent, may in time weaken in its mastery. There is a surgical procedure that can provide a vigorous supplement to Mestinon therapy. This involves the removal of the thymus gland. The thymus is one of the endocrine glands and is situated in the windpipe, just below the thyroid gland. Its function has to do with the development and maintenance of the immunological system. The thymus develops rapidly in infancy, grows more slowly until around puberty and then, in most people, begins to wither away. It has been established that in victims of myasthenia gravis the thymus is abnormally intact, and its functions seem to have been perverted into the phenomenon of autoimmunity. In the course of the summer of 1979, Dr. Baughman decided that Miss Allen would benefit from the removal of her thymus, and he arranged with a thoracic surgeon named David M. Long to perform the operation. Miss Allen was admitted to El Cajon Valley Hospital on September 25 and underwent a transcervical thymectomy the following morning. The operation was a complete success, and its good results were immediately apparent. Dr. baughman noted that within less than two hours Miss Allen's muscular powers were stronger than at any time since he had diagnosed her illness. On September 29, she was discharged.
"Oh, sure," Miss Allen told me toward the end of our talk in Dr. Baughman's office. "I knew that a thymectomy was a serious operation. Dr. Baughman told me all about it. But of course I agreed. Whatever he suggested was gospel to me. He was the messiah. He is the messiah. I moved back to Los Angeles in January of 1980, but we keep in touch--I'm still on Mestinon; I always will be--and I see him here once a year. It seems to me that I feel better every day. When I moved back to L.A., I had finished my real-estate course, and I went in with my mother and father in their real-estate business. I also did volunteer work for a while in a hospital in the neighborhood. I did physical counseling. Then, in 1982, I heard about a new airline opening up with flights between L.A. and Hawaii, and I applied, and they took me on. I'm a senior flight attendant, and I do seven turnabout flights a month. Between trips, I still work in real estate. The only trouble I have now is trying to hold myself back. I don't want to walk--I want to run. I'm so full of strength and energy. I guess I'm making up for lost time. I told my new boyfriend about my myasthenia. He said, 'So what?' We dance all night."
|Printer friendly Cite/link Email Feedback|
|Title Annotation:||myasthenia gravis patient Sheila Allen|
|Publication:||Saturday Evening Post|
|Date:||Oct 1, 1984|
|Previous Article:||How do you shape up?|
|Next Article:||The toy that built a town.|
|ProSeal[TM] Laryngeal Mask Airway in a myasthenia gravis patient for thymectomy.|
|DR scoot's surgery.|