The Medical Detectives Volume I

by Berton Roueche

  [1974 ]

  CHAPTER 18

  Two Blue Hands

  Dean M. Berger is a big, tall, smiling man, and on the night of January 10, 1974—a cold night with a light snow blowing, a night he still remembers with a shudder—he had just turned fifty-two. Berger is a paint chemist. He is associated professionally with Gilbert Associates, Inc., a firm of engineering consultants in Reading, Pennsylvania. His avocation is bridge, a diversion in which he has achieved the gratifying rank of Life Master, and whenever possible, whenever he is not on one of his many out-of- town consultations, he plays in the regular Thursday-night games of an American Contract Bridge League club in the nearby town of Lancaster. His usual partner, until she moved to Michigan with her family, was his daughter Cheryl—Mrs. R. Douglas Crews— also a talented amateur. The Crewses lived in Palmyra, twenty- five miles from Lancaster, and on his occasional bridge nights, Berger would dine with them at their home. January 10, 1974, was a Thursday, and it was such a night.

  The event that evening was a tournament, and it was held in a private room at the Distelfink Restaurant, on the outskirts of Lancaster. Berger and his daughter arrived there, in Berger's car, at around seven-thirty. The meeting was well attended, with thirteen or fourteen tables in play, but the room was cold. Everybody complained about it. Berger felt it in his hands, in his fingers. He was warmly dressed, in heavy socks and a flannel sports shirt, but his fingers were like ice. He sat for a while, between games, between rubbers, whenever he was dummy, with his hands tucked in his armpits. Then he tried keeping them warm in his trouser pockets. He went back to warming them under his arms. The room got colder. Finally, a little before eleven, the meeting broke up. Berger and his daughter placed second. They were sitting at their last table recalling the more decisive plays of the evening when Mrs. Crews gave a sudden gasp.

  "Daddy!" she said. "Your hands! Look at your hands!"

  Berger looked. He stared. His hands were blue—a gray, slate blue, about the color of a ten-cent postage stamp.

  Mrs. Crews said, "Are you all right?"

  "I don't know," Berger said. He stared at his hands. They looked dead. "I feel a little woozy."

  "I think we'd better go," Mrs. Crews said.

  When they got out to the car, Berger gave Mrs. Crews the keys. He didn't feel like driving. It had just occurred to him that his hands looked like the hands of a corpse. He didn't feel he could trust them on the wheel. They drove in silence. Berger was too worried, too bewildered to talk. Mrs. Crews was trying to think what to do. A fork in the highway loomed ahead. She abruptly made up her mind, and took the turn to the left. Berger sat up.

  "Cher—where are you going?"

  "I'm taking you to Hershey Medical Center," she said. "I'm worried about you, Daddy. I want a doctor to look at your hands."

  The Hershey Medical Center, in the chocolate town of Hershey, is no ordinary hospital. It is the Milton S. Hershey Medical Center of the Pennsylvania State University, and it consists of a college of medicine and a teaching hospital—a three-hundred-and-forty- bed hospital—of the first rank. Just before midnight, Berger, with Mrs. Crews at his side, walked into the emergency room there. He was received by a nurse, who took one look at his hands and called the physician on emergency duty that night. The physician was a first-year resident named Robert D. Gordon. Dr. Gordon and the nurse both stood and stared at Berger's hands. They had never seen a case of cyanosis so chromatically arresting.

  Cyanosis (which takes its name from the Greek kyanos, meaning "blue") is a discoloration of the skin that reflects an insufficient concentration of oxygen in the blood. The presence of oxygen in the protein known as hemoglobin is what gives normal blood its rich red color, and when for any reason the normal oxygen supply is reduced, the color fades and dulls. This phenomenon most commonly manifests itself in the face (especially in the thin skin of the lips) and in the extremities—the toes and fingers. The appearance of cyanosis, if at all pronounced, is a serious sign of warning. It signals either a circulatory problem or some ailment affecting the lungs. The number of diseases in which cyanosis is an early symptom is very considerable. Even those in which the cyanosis involves the hands are numerous. They passed in baleful procession through Dr. Gordon's mind—congestive heart failure, Raynaud's disease, polyarteritis nodosa, Buerger's disease, scleroderma, dermatomyositis, systemic lupus erythematosus, arteriosclerosis, polycythemia, obliterative vascular disease, syringomyelia, congenital heart disease, arteriovenous aneurysm, myxedema, and several forms of poisoning, including the gangrenous agony induced by the ingestion of fungus-infected rye grain, which has been known since medieval times as ergotism. The parade of possibilities halted only once: at the phonetic coincidence of Buerger's disease. It then moved evenly, and uninstructively, on to the end. Dr. Gordon had, of course, expected nothing else. Cyanosis of even the deepest lividity is merely an indication of the presence of disease. It is not—like certain rashes, like certain lesions, like certain neural responses—a definite diagnostic sign. It is a symptom to be explained.

  Dr. Gordon left Mrs. Crews to the hospitality of the emergency- room nurse, and led Berger down the hall and into an examination cubicle. Berger seated himself on the examination table. Dr. Gordon leaned against the wall and guided him through the usual interrogation. Berger's past was uneventful. There was nothing in his medical history (scarlet fever as a child, an appendectomy in 1941, a hemorrhoidectomy in 1961) that cast any light on his present problem. Dr. Gordon brought him up to the present. Berger didn't smoke or drink, but he had that night drunk several —maybe four—cups of coffee. His hands were neither numb nor painful. They were sensitive to the cold. He had no pain of any kind, but that evening he had had a couple of moments of lightheadedness. Dr. Gordon interrupted only once. Berger had mentioned eating rye bread at dinner. Did he often eat rye bread? Yes, he did—quite often. He much preferred it to white. Dr. Gordon mentioned ergotism, and explained it. Berger nodded. As a matter of fact, he said, he had found some mold on a rye-bread sandwich the other day, and had simply scraped it off. That sort of thing didn't bother him. In the Second World War, in the Navy in the South Pacific, he had thought nothing of eating bread full of embedded Oriental beetles. Dr. Gordon reflected, looked again at Berger's hands, and put the thought of ergotism aside. Cyanosis alone was not enough. He moved methodically on. Berger continued fully cooperative. He said he suffered occasionally from headaches. He had been told that he had high blood pressure, but had never done much about it. He had an occasional high-pitched ringing in both ears. He had been afflicted off and on for several months with a dry cough. He had no abdominal pain, no nausea or vomiting, no urinary-tract symptoms. He suffered occasionally from constipation. That, to Dr. Gordon's satisfaction, completed the standard review of systems. He made a perfunctory note: "Tinnitus, cough."

  The standard physical examination came next. Dr. Gordon took a sample of blood and a sample of urine, and sent them off for laboratory analysis. He then proceeded with the examination. He noted down his findings in the standard mode. He found Berger to be "a well-developed, well-nourished white man, in no acute distress, with dusky hands." Berger's blood pressure, in his right arm, was 212/152. His pulse rate was 80, respirations 12, temperature 36.8° C. (or 98.2° F.). There was no evidence of any intradermal, submucosal, or subcutaneous hemorrhaging. There were normal tympanic membranes. Berger's teeth were "in poor repair," but there was no mucosal cyanosis. There was no neck- vein distension, no back tenderness. Lungs were clear to percussion and auscultation. Abdomen was soft and non-tender. There were normal bowel sounds. Genitalia were "within normal limits." Rectal examination showed normal sphincter tone. Prostate was normal. Examination of the extremities "revealed the absence of peripheral edema." There was "no pedal cyanosis, but the patient's hands appeared definitely dusky in color." There was no calf tenderness. Chest X-ray was normal. Electrocardiogram was normal. That completed the general physical examination. Only one finding was of a
ny pathological interest. Berger's blood pressure was elevated. Even allowing for his history of high readings, and for the usual rise in moments of stress and tension, it was high—ominously high. Dr. Gordon added "hypertension" to his diagnostic notes. The source of Berger's trouble was still anything but clear. There was, however, sufficient evidence to consider his admission to the hospital. Dr. Gordon excused himself and went in search of the admitting resident.

  Berger sat huddled on the edge of the examining table, and watched Dr. Gordon go. Hershey Medical Center felt as cold as the Distelfink Restaurant. He tucked his cold blue hands in his armpits. He sat alone and waited. "I was scared," he says. "I mean I was getting really frightened. What the heck was the matter with me? What was going on?"

  The admitting resident on duty that night was a second-year resident named John M. Field. Dr. Field accompanied Dr. Gordon back to Berger's cubicle. He greeted Berger and looked at his hands—his dusky, cyanotic hands. He was uncomfortably struck by the fact that the cyanosis included the nail beds. He talked with Berger for a moment. He reviewed Dr. Gordon's notes and findings. He rechecked Berger's blood pressure, and confirmed Dr. Gordon's reading. Berger was indeed seriously hypertensive. That, however, did nothing to explain his dusky hands. An explanation of that, at this point, could come only from the laboratory. Dr. Gordon reported that the results of the blood and urine studies were on the way. While they waited, he shared with Dr. Field his lingering suspicion of ergotism. Dr. Field was interested in so exotic a thought, then skeptical. He finally shook his head. The results arrived from the laboratory. Berger's urinalysis was normal. So were certain aspects of his blood chemistry. These included the white-cell count, hematocrit, prothrombin time, blood urea nitrogen, calcium, phosphorus, creatinine, and bilirubin. Certain other aspects, however, were not. These were in the category known to hematology as "blood gases." Berger's blood pH was 7.24, or excessively acid. His blood-carbon-dioxide pressure was 38, compared to a normal pressure in the middle thirties. His blood-oxygen pressure was 78, compared to a normal of at least 80. And his blood-oxygen saturation was 92 per cent. Normal is upward of 94.

  Dr. Field reread the blood-gases report. He found it disconcerting. On the basis of the laboratory findings, Berger was suffering from both hypoxemia (low blood oxygen) and acidosis. That would seem to account for his cyanosis. But that would also indicate that he was seriously ill. And he didn't seem to be. Except for his dusky hands (and the hypertension, irrelevant in this connection), he showed no clinical signs of illness. Moreover, and inexplicably, his chest X-ray was normal. "Not only that," Dr. Field says. "There was also a conflict that I didn't understand. I didn't think his high carbon-dioxide pressure was entirely compatible with his low pH. Well, we have a rule here on admissions. It requires that we either admit or discharge after two hours. I didn't do either. I couldn't possibly discharge a man with that degree of cyanosis. But I wasn't quite ready to admit him. I wanted some more information. I decided to keep him where he was for the time being. It was now around two o'clock. I wanted his blood pressure taken every hour, and at six o'clock I was going to run another check on his blood gases. Then I would know. At least, I hoped I would."

  Berger was left alone again. Dr. Field and Dr. Gordon had gone. His daughter had been in to say goodbye. The doctors had advised her to leave; she had talked to her mother; she would be back again in the morning; she knew everything was going to be all right. The nurse appeared and took the first of the hourly blood-pressure readings. Berger tried to make himself comfortable on the narrow examining table. The room was dimly lighted, and cold. He tried not to worry. He tried not to think. "I wanted to leave with Cher," he says. "They told me they couldn't let me. It would be against their better judgment. But they couldn't tell me what was wrong. I had high blood pressure. There was also something wrong with my blood chemistry. They weren't sure what. But I got the impression it was bad news. I dozed off. Then the nurse came in for another blood pressure. I don't think I slept at all after that."

  But, of course, he did. He was asleep, hunched up on the table with his feet hanging over the end, when Dr. Gordon looked in a little after six. Dr. Gordon awakened him gently. He took a sample of arterial blood from Berger's wrist. He gave him a reassuring word or two. He then went along to the laboratory and arranged with the night technician for another blood-gases analysis. The results were ready by seven o'clock. Dr. Gordon and Dr. Field read them together. The second report more than confirmed the first. Berger's blood pH was much the same as before. It was now 7.25, compared to the earlier 7.24. But his blood-carbon-dioxide pressure was up from 38 to 41. His blood-oxygen pressure was down from 78 to 62. And his blood-oxygen saturation was down six points, to 86 per cent.

  Berger lay awake after Dr. Gordon had gone. He and his boss at Gilbert Associates were scheduled to fly to a business meeting in New Orleans tomorrow—today. The nurse came in for the seven- o'clock blood-pressure reading. When she left, he got up and found his way along the hall to the men's room. He straightened his clothes and combed his hair and washed his cold blue hands. "Dr. Gordon came in while I was drying my hands," Berger says. "He watched me for a moment. Then he said he'd like to try something. He took me back to the washbasin and had me hold my right hand under the cold water. And was that water cold! It couldn't have been more than forty degrees. He kept it there for a full two minutes. And then—my God! It was the damnedest thing anybody ever saw. My hand began to change like a damn chameleon. First it was blue. Then it was red. Then it was blue again. There was something about it that really scared me. That's when I knew I was a goner."

  The attending physician on call from the medical service to the emergency room on Friday, January 11, was an assistant professor of medicine named Joseph J. Trautlein. Dr. Trautlein arrived at the medical center that morning at his usual time, a minute or two before eight. He parked, as he always did, in the staff parking lot, and came in through the emergency-room entrance. Dr. Field knew Dr. Trautlein's habits. He was waiting for him in the foyer of the emergency room. He greeted him there and walked with him while he put away his overcoat and got into his long white coat. He told him about Berger.

  "He said he hadn't wanted to bother me during the night," Dr. Trautlein says. "But he had this man with two blue hands and abnormal blood gases and a negative chest X-ray. And high blood pressure. He said he didn't know what the hell was going on. Would I take a look at him? Just a curbside consultation—that's all. Just an opinion. I said O.K.—be glad to. It was entirely appropriate. I was a physician of record. And it sounded interesting. So I went along to the examining room and met Mr. Berger and looked at the resident's notes and talked for a while—and I was just as puzzled as John Field was. That acute onset, those two grossly cyanotic hands, those severely altered blood gases. It was strange. I thought, for one thing, that a lung scan was indicated, and a lung scan is an in-patient procedure. Dr. Field had come to the same conclusion. So it was agreed. I admitted him to my service. I then tactfully withdrew. I was the attending, but this is a teaching hospital here, and our residents are given responsibility. It's an observed responsibility—we're always there within an arm's reach. But it's still responsibility. Mr. Berger was Dr. Field's patient."

  Berger was admitted to the hospital under a tentative diagnosis of hypoxemia and acidosis, and shown to a private room on the fourth floor. After a night on an examining table, he was more than ready for bed. "But first I had some telephone calls to make," he says. "I wanted to talk to Marilyn—my wife. She would have heard by now from Cher, but that wasn't enough. I wanted her to hear the worst from me. I arranged for some clean clothes and toilet articles and all. Then I called Cher and brought her up to date. Then I called my boss, and we cancelled the trip to New Orleans. I can't tell you how hopeless I felt. When I talked to Marilyn, I told her to go out first thing and increase my life insurance. I was joking—but not really. Then I undressed to my shorts and got into bed. At least I had things organized. That made
me feel a little better. And I was warm and comfortable for once. I began to feel hungry. But nothing was said about breakfast. Around nine o'clock, a nurse came in with some apparatus, and the next thing I knew I had a tube up my nose and they were giving me supplementary oxygen. Then a beautiful woman doctor came in and took some more blood samples. I hardly felt a thing. Then I was left alone again, and I knew I wasn't going to get any breakfast. Or any lunch. Or anything. I was too far gone to waste food on."

  By nine o'clock, the news of Berger and his perplexing case had spread through his floor and beyond. Dr. Graham H. Jeffries, the chairman of medicine, heard the news as he stepped out on his regular morning rounds, and he at once added Berger to his itinerary. There were eight physicians in his entourage—Dr. Trautlein and two other staff members, and five house officers, including Dr. Gordon and Dr. Field. The group arrived at Berger's bedside at a little past nine-thirty. Dr. Gordon made the presentation. Berger was asked to display his hands. Dr. Jeffries examined them with interest. He had never seen anything quite like them. They made, however, an unusually challenging teaching exposure. Questions were asked and answered. Opinions were solicited. Ergotism was mentioned, and again discarded. Berger's work as a paint chemist was discussed. It was established that he was exposed almost daily to paint and solvent fumes. The order for a lung scan to determine the presence of a possible obstruction was approved. It was scheduled for one o'clock. There was an evaluation of Berger's blood pressure. It had dropped significantly in recent hours—to 160/100. There was still a degree of hypertension, but it was agreed that his condition could satisfactorily be treated on an out-patient basis. It was also agreed that his hypertension was unrelated to his other, more urgent troubles. Dr. Jeffries endorsed the admitting diagnosis, and the reservations of the two residents about it. He thanked Berger for his patience and cooperation. He and his entourage moved on.