Fascinomas- Fascinating Medical Mysteries

by Clifton K Meador

  Dewayne had worked as an Emergency Medical Technician (EMT) for the county for more than five years. Before that he worked as a nurse practitioner in a city hospital emergency room. He thought he had seen it all.

  A month after moving into the log cabin, Dewayne woke with a severe headache and fever. His temperature was 104 degrees. He hadn’t felt well for two days with symptoms he thought were the flu, but this was clearly something else. He told Edwena they needed to get to the emergency room and she needed to drive.

  When Dr. Paul Barnett saw Dewayne in the ER, he immediately recognized signs of a serious, even life-threatening infection. Dewayne was “sick out the eyes,” a poorly defined term that pediatricians used to describe very sick children. Experienced physicians knew it when they saw it.

  There are very few infections that can flatten a healthy person in a few hours. Meningitis would be high on the list. In addition to the severe headache and fever, Dewayne complained of abdominal pain, aching joints and nausea. Soon after he arrived in the ER, he vomited several times.

  The physical exam was normal except for the fever and a faint pink rash appearing on the skin of his chest and abdomen. There was no rash on the arms or legs. Barnett thought Dewayne’s neck was a bit stiff, enough so to order a spinal tap to check the spinal fluid for meningitis. Without waiting for any lab test results, he started intravenous antibiotics to cover any known infectious organism.

  Just after the spinal tap was done, Edwena came into the exam room.

  “Dewayne,” she said, “did you tell Dr. Barnett about the rats?”

  Her sick husband could barely shake his head, “No,” he muttered. “You tell him.”

  Edwena began telling Dr. Barnett a long story that seemed to be going nowhere. One evening at dusk, she said, she was sweeping the front porch of the cabin when she saw what she thought was a limb from a tree. She tried to sweep it off the porch when it started to move. She screamed for Dewayne, and then remembered he wasn’t home yet from his shift on the emergency crew.

  Dewayne interrupted Edwena. “He doesn’t need the whole background. Just the facts.”

  Edwena continued her story. “When the thing started to move, I knew it was a snake. So I ran to the woodpile and grabbed a big ol’ axe. I took it back to the front porch and began choppin’ that snake into as many pieces as I could, as fast as I could.”

  The term “overkill” came to mind as Barnett listened to Edwena’s description of how many times she hit that snake. But each time she would lop off a piece, it would squirm a bit. So she kept chopping, determined to be sure that snake — all of it — was dead.

  “When Dewayne got home that night, I thought he would be proud of my being so brave,” Edwena said. “But he fussed at me! He said it was a rat snake which eats rats, and I shouldn’t have killed it. He said it was helping us around that old country cabin. Well, dang it, how was I to know? A snake is a snake.”

  Sure enough, over the next few weeks the place was overrun with rats. In any given day, they would see at least a half dozen of the nasty rodents running out of the closets, behind the furniture and into the kitchen.

  “When Dewayne got sick this morning, he told me it was because of all those rats that come from my killing that snake,” she said. “He thinks he’s got rat fever, whatever that is.”

  Dr. Barnett told them he also thought Dewayne had “rat fever,” called murine or endemic typhus. He had begun doxyclycline, the antibiotic needed to treat murine typhus. He also used several other antibiotics to cover other possible infections. The spinal fluid was normal, ruling out meningitis. They would have to wait on the test results to know exactly what infection Dewayne had.

  Murine typhus is transmitted by the fleas of rats, most frequently in the summer and fall. The infectious organism is classified as a rickettsia. It is difficult to culture, so the diagnosis is confirmed by finding antibodies to the rickettsia in the blood. In this case the suspected organism was rickettsia typhi. The infection can be lethal if not treated.

  Several days later, Dewayne’s serological test for rickettsia typhi showed a high level of antibodies. The diagnosis of murine typhus was confirmed.

  Epidemic typhus, the one responsible for lethal epidemics throughout history in Europe, is transmitted by body lice from person to person. That organism is rickettsia prowasekii. Mortality of epidemic typhus can reach over 50 percent.

  The state health department sent a team out to the log cabin and found rickettsia typhi in the feces of the rats. Dewayne had intuitively known what made him sick. The health department instigated a fumigation of the cabin to kill the fleas and put out poisons to kill the rats.

  In a few days, Dewayne was much better and ready to go home. He would continue the oral antibiotic for another 10 days. Dr. Barnett wrote out the prescriptions and handed them to Edwena. He asked her to read one of them out loud to her husband:

  “No. 1,” she began, “Get one black rat snake, two if possible.

  “No. 2: Put the snakes under your house.

  “No. 3,” she paused with a big grin, “Do not kill the snakes.”

  *Case shared by:

  Paul Barnett, M.D.

  Associate Clinical Professor of Medicine

  Department of Medicine

  Vanderbilt University School of Medicine.

  Chapter Six

  A Lag in Medical Knowledge.*

  Marilyn Potter was rushed to the ER of the local hospital by her fiancé, Boyd Sutterfield. Marilyn had just swallowed all of the pills she had on her bedside table, telling Boyd she hated him and wished to die. She had just celebrated her 29th birthday. The year was early 1977.

  In the ER, the team of nurses and Dr. Alex Witherspoon hearing of the suicide attempt rushed to quickly begin IV fluids. They then aspirated and irrigated Marilyn’s stomach with saline solutions. Marilyn’s blood pressure and pulse were normal. Her breathing appeared normal, as did her blood gases.

  Dr. Witherspoon was perplexed by the array of pill bottles brought in with Marilyn. There were all sorts of medications listed on the labels of the empty bottles, including birth control pills, multivitamins, an antihistamine and capsules of vitamin A, vitamin C and Tylenol. What puzzled Witherspoon was the fact none of these pills were thought to be lethal at the time, not even taken all at once as Marilyn claimed she’d done.

  Witherspoon began questioning Marilyn about her intent and her state of mind. After several minutes, she began to sob uncontrollably. She told Witherspoon that she wanted Boyd’s attention and the only way she knew to get it was to fake suicide. She was careful to use only the pills she thought were safe. She really did not intend to kill herself.

  Being extra cautious, the doctor decided to hold Marilyn overnight for observation of her physical and mental states. It was a good call because by the next morning, screening tests showed her liver function was markedly abnormal. Not knowing the cause for this troubling change and concerned for her rapid clinical deterioration, Witherspoon transferred Marilyn to the University Hospital for further evaluation.

  By the time of transfer, Marilyn was becoming severely nauseated and vomiting all oral intake. She also was noticeably jaundiced and exhibiting signs of severe liver damage.

  The University Hospital had just instituted a clinical pharmacy program. Pharmacists with special training in clinical pharmacology and therapeutics were just beginning to be hired as staff to the larger U.S. hospitals. These new, highly trained pharmacists were now receiving Doctor of Pharmacy (PharmD) degrees. The University Hospital had just engaged Dr. Al Roach as a consulting Clinical Pharmacist. Roach made himself available for any medication or drug problem, be it for information on dosages, interval of administration, potential toxicity, indication, interaction with other drugs, contraindications and other vital information. Soon medical staffs knew to call the clinical pharmacist for any and all medication issues. This new breed of pharmacists changed and improved the face of in-hospital drug administration.

 
Dr. Roach was called to see Marilyn Potter on her arrival at the University Hospital. The unanswered questions were: If all the drugs she took were not toxic, what was the cause for her deterioration in liver function? Was this due to some combination of the drugs, or was this the result of some drug she hadn’t told them she had taken? Most importantly, what could be done to protect or reverse her now failing liver?

  Roach, on speaking with Marilyn and her family, determined that the largest dosage of any drug she took was Tylenol in its generic form acetaminophen. A careful search of the usual North American literature revealed no reported toxicity of acetaminophen. Not satisfied, Roach began searching foreign and European literature. It was here he uncovered a drug named paracetamol in the United Kingdom. He quickly discovered it was another generic name for acetaminophen and that the two drugs were identical. What’s more, the incidence of liver toxicity for paracetamol was well documented in the European literature.

  Now knowing the source of liver damage, Roach found and obtained the known antidote for paracetamol: acetylcysteine whose trade name is Mucomyst. Acetylcysteine acts as a precursor to glutathione, thus helping the body regenerate enough glutathione to prevent further liver damage from the metabolites of acetaminophen (Tylenol).

  There are two lessons here: Because none of the existing medical specialties could track all drugs, advanced clinical pharmacists as a new specialty with PharmD degrees provided a necessary — and sometimes critical — piece of the puzzle as newer and more complex drugs were being introduced. Their contribution has greatly enhanced in-hospital care. The perfect example is Marilyn Potter, who may not have survived the liver damage without acetylcysteine discovered by such a pharmacist.

  The second lesson, however, lies in the fact that this particular pharmacist wasn’t satisfied by his findings in the North American Literature regarding the absence of reported toxicity. Where others might have stopped at this authoritative source, he kept digging. That tenacity most likely saved the patient’s life.

  Case shared by:

  Al Roach, PharmD, FACG

  Medical Scientist Director

  Ironwood Pharmaceuticals

  Nashville, Tennessee

  Chapter Seven

  A Dose by Any Other Color...*

  Dr. Alan Siegal was finishing his check-out sessions reviewing charts with the medical residents, when Dr. Maxwell Fisher asked him to see one of the clinic patients. Fisher was a second-year resident headed toward a practice of general medicine. Walking down the hall toward the exam room, Fisher quickly briefed Siegal on the case of Jonas Stillman.

  Stillman was a 28-year-old mechanic back for a follow-up visit. Dr. Fisher had seen Stillman a month ago and made a diagnosis of hypothyroidism. The patient then had cold intolerance, very dry skin, puffy eyes and poor exercise tolerance. The blood tests had confirmed very low thyroid levels, so Dr. Fisher began Stillman on thyroid replacement therapy with Synthroid , a synthetic thyroid hormone. In the month since Fisher had seen Stillman, all of the symptoms of low thyroid hormone had disappeared. Fisher however said Stillman had a new and unexplained symptom.

  When the doctors entered the small exam room, Stillman was sitting on the exam table pulling a towel back and forth across his shoulders. “This itch is killing me,” Stillman said. “It’s all over, like insects. It won’t go away even when I scratch it.”

  Dr. Siegal examined carefully Stillman`s skin and found no lesions or hives. The itching had no visible cause. The patient went on to say how much better he felt on the thyroid replacement generally and he would be well if the itching would quit. “I started to itch about a week after I last saw you,” Stillman told Dr. Fisher.

  Siegal asked the young resident if he had any idea what might be causing the itch. Fisher said he had no clue.

  “What dose of thyroid replacement did you give?” Dr. Siegal continued.

  “He is on Synthroid 0.1 milligram daily.” Dr. Fisher said. Synthroid is commonly prescribed for hypothyroidism. The dose was about average for replacement.

  “Stop the Synthroid 0.1 milligram tablets. Change him to Synthroid 0.05 milligram tablets, two a day,” the senior physician said.

  Fisher now looked completely puzzled. “But two 0.05 milligram tablets is 0.1 milligram. That’s the same dose he’s on now.”

  “I know. Trust me,” Dr. Siegal said. “I believe Mr. Stillman’s itching will go way.”

  Fisher wrote a short note in the chart, shaking his head in disbelief. He had no idea what Dr. Siegal was thinking.

  When Jonas Stillman returned to see Dr. Fisher a few weeks later, he was a happy man. “Itching went away just like Dr. Siegal said it would.”

  Fisher was delighted, but still confounded as to how two pills instead of one made a difference, given the same dosage. He rushed to find Dr. Siegal.

  Dr. Siegal also smiled at the news and began to explain how he pinpointed the source of the itching.

  “I knew 0.1 milligram Synthroid was yellow and I know that the yellow dye in the tablet is Tartrazine,” he said. “Tartrazine is also a food coloring with a documented history of occasional allergic reactions. Mustard gets its yellow color from the dye. I recalled another case where Synthroid 0.1 milligram had also caused itching, relieved by using two 0.05 milligram tablets that don’t contain the yellow dye. The 0.05 mgm tablets are white.”

  Dr. Fisher was amazed. “But why don’t people get itching from eating mustard?” he asked.

  “I suspect they would if they ate it every day and they were susceptible to tartrazine itch, whatever that is. Taking the yellow Synthroid every day sets up whatever tratrazine dye causes. I don’t know the precise allergenic cause for the itch, but it is uncommon.

  “In 30 years, this is only the second case I have seen,” Dr. Siegel said, “and it’s your first, Dr. Fisher. But I bet you will not miss another Synthroid 0.1 tablet itch if you ever see another one.”

  *Case shared by

  Dr. Alan Siegal, M.D., M.A.C.P.

  Faculty, Medical Education

  Baptist Health Systems

  Birmingham, Alabama

  Chapter Eight

  First Diagnosis of Death*

  It was my first night on call as a medical intern at Columbia Presbyterian Hospital. I was assigned to cover the private area known as Harkness Pavilion. The year was 1955.

  Harkness Pavilion was the hospital for the rich and famous of Manhattan and, I suppose, for much of the world at that time. Madam Chiang Kai-Shek, for example, took over an entire floor when she came for her annual checkup. During the year of my internship, the private physicians admitted Elizabeth Taylor, Edward R. Murrow, Rex Harrison and many other celebrities from Broadway, Hollywood and the world of politics. It was one of the main U.S. hospitals for the royal families of Saudi Arabia.

  I had lived all my life in the South, went to college and medical school there. New York was a strange territory for me. Southerners were still looked down on as stupid and uneducated. The prejudice was at times palpable. I was a stranger in a very strange land.

  Around 2 a.m., I was called to pronounce a patient dead. I jumped out of bed. We slept in our white uniforms, so we were always ready for calls.

  After finding my way to the 5th floor, I met the nurse in the dimly lit hallway. She was very official, stern and appeared to be older than my grandmother. Wearing a long white dress with a blue apron and large white nurse’s cap, she was from another century. She looked like I imagined Florence Nightingale might have looked in her later years.

  She peered over her glasses as she spoke to me. “Mr. Williams in room 553 quit breathing at 1:54 AM. We need you to pronounce him.”1*

  This being my first official patient, my overriding fear was that I would make a diagnosis of death on a man who was still alive. In some Edgar Allen Poe-like ghoulish scene, I could imagine the poor man waking in horror in the morgue, calling out to some attendant, who would immediately call the chief resident, who would then call the Chairman of Medicine
. I shuddered at the thought of being called into his office to be told I that I could not tell a live person from a dead one. I would be banned forever and on my first night as a real doctor.

  I had only one thing on my mind when I walked into Mr. William’s room: I would not make an overdiagnosis of death. I would make sure he was dead before I said he was dead. I had seen dead patients in medical school, but I had never had to say they were dead. My obsession with making an accurate diagnosis I am sure buried my deeper emotions of facing death. I displaced the thought that here was a person who only a few moments before was an alive human being.

  I looked at the chest for several moments. No movement. Then I listened to his heart with my stethoscope for several minutes and at each valve area. No sounds. Then I felt for a pulse at his wrist, then his neck, then for a femoral pulse at his groin. Nothing. No pulse. I actually checked for rigor mortis, hoping to find it, but his joints were quite limber.

  Then I remembered reading that after death, the arteries in the back of the eye change dramatically. I asked for an ophthalmoscope. While the nurse went for the scope, I went back over each pulse area, listened some more to the heart and even put my ear close to his nostrils, listening for any movement of air. I was becoming more and more confident that Mr. Williams was dead. More confident but still not absolutely sure. No half-dead person was going to get by me and wake up in the morgue.

  I looked carefully through the ophthalmoscope and there they were – the small arteries lacing across the retina at the back of the eye with the telltale sign. The red cells had separated from the plasma in alternating dark and clear segments called “boxcars.” I knew with certainty that Mr. Williams was dead. His blood had begun to separate.