In Praise of Eponyms and the Knowing of Rare Diseases

As expected of a medical student, I had difficulty appreciating that rare diseases are really rarely seen;
sixty years later I continue to look for Osler-Weber-Rendu, pseudoxanthoma elasticum and systemic
amyloidosis in any patient with unexplained gastrointestinal bleeding.  Coughing up blood?  Could be paragonimiasis, perhaps the most common cause of hemoptysis in the world, this lung-fluke is not seen
in the Western hemisphere, but who knows? As was said about the fishing in Dr. Seuss’ McElligot’s Pool,
who knows? 

Not withstanding reality, probability and common sense, I relish looking for zebras, even though I know
the sound of the hoof-beats is most likely a horse. 

One recent morning I was on the hospital’s 4^th floor, in a room given over to countertops and computers,
waiting for the medical students who were to join me for bedside rounds, an internal medicine resident,
who was assigned for a month-long rotation on the gastroenterology service, passed by.  As we exchanged greetings, and as he was leaving the room, apropos of nothing, I said in jest go find me a case of Whipple’s Disease! 

Whipple’s is a rare condition now recognized to be caused by a bacterium Trophyrema whippelii.  This germ rather silently infects and alters many types of tissues brain, joints, heart and gut among others. 
Apparently I was first drawn to Whipple’s as early as 1965, my 3^rd year in medical school for I recently found in my 1962 Fourth Edition of Harrison’s Principles of INTERNAL MEDICINE extensive underlining of the three short paragraphs dedicated to this disease while leaving the neighboring topics untouched.

I was again drawn to this disease in 1970 when W.O. Dobbins 3^rd, of Johns Hopkins College of Medicine, published a series of eighteen patients in the journal Medicine (Baltimore).   This paper was what we now
label a narrative review.  Medicine was then an august and respected journal redolent with and renown for
these Osler-like, pre-Bayesian case series with a review of the literature.  Since the rise of Evidence-based-Medicine, the journal Medicine has slipped into intellectual opprobrium.  But 50 years ago I had read this extensive review of narrative and tables with no illustrations; and I can only suppose that I knew that this
was a waste of my time as this was just a rare disease.  

Parenthetically, Whipple was 1905 graduate of Johns Hopkins who spent a year as a graduate pathologist
in Panama where he described the autopsy findings of a 37 year-old missionary:  “A hitherto undescribed
disease characterized anatomically by fat and fatty acids in the intestinal and mesenteric lymphatic tissue”.
He wrote that he had seen bacilli in the lymph nodes.

With that prologue as background, I had jokingly challenged the resident to go out and find me a case.  

Soon my students joined me and escorted me to the bedside of one of their patients.  As it turned out, a
hospitalist team was making rounds on the patient that the student had chosen for us to visit.  So, I stood
in
the background with the three students as the team members discussed a puzzling case:  a middle-aged,
middle-class white man, who had been ill and disabled by a puzzling polyarthritis, fever, weight loss and
now a stroke. As we listened, the team reviewed the latest: an echocardiogram had shown vegetations on
his mitral valve.  Despite multiple and recent sterile blood cultures, he had endocarditis. The family who
were at the bedside was obviously distraught.

The clinical information washed over me and I blurted out “He’s got Whipple’s Disease!”.  The hospitalist, resident and the intern looked at me with puzzlement and no little skepticism.  But up to the challenge,
they took up their various electronic fact checking devices and rapidly agreed that he matched a classic description of this rare disease.

My heart and my head swollen, with the students I left the room to go to another bedside.

After further investigation, a duodenal biopsy showed “…fat and fatty acids in the intestinal and
mesenteric lymphatic tissue”.  Bacilli were seen and a genetic analysis showed the DNA of T.whippelii 
in both the tissue and in the blood.  After five decades I had found my Whipple’s. 
Most importantly, the patient has a diagnosis and has a treatable disease.

I am still making teaching rounds with students and still following the improbable dream of systemic amyloidosis, pseudoxanthoma elasticum, Osler-Weber-Rendu, paragonimiasis and other rare diseases. 
I continue to follow a commandment “Patients with rare diseases deserve diagnosis too”.

Bacterial Overgrowth

 My Bovine-Like Life with Methane-Making  Gut Germs 

Methane has become very important to me.  In many ways this molecule composed simply of hydrogen and carbon dictates to me my daily life.  It tells me what to eat and how much.  Microrganisms wait patiently in my stomach for me to feed them the carbohydrate they need to produce methane.  The methane then inflates my gut causing bloating and lots of burping.   These methane-producing microorganisms are like Al Qaeda terrorists resting in the hills prepared to strike whenever the opportunity comes.  They are nearly impossible to dislodge from the mountains, hills and valleys of my gut.  I now view them as inscrutable and indomitable.

Bacteria have been known to us since the research of Pasteur and Koch at the turn of the nineteenth century.  However, the importance of another group ofmicro-organisms, the Archaea was unknown until the last few decades.  Indeed, they had been unknown to me during my infectious disease consultation practice in the 1970s & 80s; even though I was ignorant of the Archaea, they were not ignorant of me.

Wikipedia provides this background:  “The word archaea comes from the Ancient Greek ἀρχαῖα, meaning 'ancient things" … For a long time archaea were seen as extremophiles that only exist in extreme habitats such as hot springs and salt lakes. However, by the end of the 20th century, archaea had been identified in non-extreme environments as well. Today they are since the 1970s known to be a large and diverse group of organisms that are widely distributed in nature and are common in all habitats. This new appreciation of the importance and ubiquity of archaea came from using polymerase chain reaction ... This allows the detection and identification of organisms that have not been cultured in the laboratory.”

The microscopic Archaea like bacteria have no nucleus. They have been awarded their own separate domain or kingdom, leading to the recognition three living kingdoms, Archaea, Bacteria and Eukaryocytes.  The Eukaryocytes are us.  The cells of Eukaryocytes have a nucleus and include the life forms we are able see as well as a few micro-organisms.  The microscopic Archaea and Bacteria are known as procaryocytes. They have no nucleus—but enough microbiology.

 Unidentified, and certainly uninvited, they have been living in my gut.  The Archaea and some bacteria are methanogens.  They produce methane by fermentation of ingested carbohydrates and are a large source of human intestinal gas.  In most humans they live mostly in the large intestine, however, in some people they extend from their home territory into the small intestine and the stomach.  Here they have easy and rapid access to the carbohydrates that we are encouraged to eat instead of fats and red meat.

In the upper parts of the gastrointestinal tract the gas these microorganisms make is not easily expelled as it is from the colon and causes distention of the stomach and small bowel.  From the stomach, the gas may be released as eructations—belching.  Clinically this aggregate of symptoms certainly is not unique, but it is consistent with the syndrome of Small Intestinal Bacterial Overgrowth Syndrome.  This is somewhat of a double misnomer since both Bacteria and Archaea, produce the gas, and this may occur not only in the small bowel but also the stomach.  A better name, although unwieldy, is the Gastric and Small Intestinal Bacteria & Archaea Overgrowth Syndrome.

While not free of microrganisms in the natural state, the stomach and upper gut, harbor only transient germs which number in only in the hundreds.  When large numbers invade and conditions allow for them to set up habitation, i.e., to colonize, with their population numbers now in the tens of thousands, the effects of their fermentation products are felt.  

Why in some humans do Bacteria and Archeae colonize the upper reaches of the gut with such a large and gaseous population?  Why does his not happen to everyone?  There are two principle actions of the upper gut that keep the potential hordes of microbes in check:  toxic chemicals and sweeping.  This is similar to the work of a German housewife keeping a clean kitchen floor.  The gut’s chemical effect is created by liters of hydrochloric acid that is normally produced daily.  The wildly active acid in the gastric juices is there for a key purpose, to break down meats and other proteins.  As a by-process the acids dissolve the micro-organisms which we unavoidably swallow as well as the germs that may wander from their home in the distal bowel, the colon.  The chemical effective of the gastric acid is aided by the sweeping action, peristalsis, of the alimentary canal.  Well-coordinated peristaltic waves are highly effective in sweeping along food and along with the food, microbes.  The housewife uses her lye and her broom.  We use acid and peristalsis.

In my stomach and small bowel the methanogens have survived in large enough numbers to produce copious amounts of methane.  Enough to make me not only uncomfortable, but regularly sick and impaired from this sickness.  As lamented Job, “Why Me?”  Well, as with many of the ill-fortuned ill, it was of my own doing.  The pump that pushes hydrochloric acid into the stomach was stopped cold by a so-called protein pump inhibitor.  I have taken this type of medication for 20 years after I was found to have an esophageal ulcer from excess acid leaking up from my stomach.  That is another self-induced disease of which you may read in the story of the “Esophageal Ulcer”.

Twenty and more years passed with reduced acidity in my stomach.  More and more I was bothered with eructations and belly distention, and in particular I was distressed after meals.  Such post-prandial discomfort can be the symptom and the sign of truly pernicious conditions, e.g., cancers of the stomach and pancreas among others.  Worried about these, I worried my physicians, and over recent years I had had series of diagnostic tests:  upper endoscopies, CT scans, abdominal ultrasounds examinations and gall-bladder studies.  All had been normal.

The symptoms worsened and started to interfere with my well-regulated life.  One morning I was unable to help with the case discussions with the house staff.  My belly was distended, I was nauseated and felt weak and generally sick, i.e., malaise.  By chance my gastroenterologist was also teaching that Wednesday morning.  As he left the conference, by way of politeness, he asked how I was doing, and unexpectedly for him, I told him.  He immediately said, “You have small bowel overgrowth.”

He arranged for me to have a breath-test.  I drank a solution of  a nonabsorpable sugar, in this case lactulose, my breath was tested for the presence of hydrogen and methane every 20 minutes for 3 hours.  Normally the breath is free of these gases until the sugar reaches the colonic bacteria and archaea; this is a trip that takes about two or three hours.  Large amounts of hydrogen or of methane in the exhaled breath before 2 hours is evidence of bacterial or archaeal colonization upstream from the colon.  Dramatically, at baseline and thereafter my breath contained a large amount of methane.  A second test gave the similar results.  I had methanogens in my stomach and small instestine.  



We now had evidence that methane is the source of my discomfort.  The task now was to reduce the quantity of methanogens and/or to reduce the carbohydrates from which they make the methane.  The first approach has not been successful.  I have taken multiple courses of multiple antimicrobials which has not abated the bloating and belching.  Concurrently I have altered my diet to reduce fermentable carbohydrates. Meats and fats do not  contain carbohydrates, but steady diet of steak while sounding good, shortly becomes tedious and undesirable.  So goes it, and the struggle goes on.

As an after thought, my stomach is similar to the first stomach of the bovine breeds.  Cows have four stomachs, and in the first two these, the rumen and reticulum, the mucosa of the walls are lined with and the lumen is filled with micro-organisms which ferment the fibrous grasses and hay which are the sustenance of cows.
The fermentation products include hydrogen and methane.  Indeed cows are an important source of green house gases and climate change.

I have been joined in my struggle, or perhaps I have joined a struggle.  For decades patients and clinicians have labored over a vague set of signs and symptoms, a syndrome, which include abdominal bloating with discomfort, diarrhea or constipation and more.  Despite much effort and many blind alleys of investigation, this syndrome, known as the Irritable Bowel Syndrome (IBS), continues to plague patients.  In recent years however, IBS has become linked to SIBO since a portion of IBS patients too will have a positive breath test and do respond to oral antimicrobial agents.  

The bloating and eructations are easily explained by the methane in the stomach, but what accounts for the generalized symptoms of malaise and nausea?  It is now recognized that the gut by way of afferent vagal nerve fibers acts something like a second nervous system.  Within the small bowel mucosa, in addition to the usual mucoal cells there are  enterochromaffin cells that produce and secrete neurotransmitters.  This gastrointestinal brain helps coordinate peristalsis, and when these cells are squished by the distention of the gut they give off large amounts hydroxytryptophan which is converted to serotonin.  Serotonin then acts on reflex centers in the brain stem that cause nausea.

Why was the overgrowth of my gut not recognized earlier?  Firstly the hydrogen-breath test although developed years ago was only performed in certain specialized research laboratories.  Now it is more widely available in hospitals and clinics.  Secondly, my symptoms were not disabling until recently and by that time a breath test could be easily obtained.  Importantly my gastroenterologist is young, and had been trained at an academic center with a strong investigational interest in SIBO. He knew about the condition and he knew how to use the test.
Now, 2018, we have diagnosed the cause of my distress but have yet to find a cure.  I go through life belching and bloated.  I am nauseated and regularly use medical cannabis whenever indicated, and I am the judge of that.  Life could be worse!

Thorough

A Thorough Physician

My nearly ninety year-old mother was failing.  She had always been more active than her age-defined cohort, she had cooked, quilted, hooked rugs and was a star personality in the local Presbyterian Church.  She was proud of herself:  her skills, her know-how and her energy.  Now she had become too tired, too fatigued to enjoy these skills.  

Although in part we could attribute her condition to advancing age, her internist, an excellent physician, took our observations earnestly; he was by all standards an earnest man.  She may have not complained to him, but I did, and years before I had been his Chief of Medicine and he my Chief Medical Resident (CMR). Our CMR’s were chosen from among their peers to assist the teaching faculty.  Nearly all CMR’s have been luminary physicians both on the wards and in the clinic.

The physical examination, and the routine focused laboratory examinations were normal:  no anemia, kidney failure, diabetes nor an occult cancer, nor evidence for polymyalgia rheumatica.  The thyroid stimulating hormone level was within the normal range. After this thorough inquiry and examination, he had no explanation for her failure. He was earnestly puzzled

Months passed and repeated evaluations showed no change.  Then he retired and a new physician took his place.  She as he before him had been one of our CMR’s.  

While assisting with the transition of Mother’s care, I told her of the downhill trajectory.  Before Mom was see in her clinic, her new physician had reviewed the medical records, and she found no new data, but she reinterpreted existing data:  although the thyroid stimulating hormone (TSH) level had always measured within the normal range, in the last years it had fallen from a high normal to a low normal level.  

TSH, which comes from the pituitary, the master gland, is lowered by thyroid hormone (TH) which comes from the thyroid gland, and in normal events they act in feedback to each other to balance the levels, much like a teeter-totter.  

Using a graphing function embedded within our new electronic medical record (EMR), she tracked a dramatic steady linear decline in Mother’s TSH.  Her observation that the TSH had persistently fallen meant that the TH had probably risen during that same time to a higher level. TH levels are not routinely measured unless the TSH is in an abnormal range.   In response to the trend of the falling TSH, she measured the TH and it was high.  Mom’s thyroid was overriding the low TSH.  The thyroid gland had become an autonomous force not responding to the signal from the master gland that enough was enough.  

My mother had hyperthyroidism, and additional testing and consultation with an endocrinologist, a thyroid expert, confirmed this.

Mother’s condition is known as Apathetic Hyperthyroidism.  It is unusual, and when it happens it is almost always in old people.  The usual presentation of hyperthyroidism has to do with the TH’s stimulation of the sympathetic nervous system, best know as for the production and release of epinephrine.  This explains why hyperthyroidism usually presents with the tremors of sweaty hands, sleeplessness, and overactive reflexes.  It resembles a case of continuous performance anxiety.   In old people, however, the sympathetic nervous system has usually, to a great degree, withered away, and epinephrine is not abundantly released.  Old patients therefore don’t present with the usual outward signs of the condition, but with manifestations common of old age:  loss of weight, loss of energy and apathy. 

My mother’s highest praise for a doctor was that they had been thorough.  She seemed to reserve this adjective exclusively for physicians.  Both of her physicians had met her approval as thorough, and they were.  Her second physician just happened to synthesize a fresh look at old data using a new tool, the EMR.  

Near-Death by Peaches, (Well Perhaps)

A sixty-two year-old woman was admitted to the hospital with hypotension, metabolic acidosis and acute renal failure.  She had been in her usual stable but poor health until two days prior to her admission. 

Because of Crohn’s Disease with colitis she had had a proctocolectomy with an iliostomy seven years prior to admission.  This was followed by persistently high output from her iliostomy, a folate-deficiency anemia, hypocalcemia and hypomagnesium.  She had frequently required outpatient saline infusions because of volume depletion, approximately once weekly.   She required oral supplementation of calcium, magnesium and vitamins.  She recently had had an upper endoscopy with duodenal biopsies and both the gross and the microscopic examinations were interpreted as normal.

 For the two days before admission she had had an abrupt increase in the volume of fluid in her iliostomy bag that she described as profuse and watery.   She also was nauseated, had abdominal cramps that were then followed by leg cramps.  She attar

ibuted this illness to having eaten “too many peaches”.

At admission her blood pressure was 99/64 and the pulse 126.  Her neck veins were not detectable when she was supine, and she was thought to be volume depleted.  The sodium was 136; potassium 3.6; chloride 106; and the bicarbonate 16. The creatinine was 5.2 which  four months earlier had been 2.3 mg/dl.  The anion gap was calculated to be 4.  The serum albumin was 3.1gm/dl.  The venous lactate was mildly elevated at 2.3 mol/L.

She was given large amounts of saline, calcium and magnesium and her vital signs normalized as did her creatinine.  The iliostomy effluent returned to her normal 3 bags per day. 

She wondered if the peaches had been tainted, but others in her household who also ate the fruit disagreed and no one else had had diarrhea, nor did the peaches have an abnormal odor, taste or consistency. 

More likely, the peaches did cause her illness, and this was most likely due to their high sorbitol content.  The average peach contains 3 grams of sorbitol, and she ate four, therefore, she ingested ~12 grams of sorbitol. As a laxative sorbitol is usually given in a 70% solution or 70 grams in 100ml of water, and the usual dose is 30 to 150ml or 21 to 105 grams.  

The sorbitol packed into the four peaches that she ate cannot alone explain her acute illness.  It seemed obvious that something else must be going on.  I suspect that after her colectomy she has developed chronic small intestinal bacterial overgrowth (SIBO). 

People with SIBO have excess production of fermentation products, gases such as hydrogen and methane, and are bothered by gut distention, flatulence, belching and diarrhea.  There is some evidence that the distended small bowel releases cytokines and other vasoactive molecules and that these cause extra intestinal symptoms such as nausea, anorexia, and vague neurologic symptoms often referred to as “brain fog”.

Avoiding easily fermentable carbohydrates ameliorates these symptoms.  These are referred to by the acronym FODMAP for Fermentable Oligosaccharides, Disaccharides, Monosaccharide’s and Polyols.  Sorbitol is a potently fermentable disaccharide and is found in among other foods the so-called stone fruits such as peaches and these are forbidden for people with SIBO.  I believe that this patient has undiagnosed SIBO and that the four peaches abruptly in creased the level of fermentation products in her small bowel.

As it turns out, Crohn’s Disease is regularly associated small bowel bacterial overgrowth and should be considered and a breath test obtained. *  I can find no reliable data about SIBO in patients who have had a colectomy.

*Jochen Klaus,Ulrike Spaniol, Guido Adler, Richard A Mason, Max Reinshagen and Christian von Tirpitz:

Small intestinal bacterial overgrowth mimicking acute flare as a pitfall in patients with Crohn's Disease

BMC Gastroenterology 2009; 9:61

This is a case from the notes that I keep from my Chief Service Rounds.  For these last four years I had been content with the catchy title “Near Death from Peaches” and I did not look into the actual sorbitol content of peaches.  The patient has disappeared from our clinics, and now I need to find her, and to see if my hypothesis can be tested with a breath test looking for excess hydrogen and/or methane after she drinks a solution of a standard dose of a fermentable carbohydrate such as lactulose. 

Brisket Disease

Shortly after moving to the scenic mountain forests of western Arizona, near the border with New Mexico, a friend of mine, a middle-aged woman in previous good health, began to experience progressively worsening shortness of breath with exertion. She had been vigorously active helping her partner tend acreage, and she was unable to keep up this effort.  The altitude of this land is over 7,000 feet above sea level.

She was evaluated by her family physician:  a thorough physical exam, a chest x-ray, and ECG and screening laboratory tests were normal.  He found no cause for her dyspnea.

Her symptoms worsened, and she was referred for pulmonary function tests that were interpreted as normal.  The arterial blood gases were normal for the altitude.

She saw a cardiologist who performed an echocardiogram.  The valves were normal; the ejection fraction was greater than 60%.  There was mild pulmonary hypertension.  She was considered to have become a victim of her own imagination.

I talked to her partner, and I reviewed her medical records, and I suggested that she might have high-altitude pulmonary hypertension, and I recommended that they should try living at a lower altitude.  This idea was resisted until winter when they vacationed in Florida:  after a few days at sea level her dyspnea greatly diminished and then disappeared; it returned when they returned to the mountains.

Her pulmonary artery pressures were never measured, but based on her medical history, I felt, more likely than not, that she had high altitude induced pulmonary hypertension.

It turns out that some humans are more susceptible to hypoxic pulmonary hypertension than is the general population; perhaps she was one of these individuals.

Susceptibility to constriction of the pulmonary arteries when exposed to reduced levels of oxygen is modeled by a dramatically instructive animal condition known as Brisket Disease of Cattle. 

Cattle ranchers with herds in the Rocky Mountains are well acquainted with Brisket Disease of Cattle. As their herds are moved from lower winter pastures that are less than five thousand feet to the high altitude pastures above 7,000 feet in the spring and summer. There, some of the herd, as many as twenty per-cent, develop high altitude pulmonary hypertension and as a consequence failure of the right side of the heart. 

In cattle the congested state caused by right heart failure does not cause swelling of the ankles and legs, known in humans by the archaic term dropsy, instead the fluid gravitates to their most dependent part, the brisket.

Beefeaters recognize the brisket as that cut of meet from the chest muscles, the pectoralis major and minor.  The brisket is the preferred cut for barbecued beef and is used as well for corned-beef and pastrami.  

          

Above, The Bovine Brisket

Below, a Bovine with Brisket Disease

                             

Colorado ranchers take brisket disease seriously because of its grave economic consequences, and in the spring a veterinarian goes up to the high altitude pastures with the herd.   The vet then does a right heart catheterization on each of the bovines, and if the pulmonary artery pressure is at all elevated, these animals are returned to lowland pastures and feed.

Well, my friend never developed overt right heart failure with dropsy, and she was never interested in getting a right-heart catheterization, but the ameliorization of her dyspnea when she lived at sea level, convinced her to avoid the mountains, and they moved to Iowa altitude 1,503 feet above the sea level. 

Recently, while on vacation from their new home they returned to visit old friends in the mountains.  In a few days her dyspnea returned, and they promptly returned to Iowa.  Her dyspnea again disappeared.

Premature End of Life Care

I had returned to the Hospital following my longest-ever vacation, four weeks in Portugal and Southern Spain and was renewing my “management by walking around” skills when I dropped by our Medical Intensive Care Unit (MICU).  There was a buzz in the air, a friend and colleague of mine had just signed-off as the physician of an 87 year-old woman who was intubated and had been on a ventilator for several weeks. 

This was a transitional time in the history of critical-care medicine.  We were moving from open to closed units.  At that time general internists were allowed to admit and care for their own patients with or without consultation with a pulmonologist or cardiologist. Inpatient medicine has evolved, and most MICU’s are now closed, and critical care specialists manage nearly all MICU patients. 

 The day of my visit was during the good-old days of the open unit.  My colleague caring for this elderly woman was an excellent general internist and a truly humanistic individual.  He had been confronted with a seemingly insoluble end-of-life crisis.  He, along with concerned medical and nursing clinicians, believed that her endotracheal tube should be removed so that she could die.  She was unresponsive and a feeding tube, intravenous lines and a bladder catheter were also supporting her.

When I learned that she had no physician, I volunteered to take over her care.  In my formal medical training in the 1960’s there were no critical care units.  Unstable patients were cared for in the usual four-bed rooms.  Curtains separated the beds, the respirators were primitive, but the nurses were the best, although not formally trained in critical care.

Standards have evolved over the last quarter-century:  the physical units changed and now patients have private rooms; there is one-on-one care by specialty trained and certified nurses; the physicians are Board Certified in critical care; there are clinical pharmacists who help manage complex regimens of medications; and respiratory therapists are on site. 

Importantly, healthcare ethics has evolved along with critical care, and the clinicians are familiar with ethical principles.  If necessary, expert consultation is available.  Families are encouraged to come to the bedside and to talk with the nurses and physicians. When I volunteered to help that day, a quarter of a century ago, critical care had impressively evolved, but most certainly not as far as today.

This woman posed an ethical dilemma.  The hospital staff agreed that the current care that was sustaining her life was futile.  Her three children disagreed and wished to continue the intubation, ventilation, nutrition and hydration.  The major family decision-maker was a physician daughter, an academic pediatrician, who lived an inconvenient distance from Portland.  The son was a postal worker and the other daughter was the caregiver for the mother.  Their home was in a pleasant middle-class neighborhood.  End of life decisions had been appropriately delegated to her family, as she had been unresponsive since her admission for pneumonia weeks earlier. 

That was the stage set as I opened the sliding glass door to her room.  At her bedside I confirmed her physical exam, and then I attempted to arouse her:  “Mrs. X wake up…open your eyes”.  There was no response.  I then took a hearing assistance device that resembles an old-fashioned hearing aid from the pocket of my starched white coat, placed the buds in her ears, hooked up the leads and turned it on. 

Again I asked, “open your eyes Mrs. X”, and this time she did.  She looked directly up at me, and I asked her what she wanted.  She bent her right elbow and put her hand to the endotracheal tube and motioned it forward.  It was obvious that she wanted the tube removed.  After consultation with the nursing staff, all agreed that she clearly had the capacity to make decisions for her self.  We did as she directed. 

After we removed the tube she was certifiably awake.  She went home and lived for many months with the care of her daughter and son.

Deafness is a major problem in the care of the elderly.  I have helped manage more than one patient who had been thought demented but instead were extremely hard of hearing, and who, like our seemingly comatose patient, had simply tuned-out of life. 

What Did You Do? Additional "Interesting" Patients

Each Tuesday morning I am honored to talk with patients at their bedside.  I am advantaged to see them after they have been stabilized and are thinking more clearly with less anxiety than immediately after the crises of their admission, and I am not obligated to take a complete history or do a complete exam; I may ask them non-urgent and seemingly irrelevant questions.  My go-to query is to ask what they did for a living, and if a work history seems unlikely, to ask them what they have done with their life.
Nearly all of the patients I see were born shortly before or during the post World War baby boom and are now in their seventies, most often they are retired.  Younger people on our wards often suffer from serious mental illness that has led them to practice the serious use of tobacco, alcohol or more dangerous street drugs.  
Over the years I have made brief note of a few of their memorable answers:

From a playful 70 year-old man, “I made weapons of mass destruction.”  Parenthetically this response came during the half truths about “weapons of mass destruction” formulated and spread by our Nation’s deadly duo, Cheney and Rumsfeld, concerning the nature and the threat of Sadem Hussein’s arsenal.  The patient had worked in Albany, Oregon, at the Wah Chang plant that isolated purified zirconium from beach sand.  Zirconium is used to clad the reactors for our nuclear submarines, indeed a weapon of mass destruction.

A wrinkled eighty-three year-old woman, who had been admitted to the hospital to help with her symptoms of tobacco-related lung disease, was proud that she owned and operated a honky-tonk bar on the outskirts of St Helens, a community that, with tongue in cheek, I refer to as the Appalachia of Oregon.  There on a daily basis she tends the bar. 

An eighty-two year-old Japanese-American woman who remembered that as a teenager she had walked from her family’s village into the rubble of bombed-out Nagasaki two weeks after it had been the target of our second atomic attack.  She apparently had suffered no ill effects.

An eighty-two year-old woman was admitted for acute pyelonephritis and proudly reported that she had smoked marijuana since the mid-1960’s when she had also raised enough for her children.

A frail elderly Jew who had been imprisoned for nine years in a Siberian gulag, ironically he had been arrested almost immediately after the Russians had liberated him from a Nazi concentration camp.

These are stories from the people, the patients, which have strengthened my advocacy for teaching at the bedside.  These stories have allowed the house staff, students and me to recognize additional dimensions of the humanity of those for whom they care.