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Three Dutchmen Walked Into an Eye Clinic

Published September 22, 2016 Medical History 4 Comments

Three Dutchmen Walked Into an Eye Clinic and the Rest is History.

As a severe myopic, it is no wonder I have always had a certain interest in ophthalmology. And just the other day I had reason to ponder the peculiar Dutch dominance in the history of optics and ophthalmology.

When I was a nearsighted young school boy in Sweden, my mother brought me on the bus into town every fall to see the eye doctor. He must have been in his eighties, a tall man with a bow tie and a long white lab coat. His office was adjacent to his apartment in a white stucco building from the early 1900’s. It was a dimly lit space with dark, angular furniture. The doctor said very little as he made me read the letters on the Snellen eye chart while placing varying lenses in front of each of my eyes in an antique looking device, and while he peered into my eyes while holding a thick magnifying lens that focused a piercing light into my tearing eyes one by one. I could smell his skin and his hair as he leaned into me.

After each of my annual exams, he always sighed and wrote out a stronger eyeglass prescription with a old black fountain pen. He carefully blotted the prescription paper and always said to my mother “don’t let him read too much in bed”.

As my glasses got stronger, I became aware that if I looked at road signs or traffic lights out of the corner of my eye, the colors didn’t line up. The red outer circle of the Swedish no-parking signs would overlap one end of the inner blue circle and there would be a space between the two colors on the opposite side. In the same way, the red, yellow and green traffic lights wouldn’t be straight on top of each other, but at an angle. I learned in school that red light passes straighter than blue or green light through a prism, like the outer edges of my old-fashioned glass lenses.

As I approached my teens, working with an old viewfinder camera and black and white darkroom equipment, I understood why it was harder to read in dim light: a dilated pupil, just like a wide aperture, creates a shallower depth of field than a smaller one, and the ultimate small aperture, a pinhole, can replace the lens in a simple camera or even your high powered eyeglasses in a pinch.

In medical school I learned to do a neurologic exam, and the bedside test for visual fields – Donders’ confrontation, as we called it. I figured Donders was a Dutch name, but never gave it much thought.

The other night, wondering why my EMR incorrectly defines visual acuity by “Snelling” rather than Snellen, it struck me that Snellen was probably a Dutch name, just like Donders. A few minutes with my iPad and Dr. Google made me rediscover how much I enjoy medical history.

It turns out Donders built an eye clinic and hired Snellen to run it. They invited their friend Einthoven, who would later invent the EKG, to help in their research. Einthoven studied chromosteropsis, the phenomenon whereby red objects seem closer than blue objects. Donders, Snellen and their wives were the subjects, and Einthoven’s paper became his doctoral thesis. It seems that chromosteropsis has something to do with the fact that red light travels straighter and that our eyeballs are angled inward when we look at objects up close, which makes blue objects seem ever so slightly blurry.

So, anyway, my little exploration reaffirmed that if I ever cut back my clinic hours, I’ll read more about the history of medicine.

The Legend of the Avoidable Hospital Readmission

Published October 3, 2015 Medical History 1 Comment

A long, long time ago, hospitals existed to admit patients when they were sick, treat them with medicines or surgery and good nursing care, and discharge them after they became well.

Hospital care was at one time a charity, which evolved into a nonprofit service, before it became a Very Big Business.

In olden days, nonprofit hospitals charged patients straightforward fees for their services. Then, when you were just a young whippersnapper or perhaps merely a gleam in your father’s eyes, Medicare and Big Insurance started collecting premiums from workers and dole it out to hospitals when the workers or retirees needed hospital care.

At that point, hospital fees became confusing. The people who received care didn’t see what the charges were, and the payers didn’t really know how much care was medically necessary or even actually delivered by the increasingly profit-driven hospitals, let alone how much it cost to provide those services.

Insurers demanded deep discounts, and hospitals raised charges. Billing became more and more convoluted and required more hospital documentation and more business staff at both the hospitals and the insurers.

When an aspirin became as expensive as a four course meal and an overnight hospital stay became more expensive than the monthly lease payment for a Bentley, Medicare thought they had figured out a way to outsmart the hospitals: They started paying a flat rate for each hospital stay, based on the diagnosis. Suddenly, the hospitals were penalized if patients stayed longer or required more procedures or more aspirins than the average case.

That’s when patients no longer got to stay until they were well. People were discharged home at five o’clock on Friday afternoons, only partway cleared of their symptoms, with promises of a visiting nurse the following week and instructions to call their family doctor first thing Monday morning for an appointment.

A few years went by, and Medicare realized patients often ended up back in the hospital shortly after their discharge. Hospitals, of course, got to bill twice for each such episode and Medicare was obligated to pay the hospitals twice – not what they had expected would happen.

Medicare’s next move came swiftly: They didn’t retreat and say “we were wrong, keep patients in the hospital until they are well enough to go home”. Instead, they announced they would penalize hospitals if patients with certain hot button diagnoses got readmitted within thirty days of discharge.

This was an ingenious move on Medicare’s part. They are now imposing this penalty not just for patients who were sent home before they were stable, but also for patients who have severe chronic or near-terminal illnesses. For these patients, even the best possible prognosis is multiple admissions or a lengthy stay until they die. Medicare is now forcing the hospitals to spend more money than they receive during each such hospitalization, and, through the penalties, Medicare is giving itself a rebate every time one of these chronically ill patients gets readmitted appropriately, weeks after any shortcomings in the initial care would have been compensated for by the follow-up care or the passage of time.

Today, Medicare is looking outside the hospital wards for a happy ending to this situation. They are starting to spend money (presumably the money they are taking away from the hospitals) paying primary care practices for reaching out to patients immediately after they come home from the hospital in order to identify gaps in care and plan for follow-up visits. We are now becoming more and more involved with the social and economic barriers to health.

So the legend continues to evolve. But, like all legends, it is only partly true: Hospital care doesn’t cure everyone or everything. Primary care practices and their new partners – Community Care Teams and all the other agencies they network with – can only do so much to help patients overcome the obstacles that our society as a whole cannot remedy. And as primary care practices shoulder more and more chronic disease management responsibilities, even with some extra money thrown in, will we be able to also provide the timely urgent medical care our patients need in order to stay out of the Emergency Department and the hospital?

Children Who Never Grew

Published January 6, 2014 Medical History , Progress Notes 3 Comments

I have two patients with phenylketonuria. Both are about my age. Laura, a non-verbal, slender woman with weathered features but the mind of a very young child, lives in the community. Her sister, Regina, has lived all her life in a nursing home. She doesn’t have a wrinkle in her face, and seems mostly unaware of her surroundings.

The two girls were born several years before Dr. Robert Guthrie developed the blood test for phenylketonuria, and a decade before routine PKU screening was introduced in this country. I often wonder what the parents of these two girls knew about their condition, where they went for a diagnosis, and if they even got one while Laura and Regina were still young. In many cases back then, PKU went undiagnosed as the specific cause of mental retardation.

Pulitzer and Nobel Prize winning author Pearl S. Buck gave birth to a daughter, Carol, in 1921. Carol did not develop normally, and on the advice of her Chinese doctors, Pearl Buck traveled to the Mayo Clinic to have her evaluated. She left the clinic and the United States without a diagnosis, except “I don’t know. Somewhere along the way, before birth or after, growth stopped”.

Pearl Buck cared for Carol at home until age nine. At that point she returned to America. She wrote “The Good Earth”, her book about her experiences in China, in 1931 with the hope of making enough money to support her daughter, who was institutionalized around that time. In 1950, she wrote “The Child Who Never Grew”, a memoir about her daughter. It wasn’t until ten years later that the cause of Carol’s mental retardation was finally diagnosed as phenylketonuria, the genetic disease that wasn’t even known until Carol was in her early teens.

The disease had first been described in Norway twenty years before Laura and Regina were born. Its discovery involved another set of siblings:

Dr. Asbjørn Følling, who had been a chemist before studying medicine, was asked to evaluate a brother and sister with severe mental retardation. His son, Ivar, told the story in a speech on the sixty year anniversary of this event in 1994:

“The stage is set in 1934. A mother with two severely mentally retarded children came to see my father, and to ask for his advice…She had also noticed that a peculiar smell always clung to her children…

The girl, 6.5 years old, could say a few words, was fond of music, had a spastic gait and a whimsy way of moving about, apparently at random. At times she had an enormous appetite, at other times none. The boy, almost 4 years old, could not speak or walk, eat or drink on his own. He was unable to fix his eyes on anything, and stool and urine habits were those of a baby.”

Dr. Følling’s son went on to describe his father’s painstaking chemical analyses of the children’s urine over the next several months that led to the realization that they both excreted phenylpyruvic acid, which healthy individuals don’t. The disease, phenylketonuria, is still called Følling’s disease in Norway.

The diet necessary for PKU patients was slowly established once Dr. Følling’s chemical analyses of urine hinted at their abnormal breakdown of the essential amino acid phenylalanine. An infant formula was developed in 1951. There are now protein supplements with low levels of phenylalanine, and also a pill that lowers phenylalanine levels, Kuvan (sapropterin), developed in the last decade.

Laura comes to see me every three to four months. I see her sister, Regina, every week during my nursing home rounds. When I see her, I always think about the life changing benefits of the newborn PKU test that came about in my own lifetime. Laura and Regina are part of the history of medicine, some of the last few with a cruel disease few doctors today have ever seen. I feel sad and humbled in the presence of these two contemporaries of mine, two children who never grew, but I also feel inspired by the steady progress of basic science.

Signed, Harvey Cushing

Published December 11, 2013 Medical History , Progress Notes 1 Comment

A couple of birthdays ago, my wife wanted to buy me a book by or about William Osler. She had watched and listened as I read from his book on the history of medicine and as I searched online for quotes by him.

We had both heard of Harvey Cushing’s biography of Osler. On one of the larger online booksellers’ websites, she found the only available set at the time of this two-volume work, and ordered it.

A week or so before my birthday, I was on the phone with her during my lunch break when she opened the package. Of course, I didn’t know what she was doing. All I heard was the rustling of paper and then her words:

“Oh, my gosh!”

On July 18, I said the same thing when I opened volume one. There, on the first page, was a flowing inscription in brownish-black fountain pen ink, signed “Harvey Cushing”. The books were not sold with this fact stated, and would have fetched thousands of dollars if they had been.

This biography of the father of American medicine, written and signed by the father of modern neurosurgery, is a source of inspiration I often return to. It provided most of the phrases I used in my 2011 post “A Christmas Message to All Physicians from Sir William Osler”.

Osler, our continent’s foremost internist, may be many physician’s imaginary mentor, but Cushing was quite a man himself, and was America’s most renowned surgeon. He introduced blood pressure recording to the United States, for example. He became a professor at age 32, pioneered brain research and neurosurgery, described the disease we now call Cushing’s Disease, wrote 14 books, only 9 of which were about surgery, and earned honorary degrees in literature, science and the arts. After reaching the mandatory retirement age of surgeons in Massachusetts, 63, he continued to teach and also worked extensively as a military surgeon.

Thomas P. Duffy, in a 2005 article entitled “The Osler-Cushing Covenant”, writes about the two men:

“In 1900 William Osler established a friendship with Harvey Cushing that encompassed the personal and professional aspects of their lives for over two decades. Their shared participation in the covenant of medicine shaped an intense friendship and mentoring relationship that profited both individuals immeasurably. The choice of Cushing as the recipient of Osler’s mentoring had its origins in their rearing, avocations, and in the way of life that they shared. In Cushing, Osler identified a surrogate son who joined with him in defining the course of medicine and surgery over the next century.”

Osler, twenty years older than Cushing, opened his home to Cushing, as he had done to many other students, but with Cushing, the friendship also included Cushing’s young wife and their children, who knew William and Grace Osler as Aunt and Uncle.

In a twist of fate, Osler’s own son, Revere, born around the time Osler and Cushing first met, was critically wounded at age 21 in World War I, and was taken to a field hospital where the surgeon on duty was Harvey Cushing. Revere’s life could not be saved, and every year on the anniversary of his death, Osler wrote to Cushing, expressing his relief that his son had died in Cushing’s presence.

At Osler’s funeral, Cushing delivered a eulogy, in which he referred to Osler as his “spiritual father”. Osler’s widow then asked him to write her husband’s biography, a task that took him four years to complete. The 1,400 page book earned him a Pulitzer Prize in 1926.

Two quotes by Harvey Cushing speak of his own compassion and optimism:

“A physician is obligated to consider more than a diseased organ, more even than the whole man – he must view the man in his world.”

“The capacity of man himself is only revealed when, under stress and responsibility, he breaks through his educational shell, and he may then be a splendid surprise to himself no less than to this teachers.”

Of Osler, his mentor and father figure, Harvey Cushing writes:

“He advanced the science of medicine, he enriched literature and the humanities; yet individually he had greater power. He became a friend of all he met – he knew the workings of the human heart metaphorically as well as physically. He joyed with the joys and wept with the sorrows of the humblest of those who were proud to be his pupils. He stooped to lift them up to the place of his royal friendship, and the magic touchstone of his generous personality helped many a desponder in the rugged paths of life. He achieved many honors and many dignities, but the proudest of all was his unwritten title, the Young Man’s Friend.”

Words of a son; signed, Harvey Cushing.

Hippocrates’ First Aphorism

Published November 11, 2013 Medical History , Progress Notes , The Ghosts in the Exam Room 2 Comments

“Life is short, and Art long; the crisis fleeting; experience perilous, and decision difficult. The physician must not only be prepared to do what is right himself, but also to make the patient, the attendants, and the externals cooperate.”

Hippocrates was a wise man. In an era where the causes of most diseases, even the functions of many organs, were unknown, he made detailed and astute observations that allowed him to become a master of prognosis.

His first aphorism is not often quoted in its entirety. When I first read it, I was struck by especially the last two words, “externals cooperate”. It makes me think that perhaps doctoring a few thousand years ago might have been a lot like today. We aren’t the first physicians to deal with demands beyond those of the clinical circumstances, the patient and his or her family.

I can only imagine who the externals were in Hippocrates’ time, but I am thoroughly familiar with who they are today: They are insurance company doctors and executives, pharmacy benefit managers and others, who say yes or no to our diagnostic and treatment plans. They are the ghosts in the exam room when patients and doctors try to make plans together.

The phrase “experience perilous” seems to imply that the gaining of experience is fraught with danger, but an alternate interpretation is that relying only on one’s own experience can be misleading. Hippocrates often emphasized the need to apply the known science to the practice of medicine.

The central point is that it takes time and effort to learn the art of medicine, which is sometimes glossed over in our fast moving society.

The volume of known science has obviously multiplied since Hippocrates’ time, and we can each know only a small fraction of it. But with all the available information now instantly searchable, we can become dangerous to our patients if we apply things without knowing them well through education and experience.

So, in the spirit of Hippocrates:

The artful practice of medicine requires understanding of the science, technical mastery of the craft, and wisdom

and

Facts can be learned by anyone; experience is earned through years of practice; wisdom is granted to us only if our hearts and minds are open, in our practice of medicine, and in our lives.