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Wrestling the Rooster

Published January 26, 2014 Progress Notes 4 Comments

“I used to be strong, I wrestled the bull”, Sumner Ball said, “but now I can’t even wrestle the rooster”.

On the far side of eighty years old, he looked lively and trim, and his weathered face hinted at a smile as his blue eyes peered straight into mine.

“I think these cholesterol pills are hurting my muscles”, he declared. “I don’t think they’re good for me”.

“Is it your back?”

I scanned through his last few visits and saw he had mentioned some low back pain while gardening this summer.

“No, Dr. Tom took care of my back”, Sumner said, referring to our local chiropractor. “My arms and my legs hurt, even my shoulders hurt.”

Years ago, Sumner had developed polymyalgia rheumatica, and it took almost two years to get him through it with the help of gradually decreasing steroid doses.

“Let me get a blood test, and why don’t you stop the cholesterol pills for a while and see how you feel”, I offered. We agreed to have a follow-up visit in a few weeks.

Three weeks later, Sumner Ball was a changed man. His faint smile was now a big grin.

“I knew that medicine was not good for me. I feel better now, not so many muscle aches. And I stopped the other one too. It was making me dizzy.”

“What about the blood thinner?” I asked.

“No, that one I take. I know it can keep me from having a stroke. But I know my body, and I know what my body needs, just like when I had goats and horses – they knew what to eat and not to eat.”

“My goats don’t always know the difference”, I said. Sumner grinned as he continued, “and we didn’t have the vet come out all the time. We treated them with herbs, good feed and common sense.”

His blood pressure was still OK, but his pulse rate was just under 100, a little high for someone with atrial fibrillation.

“Your diltiazem was keeping your heart from going too fast”, I reminded him.

“I feel good now. I like to stay this way. Do you think I am making a mistake?” His penetrating, small blue eyes told me he didn’t want me to disagree with him.

“Your heart could start racing”, I warned him. “Let me see you in a few weeks to make sure you’re not going into heart failure.”

Two weeks later, Sumner had gained five pounds. His legs were swollen, he was short winded and his irregular pulse was 130.

“Your heart is missing the diltiazem”, I said.

“I can’t take it”, Sumner answered. I knew he had already tried a beta blocker a few years ago, and his pulse had dropped to 40 on the lowest dose.

“How about trying something natural?”

His eyebrows rose. I continued: “There is an old herbal remedy, made from foxglove, called digitalis. It may slow your heart down enough to get you out of trouble.”

A week later, Sumner looked like he’d take on something much bigger than the rooster again. His pulse was 80 and his weight was back to its baseline. Leaning back in his chair, he said:

“The third day I took your medicine, I could feel my heart slow down. I knew that herb medicine would be good. You do all right by me.”

“I figured something more natural might work for you”, I answered. “Besides, it was the only other thing I could think of.”

“I like you. You have common sense”, he said as he offered me his large hand.

It was the firm handshake of a man who had worked hard all his life.

Good, Strong Heart Beat – 140 and Regular

Published January 9, 2014 Progress Notes 1 Comment

“Welcome back. How was your trip? Or exile… you were away for a long time.”

“Almost a year”, my nine o’clock patient answered. A woman just over forty, she looked tan and physically strong. Her short hair was peppered with gray, different from the last time I saw her. She had gone abroad on assignment for a magazine and a film production company, and before she left, she had joked that she would have to be her own doctor until she could come back to see me again.

“So, what’s going on”, I asked.

“I’m pregnant, pretty far along. I thought it was early menopause, like my mother and my sister, but that doesn’t come with morning sickness. And I can feel my uterus.”

“And you haven’t seen a doctor?”

While getting ready on my exam table, she told me about her work in small villages far away from clinics or hospitals and her decision not to seek care until she came back home.

Her uterus almost reached her navel. I took out the hand held vascular doppler we use to measure blood pressures at the calf of people with circulation problems. I changed the probe to the one used for fetal heart tones, an attachment I had never used; I stopped doing obstetrics the day I graduated from my residency thirty years ago.

“I never thought I would be pregnant again after my miscarriage when I was twenty-five”, she said with sadness in her voice as I applied gel to her abdomen and turned on the device.

There was the loud, swoshing sound of the placenta following her own elevated pulse rate. I pressed deeper and aimed the instrument downward with all kinds of static from the movement against her skin. Then, suddenly, there it was, rapid and perfectly regular; a sound I hadn’t heard for thirty years.

“Is that the baby?”

“Sure is.” I counted. “Good, strong heart beat, 140 and regular”.

She reached down and grabbed my hand.

“Please leave it there. I want to listen to it longer.”

Her eyes moistened and her lips began to quiver. She placed her top teeth on her lower lip as if to keep it still. I rested the probe and we both listened in silence.

I remembered that sound, the rapid heartbeat of unborn babies, from many long nights on duty during the sleep deprived years of residency. I remembered catching a few moments’ rest in the on-call room down the hall from two or three monitors with intertwining rhythms of babies waiting to be delivered.

Vividly, I remembered my first delivery, a precipitous double footling breech with no other doctor on the ward than this frightened first-year resident. Just in time, as I stood there with my right hand assessing the situation, old Doc Walker appeared in his street clothes in the delivery room door.

“What’ya got, son? Nurses tell me you got two feet there.”

“Yessir,” I tried not to quiver.

Doc Walker’s slow and gentle words calmed the young mother and guided my hands as they in turn guided the baby, feet first, across the symphysis and onto his mother’s belly.

As the doppler continued to tap out its rhythm, I remembered faces with smiles and tears, happy couples and frightened, single young mothers in the delivery rooms. I remembered blue babies, me slipping in umbilical catheters, the neonatal intensivists watching and supervising.

I remembered my own son, hooked up to an apnea monitor at my own hospital. Years later, as a new grandparent, I was a visitor, strangely out of place in a different neonatal intensive care unit, watching my granddaughter through the walls of an incubator.

Thirty years since I heard that kind of heart sound, and it still touched me in inexplicable ways. I remembered, my whole body remembered, the mixed feeling of dread and excitement when my pager used to go off in the middle of the night: “Call 2350 STAT.”

Thirty years ago, I saw more births than deaths. Now I only attend departures. For a minute or two that morning I was again participating, ever so briefly, in the greatest miracle a physician is privileged to be part of.

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.

Twenty Questions

Published December 7, 2013 Progress Notes 6 Comments

Adrian Bell didn’t look dehydrated, but his diarrhea had come and gone for a week and a half when I saw him a few weeks ago.

“Is anyone else sick with the same thing?” I asked, beginning my usual line of questioning.

“No”, answered Eleanor, his wife.

“Have you had any water to drink from a new or unknown source, or have you traveled away from home?”

“No”, both answered in unison.

“Any new foods that only you ate or that you don’t normally eat? Are you a big milk drinker?” I added, thinking about secondary lactose intolerance.

Still, negative answers.

“Any chills, fever, belly pain…” my questioning continued.

Nothing.

“Have you had any antibiotics prescribed by any other doctor?” I asked, because we have had a flurry of Clostridium Difficile infections in our community, which is something we didn’t have to worry about years ago. We had three cases recently at the nursing home, where Eleanor volunteers.

Still, “no”.

“Anything else going on, even if it seems unrelated?” I finished my questioning as I motioned for Adrian to get up on the exam table.

“I have had some joint pains”, he answered.

After an unremarkable physical exam, I ordered some lab tests, including inflammatory markers, a stool culture and C. Difficile test. I gave dietary instructions and we set up a follow-up appointment for a few days later.

At his follow-up visit, everything was the same and all the tests were normal. I sighed internally.

“Do you think it may be Beaver Fever?” Adrian and Eleanor both leaned towards me. “We’ve heard of an awful lot of people downstate who’ve had that.”

“I haven’t seen a case of giardiasis around here in years. How do you think you may have gotten that?”

“Well, two weeks before this started, I fell in a beaver pond in the woods in back of our property. I was checking out an old four wheeler trail….”

“Fell in a beaver pond…” I kicked myself for not having ordered a test for ova and parasites, but, of course, they can be unreliable.

“I think we’ve got to put you on some medication and do another stool test”, I said, thinking to myself that I now have one more question for future diarrhea assessments.

Medicine is like twenty questions sometimes. If you don’t ask the right questions, you don’t get the right answers.

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