Urology Pearls: The mystery of the corkscrew hair, Part 2

Shahar Madjar, MD

Here is a recap of my last story: George, 71, lived alone in a trailer park at the edge of a Midwestern town. When his refrigerator’s rubber door seal broke, he taped the door shut and used only the top freezer. He stockpiled on frozen tater tots and ate them in every meal. For months, he had been bleeding and bruising easily and his stool had turned black. His head felt light and he was short of breath. In the Emergency Room, Dr. Wise noticed that George was skinny, his gums were bleeding, and he was missing a few teeth. The doctor also noticed a rash of purple spots on George’s chest, arms, and legs, and later, on re-examination, an astounding finding–the hair on George’s chest wasn’t straight, nor curled. Instead, it took the shape of a corkscrew.

Dr. Wise suddenly realized that George might have the same disease that had afflicted hundreds of sailors aboard British battleships in a voyage that took place in the 18th century.

Several of you told me that the puzzle was too easy. One patient told me, “Doc, you have to do better than that! Everyone knows it was Scurvy.” And she was right! But, wait, the solution to the puzzle — a mere diagnosis — isn’t all I have to tell you.

First, a bit about scurvy: The disease is caused by vitamin C deficiency. It is quite rare today in industrialized nations because vitamin C is readily available in fruits, vegetables, and food items, such as your morning cereals, which are fortified with Vitamin C. Vitamin C is essential in the synthesis of collagen. Collagen is a protein which underpins the integrity of almost any connective tissue in the body: skin, blood vessels, mucous membranes, and bones.

A diet poor in vitamin C will lead to the disruption in the synthesis of collagen — the substance that connects tissues, a kind of “glue” that pulls and keeps our body together. And as in George’s case, blood vessels break causing bleeding gums, skin rash, and intestinal bleeding. Tiny fractures form in bones and teeth would start to fall out. And the body falls apart.

Vitamin C is also critical in energy production. The body creates energy through oxidation. This process takes place in the mitochondria within the cells, partly by breaking down fatty acids. Without vitamin C, energy production within cells would halt, and weakness, fatigue, sometimes muscle cramping would result.

Each of George’s symptoms could be attributed to a different disease, to a different factor. The lightheadedness may be a sign of a heart disease. The easy bruising and bleeding gums may indicate a problem with George’s platelets, or a failing coagulation system. The dark stool may indicate that George had been bleeding into his digestive system, from a stomach ulcer, for example. His shortness of breath may be the result of a lung disease. The weight loss may be caused by malnutrition, severe infection, even cancer.

But in George’s case a single factor — vitamin C deficiency — was the cause of all of his symptoms. Doctors are always tempted to look for an elegant solution. It follows Occam’s razor–the idea that when competing explanations present themselves one should select the explanation that makes the fewest new assumptions. There is beauty in simplicity and great satisfaction in finding a single, simple solution to what seems to be a complex problem.

A realtor might recite the mantra “there are three important factors in real estate: location, location, location.” It is simple and catchy, even funny. It is tempting. But is it true? No! There are other factors to consider when buying a home, or determining its price: is the roof new? Are the windows facing the lake? Is there a neurotic dog across the street that keeps barking?

In medicine, complexity might present itself in many ways. Every symptom, every sign, might point in a different direction, a different diagnosis. And to keep it interesting a patient may have several diseases at the same time. Shortness of breath may be the result of a lung disease, but also of heart disease. Treat just one, and the patient would still be sick. So as an antithesis to Occam’s razor, Dr. Hickam came up with his own dictum, “Patients can have as many diseases as they damn well please.”

When facing a problem, whether professional or personal, I try to consider the complexities of life. But when a simple solution presents itself, I celebrate the beauty of occasional simplicity.

The doctors gave George the vitamin C he was lacking, and a few days later, George returned to his home better than he had been for a long time.

Editor’s note: Dr. Shahar Madjar is a urologist at Aspirus and the author of “Is Life Too Long? Essays about Life, Death and Other Trivial Matters.” Contact him at smadjar@yahoo.com.


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