Monday, June 27, 2022 Jun 27, 2022
82° F Dallas, TX

SCENES FROM A PRACTICE George and Josephine

He was a giant; she was tiny and childlike. Together, they were a perfect match.

GEORGE WAS A GIANT. HE WAS ALSO very sick, very near death, when I first met him in the emergency room. His 6-foot-6-inch frame extended over the end of the litter. I lost my hand in his palm. Two hundred fifty pounds seemed slapped on his huge frame, but it was not a proportionate fit. His arms and legs were longer than they should have been. He had large floppy ears, great fleshy folds on his face, thick bifocal lenses, a pigeon breast, and very flat feet. The parts were ugly but the whole was impressive. He was gray-green in color, there was a sheen of sweat on his brow, and he was afraid. Angor animi-death was near. George knew it and so did I. He was complaining of chest pain-a great tearing pain beneath his breast and into his back between his shoulder blades.

It was not hard for the surgeons to diagnose what was happening to George. He had Marfan’s syndrome, a disease of connective tissue, the scaffolding web around which we build our organs. Instead of being strong and flexible, George’s connective tissue was flawed-it was lax and brittle, His growth had been distorted by this absence of a firm blueprint, and now he had the most feared complication of the disease. His aorta was ripping apart, forming two channels of blood-one leading to the vital organs, the other leading nowhere. The diagnosis is easy but the treatment is not.

The surgeons nodded and withdrew.

I WENT TO THE WAITING ROOM TO FIND George’s wife. Scanning the worried and bored faces, I almost missed her. I was looking for a likely match, not the tiny little wisp at the periphery of my vision.

“Mrs. Stark…’’’

“Call me Josie.”

“Josie, your husband is very ill,” I said, hating this way of meeting someone.

“Yes, I know,” she said gently. “I knew that when he woke up with such pain. Don’t worry. Doctor. He’ll be all right. He’s so strong.”

She was actually comforting me! My surprise was dual. I was surprised at her response and more so when I looked at her closely. Josie was as strangely small as George was large. Tiny and childlike, she looked old and young at the same time. She had alabaster skin and very blue eyes which graced a somewhat plain face.

THE KING’S HORSES AND THE KING’S MEN swirled about shortly after George’s admission, and we decided to “manage conservatively,” which means sit and wait. We controlled his blood pressure and kept George quiet. His pain continued but moved slowly down his back and into the pit of his stomach as the dissection continued. The pulse in his left groin became weak as the false channel of blood squeezed the great vessel to his leg.

Josie set up vigil in George’s room. Not a hysterical vigil, not even a seriously worried vigil, more a presence. The tiny woman and the giant man talked quietly, and they occasionally shared a small laugh. When we came in. Josie quickly stood against the wall, a shy smile on her face. We felt we were in her home and she was a simple, gracious hostess.

On the third day, George suddenly had relief from his pain and his leg pulse became strong again. Josie smiled tolerantly at our elation. She seemed critical of our previous doubts that George would survive. He went home with medication to keep his blood pressure low and admoni-t ions to avoid heavy labor. He expected the latter advice, and I guessed he would not follow it.

OVER THE NEXT SIX MONTHS, GEORGE AND Josie reported regularly to my office, and their mutual affection became even clearer. George returned to work, hauling engine blocks, grunting with torque wrenches, and contorting his huge frame under and around automobiles. Josie knew this was bad medicine, but she also knew George. Rather than nag or cajole, she supported him, making it easy for him to rest when she could. She knew that not working was more deadly than working, and she accepted the risk gladly. George showed equal concern for his wife. His manners with her were courtshiplike and thoughtful. He always nodded at her near-whispered comments and suggestions.

ON ONE VISIT THEY BOTH APPEARED SOLEMN and resolved. They had decided thai I should now attend to Josie. The gravity with which they made this request should have alerted me, but I missed the gesture’s significance.

Josie had few physical complaints- some easy fatigue, dispersed aches and pains, an occasional headache, dizziness once in a while, and a rare bout of palpitations. I asked about her menstrual and sexual history and got a blunt reply. “You’ll see.”

Indeed, 1 did see. When she had disrobed and dressed in a hospital gown for a physical examination, I discovered the reason for her old-child appearance: Josie had never sexually matured. She had never gone through puberty. She had no breasts and no genital maturation. Josie was virginal and childlike. Startling findings like this really rattle an internist’s bone box, arouse intense professional curiosity. Internists love puzzles, especially when they are the first to encounter them. I had to hold myself back from a rapid-fire cascade of questions to get to the bottom of this puzzle. Josie looked me straight in the eye. waiting for my response. She was not fearful or embarrassed, just expectant.

I WORRIED OVER WHAT TO DO. SOME DELIcate switch in Josie never closed years ago. Should we even try to bring menarche to a woman of 58? Breasts are important, orgasms are important, female identity is important-important for every woman. Even if Josie couldn’t mourn the loss of something she had never had, wasn’t there some bitterness that she had been denied the substance of so much art. literature, and story? Yet this was my mind at work, not Josie’s. She had wrested from this natural misadventure happiness, devotion, hospitality, and love.

“What shall we do about your condition, Josie?”

“Am I sick. Doctor?’’ she replied, with a little smile.

“Well, not sick, Josie, but not normal either, as you know.”

“Oh yes, Doctor. I know I’m not normal, as does George, but I feel well. And we are happy,” she said.

A disease but not a dis-ease. A few lab-orator)’ tests confirmed that Josie’s condition was not a threat, either. The remaining functions of her pituitary gland were normal, and there was no slowly growing tumor. When Josie and George both knew this, they were content and decided to come back to see me only in need.

The need didn’t arise until a few years later, when a blood vessel burst in Josie’s brain. She nearly died as the hemorrhage flooded her left brain, taking away her speech and all motion on her right side. Her communication was a series of inflective sounds: “na, na, na, na.” With this single syllable and an octave of tones, Josie could be understood by George, who tended her with the same care that he had received from her. She still seemed happy. It seems to me that basic dispositions are preserved in people who have strokes. Mean and ugly people tend to become more so, and basically happy people remain so, even when the controlling brain cortex is destroyed.

JOSIE PROGRESSIVELY DECLINED AND DIED, and George went into mourning. He came to see me only to weep and re-examine the details, looking for reassurance that we had done all we could for her. “Did we do everything?” “Are you sure she wasn’t in pain?” His only forward thought was for his own death.

’it won’t be much longer will it, Doctor?”

It wasn’t.