McArdle disease

Monday

When Maude Johnson, a serene 71-year-old woman with schizophrenia, congestive heart failure, and hyperlipidemia, begins her visit by handing me a letter, my curiosity is piqued. "Dear Dr. Gross," it begins, "I am writing to let you know that I have an understanding with God. His will was that I shouldn't take my medicine any more, so I haven't taken my pills for four months." I know that Mrs. Johnson speaks with God, but this is the first time my prescriptions have been directly overruled. I shoot a glance at Mrs. Johnson's visiting psychiatric nurse. "Welcome to my world," she murmurs. I'm fond of Mrs. Johnson, who has a gentle southern accent and a radiant expression. Mrs. Johnson's nurse clarifies that she actually has been taking most of her medicines, including her antipsychotic, but not atorvastatin or carvedilol (Coreg). I consider telling Mrs. Johnson that God has given me clarifying orders, but instead relate the tale of the true believer caught in a flood who refuses every attempt at human rescue because he's convinced that God will save him. The true believer drowns and in Heaven, a puzzled God tells him, "But I sent a rowboat and two helicopters!" Mrs. Johnson laughs, but she remains adamant about the pills. "Doctor Gross," her letter ends, "one day you will look back and say there was something about that woman." Gazing at her beaming face, I nod, and I know that she's right.

Tuesday

I learned today that Maria Ruiz's 90-year-old heart gave out last night in the intensive care unit (ICU). Born in Puerto Rico, Mrs. Ruiz had a history of congestive heart failure and diabetes. For years, she was erratic about keeping appointments. Then, in 1996, after two intubations for heart failure exacerbations, her daughter-in-law, Miranda, took charge-dispensing pills, recording blood sugars, and bringing Mrs. Ruiz in for monthly visits. Given her ejection fraction of 15 percent, I wasn't optimistic about her prognosis. But for the next eight years, she stayed out of the hospital, walking a tightrope between pulmonary edema and renal failure, her systolic blood pressure hovering around 90 mm Hg, and her weight steady at 75 pounds-less than her age. She was a crooked little feather, with no teeth, bright eyes, and an infectious smile. "Give your daughters a kiss for me," she'd say. Last May, she was hospitalized with pneumonia, her ejection fraction down to 10 percent. Our cardiologist offered her an implantable defibrillator and biventricular pacemaker, but she refused. This past week-end while I was away, fluid filled her lungs. When I heard she was in the ICU on pressors, my heart sank. Illogical as it sounds, I was hoping she'd go on forever. I wanted to visit, but didn't have the time yesterday, and now it's too late. She's gone and I'm shocked. How foolish is that? It just goes to show that love, even a doctor's love isn't rational.

Wednesday

Junior Serafin, age 12, is brought in by his mom Linda because of "behavioral problems." Junior is silent today, which is typical. "He's gotten into trouble in school, and he's been fighting more with his sister," says Linda, looking annoyed and wrung out. In addition to raising three children, she works full-time at a fast-food restaurant. "What happened at school?" I ask Junior. Slowly, painstakingly, the story comes out. "I threw a desk because the teacher didn't give me candy." Because Junior's never done this before, I wonder what's going on in school. According to Junior, he has a few friends, but there are kids in his class who curse at him, and he's scared. "They might jump me." I pause to wonder how I would feel working under such circumstances. "Sounds pretty awful," I say. Meanwhile, Linda's expression has softened and she's dabbing her eyes. Do I detect a hint of self-reproach? "He never told me that," she says quietly. We make a plan for Junior and Linda to schedule an appointment with his teacher and guidance counselor. Meanwhile, I encourage Junior to be open with his mom about his struggles in school. "And you," I tell Linda, "should get Junior's okay before you take any steps." We agree on a follow-up date, and I cross my fingers, hoping that something positive will come out of our talk today.

Thursday

Do other physicians feel as tormented as I do by bad outcomes? Mr. McArdle, a pleas-antly gruff 75-year-old smoker, comes to our inpatient service after a combined operation to remove half of his colon (because of a cancer found on colonoscopy) and one kidney (because of a tumor found while staging the cancer). His bad luck is tempered by some postoperative good news: both primary cancers are in early stages. This might be a cure! But then, his remaining kidney takes a nose-dive, with his creatinine shooting up from 2.0 to 8.2 mg per dL over three days. The morning after he's dialyzed, while still on subcutaneous heparin, he tells an alert resident about slight breathlessness. A spiral computerized tomographic (CT) scan confirms bilateral pulmonary emboli. The next day, Mr. McArdle begins wheezing. Then today, just when it feels like we've hit bottom, it gets worse. A radiologist reviewing the CT scan from two days ago points to a haziness in the lung. "That's not caused by infarct," he says. "It's an infiltrate." By mid-afternoon, this nice man, now growing more hypoxic and burdened with cancer, renal failure, a pulmonary embolism, chronic lung disease, and pneumonia, is en route to the intensive care unit. I shake my head and wonder, "Can we get a break, please?"

Friday

What happens when a challenging patient drops out of sight? Today, I find out when she resurfaces. I last saw Federica Colon in March 1997, when she was 76 years old. She has a history of chronic abdominal and back pain, multiple surgical procedures, smoking, anxiety and depression, and histrionic agony, which I did little to quell. At her last visit, she presented with belly and rectal pain. "Clutching abdomen, practically writhing on the table," my notes read, "but cheerful moments later." My intervention: "Resume lactulose at increased dose." She never returned, causing me to wonder: Did her bowel finally explode, as she suggested it might? Did I minimize her symptoms once too often? Did she die in excruciating pain? None of the above; she moved to Puerto Rico to live with a daughter for seven years. Now she's back, snowy-haired and more gnarled, all smiles, and happy to see me until her hand begins vigorous, circular motions around her navel. "I've got pain here and in my back, I'm constipated, my nerves are acting up, my ears are clogged, and my right ankle swells and turns black." She hasn't changed a bit. Turns out, neither have I. After taking in her symptomatology, what do I prescribe? Lactulose! It's like deja vu all over again. Maddening? Yes, but it's also pleasantly invigorating to see her once more.

Saturday/Sunday

One of the advantages of being married to a writer is being invited to tag along when my wife goes on assignment. This time, I'm intrigued. Diane's been asked to write about "Partners, Pleasure, and Passion," a couples retreat being conducted at Miraval, a luxury resort and spa outside Tucson, Ariz. The retreat is organized by two family physicians, Lana Holstein, M.D., and David Taylor, M.D., who met at Yale University School of Medicine and, after years of treating patients' sexual issues, developed this four-day pro-gram as a married duo. Diane and I join 13 other couples with common obstacles to intimacy and sex (i.e., work and worry, non-stop intrusions, too little time). Brief lectures challenge us to recast our notions of sexuality, then we take up couples-based exercises and daily "homework" assignments. By retreat's end, we're all purring like contented kittens as we fashion written plans to maintain our newfound intimacy at home. Looking around, I see fellow middle-aged couples practically aglow with the transformative power of sexual intimacy. "You are the most fantastic woman," says one tearful entrepreneur to his wife. I grip Diane's hand, taking in my good fortune, realizing how drab even this doctor's life could seem without my soul mate.

For the past 13 years, Dr. Paul Gross has been on the residency faculty of New York Medical College at St. Joseph's in Yonkers, New York, a city with a population of 196,000. He divides his time between patient care, resident supervision, teaching, and life with his own family-a wife and two daughters.

Address correspondence to Paul Gross, M.D. (e-mail: pgross@pol.net).

To preserve patient confidentiality, the patients' names and identifying characteristics have been changed in each scenario.

COPYRIGHT 2005 American Academy of Family Physicians
COPYRIGHT 2005 Gale Group