“Are you sure you don’t want to give him a mega-dose of IV steroids?” the attending asked.
“No. Do me a favor. If you don’t want to stop the current antibiotics, add IV Bactrim tonight. Give me a day, and if the slides don’t show evidence for Whipple’s we can stop the Bactrim. Can you do that?” I held my breath.
The next morning, after a second dose of Bactrim, Leon’s fever broke. Art Vanderburgh, MD, the taciturn pathologist, called and wanted me to take a look at the slides. The PAS stains were positive for Whipple’s. Everywhere we looked: the lymph node, the fluid from around the heart and lungs, every sample he restained was flooded with the organism. He marveled at how the organism, now so obvious, could have remained hidden so long.
The Road to Recovery
And ever so slowly, Leon made headway. His arthritis melted away and the diarrhea resolved. Follow-up scans showed that the fluid around his heart and lungs and the ascites in his abdomen were in full retreat. On the day his oxygen was discontinued, he asked for a pass to go outside to smoke a cigarette. His strength had returned. He’d had enough of hospitals. He was going outside, regardless. He was a changed man—but not that changed.
The Great Imposters
We call them zebras—rare diseases hiding in plain sight. They are the great imposters, mimicking everyday infections, cancer or autoimmune disease. And they keep me sharp, thinking, reviewing, adjusting my differential diagnosis when at the end of a consult, it just doesn’t add up. On the day Leon Woodle was discharged, the lab technician handed me the extra red-top tube of spun-down blood I’d requested. As a mystery solved, I decided to give it to Leon, who appreciated the fact that he was still alive. “You never know,” he informed me, “when Tropheryma whipplei might appear in a Times crossword puzzle.”
I wrote out the name of the causative organism of Whipple’s disease on the tube. We both agreed we’d never forget the spelling. I know he won’t.
Charles Radis, DO, is a rheumatologist in Portland, Maine, and director of clinical research for Rheumatology Associates.
Fast Facts: Whipple’s Disease
- Originates in the small bowel;
- Tropheryma whipplei can’t be cultured from stool samples or grown from blood;
- There’s no antibody test to definitively confirm the diagnosis;
- It can be seen on microscopy if the proper stain, a PAS (Periodic acid-Schiff) stain, is applied to the biopsy material;
- Untreated, the infection spreads from the GI tract to adjacent lymph nodes and slowly overwhelms the immune system;
- Arthritis, sometimes mimicking rheumatoid arthritis, may be present;
- It may be more common in workers exposed to manure or raw sewage;
- Untreated, the disease is usually fatal;
- Treatment with corticosteroids is generally ineffective; and
- Treatment with the antibiotic Bactrim is usually curative.