It started with a sore thumb and ended with a lifetime of medication. In between there was an emergency room visit at one small hospital, an ambulance transfer to a big hospital, a Medevac flight to Massachusetts General Hospital, multiple invasive procedures and a diagnosis of an often lethal condition. And I was not the one who made that diagnosis.
Paul Allard had developed severe heartburn and indigestion earlier this fall, and had just recovered from a bout of wrist pain when I saw him a month ago. The pain and swelling had been there for a week or so, did not seem to be caused by any trauma or overexertion. It had cleared up after just three days of prednisone, and his rheumatology blood profile was completely negative.
This time, Paul had a pain along half of his left thumb and in the web space between his thumb and his index finger. It was sharp, burning and persistent.
As I asked all kinds of questions and checked his hand strength, skin temperature, monofilament and temperature sensation, arm strength, neck movements, axillary and supraclavicular lymph nodes, Paul clearly seemed uncomfortable. His partner, who usually seemed a little disinterested in Paul’s medical concerns, was leaning forward in his chair watching our exchange and my exam intently.
“I’m not sure what’s going on”, I said to the two men. “It could be something arthritic, like that episode of wrist pain, or maybe some type of vascular inflammation in a very small vein or even one of the four arteries that supply the thumb, but it could also be a pinched nerve, especially because it involves the space between the thumb and index finger.”
I suggested that Paul finish the course of prednisone he had been able to stop early when his wrist pain resolved. Paul and John agreed and we set up a five day followup.
That was Friday. Monday morning, my inbox had the next several installments of the story:
Late Friday night Paul suddenly developed nausea, severe abdominal and left flank pain, and went to our small hospital emergency room. They did a CT scan of his abdomen, which showed an infarction of his left kidney. When that happens, the cause is usually a blood clot, so they transferred him to Cityside Hospital for evaluation by the vascular surgeons.
A CT angiogram showed a large clot in Paul’s thoracic aorta. He was started on a heparin drip and airlifted to Boston. There, they didn’t see a clot in the thoracic aorta, but it had apparently just moved down to his abdomen. The clot was removed surgically, and while his kidney showed signs of recovery, and several specialists were working out his diagnosis, his left lung suddenly filled with blood. He had three quarts of blood drained through two chest tubes and was finally allowed to return to Maine with a diagnosis that explained what had happened and committed him to a lifetime of warfarin to prevent future blood clots.
“So, you have Lupus Antigen”, I said, rhetorically. “But they didn’t think that you have lupus?”
“Right”, Paul and John answered in unison.
“Sure, the wrist pain could have been something else. What did they think of the thumb pain, a small embolus in one of the four little arteries that supply the thumb?”
“They weren’t sure”, Paul answered.
“Who would have known…”, was all I could say.
“He could have died, they told us”, John said.
“Most people with this kind of clot do”, Paul filled in.
I half shook, half nodded my head as I punched in Paul’s warfarin dosing order in the computer. I thought, not for the first time, about how you see things on the front lines of medicine that turn out to be the first sign of a condition that other colleagues diagnose hours, days or even weeks later, as symptoms evolve and the clinical picture comes into clearer focus. It is a humbling experience.
It has been said about the Lupus Antibody Syndrome’s sister condition, “If you know lupus, you know medicine”.