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The disease that led to the death of Larry H. Miller is unfamiliar to most people because it is so extremely rare. In fact, in Miller's case, the disease was even more unexpected.
Despite the severity of Miller's diabetes and the fact doctors had to amputate both his legs below the knees, they really thought he had a chance to make it. What caught them off guard was the disease called calciphylaxis, a rare complication that develops inside the blood vessels.
Only 1 percent of patients in end-stage renal disease develop calciphylaxis. Even within that population, it's after they've been through a lot of dialysis.
"Mr. Miller was on a very short course of dialysis. It's pretty unusual for as short as his course was to have such a severe problem," said Dr. Michelle Mueller, a vascular surgeon at the University of Utah.
Mueller describes the cascading events behind this rare condition that's 60 to 80 percent fatal: End-stage renal disease leads to more dialysis, to a hyperparathyroid condition, which triggers high phosphate levels. That then causes the leaching of calcium from bones into the blood stream. Once inside blood cells, small arteries literally calcify.
"It looks like bone. It's calcium deposits that look like bone. So, on patients that have a lot of calcium in their blood vessels, when you take just a plain X-ray of it, you can see the outline of the blood vessel wall which, on plain X-ray, you couldn't normally see," Mueller said.
The calcium blocks off the flow of blood, killing skin and soft tissue. Dying tissue progresses rapidly in feet, thighs, buttocks, the breast, and even the tips of the fingers.
"Almost in front of your eyes, from one night to the next morning, it looks worse," Mueller said.
Nobody knows why just a few patients develop calciphylaxis while most don't. "There is some sort of perfect storm that we don't quite know yet what causes that calcium to be deposited into the arteries. It's something that progresses to the point where you just can't live through it," Mueller said.
Medications that bind excess phosphate or liquefy the calcium are sometimes used successfully in the 20 percent or so of those who do survive. The university knows of only one current survivor they've treated. That person lives out of state.
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