Diagnosis: calciphylaxis

By early January, 2016, Stephanie's leg infection had grown progressively worse, and the only way to save her life was by amputating her left leg. As with all the difficulties she faced before and after, Steph had come to terms with this. We held hands a lot, but she was tough. She could deal.

And then, suddenly and unexpectedly, there was even more to deal with. A few hours before Steph's amputation, a nurse examining her noticed a large, unusual dark area on her chest, and several more dark areas extending around her back. "Any idea what these are?", he asked her.

"I've noticed them for a few weeks," Stephanie said. "I Googled it, and I'm pretty sure they're just bedsores." Stephanie had been confined to her bed at home, 24/7, for several months leading up to the amputation of her leg.

"Hmmm," said the nurse. After he left, Stephanie showed me the lesions. The largest was about the size of the palm of your hand, and some but not all of them were moderately painful to the touch, she said.

I asked why she hadn't told me about these things on her skin, or shown them to me, and Stephanie said that she knew I was worried about her amputation, and she didn't want to worry me even more. It was one of the few conversations where I scolded her. Told her that I'm part of her, she's part of me, and we can't keep such things a secret. "Tell me, please, Love. Even bad things, worrisome things."

It was an hour before her surgery, though, so I didn't scold much. To my knowledge and recollection, this was the only big thing Steph ever kept from me, and I wish she hadn't, but it was forgiven long ago and I'm not going to scold her for it again today.

The surgery went well, and Stephanie had become a one-legged woman, but she was the sweetest, smartest, cutest, all-around finest one-legged woman in the world, same as she'd been when she'd had two.

A parade of doctors came into her hospital room, several times daily, to poke at the stump where her leg now ended, and other doctors came to poke at the dark splotches on her torso. The leg-minded doctors said she was healing well, and the splotch-minded doctors said hmmm a lot, and had a small piece of one of the lesions snipped off and sent for testing.

The diagnosis was calciphylaxis, and it was bad news indeed. I'll quote from the Mayo Clinic's page on the disease:

"Calciphylaxis (kal-sih-fuh-LAK-sis) is a serious, uncommon disease in which calcium accumulates in small blood vessels of the fat and skin tissues.

"Calciphylaxis causes blood clots, painful skin ulcers and may cause serious infections that can lead to death. People who have calciphylaxis usually have kidney failure and are on dialysis or have had a kidney transplant. The condition can also occur in people without kidney disease."

As explained to us by the experts, the black things on Stephanie's chest and back were calciphylaxis lesions, and having them once meant she'd have them always. It sounded horrible — a little like leprosy.

We were told that when the lesions on her torso faded, she would develop lesions elsewhere, perhaps on her arms or neck, and as those lesions were treated and faded, new lesions would appear on her leg, or on her stomach. The lesions could only be treated, not prevented, and even with treatment there would always be more lesions to come. There's no cure for calciphylaxis, and in the long term, it's fatal.

Stephanie had already been diagnosed with diabetes, which can be fatal.

She'd been diagnosed with kidney disease, which is always a fatal diagnosis.

And now she'd been diagnosed with calciphylaxis — her third fatal diagnosis.

There were tears, of course. We spoke of mortality, death, fear of the unknown and fear of the end. As I'd requested, Stephanie held nothing back from me, and I thanked her for that. Our conversations were dark for a few days, though as always, no matter how bleak things were, Steph had her sense of humor to protect her. There was nothing that woman couldn't laugh about, or make me laugh about.

She'd brought her laptop computer to the hospital, and of course we were Googling everything about calciphylaxis, a disease previously unknown to both of us. After days of intense reading about it, though, Steph became skeptical of this new diagnosis.

She discussed her doubts with three different doctors, who were polite but dismissive of her concerns. She spoke with them separately, but their responses were remarkably similar, and their unanimous implication was that Steph was in denial of an unpleasant diagnosis.

Hearing that, three times, made me more certain that Stephanie was right and the doctors were wrong. They maybe knew medicine, but I knew Stephanie, and Stephanie Webb never did "in denial." Her health was in decline, and she knew it, and she fought it, but she never pretended it wasn't happening.

She asked to see the test results. There was some reluctance to provide the actual documentation — not because there was anything to hide, I'd say, but more because letting a patient see her test results is out of the ordinary. Well, Steph was always out of the ordinary, and she politely but insistently asked and asked again.

It took a couple of days, but the test results — on paper — were brought to Stephanie's bedside by her attending physician. He spent ten minutes explaining what was printed on the pages, and then left the documents for Stephanie to peruse. And being Stephanie, peruse she did indeed. I perused too, but Stephanie understood the data better, both because of her background in chemistry and because her IQ was noticeably higher than mine.

She read the test results with, I believe, an open mind. She was willing to be convinced that she was wrong. We Googled for more information about every term and every number, and shared web-links with each other, and for a couple of hours we didn't speak much; we just poured over those results and clicked our keypads.

Finally, Stephanie stared at me for what seemed a minute or two but was probably just seconds, and she smiled. I always loved it when Stephanie smiled, but this smile was even more special than most of her smiles. This was the smile of a brainy dame who'd figured something out.

"It's a collection of symptoms," Stephanie said, "more than it's really a disease. The test isn't for calciphylaxis, it's a test for certain symptoms of calciphylaxis, but none of it adds up to an absolute 'yes' or 'no'. It's not like a pregnancy test. It's not like a math test. There's no actual answer here," she said, waving the papers, "only assumptions."

"One factor is, are there calcium deposits in my blood vessels? Yes, there are, and that's a symptom of calciphylaxis — but that's also a symptom of diabetes, and hello? I've had diabetes.

"Another factor is, they measure the chemicals in your blood — calcium, phosphorus, creatinine and albumin, and others, and their presence can suggest calciphylaxis. But you know what? Those are the chemicals in my blood that the kidney doctors are always monitoring. It's an indication of kidney disease, and I have kidney disease.

"Now I have an ugly sore and a collection of symptoms, and the doctors think it all adds up to calciphylaxis, but here's Catch 22 — having kidney disease is one of the key indicators that my symptoms are calciphylaxis. If I had the same symptoms without kidney disease, I don't think they'd be saying these numbers add up to calciphylaxis. There might be another explanation.

"And in my case, there is another explanation — a much simpler explanation. I was at home and in bed for all of September, October, November, and December. You moved the TV into the bedroom. You brought me three meals a day, in bed. You brought me the bedpan, and emptied it and washed it. You cleaned up the bed when I spilled, and by the way, thank you again for all of that.

"But for four months I almost never got out of that bed. No doctor had warned us to watch out for bedsores, and the idea of bedsores never entered my mind, until I noticed this thing on my chest a few days before going into the hospital. I Googled it, and bedsores was an explanation that made sense, and it still makes sense. All the sores line up on one side of my body, and it's the side that I always sleep on.

"Occam's Razor is a rule of logic — when you have two possible explanations for something, the simplest explanation is likely to be the right explanation.

"So do I have calciphylaxis, a disease so complicated that only doctors have heard of it, and there's no definitive test for it? Or, do I have bedsores from being in bed for four months?"

I'll just say this: The doctors told Stephanie that she'd be battling calciphylaxis lesions for the rest of her life. Those ghastly and deadly skin ulcers, they said, would appear at random, repeatedly, and while treatment would help, nothing could prevent new lesions from appearing.

Well, it took a few months of treatment to make the sores on her chest and back disappear, but after they were gone, she never had another. Never.

Have I mentioned that Stephanie loved being right in an argument? She had a pretty good batting average for being right, and in this case she was right again, and the doctors were wrong. Calciphylaxis? Nope. Bedsores.