Memories

During the first month on my medicine rotation, I met one of the most memorable patients I have ever known. She was a 23-year-old woman with scleroderma who presented to the ER with nausea, vomiting, and decreased appetite. When I went down there to see her, I practically stopped dead in my tracks. She was a textbook example of all the complications of scleroderma, but I was wholly unprepared for what exactly that looked like.

Cachexia is a poor descriptor of the degree to which she was wasted; she barely weighed 80 pounds. Her skin was pulled taut around what little muscle remained. Her gaunt face was accentuated by a small oral aperture, insensitively termed a “fish mouth.” Contractures deformed her upper extremities, with digital ulcerations affecting the distal tips. Large amounts of calcium had deposited along her arms and knees and they constantly elicited excruciating pain. Her legs were wrapped in gauze and hung over the edge of the bed with a special pillow underneath her knees.

Removing the gauze caused such significant pain that it wasn’t until the next day on rounds that I was able to see what was underneath. Her foot had been amputated and a massive non-healing ulcer had formed around her heel, encompassing the entire stump and delving deep into her flesh. Watching her mother unwrap that dressing was a harrowing, heartbreaking experience for me. Instead of screaming in pain—which would have been easier for me to handle—she simply became quieter as her eyes filled up with tears and her mouth transformed into sobs. And she needed dressing changes twice a day.

But despite all her disease, she was a pleasant and gregarious young girl. She was taking online courses at Arizona State. She had gone on a family vacation to Puerto Vallarta a few weeks prior. For all intents and purposes, she had been leading a relatively normal life. That is, until this hospital stay.

Listening to her heart revealed tachycardia. We tried repleting fluids to counteract her decreased PO intake, but all the extra volume would drain out of her leg ulcer and require TID dressing changes. We gave her a Dilaudid PCA to help resolve any pain that might be increasing her heart rate, but we could neither control the pain nor the pulse. We eventually sent her for a CT angio to rule out PE, which again came back negative. Out of options, we consulted a cardiologist who specialized in scleroderma. None of us were expecting the cardiac MR he recommended to show severe ischemic damage with reduced ejection fraction, but that is precisely what happened.

Earlier in her stay we had consulted plastics to examine her ulcer. They were not impressed by visual inspection, but they recommended an MR anyway. The scan revealed osteomyelitis, but her arterial supply was so severely impaired that she would not tolerate a bone biopsy or surgical debridement. She would need 6 weeks of empiric IV antibiotics and follow-up discussion of a possible above-the-knee am­putation (where her blood flow remained strong) sometime in the future.

To make matters worse, she was started on TPN (a poor prognostic indicator) and was still losing weight. We discharged her home from the hospital on my last day on the floor. She was still nauseated and in severe pain, but she wanted to be with her family for Easter Sunday. We were powerless to make her better. Instead we just made her parents all the more aware of just how sick their little girl was. I felt awful.

I took care of her during my entire month on service and she has stayed with me every day since.