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Scleroderma: Incurable, Not Untreatable

Scleroderma: Incurable, Not Untreatable

During the winter of 2000, 66-year-old Nancy Bechtle’s hands morphed into alien appendages—blanching and going numb at the merest temperature dip, aching with carpal tunnel pain, ballooning to clumsy paws. Soon her feet followed suit, swelling to the point that donning shoes was impossible. Then, her skin grew hot, stiff and tight. Four months passed before the San Franciscan found a physician who told her that what was sabotaging her appearance, her independence and her outlook was the potentially deadly autoimmune condition called scleroderma.

scleroderma

Tight, thickened skin has curled the fingers of this scleroderma patient.

At that point, Bechtle, who’d been presiding over the board of the San Francisco Symphony for 13 years, went to the heads of three of the nation’s top medical schools to ask for the name of the finest scleroderma specialist in the country. Each gave her the same response: Fred Wigley. More than 1,700 patients have made their way to Wigely, director of the Johns Hopkins Scleroderma Center, in the last dozen years, some from as far away as Iraq. Nearly all arrive in desperation.

As he does with all new patients, Wigley spent two hours with Bechtle, literally drawing her pictures of the disease process as it was affecting her. “One of the most challenging things,” he says, “is to get patients not to talk in terms of their diagnosis and what people have told them about it, but to tell you their experience. Most have mild disease, but the advanced cases get all the press.”

Bechtle found it oddly comforting to hear Wigley explain that—since there is no magic bullet, no single drug or treatment that can sweep away scleroderma’s myriad ills—he would work with a Hopkins team of specialists in every aspect of her disease to fight each symptom as it comes up. “You will get better,” he said.

For Bechtle’s treatment, Wigley selected an immunosuppressant ordinarily used to prevent organ rejection in heart or kidney transplant patients. But for weeks, the drug seemed to have little effect. Then, one day, her husband, Joachim, noticed when he was massaging her hands and feet “that I could squish a little more skin together.”

Soon, she could wriggle her feet into her ski boots. Eventually, it was clear that Bechtle had improved so much that she could go off the drug. Today, even though she is not technically cured, she looks—and feels—her pre-scleroderma self. Which to Wigely is not unexpected. “What is clear,” he says, “is that the key to improvement lies in tailoring care to each person’s symptoms and stage of disease.”