Being Difficult

Maryland pediatrician Daniel Levy is among them. Levy said that a few weeks ago he received an emergency page on a Sunday afternoon from a mother of three who said that her youngest, a 2-year-old, had a fever and that the pain reliever was wearing off after four hours, not six, as the label indicated. The child was otherwise healthy and did not seem agitated or lethargic -- two danger signs -- and Levy knew that the mother had faced similar situations with her older children without a problem.

"Everyone in my practice knows that when they get an after-hours page [from certain people] it's not an emergency; the parents are decompensating," said Levy, who gave the mother the same simple instructions he had when her older children were ill. Later that evening, he called to see how things were going.

"I knew the kid was fine, but I knew that a strategic call to the mother would let me see how she was doing," he said.

Mayer, the scleroderma patient, said that in her experience, such demonstrations of concern were rare.

Living in the South, she said, she quickly learned to tone down what she calls her native Philadelphia feistiness.

For months after she first developed symptoms, she said, she was told she did not have scleroderma, although she was sure she did. One specialist told her to take a chemotherapy drug that had been found to be ineffective against the disease in randomized clinical trials; when she asked about this, he brusquely rebuffed her inquiry. Another specialist told her she was likely to become "very sick, very fast." A third spent less than 10 minutes with her before telling her that although she had flown hundreds of miles to see him, there was nothing he could do. "I have a waiting room full of patients," he said, visibly irked when she persisted in asking a question.

"All this expert advice left me feeling as if I should lie down and die," she wrote.

After much trial and error, and with the support of her husband, an infectious-disease specialist, she found a rheumatologist 200 miles from her North Carolina home, and a sympathetic family practitioner nearby who helped coordinate her care.

"I have found that those providers who saw me as a partner rather than a passive recipient of their knowledge and advice were the most supportive and helpful," she said. Mayer said she wrote the Health Affairs essay out of frustration and as a plea to physicians to listen to patients.

In the end, although Mayer derived strength from doing things her way, which may have prolonged her life, her disease won. She entered the Duke hospice in late September and died there on Oct. 11, her 11th wedding anniversary. A few days before her death she left the hospice briefly to spend time at home with her children, ages 7 and 9.

"She was just trying to live the best she could as long as she could," said her husband, William Stainbach. "It's more difficult if you have a rare disease, but she found doctors who would work with her."

Shortly before her death, Mayer said in an interview that she worried about the ordeal experienced by other patients, difficult or not.

"We speak English, have great insurance, money, tons of education and my husband has connections, and we couldn't get the system to work," she said. "I fear what happens to other people."

Comments:boodmans@washpost.com.