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The days when infant sickle cell patients simply caught cold and died without even being diagnosed are mostly, though not entirely, in the past. Befitting his profession, Lorenzo and his wife, Veronica, 49, have taken no chances in Zakee's care. They brought their only son up with the conviction that sickle cell could be survived if its dangers were respected, and made sure he avoided cold drafts, hard exercise and other behaviors that put patients at risk for pneumonia, pain crises and other ravages of the disease. But there were things even they couldn't control. Around the time his father began peeking into the history of the family's sickle cell genes, Zakee, a seriously thin, bespectacled youth, left the pediatric clinic and entered the world of adult care. And that, say advocates of patients with chronic illnesses, is a dangerous place to be.

Washington Children's Hospital Center had been Zakee's home away from home from the time he could crawl. Children's coddled its young charges with social workers and play rooms, treats and family services. The adult clinic at the hospital where Zakee goes now has none of that. To be sure, Zakee had the good fortune to be living at home, comfortably, in a small subdivision in the countryside south of D.C. He was under the protection, as a college student, of his father's excellent government-issued insurance. But the future loomed menacingly. Once he entered the workplace. his "pre-existing condition" would be an albatross, warning away prospective employers leery of jacked-up insurance premiums.

Sickle cell disease is a genetic illness characterized by fragile, crescent-shaped red blood cells that are inadequate oxygen carriers and cause painful clogging of the blood vessels. The adult sickle cell patient's world is laden with obstacles -- unpredictable, wrenching pain episodes; complications of the spleen and liver; leg ulcers and hip replacements; stroke. Worst of all, for many patients, is the vulnerability -- the fear of being forced to enter a strange emergency room whose staff, unfamiliar with sickle cell, are skeptical of the typical patient's need for large doses of powerful narcotics like morphine, Demerol and Dilaudid.

The Nicholses were taking no chances. "I'm studying business," said Zakee, now 19, "because I'm going to have to be independent, and well off."

Increasingly, as science gains new footholds in its comprehension of genetic disease, patients with inherited disorders are surviving well into adulthood. Sickle cell and cystic fibrosis are two good examples. In a sense, the diseases are shadow images of each other: Both are caused by mutations in a single gene that, in our evolutionary past, conferred advantages: A single sickle cell gene protects the carrier against malaria; a single cystic fibrosis gene helps stave off cholera. But until the last few decades, children who inherited copies of the mutated sickle cell or cystic fibrosis genes from both parents did not survive. Credit molecular medicine with changing that. Today, the 90,000 or so Americans with sickle cell have a median life expectancy of about 45; the 30,000 cystic fibrosis patients, on average, will live into their 30s -- and beyond, if new treatments live up to their promise.

But in the era of cost-cutting managed care, the world in which these patients survive is not all that welcoming. These patients are at the crux of the central paradox of American medicine -- increasingly sophisticated diagnosis and treatment, and increasingly short funds to pay for them. The 20 or so top sickle cell centers across the country are struggling financially because of low reimbursement from insurers for the myriad services required to take care of this chronic disease. Patients too poor to afford good private insurance (or the supplemental care required) find themselves switched from doctor to doctor, struggling to get referrals to specialists who know how to take care of them. When their regular care suffers, they end up being treated in the emergency room, and suffering unnecessary complications.

Next page | Some drugs cost up to $50,000 a year for one patient's treatment