Adults living with sickle cell disease are underserved, according to Carlton Haywood Jr., an associate professor at the Johns Hopkins School of Medicine who lives with the disease.

The tall, lanky patient enters the room bent over and shuffling like a man twice his age. He climbs on a gurney and lies back, head throbbing. Then Carlton Haywood pulls out a bottle of Tums, relief-in-waiting for the nausea he fears will come.

"You never know how bad it's going to get," he says with a cordial smile.

Haywood, 37, belongs to the 0.003 percent of the U.S. population that suffers from sickle cell disease, which predominantly affects blacks and which he has battled since birth. A health care team at the Johns Hopkins Outpatient Center is about to remove 75 percent of his blood and replace it with donated units, a procedure he undergoes monthly.

As rare as the blood disorder is, Haywood's situation is even rarer. A bioethics and hematology professor at the Johns Hopkins University, he's one of the few academicians who root a career studying the illness in personal experience.

Haywood the scholar knows Haywood the patient, a fact that imparts a special meaning and power to an often lonely mission.

"There's so much Carlton brings to our work that no one else can," says Dr. Sophie Lanzkron, the director of the Adult Sickle Cell Clinic at Hopkins. "He has significant illness, lives with this discomfort every day and manages it incredibly well. It's one reason he knows as much about [sickle-cell] as anybody I've met."

On this June morning, Haywood reclines in fatigue as the bad blood flows from his body and the good enters through tubes in his chest. If he's lucky, he'll be well enough next week to return to a schedule of lecturing, writing and researching the state of sickle cell treatment in the U.S.

On that last subject, he turns learned professor.

Most sufferers, Haywood says, have horror stories of locking horns with their health care providers. Many in the general population still believe the "black disease" they heard so much about 40 years ago has been cured. Worse, funding for research is 1/300thsof that available for comparable genetic illnesses.

"Sickle cell disease represents a uniquely bad confluence of problems," he says in a voice far gentler than his disorder.

Haywood pops a Tums.

"We have a long way to go, mm-hmm," he says

A full-blown case

Medical historians have traced sickle-cell disease to four regions of Africa, where they say it developed thousands of years ago and was first described in medical literature in 1870. Today it affects about 100,000 people in the United States, nearly 3,300 of them in Maryland.

It strikes individuals of many ethnicities, including Caucasians, though more than 90 percent of sufferers are black.

The medical system calls it an "orphan disease" — that is, like muscular dystrophy, cystic fibrosis and other disorders, it affects fewer than 200,000 people. But to those who have it, the numbers are more than big enough. And the problems start early.

Haywood was born in Atlanta, and for the first three years of his life, he screamed so often and so terribly that his parents, Carlton Sr., and Harriett Haywood, thought they must be doing something wrong. By 1979, when his sister Tammy was born, they had moved to Alabama, a state that mandated sickle-cell testing for all newborns. She didn't have the disease but carried some of its traits, so doctors suggested they test their son.

The results were "devastating," says Harriett, now 61 and living in Columbia, S.C. "The doctors told us Carlton wouldn't live through childhood. It was very, very difficult to deal with."

The condition refers to misshapen red blood cells, according to the National Institutes of Health.

Normal red blood cells are doughnut-shaped and malleable, and contain hemoglobin, a protein that allows the cells to carry oxygen throughout the body. Hemoglobin-A — the normal kind — is ball-shaped, allowing the cells to move smoothly around even the sharpest turns in the body's vascular system, reaching every organ.