When Janoi Burgess was a child, he thought doctor appointments were fun. "They were really nice and friendly," said Burgess, who was born with sickle cell anemia, an inherited blood disorder.
But when he turned 21, the South Florida resident could no longer go to his pediatric specialist. Instead, he "bounced around" to adult primary care doctors, none of whom seemed well-versed in the details of his condition. When he had a crisis two years later, his only choice was to go to a hospital emergency department, where, he says, he waited three hours for pain medication.
"They triage you based on severity, and pain is not something that they consider as severe" as other conditions, he recalled. "One doctor even said, 'Your labs are OK so you're not in pain.' It was crazy and insulting at the same time."
Burgess' experience is not unusual among many adults with sickle cell anemia, which affects up to 100,000 people in the United States, most of them black. For many years, most people with sickle cell died in childhood or adolescence, and the condition remained in the province of pediatrics. During the past two decades, advances in routine care have allowed many people to live into middle age and beyond.
"Some people with sickle cell disease are actually living to be elderly, and the majority of patients are adults," said Dr. Wally Smith of Virginia Commonwealth University Medical Center. "We don't have a health care system ready for that."
Early adulthood, a time when patients make a switch from pediatric to adult care, can be perilous for these patients. A 2010 study of 940 Dallas people with sickle cell born after 1982 reported that the period immediately after they "aged out" of pediatric care was the riskiest for death.
One explanation for the increased deaths could be that early adulthood is a time when the repeated stresses of sickle cell "catch up" with the body. But social and health system factors also play an important role.
There also is an inadequate number of physicians with expertise in the condition. And finding specialists who accept Medicaid's lower reimbursements can be difficult.
In addition, sickle cell day hospitals — dedicated infusion centers where patients can get intravenous treatment for acute pain episodes — have been shown to reduce hospitalizations and reduce the length of crises. Yet fewer than a dozen such centers exist nationally, experts said.
Pain is a hallmark of sickle cell disease, which is caused by abnormal hemoglobin, the protein that allows red blood cells to carry oxygen to the body's tissues. Under certain conditions, these affected red blood cells lose their characteristic disk shape and morph into rigid crescents, clogging up small blood vessels and disrupting the flow of blood.
"There is no disease bigger than sickle cell in terms of bias and disrespect," said Dr. Mary Catherine Beach, a professor of medicine at Johns Hopkins. "Many clinicians dislike taking care of people with sickle cell disease because of issues around pain management. When you add in race, it's a perfect storm."