Janoi Burgess, who was born with sickle cell anemia, studies in his room. (Liam Crotty for Kaiser Health News)

When Janoi Burgess was a child, he thought doctor appointments were fun.

“I used to love it because they had a section where you could play games,” said Burgess, who was born with sickle cell anemia, an inherited blood disorder. “They were really nice and friendly.”

But when he turned 21, the South Florida resident could no longer go to his pediatric specialist. Instead, he “bounced around” to various primary-care doctors for adults, none of whom seemed well versed in the details of his condition. When he had a painful sickle cell 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 recently recalled. “One doctor even said, ‘Your labs are okay, so you’re not in pain.’ It was crazy and insulting at the same time.”

Burgess’s experience is not unusual among many adults with sickle cell anemia, which affects approximately 100,000 people in the United States, most of them African Americans. 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.

Burgess gets some exercise near his home outside Miami. (Liam Crotty for Kaiser Health News)

“Some people with sickle cell disease are actually living to be elderly, and the majority of patients are adults,” said Wally Smith, a professor of internal medicine at the 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 who were born after 1982 reported that the period immediately after they “aged out” of pediatric care was the riskiest for death. Other research found that Wisconsin Medicaid patients with sickle cell were especially likely to rely on emergency departments for care during this transitional time period.

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.

Compared with other genetic diseases, a disproportionate number of patients with sickle cell rely on Medicaid, the federal-state health program for low-income people. But finding specialists who accept Medicaid patients can be difficult.

There also is an inadequate number of physicians with expertise in the condition. Few adult hematologists — specialists in blood disease — focus on sickle cell treatment, which is less lucrative than that of conditions such as leukemia.

“The number of hematologists available to provide that care is far too small to address the need,” said Michael DeBaun, director of the Vanderbilt-Meharry-Matthew Walker Center for Excellence in Sickle Cell Disease in Nashville.

Sickle cell day hospitals — specialized infusion centers where patients can get intravenous treatment for acute episodes of pain — have been shown to reduce hospitalizations and reduce the length of crises. Yet fewer than a dozen such centers exist nationally.

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 cells lose their characteristic disk shape and morph into rigid crescents, clogging up small blood vessels and disrupting the flow of blood. Nearly a third of adults with sickle cell disease experience pain, often moderately or severely intense, almost every day, and opiates are an important part of managing the condition. Often, physicians and nurses are skeptical of adult sickle cell patients’ motives in asking for pain medication, even though narcotics addiction is no more common in people with sickle cell disease than in the general population.

“There is no disease bigger than sickle cell in terms of bias and disrespect,” said Mary Catherine Beach, a professor of medicine at the Johns Hopkins School of Medicine. “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.”

Silent strokes, which do not cause any obvious sign of injury, also complicate the transition to adult care for some patients. They occur in more than 1 in 5 people with sickle cell by the late teen years. These strokes can lead to problems with understanding and decision-making, preventing effective navigation of a confusing adult health system.

One strategy to improve care is for children with sickle cell to see a family medicine or a “med-peds” physician who can care for them from birth through adulthood. (Med-peds physicians complete both a pediatrics residency and an internal medicine residency, and most take separate exams to become board-certified in both fields.)

But there is a shortage of primary-care physicians generally, and fewer than 400 doctors graduate from med-peds residencies every year, according to Niraj Sharma, who directs the Harvard Brigham and Women’s/Children’s Hospital Boston Med-Peds Residency.

One obstacle to smooth transfers has traditionally been physician reimbursement, said Patience White, co-director of GotTransition.org, a federally funded center that aims to improve the transition process. Her group has been working with medical groups on a new billing code that would allow internists to be paid for communicating with pediatricians and reviewing records as patients transition.

Another barrier for adults has been primary-care providers’ lack of familiarity with routine management of the condition. In addition to their discomfort prescribing narcotics, nonspecialists often aren’t comfortable administering hydroxyurea, a medication that has been shown to reduce painful crises and save lives in patients with sickle cell.

Burgess, now 28, finally found an adult specialist who stabilized his sickle cell, enabling him to complete a college nursing degree. “There are a few nurses who made an impact on my life, and I’d like to add to that,” he said. “I have a need to help, and I feel like I can do it.”

— Kaiser Health News