When Janoi Burgess was a child, he thought doctor appointments were fun.
鈥淚 used to love it because they had a section where you could play games,鈥 said Burgess, who was born with sickle cell anemia, an inherited blood disorder. 鈥淭hey were really nice and friendly.鈥
But when he turned 21, the South Florida resident could no longer go to his pediatric specialist. Instead, he 鈥渂ounced around鈥 to various adult primary care doctors, 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.
鈥淭hey triage you based on severity, and pain is not something that they consider as severe鈥 as other conditions, he recently recalled. 鈥淥ne doctor even said, 鈥榊our labs are OK so you鈥檙e not in pain.鈥 It was crazy and insulting at the same time.鈥
Burgess鈥 experience is not unusual among many adults with sickle cell anemia, , 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 .
鈥淪ome people with sickle cell disease are actually living to be elderly, and the majority of patients are adults,鈥 said of the Virginia Commonwealth University Medical Center. 鈥淲e don鈥檛 have a health care system ready for that.鈥
Early adulthood, a time when patients make a switch from pediatric to adult care, can be perilous for these patients. A of 940 Dallas people with sickle cell born after 1982 reported that the period immediately after they 鈥渁ged out鈥 of pediatric care was the riskiest for death. Other research found that Wisconsin Medicaid patients with sickle cell were especially likely to during this transitional time period.
Disease Rife With 鈥楤ias and Disrespect鈥
One explanation for the increased deaths could be that early adulthood is a time when the repeated stresses of sickle cell 鈥渃atch up鈥 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聽, the federal-state health insurance program for low-income people, but finding who accept Medicaid鈥檚 lower reimbursements .
There also is an inadequate number of physicians with expertise in the condition. Few adult hematologists 鈥斅燽lood disease specialists 鈥斅爁ocus on sickle cell, which is less lucrative than conditions such as leukemia.
鈥淭he number of hematologists available to provide that care is far too small to address the need,鈥 said Dr. Michael DeBaun, director of the in Sickle Cell Disease in Nashville, Tenn.
In addition, 聽鈥 dedicated infusion centers where patients can get intravenous treatment for acute pain episodes 鈥斅爃ave been shown to and reduce the length of crises. Yet fewer than a dozen such centers exist nationally, according to medical experts who have studied them or set up such facilities.
Pain is a hallmark of sickle cell disease, which is , the protein that allows red blood cells to carry oxygen to the body鈥檚 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. Nearly , often moderately or severely intense, almost every day, and . Often, and are skeptical of adult sickle cell patients鈥 motives in asking for pain medication, even though in people with sickle cell disease than in the general population.
鈥淭here is no disease bigger than sickle cell in terms of bias and disrespect,鈥 said Dr. Mary Catherine Beach, a . 鈥淢any clinicians dislike taking care of people with sickle cell disease because of issues around pain management. When you add in race, it鈥檚 a perfect storm.鈥
, which do not cause any obvious sign of injury,聽also complicate the transition to adult care for some patients. They occur in more than one in five 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.
鈥淚n pediatrics, if you miss your appointment, a nurse will call, or someone might even go to your house,鈥 said Dr. Charles Quinn, . 鈥淚n adult medicine, if you miss your appointment, you鈥檙e on your own. It鈥檚 an entirely different system of health care delivery that happens abruptly.鈥
Easing The Transition
One strategy to improve care is for children with sickle cell to see a family medicine or a med-peds physician, who can provide care for them from birth through their adult years. specialists complete a three-year residency after medical school that includes rotations in obstetrics and gynecology, surgery, geriatrics, psychiatry and medicine and pediatrics. physicians 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,聽and fewer than 400 doctors graduate from med-peds residencies every year, according to Dr. Niraj Sharma, who .
Instead, he said, all pediatricians should start to discuss the transition, including educating their patients with chronic conditions about their illness, at age 12.
One obstacle to smooth transfers has traditionally been physician reimbursement, said Dr. 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 professional societies to propose a new billing code that would allow internists to be paid for the work of communicating with pediatricians and reviewing extensive medical records as patients transition.
Another barrier for adults has been primary care providers鈥 lack of familiarity with routine management of the condition. In addition to their discomfort prescribing narcotics, nonspecialists often administering hydroxyurea, a medication that has been shown to reduce painful crises and save lives in patients with sickle cell.
A team of experts at Johns Hopkins Medical Center, led by Dr. Rosalyn Stewart and Dr. , seeks to deal with such problems by helping doctors improve their knowledge of sickle cell through a .
On another front, Coretta Jenerette, an associate professor of nursing at the University of North Carolina at Chapel Hill, works with patients to help them explain their symptoms so that medical professionals can better understand what they need. 鈥淲hen you come to adult care, there鈥檚 an expectation that you鈥檙e able to give your history and the reason you are seeking care yourself.鈥
Burgess, now 28, finally found an adult specialist who stabilized his sickle cell, enabling him to complete a college nursing degree. 鈥淭here are a few nurses who made an impact on my life, and I鈥檇 like to add to that,鈥 he said. 鈥淚 have a need to help, and I feel like I can do it.鈥
The reporting of this article was supported by a grant from the Ford Foundation.