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Report: Sickle Cell Care Is Hard to Find for Adults

Few physicians are familiar with the disorder

During the past 20 years, advances in routine care have allowed many people with sickle cell anemia to live into middle age and beyond. But early adulthood, a time when patients make a switch from pediatric to adult care, can be perilous for these individuals, according to a report from Kaiser Health News.

Most people with sickle cell anemia are of African ancestry or identify themselves as black. Approximately one in 13 African-American babies is born with the sickle cell trait, and approximately one in every 365 black children is born with sickle cell disease. Up to 100,000 people have the disorder in the United States.

Sickle cell anemia 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 small blood vessels and disrupting the flow of blood.

Nearly one-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 medications, even though narcotic addiction is no more common among people with sickle cell disease than in the general population, according to the Kaiser report.

Compared with other genetic diseases, a disproportionate number of patients with sickle cell anemia rely on Medicaid, but finding specialists who accept Medicaid’s lower reimbursements can be difficult. In addition, an inadequate number of physicians have expertise in the condition. Few adult hematologists focus on sickle cell disease, which is less lucrative than other conditions, such as leukemia.

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 the length of crises, and yet fewer than a dozen such centers exist in the U.S., according to medical experts who have studied them or set up such facilities.

Silent strokes, which do not cause any obvious sign of injury, also complicate the transition to adult care for some sickle cell patients. Such strokes occur in more than one in five people with sickle cell disease by their late teen years, and they can prevent effective navigation of a confusing adult health system, Kaiser notes.

One strategy to improve care is for children with sickle cell disease to see a family or med-peds physician, who can provide care for them from birth through their adult years. Family- medicine specialists complete a three-year residency after medical school that includes rotations in obstetrics and gynecology, surgery, geriatrics, psychiatry, and medicine and pediatrics. 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 fewer than 400 doctors graduate from med-peds residencies each year in the U.S., according to Dr. Niraj Sharma, director of the Harvard Brigham and Women’s/Children’s Hospital Boston med-peds residency. 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, Dr. Patience White, co-director of, told Kaiser Health News. 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 sickle cell patients transition.

Another barrier for adults with sickle cell disease has been primary care providers’ lack of familiarity with routine management of the condition, Kaiser points out. In addition to their discomfort prescribing narcotics, nonspecialists often are uncomfortable administering hydroxyurea, a medication that has been shown to reduce painful crises and save lives in patients with sickle cell disease.

Source: Kaiser Health News; March 22, 2016.

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