‘Disrespected’ disease

Sickle cell patients survive despite challenges


MIAMI-DADE COUNTY – When Janoi Burgess was a child, he thought doctor appointments were fun. He was born with sickle cell anemia, an inherited blood disorder.

Janoi Burgess studies in his room at his Miami-Dade County home.(LIAM CROTTY/KAISER HEALTH NEWS/TNS)
Janoi Burgess studies in his room at his Miami-Dade County home.

But when he turned 21, the South Florida resident could no longer go to his pediatric specialist.

Instead, he “bounced around” to various adult primary care doctors, none of whom seemed well-versed in the details of his condition.

Waited for hours
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.

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 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.

Medical costs matter
Compared with other genetic diseases, a disproportionate number of patients with sickle cell rely on Medicaid, the federal-state health insurance program for low-income people, but finding specialists who accept Medicaid’s lower reimbursements can be difficult.

There also is an inadequate number of physicians with expertise in the  condition. Few adult hematologists – blood disease specialists – focus on sickle cell, which is less lucrative than 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 reduce the length of crises. Yet fewer than a dozen such centers exist nationally.

Daily pain
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.

Nearly a third of adults with sickle cell disease experience pain 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 narcotic 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 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.”

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 provide care for them from birth through their adult years.

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 Dr. Niraj Sharma, who directs 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.

Various barriers
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.

Another barrier for adults has been primary care providers’ lack of familiarity with routine management of the condition. 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.

Became a nurse
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.”


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