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September 20, 2017

Why sickle cell patients face barriers to care: Inadequate training, racial bias, and more

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Inadequate provider training, racism, and the opioid misuse epidemic are creating barriers to needed care for patients with sickle cell disease—at times with fatal consequences, Sharon Begley writes for STAT News.

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Background on sickle cell care

Sickle cell disease is a genetic disorder that affects about 100,000 U.S. patients and is predominantly found in African-Americans. The disease affects a patient's hemoglobin, making it difficult for red blood cells to deliver oxygen to cells throughout the body. It can trigger an acute sickle cell episode, called a vaso-occlusive crisis, which causes excruciating pain by restricting a patient's blood flow and if left untreated can damage vital organs.

Proper treatment typically involves fluids, pain medication, and oxygen, but according to STAT News, patients often struggle to receive adequate care. Data from the American Society of Hematology show the mortality rate for adults with sickle cell disease has increased 1 percent annually since 1979, and half of adults with sickle cell die by their early 40s, according to STAT News.

Keith Quirolo, a retired pediatric hematologist, said every physician who cares for adults with sickle cell patients "knows of one or more who were not treated properly and died."

STAT News examined the factors contributing to the care delays and rising mortality rates. To do so, STAT News interviewed physicians and a dozen U.S. patients with sickle cell disease, including patients who were old, young, male, female, students, employed, and unemployed. STAT News reached out to the sickle cell patients through social media and physicians.

Contributing factors to care delays

STAT News identified three main factors contributing to care delays among sickle cell patients: inadequate provider training, the opioid misuse epidemic, and racism.

Research shows few physicians feel confident treating sickle cell disease, in part because the disease is so complex, requiring physicians to have "knowledge of, and access to specialists in, cardiology, pulmonology, nephrology, and more," STAT News reports.

Paula Tanabe of Duke University said, "[M]ost primary care doctors don't know how to take care of these patients." A 2015 survey of more than 3,000 family physicians found only 20 percent of such physicians said they felt comfortable treating sickle cell disease. And even those whose specialization prepares them to treat the disease, hematologists, often prefer to treat patients with blood cancers, which STAT News reports "is much more lucrative."

As a result, many patients with sickle cell do not receive routine preventive care and end up seeking care in the emergency department (ED). But ED personnel may not have proper training either: Tanabe conducted a study that showed emergency nurses on average scored a 65 on a test about basic emergency care for sickle cell patients.

Yvonne Carroll, director of patient services in the hematology department at St. Jude, said "Hospitalists know a little bit about everything, but with sickle cell, we're talking about a multi-organ disease."

On top of these issues, STAT News reports that the opioid misuse epidemic has made ED providers more reluctant to give out pain medication to sufferers of sickle cell disease.

According to STAT News, providers are increasingly wary of patients who request specific pain medications, make multiple visits for the same complaint, report pain symptoms that do not match an examination, and seek treatment at an ED—all of which are common amongst sickle cell patients.

Alexis Thompson, president of the hematology society, said, "We want to have informed, empowered patients, so we've taught them to be aware of what their medical needs are, what they take, and at what dose," but being aware of the treatment great detail "is perceived as drug-seeking."

Further, research has shown that racial bias may play a role in sickle cell patients' care. Keith Quirolo, a retired pediatric hematologist who used to treat adults with sickle cell disease, said, "If you are African-American and are in the ED for pain, you are almost automatically tagged as drug-seeking," though studies show the rates of opioid misuse are no higher for individuals with sickle cell than people in the general population. In fact, African Americans with sickle cell disease accounted for only 8 percent of opioid overdose deaths in 2015, according to STAT News.

At one hospital, researchers found patients with sickle cell waited 60 percent longer than other patients who reported less severe pain to receive pain medication, and they also often were triaged into a less serious category.  Outside the hospital, the American Society of Hematology found less than a quarter of adult patients in need of pain medication approved to prevent sickle cell crisis receive the treatment.

According STAT News, these delays in care could have fatal consequences, as the number of sickle cell patient dying of chronic kidney disease, organ failure, and sepsis has steadily risen.

Providers caught in tough situation

Some physicians say they find it challenging to ensure they are not fueling the opioid misuse epidemic while also properly treating patients, STAT News reports.

Howard Mell, an emergency medicine physician in Ohio and spokesperson for the American College of Emergency Physicians, said when a patient requests opioids for pain, "I have to verify the need for these powerful medications." Mell said the call can be tough: "99 out of 100 (sickle cell) patients will have a legitimate need (for the pain medication they request), but the 1 in 100 could cost me my license."

Similarly, Jon Hirshon, an emergency physician at the University of Maryland, said, "When a patient tells me that this dose of this pain medicine works for them, I need to have some type of objective information to support that." Still, Hirshon said, "The vast majority of the individuals with sickle cell disease who go to the emergency department get excellent care."

Several ED physicians told STAT News that sickle cell patients are likely to receive appropriate treatment if they bring to the ED their so-called care plans—a document detailing the patient's diagnosis as well as their drug, intravenous hydration, and possibly transfusion treatments in the case of a sickle cell crisis.

Path forward

According to STAT News, physicians and patient advocates are working to address the problems and improve care for sickle cell patients, such as by educating ED staff about the disease and appropriate care. But both patients and doctors said "that the failings in clinical care won't be solved without addressing racial prejudice," STAT News reports (Begley, STAT News, 9/18).

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