Yes, sickle cell is a pain

For most patients, dealing with pain will be a temporary issue. For sickle cell patients, it's a lifetime struggle.

For most patients, dealing with pain will be a temporary issue. For sickle cell patients, it's a lifetime struggle, and many of them come to hospitals and hospitalists looking for help.

In some cases, they come many, many times, causing frustration and misunderstandings. “If you haven't had experiences with sickle cell patients, they start to get labeled as drug seekers or pretending to be in pain,” said Leonard Feldman, MD, FACP, assistant professor of medicine at Johns Hopkins University School of Medicine in Baltimore. “Obviously, that leads to all sorts of bad outcomes for these patients.”


Dr. Feldman and other physicians knowledgeable about sickle cell disease are working to improve these outcomes. A little more information about this complex disease could help both hospitalists and patients come away from their encounters more satisfied, and maybe even reduce the occurrence of them, the experts said.

At Johns Hopkins, the improvement effort is part of the iHOMES (Improving Health Outcomes and Medical Education for Sickle Cell Disease) project, a federally funded initiative to address shortcomings in sickle cell patients' outpatient and inpatient care.

The inpatient component starts right when patients walk in the door. “It's an area that patients with sickle cell disease will point to as a real deficiency in their care—how long it takes someone with sickle cell disease to receive parenteral pain medication when they present with an acute pain crisis,” said Dr. Feldman.

The goal is to get patients these medications within half an hour of presentation. Right now, the Johns Hopkins emergency department is at about two and a half to three hours but is working hard to reduce the wait time.

While it's important to treat pain quickly, hospitalists should not rush to judgment about what's causing a sickle cell patient's pain. “It's true that the most common reason for people with sickle cell disease to be admitted is uncontrolled pain, and most of the time, especially in children and young adults, that pain is a [sickle cell] crisis,” said Kathryn Hassell, MD, a professor of hematology at the University of Colorado.

But, especially in older patients, don't forget to consider other possible causes of pain. “Their back hurts; they feel it's crisis pain. We do an X-ray; they have a compression fracture,” said Dr. Hassell. “Think broadly…that might reduce the likelihood that they come back next time because the compression fracture wasn't recognized or the ovarian cysts weren't treated with appropriate hormonal manipulation.”

The dilemma of diagnosing a sickle cell crisis is that there's no definitive way to do it. “You can't draw a blood sample and say, ‘Oh, you're in crisis' or ‘This is high or this is low, so you're not.’ It's a subjective experience,” Dr. Hassell said.

Therefore, the diagnosis requires trust in the patient's interpretation of his condition. “Know that the patient doesn't need to come looking like they're about to die to actually be feeling pain,” said Dr. Feldman.

Hospitalists should also know that sickle cell patients may require a lot of opioids to make a dent in the pain. “Hospitalists are used to giving fairly modest doses of opioids. They're not so accustomed to giving the larger doses that may be necessary in someone who has had a lot of pain off and on all their life,” said Dr. Hassell.

The fact that most sickle cell patients are African-American may further complicate this issue. “There's a growing body of evidence that suggests that the response to opioid therapy is different in different groups, and that the response is blunted in people of African-American descent. Really, they need more,” Dr. Hassell said.

Treatment needs to be lengthy as well as intensive, given that the average sickle cell crisis lasts at least seven to 10 days. “If they're going home in two days, you say, ‘Woohoo, they don't need medication!’ They do, perhaps for as long as another couple of weeks while the event abates,” said Dr. Hassell.

To reduce the risk of readmissions in those next weeks, the patient needs to be seen by an outpatient clinician. “Make sure they're going somewhere at discharge,” said Dr. Hassell. “They don't necessarily need some fancy hematologist at an academic center. They just need someone who can follow up.”

Bringing sickle cell patients into the primary care system and making sure the system works for them are major goals of the iHOMES project. “We're connecting patients with primary care providers they feel comfortable with, and cultivating a good doctor-patient relationship, but also helping to educate the primary care physicians,” said Rosalyn Stewart, MD, MBA, FACP, an associate professor of medicine who cofounded the iHOMES network with Dr. Feldman.

The education includes both resident training and resources for physicians already in practice, including CME events, webinars (which are available online to physicians outside of the area) and community forums. “If they are seeing a patient and have questions for a hematologist, they can call our hotline,” said Lauren Whiteman, MPH, senior research data analyst for iHOMES.

Even hospitalists who don't have such a resource locally should find a way to call for help when struggling with sickle cell care, experts advised. “You don't have to go it alone,” said Dr. Hassell. “You can seek out people in your institution or your practice—someone who says, ‘I've worked with some people with sickle cell.’ It could be an outpatient doctor who would consult inpatient…but get resources around you.”