Experts Offer Roadmap for Improving Care of Patients with Sickle Cell Disease in the ED

By Dorothy Brooks

When a patient with sickle cell disease (SCD) presents to the ED, it is likely because he or she is experiencing excruciating pain from a vaso-occlusive episode — a condition that occurs when sickle-shaped red blood cells get stuck while traveling through small blood vessels, impeding blood flow. Such a patient generally will require opioid infusions to relieve the pain, but experts tell EDM that too often such care is delayed or even denied due to several factors, which may include a lack of knowledge about SCD, stigma, and/or the fact that the treatment required for this condition is under considerable scrutiny because of the country’s opioid epidemic.

A big part of the problem is that SCD is a relatively rare condition, affecting about 100,000 Americans who are primarily of African American and Hispanic descent, according to data from the Centers for Disease Control and Prevention.¹ “If you live in an area that is not diverse, you may not see a lot of patients with sickle cell disease,” explains Paula Tanabe, PhD, MSN, BS, a professor of nursing and medicine, and the vice dean of research and regulatory affairs at Duke University School of Medicine. “When there aren’t many patients, you don’t learn as much in your preparation as a physician or as a nurse.”

However, in areas that lack access to hematologists — the specialists who typically oversee the care of patients with SCD — the burden often falls on the ED to respond to vaso-occlusive pain episodes as well as other complications that can result from SCD, observes Tanabe.

To help EDs improve their performance in caring for SCD patients, Tanabe has worked closely with the Emergency Nurses Association (ENA) to make sure education on SCD is prioritized and distributed to members. Such education can become crucial very early in a patient’s ED encounter. For instance, she notes that SCD guidelines state that patients who present with vaso-occlusive pain crises should be categorized at triage as Emergency Severity Index (ESI) Level II, making expeditious care a high-level priority.

“Triage nurses should also pay more attention to anything else that might raise the index of suspicion that an SCD patient might be experiencing more than a pain crisis,” explains Tanabe. “Any abnormality in a vital sign is a super red flag in a sickle cell disease patient.”

This is important because patients with SCD can present with many complications, such as acute chest syndrome — a severe complication of SCD — sepsis, or strokes. In any of these circumstances, the triage nurse needs to make sure that care is facilitated as quickly as possible, notes Tanabe.

Regarding to the more common vaso-occlusive pain episodes, the SCD guidelines stipulate that pain medication should be administered within 60 minutes, explains Tanabe. However, she acknowledges that this goal has become increasingly difficult to meet in recent years. “The biggest barrier right now is that [ED] overcrowding is the worst it has ever been, so it is very difficult to find a place to put people to treat them,” she says.

To address this dilemma, Tanabe encourages ED leadership to find a way to administer opioids to SCD patients in the waiting room when necessary. “Make sure your policies allow the triage nurse to do that,” she says. “The takeaway is to facilitate care as quickly as possible, and usually that care is pain medication, and a lot of times it is opioids.”

Tanabe advises hospitals to form a multidisciplinary team for SCD that can help to make sure that appropriate resources are in place to expedite care to SCD patients. For example, one of the big problems that SCD patients report when presenting to the ED with pain crises is that frontline clinicians who are unfamiliar with SCD may view their complaints skeptically, thinking that they are exhibiting drug-seeking behavior. However, Tanabe notes that many such encounters can be avoided if the multidisciplinary team has taken steps to ensure that SCD patients have individualized pain plans embedded in their electronic medical record (EMR).

What the multidisciplinary team can do is ensure that SCD patients who present to the ED are connected with a hematologist, who then can devise a pain plan that includes individualized dosing recommendations. “With such a plan in place in the EMR, when the patient presents to the ED with a vaso-occlusive pain crisis, emergency providers will know how to treat the patient,” states Tanabe. “They won’t have to worry about whether a dose is too high or too low because the outpatient team has already determined [what dose is appropriate and effective for this patient].”

Another task that a multidisciplinary SCD team can take charge of is identifying an ED-based SCD nurse champion who can not only be a resource for colleagues when a patient with SCD presents, but also can deliver SCD education to staff and conduct SCD-related quality improvement work in the ED.

Tanabe pioneered the SCD nurse champion program at Duke University Hospital more than a decade ago. While the program now develops SCD nurse champions for positions throughout the hospital, the program started with the ED.

“I would provide some workshops and support for [the nurse champions] on sickle cell disease, but then their responsibility was to make sure that as new nurses came into the emergency department, they would get some kind of training on SCD so that they would understand the disease better,” states Tanabe.

Further, in addition to being a resource for colleagues with less knowledge about SCD, the nurse champions can perform audits to assess performance on key quality indicators regarding SCD care. “For example, the nurse champion can do an audit to assess what proportion of SCD patients were triaged correctly as ESI level 2 patients,” states Tanabe.

Unfortunately, the Centers for Medicare & Medicaid Services has not yet established any reportable quality indicators related to SCD, so incentives are lacking for hospitals or EDs to prioritize this kind of data gathering, acknowledges Tanabe, but she notes this is just one of many ways that SCD nurse champions can make a difference in education and quality improvement.

To be effective, though, SCD nurse champions need to be given the time and opportunity to further their education about SCD, and they need the full support of hospital and ED leadership to carry out their work, stresses Tanabe.

For example, at Duke University Hospital, where nurse champions are placed throughout the institution, including the ED, there is a new sickle cell champion committee that oversees the program, with Tanabe serving as a faculty advisor.

T. Caleb Rutan, MSN, APRN, ACCNS-AG, CEN, CPEN, the current chairman of the champion committee and a clinical nurse specialist in the ED, tells EDM that the nurse champion program has dealt with a number of challenges in recent years with strains from the pandemic and the high number of nurses who have left the profession. However, he says that there is a solid base of education and support in place for the SCD nurse champion candidates. This includes didactic education about SCD, much of which was developed by Tanabe, as well as education that focuses more on interpersonal relations when caring for patients who are dealing with SCD.

“We know there are significant health disparities in the populations that intersect with SCD, along with socioeconomic factors, so there is a lot more than just the disease that you need to understand in order to really take care of these patients in a way that is optimal,” states Rutan.

For instance, many SCD patients have neurocognitive deficits that may affect their executive function or the way they express emotions, according to Tanabe. Patients with SCD also often face challenges related to social determinants of health, such as housing and food insecurity.

Another aspect of the nurse champion program that Rutan intends to focus on in the coming months is how to train the champions to be effective communicators when addressing their peers. “One of the things that we’ve identified is that just because someone is a nurse doesn’t necessarily mean that they’re prepared to speak with their colleagues in a more formal setting,” he says. “We want them to be able to go out and maybe take 10 to 15 minutes at a staff meeting to provide an overview on sickle cell disease.”

Rutan says that the goal for nurse champions is not only to take good care of patients with SCD, but also to “proselytize” about the appropriate care of SCD patients to the rest of the healthcare system.

Rutan acknowledges that the Duke Health System has a robust array of resources for SCD patients that other clinicians may not have access to in their own systems. “We’ve got a great outpatient system set up to help with the care of patients dealing with SCD … and we have in-house pain management teams that see these patients,” he says.

Even in less-resourced settings, however, SCD nurse champions still can go a long way toward ensuring that SCD patients who present to the ED are treated with the care they deserve. In particular, Rutan’s advice to ED leaders who recognize the need for improvement with respect to this population is to take what SCD patients are telling you at face value. “There is this divide between the behaviors we expect to see vs. what we might see in a population of patients that regularly deals with acute pain,” he says. “Make sure that your staff understand that the presentation of these patients may not be what you classically think of as having acute, debilitating pain. Take them at their word if they say their pain level is a 10.”

Further, Rutan reiterates Tanabe’s point that emergency staff need to understand that patients with SCD are at risk for all kinds of complications and comorbidities. “Even with patients that you may see frequently, every time they come in, you have to assume the worst and then kind of go from there,” he says. “Nine times out of 10, it is probably going to be [a vaso-occlusive pain crisis], but there is always that one [complication] that you don’t want to miss.”

More generally, Rutan stresses ED leaders need to make sure that staff never become jaded with a patient population that has very high needs. “These are individuals who can take up a lot of resources; it is not always easy to take care of patients who are chronically dealing with sickle cell disease.”

Jontue Hinnant, RN, BSN, was one of the first ED-based SCD nurse champions at Duke University Hospital, although she has since moved on to the Baltimore-based University of Maryland Medical System where she continues to serve as an unofficial nurse champion for SCD care in the multiple EDs where she spends time. She tells EDM that she still sees plenty of opportunities to intervene when a patient with SCD presents for care.

For example, Hinnant recalls the recent case of a young man who had been complaining about pain for hours when she arrived on shift to the ED in the morning. “They were going to initially discharge the patient with a prescription, but the patient was not housed, so they had to wait until the morning for a social worker to come in,” explains Hinnant.

However, Hinnant quickly realized that the patient had been requesting medication for pain since 11:30 p.m. the previous evening, but the nurse on staff at the time had not contacted the physician in charge of the patient’s care. Hinnant apologized to the patient who, by that time, was in tears. She also reached out to the physician.

“He said he didn’t know that the patient was still having pain, and quickly ordered pain medication,” observes Hinnant. The physician then admitted the patient, concluding that he should not be discharged if his pain was not resolved.

Hinnant shares that she had a candid conversation with the nurse she took over from that morning about SCD and how patients with the disease present with excruciating pain crises that require infusions of opioid pain medication. She also tells EDM that anecdotes like this one are not uncommon. “Unfortunately, those [types of incidents] are still happening where people are very dismissive,” she says. “It’s very disheartening and upsetting.”

Hinnant’s advice to ED leaders and SCD nurse champions is to make SCD education a priority with new nurses just coming on board to work in the ED. “I think one of the main things that probably makes the biggest difference is to deliver this education during the initial orientation,” states Hinnant. “This is one of those topics that needs to be covered as far as education about the SCD disease process, what the complications are, and why SCD patients come to the hospital.”

Hinnant already had a strong interest in SCD when she was first tapped to be an ED-based nurse champion because a sibling of a good friend had SCD, and Hinnant also had a co-worker with SCD when she was in high school. At Duke, she was given the time and opportunity to become knowledgeable about the disease and to advocate for patients with SCD. This kind of support from leadership is critical to an effective champion program, whether that involves attending conferences focused on SCD or being given the time to provide lunch-and-learn sessions to the rest of the staff, she says.

“The more the champion knows and the more [he or she] brings back to the organization, the more beneficial that champion will be,” says Hinnant.

Editor’s note: Multiple groups have developed SCD-related tools, strategies, and education specifically geared toward frontline providers. The Emergency Department Sickle Cell Care Coalition (EDSC³), which was formed in 2016 and is led by the American College of Emergency Physicians, produced a decision-
support tool that can help guide frontline clinicians in caring for SCD patients who present to the ED. This tool and many other SCD resources from the group can be accessed here: https://www.acep.org/by-medical-focus/hematology/sickle-cell

There is also a wealth of ED-focused information about SCD care available through a website developed by Tanabe and her colleagues at Duke University School of Medicine. The site includes links to an SCD provider network and other organizations that figure prominently in the care of SCD patients. It also provides an array of SCD treatment algorithms and information about educational conferences focused on SCD care. Access the site here: https://nursing.duke.edu/scd

Organizations looking for guidance on how to develop an effective SCD nurse champion program are welcome to reach out to Tanabe. Her contact information is here: https://medicine.duke.edu/profile/paula-j-tanabe

REFERENCE

1. Centers for Disease Control and Prevention. Data & statistics on sickle cell disease. Last reviewed July 6, 2023. https://www.cdc.gov/ncbddd/sicklecell/data.html