For many years, there have been complaints about the care patients with sickle cell disease (SCD) receive in the ED. The reasons for this dissatisfaction can vary, but they tend to range from excessive waits and inadequate treatment to unfounded accusations of drug-seeking behavior.

Sophie Lanzkron, MD, MHS, director of the Sickle Cell Center for Adults at The Johns Hopkins Hospital, notes there are many complicated reasons for such complaints, but they raise valid concerns. “When I first started, I would see patients in clinic and they would be in crisis and having pain,” she recalls. “The only option I had was to send them to the ED where I knew on a good day they would probably wait two and half hours for their first dose of pain medication.”

To address the problem, Lanzkron opened a specialty infusion clinic in 2008, a place where SCD patients experiencing disease-related pain crises could receive rapid treatment. The clinic was well-received, and data appeared to suggest it produced superior outcomes as well, particularly regarding time-to-treatment and hospitalization rates when SCD patients were treated at the infusion clinic vs. the ED.

Lanzkron wanted to gather experts from the existing specialty infusion centers to delineate best practices. However, before anyone would fund such an effort, they wanted evidence showing whether care received in an infusion center was superior to care received in the ED. “The argument was that patients seen in the ED were probably sicker than those seen in the infusion clinic, and that was why there was a difference in [hospital] admission rates. We needed to come up with a study that compared outcomes between these two groups in an ethical way,” Lanzkron explains.

Lanzkron and colleagues identified nearly 500 patients with SCD who lived close to care sites in Baltimore, Cleveland, Milwaukee, and Baton Rouge, LA. At each study site, there was either a specialty SCD infusion clinic or an infusion clinic dedicated to hematology and oncology patients, including those with SCD. All clinics were located in hospitals that maintained EDs. The patients were followed for 18 months, with investigators anticipating each individual would log one or two acute care visits to either a specialty infusion clinic or an ED per year.1

Considering the infusion clinics are open only during weekdays, Lanzkron and colleagues excluded any acute care visits that took place when the infusion centers were not open. Ultimately, there were 1,441 acute care visits included in the analysis. Of these, 241 took place in the ED and 1,200 occurred at an infusion clinic.

Lanzkron and colleagues concluded patients who visited infusion clinics for treatment of SCD-related pain crises received their first dose of intravenous pain medication more than twice as fast as patients who visited the ED. The mean time to medication administration in an infusion clinic was 62 minutes vs. 132 minutes in the ED.

Investigators also found patients visiting infusion centers for treatment of an uncomplicated vaso-occlusive crisis (a painful condition that occurs when sickle-shaped cells block blood flow through the vessels) were much more likely to be discharged home than similar patients who visited the ED for treatment. Specifically, the researchers reported the probability that a patient’s acute care visit would end with a hospital admission was smaller by a factor of 4 when they were treated in an infusion clinic vs. an ED.

While they can point to evidence showing SCD patients treated in a specialty infusion clinic experience much better outcomes than similar patients treated in the ED, Lanzkron and colleagues did not delineate the specific reasons why this is the case. The faster treatment times in the infusion centers could certainly play a role, but so could the fact that patients in infusion centers are cared for by specialists in SCD. Often, these specialists are familiar with the specific patients they are treating. Some or all of these factors may play a role, and other researchers can look more closely at what factors figure most prominently in producing better outcomes.

What can EDs take away from this study, also considering specialty infusion centers generally are not accessible to patients with SCD who live far away from major urban centers? Lanzkron believes care would improve if many more SCD patients were equipped with specific treatment plans written by SCD experts for when they require emergency care. “We as providers of sickle cell care are trying to move in that direction,” she says. “Patients should know that they should seek out an expert in SCD care to be seen. Maybe that means once every year or perhaps once every two years if the patient lives in a rural area.”

Lanzkron notes there is telemedicine capability now, so patients can at least connect at that level with an SCD expert. However, she stresses every SCD patient should be evaluated by an expert in SCD care who can develop a patient-specific treatment plan that can be used in the ED. Without such a resource, caring for SCD patients will continue to be challenging for emergency providers.

Lanzkron works closely with emergency medicine leaders through her participation in the in the Emergency Department Sickle Cell Care Coalition. Affiliated with the American College of Emergency Physicians, this group was established in 2015 in recognition of the fact there is a pressing need to improve ED care for patients with SCD.

“We really wanted to develop a national coalition that is led by and supported by members of the emergency medicine community in order to make progress," explains Patricia Kavanagh, MD, the group’s current chair and an attending physician in the pediatric ED at Boston Medical Center (BMC).

While the group is led by emergency physicians, it includes all the various provider types that contribute to the care of a patient with SCD, including specialists like Lanzkron as well as advanced practice providers, nurses, and pharmacists. Also represented in the group is the patient voice. “This coalition is really pulling together all the voices that really need to be speaking in harmony in order to make change,” Kavanagh says.

One of the biggest obstacles impeding improvement in this area is a general lack of knowledge about sickle cell care among emergency providers.

“Unless somehow in your medical school training you came upon a hematologist who really knew a lot about sickle cell disease, there is really not a lot of training dedicated to this disease,” Kavanagh says.

As a result, when a young adult with SCD visits the ED, providers tend to place these patients behind other older patients presenting with abdominal pain or chest pain. 

“There isn’t that recognition that SCD is a very serious, life-threatening condition. When [SCD patients] are having a crisis of any kind, this could be a huge problem,” Kavanagh says. “There are so many providers who don’t realize that the average lifespan [of a patient with SCD] is in the early to mid-40s. That really has not changed that much over the last 25 years. They are at high risk of comorbidities when they present to the ED. They should be triaged at a high level ... so that they are evaluated quickly.”

Another obstacle that can emerge in the ED is the stigma associated with any painful condition. “Given the shadow of the opioid epidemic that we are all living under, patients coming in with severe pain from SCD are often lumped into the category that they are drug-seeking or they are having a problem with substance abuse,” Kavanagh observes. “This undermines the fact that they are coming in because they need help. They are not coming in because they like opioids or they want them.”

The Emergency Department Sickle Cell Care Coalition is focused on boosting the education of emergency providers so they understand SCD and learn how to properly care for these patients. Much of this education is focused on how to appropriately and quickly manage the pain that brings these patients to the ED so often.

“There are occasionally people [for whom] a hematologist recommends a different pathway with different medicines, but most [SCD] patients really do need opioids. Then, [these medicines] should be repeated every 15 to 30 minutes for as many times as it takes to get some pain relief,” Kavanagh explains.

This level of care is not always easy to accomplish in a busy ED. Some departments have developed processes that can facilitate or streamline the care of SCD patients. For example, the ED at BMC created an order for SCD patients that has both pharmacy and therapeutic approval.

“We write [a prescription] for one medicine, one opioid dose, and then we can repeat it two times as needed every half-hour,” Kavanagh reports. “The physician writes the order once, but then the nurse who is doing all the work in terms of pain assessments and then administration can work more independently and not have to keep going back to that provider.”

Further, the Emergency Department Sickle Cell Care Coalition has just unveiled a point-of-care tool that can guide emergency providers through the proper evaluation and care of a patient who presents with SCD.2 The tool is broken down into several sections with pull-down menus, making it easy for the clinician to access the specific information he or she needs quickly without searching through lengthy guidelines.

In addition, there is guidance provided for every step involved in the ED encounter. The sections cover communication, triage, history, evaluation, treatment, and disposition.

Even for large urban medical centers that operate specialty infusion clinics to provide expert care to SCD patients, both Kavanagh and Lanzkron agree ED providers still can play an important role in SCD care, especially considering most specialty infusion centers are open only during weekdays.

For example, the infusion center at Johns Hopkins Hospital employs advanced practice providers who serve as liaisons to the ED. The two departments have developed a close working relationship.

“As we developed the infusion clinic model here at Hopkins, our ED colleagues were amazing champions for our patients, and we really worked in partnership,” Lanzkron explains. “[Providers in the ED] opened their observation unit so that patients with SCD would get access to beds opened up there.”

The ED observation unit, which advanced practice providers also manage, began to see many of the same patients as the infusion center. Thus, the infusion center staff provided added training to these providers, which has benefitted SCD patients. “We are not open 24 hours a day, but obviously the ED is,” Lanzkron says.

REFERENCES

  1. Lanzkron S, Little J, Wang H, et al. Treatment of acute pain in adults with sickle cell disease in an infusion center versus the emergency department: A multicenter prospective cohort study. Ann Intern Med 2021;174:1207-1213.
  2. American College of Emergency Physicians. Managing sickle cell disease in the ED.