November 10, 2025
5 min read
Key takeaways:
- Patients with sickle cell disease who have vaso-occlusive crises often get delayed treatment in ED due to improper Emergency Severity Index assignment.
- Patients assigned ESI 3 wait three times longer than ESI 2.
Most patients with sickle cell disease who go to the ED for vaso-occlusive crises do not get triaged according to guideline recommendations, resulting in significantly longer wait times for pain medication.
Patients who receive an Emergency Severity Index (ESI) score of 3, instead of 2, can experience a 2-hour delay in analgesia, according to a retrospective analysis.
Data derived from Abu Haimed AK, et al. Blood Adv. 2025;doi:10.1182/bloodadvances.2025016538.
R. Gentry Wilkerson
“These patients have been experiencing episodes of pain their entire lives, and they have different coping mechanisms that they’ve developed over the years,” study author R. Gentry Wilkerson, MD, associate professor of emergency medicine and director of clinical research in the department of emergency medicine at University of Maryland School of Medicine, told Healio.
“They may be experiencing severe pain, but they look relatively well. It’s easy to understand why they may get under-triaged. [However], take their pain seriously. Understand the complications that can result from having sickle cell disease, and do everything you can to optimize their care. Follow the guidelines so that we can treat them appropriately.”
Pain everywhere
More than 100,000 individuals in the U.S. have sickle cell disease, according to study background.
Patients with sickle cell disease frequently experience vaso-occlusive crises, which can manifest as severe pain that can occur anywhere in the body and may require an ED visit.
The overall annual cost of these visits in the U.S. is $1.1 billion.
Abdulaziz Abu Haimed
“They have pain all over their body,” lead author Abdulaziz Abu Haimed, MD, hematology-oncology fellow at Duke University Hospital, told Healio. “This pain is severe and persistent, and patients with sickle cell disease describe it as having a tourniquet cutting the blood supply, not just across your arm, but everywhere.”
ED nurses use ESI to triage patients. Nurses use appearance, pain, ability to walk and talk, and other vital signs to categorize individuals between 1 and 5, with 1 being the most in need of attention.
Patients experiencing a vaso-occlusive crisis should be given an ESI of 2 and pain medication within 30 minutes of triage or 60 minutes of registration, according to National Heart, Lung, and Blood Institute guidelines. American Society of Hematology also recommends analgesia within 60 minutes of arriving at the ED.
However, previous research has shown that patients with sickle cell disease often do not receive that care.
They have 25% longer wait times than other individuals with similar pain scores and receive appropriate ESI scores 10% to 27% of the time. Between 5% and 48% of patients receive analgesia in the recommended timeframe.
The University of Maryland previously had a dedicated nurse for sickle cell disease, but she only worked 40 hours per week, leaving many hours without a specialist.
“We moved to a 24/7 model where patients were going to hopefully receive the same care,” Wilkerson said. “We had guidelines in place, but the one thing we didn’t really focus on during that period was the ESI category. After about 6 months, we realized patients were being triaged as ESI 2 or ESI 3. At that point, I had gotten nurse management to mandate that ESI 2 was going to be used, but we had a 6-month period where it could have gone either way. We did a retrospective review of that period looking at how the ESI category affected the time to analgesia.”
Researchers included 66 ED visits by 41 patients (median age, 31 years; range, 22-77; 58% women) between April and September 2023 in their analysis.
Significant delay
Overall, 65.2% of visits received an ESI score of 3, and 34.8% were assigned a score of 2.
Patients who received an ESI 2 had a significantly shorter median time to first analgesia than those who had a score of 3 (65 minutes vs. 178 minutes; P < .001).
Four patients who were categorized as ESI 3 left without receiving pain medication after a median wait of 349 minutes.
No patients with an ESI of 3 received first analgesia in the guideline-recommended time frame.
Age, gender and sickle cell disease genotype did not impact time to first analgesia.
Time to second analgesia did not differ between ESI 2 or 3.
Researchers acknowledged study limitations, including its retrospective nature, single-center design and short study time.
“We were actually happy that we were able to find that difference, because it gave us the data that we can then go and let our administration, our nurses — everybody — know how important it is,” Wilkerson said. “Every hospital’s different, and every hospital’s got its own nuances of what matters in their care of their patients, and they can’t exactly extrapolate our results to what they have. Hopefully they will say, ‘Hey, this probably is important, and we need to make sure that we’re following those guidelines and giving the ESI of 2 to our patients, as well.’”
‘Simple’ solution
The researchers did not investigate why patients were assigned ESI 3 instead of ESI 2, but Abu Haimed had hypotheses.
“These patients tend to have some sort of biases against them,” he said. “[Someone may think] they are drug-seeking because maybe they are so used to the pain that they don’t show it. They’re saying, ‘My pain is a 10 out of 10,’ but they’re just sitting there because they’re used to a pain of 7 or 8. Some people dismiss them by the way they look, or dismiss them by the way they behave. They might be swirling on the chair, twisting on the bed. Unfortunately, they have biases against them that limit how we look at them and how we manage them.”
Wilkerson called the solution “simple”: Assign everyone with a vaso-occlusive crisis at least an ESI 2 at registration.
“There were studies that looked at the efficacy of just introducing a system for the nursing staff, that when a patient comes in with sickle cell disease, they get a prompt that says assign them an ESI of 2. That increased ESI assignment is around 70%,” Abu Haimed said. “It didn’t reach 100%, but it was a significant and interesting increase.”
Future research could reenforce this message.
Abu Haimed suggested patient-reported outcomes from the ED could be enlightening.
Data on how ESI 2 affects time in the ED and need for hospitalization could be impactful, too.
“I’m a proponent for focusing on policy changes in order to realign what we want, to do what every guideline recommends,” Abu Haimed said. “I think introducing such policies, coupled with education, and correcting any knowledge gaps, really does help in implementing these policies.”
Future research also should focus on treatment for vaso-occlusive crises.
“There are treatments that can reduce the number of vaso-occlusive crises, but nothing really has been proven to reverse it,” Wilkerson said. “There have been a number of studies, a number of drugs that have been looked at, but so far, nothing has really shown benefit. We continue to hope that something is around the corner, but so much more research needs to be done.”
For more information:
Abdulaziz Abu Haimed, MD, can be reached at abuhaimed.aziz@gmail.com.
R. Gentry Wilkerson, MD, can be reached at gwilkerson@som.umaryland.edu.
Sources/Disclosures
Source:
Abu Haimed AK, et al. Blood Adv. 2025;doi:10.1182/bloodadvances.2025016538.
Disclosures:
Wilkerson reports research funding from Becton, Dickinson and Co., CoapTech, CSL Behring, Egetis Therapeutics AB, EndPoint Health, Global Blood Therapeutics, Novartis Pharmaceuticals, Pfizer and Roche Diagnostics; equipment/supplies from Cepheid and Eldon Biologicals A/S; and consulting for National Foundation of Emergency Medicine. The other authors report no relevant financial disclosures.
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