Emergency rooms fall short on timely pain management for sickle cell patients | ASH 2025

Adult patients with sickle cell disease are not treated as quickly in the emergency department for pain associated with acute vasoocclusive crisis as children, and more men with the disease are treated faster than women, finds a new analysis presented at the annual meeting of the American Society of Hematology being held in Orlando.

“We concluded that adherence to management guidelines in the emergency department for patients with sickle cell pain crisis is poor,” Ibrahim Gwarzo, Dr.PH., MPH, a research scientist at Nemours Children's Health and a research assistant professor in pediatrics at Sidney Kimmel Medical School at Thomas Jefferson University, said during a press briefing ahead of the meeting.

Gwarzo and his colleagues found that adults consistently had more delayed care, but men with sickle cell received care according to guidelines more often than women, and children received more consistent care than adults.

“We recommend that standardization of the protocols for the management of pain in the emergency department could help in improving guidelines and eliminate these disparities,” Gwarzo said.

Sickle cell disease is a group of inherited blood disorders that affected about 100,000 people in the United States. The disease is caused by mutations in a gene that encodes a key component of hemoglobin, the oxygen carrying molecule in blood. Normal red blood cells are disc shaped but in sickle cell, the red blood cells are a crescent shape. These cells can block blood flow, leading to painful episodes called vasoocclusive crisis.

Two leading guidelines — the 2024 National Institutes of Health's National Heart, Lung, and Blood Institute (NHLBI) guideline and ASH’s own guideline for managing acute pain issued in June 2020 — both recommend that patients be assessed and pain aggressively treated upon arrival at the emergency department.

The guidelines differ on the timing of pain administration and reassessment. The NHLBI guidelines recommend that patients be treated within 30 minutes of arrival at the emergency department. The ASH guidelines recommend that patients be treated within 1 hour of arrival.

The guidelines also differ in the follow-up assessment of patients while they are in the emergency room. The NHLBI also recommends reassessing patients every 15 to 30 minutes and readministering pain medication until pain is controlled; ASH suggests every 30 to 60 minutes.

Gwarzo and his colleagues wanted to assess how well emergency departments were meeting these guidelines for opioid administration of pain. “Despite the publication of these guidelines, there have been widespread delays reported by patients with sickle cell pain crisis when they go to emergency departments waiting for any form of intervention for their pain,” he said.

Researchers for this analysis used the Epic Cosmos database, which contains data from health systems that use the Epic system for managing electronic health records. It includes data from 1,854 hospitals and 300 million patients. They leveraged data from January 2019 to December 2024 and analyzed 398,895 emergency department visits for uncomplicated pain crises that occurred across 233 emergency departments in the United States between 2019 and 2024.

They measured first-dose guideline adherence as the percentage of visits where the first opioid medication was administered to a patient with a pain crisis within 60 minutes of emergency room arrival. They also measured the percentage of visits where the second dose was given within a 30-minute interval of the first and, separately, when the second dose was given within a 60-minute interval.

Researchers found that of the 398,895 visits, 77% of patients required a second opioid dose for their pain. In 32% of those visits, the first dose was given within 30 minutes, and guideline adherence for the second dose was 36%, the researchers found.

“We also found significant variations or differences in guideline adherence based on patient demographics and facility-level factors; patients older than 19 years old had much lower guideline adherence for the opioid medications compared with patients who were 19 or younger,” Gwarzo said.

There was a 30% overall guideline adherence for those over 19, compared with 52% for patients who are 19 or younger. Younger patients seen at pediatric emergency departments, regardless of their age, had 65% guideline adherence, which was higher than patients that were seen at general or adult emergency departments.

The study has limitations, Gwarzo said. Their analysis only considered Epic users; data were retrospective and deidentified and may not be representative of the sickle cell population in the United States.

“We recommend that future studies should specifically be designed to identify specific barriers to guideline adherence that would lead to interventions that would improve guideline adherence and timely management of pain crisis for the sickle cell population,” he said.