Individuals with sickle cell disease face long delays to pain care in emergency department

(Press-News.org) (WASHINGTON — October 8, 2025) – Most individuals living with sickle cell disease who presented to the emergency department with a pain crisis, known as vaso-occlusive crisis (VOC), were not triaged appropriately according to established national guidelines, reveals a study published today in Blood Advances. Patients triaged with a less severe category waited nearly three times as long for their first dose of pain medication when compared to individuals with sickle cell disease who were triaged appropriately.

“Pain is a significant burden in the day-to-day lives of those living with sickle cell disease, with VOC being the number one reason these patients present to the emergency department,” said the study’s lead author, Abdulaziz Abu Haimed, MD, who was an internal medicine resident at the University of Maryland Medical Center at the time of the study. “When these patients arrive, they really need help, and as soon as possible. Our study shows that assigning them an appropriate emergency severity index (ESI) score substantially impacts the length of time it takes for them to receive their first dose of pain medication.”

Sickle cell disease is the most common inherited red blood cell disorder in the United States, affecting an estimated 100,000 people and one out of every 365 Black or African American births. The disorder is characterized by abnormally shaped blood cells that can become lodged in the blood vessels, blocking blood flow and leading to organ damage, infection, and episodes of severe pain, frequently referred to as VOC.

In their study, the researchers analyzed the impact of ESI assignment (the scoring tool used to triage a patient in the emergency department) on the time it takes for a patient with sickle cell disease to receive their first dose of pain medication for VOC. The ESI ranges from a score of 1 (most urgent) to 5 (least urgent), with National Heart, Lung, and Blood Institute (NHLBI) guidelines recommending an assignment of 2 to patients with sickle cell disease. Guidelines from the American Society of Hematology further recommend these patients wait no more than 60 minutes from arrival to receive their first dose of pain medication, with reassessment and re-administration of medication every 30 to 60 minutes, as needed.

“Many patients describe VOC as the worst pain of their lives,” said study author Jennie Law, MD, a hematologist-oncologist and associate professor of medicine at the University of Maryland School of Medicine. “They sometimes compare it to the feeling you’d get from taking a tourniquet to your limb and tightening it.”

The researchers retrospectively identified 125 sickle cell disease-related visits to the University of Maryland Medical Center’s emergency department over a six-month period in response to a change in their organization’s emergency department triage policy that aimed to align with available guidelines. After excluding visits for VOC complicated by other diagnoses that necessitated an ESI 2 and visits for other acute sickle cell disease complications, the study included 66 emergency department visits by 41 patients. The majority of these patients (63.4%) had the most severe variant of sickle cell disease, HbSS, and most patients (58%) were female, with a median age of 33. On a ten-point scale, the median pain score patients reported at triage was nine.

Of these emergency department visits, ESI 2 was assigned to 23 patients (34.8%) and ESI 3 to 43 patients (65.2%). There was no significant difference in gender, pain score, sickle cell disease variant, or age group between patients assigned an ESI 2 or 3.

The median time to first dose of pain medication for individuals assigned ESI 2 was 65 minutes, and the median time for those assigned ESI 3 was 178 minutes, with four such patients leaving the emergency department without receiving pain medication after a median wait of 349 minutes. No patients fell within the recommended time range outlined by NHLBI guidelines.

“A patient with sickle cell disease assigned an ESI 2 compared to 3 is about six times more likely to receive pain medication quicker,” said Dr. Abu Haimed. “Ensuring we assign patients the recommended ESI is one small intervention that can significantly improve the quality of care and overall experience in the emergency department.”

The study has some limitations, mainly due to being retrospective and conducted at a single center. As the study took place in a busy emergency department at a major urban hospital, it may not be representative of other emergency departments across the country. Additionally, to isolate the impact of triage, the researchers defined time to first dose of pain medication as time from triage to receipt of pain medication rather than time from arrival at the emergency department to receipt of medication (as employed by ASH guidelines), which may underestimate overall delays.

The researchers plan to replicate the study’s results with a larger sample size. Additionally, they hope to conduct studies examining the impact of ESI assignment on patients’ overall satisfaction with their experience in the emergency department, as well as how ESI influences the likelihood and duration of hospitalization.

In partnership with the Health Services Advisory Group, ASH developed two facility-level quality measures to improve time to pain management for individuals living with sickle cell disease presenting to the emergency department with VOC, which the Partnership for Quality Measurement recommended to CMS, but was not included in the FY 2025 Hospital Inpatient PPS proposed rule. ASH is continuing to identify pathways to implementing these measures for individuals living with sickle cell disease.

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Blood Advances (bloodadvances.org) is an online, open-access journal publishing more peer-reviewed hematology research than any other academic journal worldwide. Blood Advances is part of the Blood journals portfolio (bloodjournals.org) from the American Society of Hematology (ASH) (hematology.org).

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