Sickle Cell Disease in the Emergency Department: Confronting Barriers to Care

“You’re too pretty to have a disease,” declared the nurse in the emergency department (ED) dismissing Constance Benson’s reports of unbearable pain, a byproduct of her sickle cell disease (SCD). Constance, who is in her late twenties, is a professional actress and model living in Kennesaw, GA.

It wasn’t until Constance’s blood tests came back with information about her blood count that she was treated with the care and attention she deserved. “If I had a broken leg, it would be different. If I was having a heart attack, it would be different. But because I had sickle cell disease and I look like a normal person, my pain was dismissed.”

Constance’s experience in the ED is not uncommon among people with SCD, a condition that causes abnormal, sickle-shaped red blood cells that can block blood flow and limit the amount of oxygen getting to the body’s tissues and organs.

Patients with SCD often seek care in the ED when their health problems cannot be treated at home or when they do not have access to a doctor who specializes in treating SCD. Pain, which can be very severe, is the most common health problem among people with SCD and the top reason for going to the ED.

A Sickle Cell Data Collection (SCDC) program study found that people with SCD living in California go to the ED an average of 3 times a year during their late teens and continue this pattern into their late 50’s.¹ A Tennessee study reported that African Americans with SCD go to the ED two to six times more than African Americans without SCD.²

Patients with SCD frequently have difficulty getting proper treatment when they arrive in the ED. One study found that they wait 25% longer to see a doctor in the ED than do other patients, even though people with SCD tend to have worse pain.³

Once patients with SCD are seen by an ED healthcare provider, they may experience further barriers to care. Examples include being perceived as drug-seeking and being doubted when they report the severity of their pain.⁴

Constance recalls landing her first contract for modeling, a lifelong dream, at age 22. Between doctor appointments, ED visits, and pain crises, a determined Constance made 13 hour road trips to reach her modeling jobs in Philadelphia. “From the time I left to the time I got back, I was in pain, but I had medication and tried to manage it as best as possible,” says Constance. “I was chasing after my dream and I wanted it to be a reality. I could taste it.”

Inevitably, Constance’s pain went from bad to worse. One day while in the hospital, she received a call from her agency about an audition for a runway show. However, her severe pain kept her from making it more than 3 feet from the hospital bed.

Constance remembers her tolerance to pain medications increasing to the point that some became like “Tic Tacs”—not very effective. When Constance asked for help, healthcare workers often failed to treat her pain appropriately. At times, Constance even felt she was perceived as a drug seeker.

Unfortunately, healthcare providers may believe that people with SCD are more likely to be addicted to opioids,⁵ even though data are not available to support this belief.⁶ Patients with SCD may not always appear to be in pain because they have often developed a high pain tolerance due to a lifetime of chronic pain. Yet, they deserve appropriate pain management.

Inaccurate provider beliefs and lack of pain management protocols in the ED may prolong pain episodes for patients with SCD. Research shows that adults with SCD experience significant delay in receiving pain medications when they go to the ED.⁷ In one study, patients with SCD waited an average of 30 minutes longer before receiving pain medications compared to patients with kidney stones, which can cause pain comparable to pain from SCD.⁸

Constance is just 1 of about 100,000 people in the United States who have SCD. Many of these people require care in the ED and they endure inadequate treatment.

Food for thought

Consider reasons why people with SCD experience barriers to care in the ED. How can healthcare professionals, policymakers, and the SCD community improve ED care for people with this life–disrupting disorder?

Thanks to CDC’s Division of Blood Disorders for capturing Constance Benson’s story and providing information about sickle cell disease. For more information about SCD, visit www.cdc.gov/ncbddd/sicklecell.

References

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2. Yusuf HR, Atrash HK, Grosse SD, Parker CS, Grant AM. Emergency department visits made by patients with sickle cell disease: a descriptive study, 1999–2007. Am J Prev Med. 2010 Apr;38(4 Suppl):S536–S541.
3. Haywood C Jr, Tanabe P, Naik R, Beach MC, Lanzkron S. The impact of race and disease on sickle cell patient wait times in the emergency department. Am J Emerg Med. 2013 Apr;31(4):651-6.
4. Haywood C Jr, Williams-Reade J, Rushton C, Beach MC, Geller G. Improving clinician attitudes of respect and trust for persons with sickle cell disease. Hosp Pediatr. 2015 Jul;5(7):377-84.
5. Waldrop RD, Mandry C. Health professional perceptions of opioid dependence among patients with pain. Am J Emerg Med. 1995 Sep;13(5):529-31.
6. Zempsky WT. Treatment of sickle cell pain: fostering trust and justice. JAMA. 2009 Dec;302(22):2479-80.
7. Tanabe P, Myers R, Zosel A, Brice J, Ansari AH, Evans J, Martinovich Z, Todd KH, Paice JA. Emergency department management of acute pain episodes in sickle cell disease. Acad Emerg Med. 2007 May;14(5):419-25.
8. Lazio MP, Costello HH, Courtney DM, Martinovich Z, Myers R, Zosel A, Tanabe P. A comparison of analgesic management for emergency department patients with sickle cell disease and renal colic. Clin J Pain. 2010;26(3):199–205.