By Pat Anson, PNN Editor

People with sickle cell disease who are prescribed a corticosteroid – an anti-inflammatory medicine often used to treat pain – are significantly more likely to be hospitalized with a severe pain episode, according to a new study.

Sickle cell disease (SCD) is a genetic disorder that causes red blood cells to form in a crescent or sickle shape, which creates painful blockages in blood vessels known as vaso-occlusive episodes (VOE), which can lead to infections, strokes and organ failure. About 100,000 Americans live with SCD, primarily people of African or Hispanic descent.

“Individuals living with SCD often suffer crippling episodes of pain, which can greatly impair their quality of life,” said Ondine Walter, MD, of Toulouse University Hospital in France, lead author of the study published in the journal Blood.

Walter and her colleagues looked at medical data for over 5,100 patients with SCD in the French National Health Insurance Database between 2010 and 2018. Patients had to have at least one hospitalization for VOE to be included, and their corticosteroid exposure was identified using outpatient prescribing records.

Researchers found that patients exposed to a corticosteroid in the month prior to a pain flare were nearly four times more likely to be hospitalized for VOE than those who did not get a steroid. The median time between filling a prescription for a corticosteroid and hospitalization was just five days.

Nearly half the patients (46%) were prescribed a corticosteroid during the study period, an indication of just how common steroid treatment is for SCD. Walter said the results demonstrate the need for better education of clinicians and patients about the potential risks of corticosteroids, especially when there isn’t a clear reason to use them.

“Based on our data, corticosteroids are commonly prescribed for conditions unrelated to their underlying SCD. Vaso-occlusive events and related hospitalization appear to follow corticosteroid prescription fairly quickly. This evidence suggests corticosteroids may be contributing to the events and should be avoided as much as possible in these patients,” Walter said. “Corticosteroids are mostly easy to avoid, and in circumstances when they are necessary, it’s important to start them in collaboration with an SCD expert and to take all appropriate precautionary measures to administer them safely.”

The American Society of Hematology’s Clinical Practice Guideline recommends against using corticosteroids for acute pain in SCD patients.

The French research team also found that SCD patients taking the drug hydroxyurea had about half the risk of being hospitalized for VOE than those not taking it, which may indicate the drug has a protective effect. Hydroxyurea is often prescribed to SCD patients to reduce the number of pain flares and the need for blood transfusions. Men benefited from hydroxyurea more than women and children.

It’s not uncommon for someone with SCD to visit an emergency room a few times each year due to acute pain or complications such as anemia. Many are disappointed by the experience. A 2021 survey of SCD patients in the U.S. found that nearly two-thirds felt ER staff were rude, ignorant or misinformed about sickle cell disease, didn’t take their pain seriously or believed they were drug seekers.