People with sickle cell disease who take corticosteroids to treat asthma or inflammation may suffer severe pain and even need to be hospitalized, researchers report.
This reaction to corticosteroids can be particularly severe among older people, women and patients not taking the drug hydroxyurea to treat their sickle cell symptoms. These were the most likely to be hospitalized, the study found.
"Individuals with sickle cell should try to seek care with somebody who has some experience with sickle cell or knows about guidelines," said Dr. Lewis Hsu, chief medical officer of the Sickle Cell Disease Association of America, who reviewed the findings.
Current sickle cell guidelines advise against the use of oral corticosteroids, said Hsu, a pediatric hematologist and director of the Sickle Cell Center at the University of Illinois in Chicago.
But, he added, many doctors are unaware of these guidelines unless they regularly treat sickle cell patients, which is why patients themselves should be aware of the dangers of taking oral or IV steroids.
"The reason for the recommendation against steroids is that people have described this pattern of pain," Hsu said. "It actually magically works for a day or two. But then a few days later, bam, the person comes back with pain that's worse and sometimes much worse than they had before. I've had that personally happen with patients who got steroids because that's the standard thing for their asthma."
He added that inhaled steroids to treat asthma are not associated with this rebound pain.
Sickle cell anemia is an inherited disease caused by a change in a gene that signals the body to make hemoglobin, a compound in red blood cells. In sickle cell patients, red blood cells become rigid, sticky and misshapen, according to the Mayo Clinic. Episodes of extreme pain are a major symptom of the disease.
For the study, published April 26 in the journal Blood, a team led by Dr. Ondine Walter of Toulouse University Hospital in France collected data on more than 5,100 French patients with sickle cell disease.
Researchers stressed that this study can't prove that corticosteroids cause pain and hospitalization in sickle cell patients, only that their use is associated with these events.
They found, however, that patients given oral corticosteroids were more likely to be hospitalized for a type of pain known as vaso-occlusive episodes (VOE). VOEs, the most common complications of sickle cell disease, can cause intense pain and irreversible organ damage.
But patients taking hydroxyurea for their sickle cell disease were less likely to suffer pain linked to corticosteroids, the study found. The risk for hospitalization was also lower in men than in women, and lower in children than in adults, researchers said.
The median time between filling a corticosteroid prescription and hospitalization was five days, meaning half of patients were hospitalized sooner and half later. In all, 46% of patients in the French study had been prescribed at least one corticosteroid.
Hsu was surprised by that high percentage.
"Their use of steroids in sickle cell patients was far more than I have ever seen in the U.S.," he noted.
Researcher Walter said the risk of hospitalization was lower in young men and in patients who were also taking hydroxyurea to reduce pain events caused by sickle cell disease and their need for blood transfusions.
"Still, based on these results, we still need to think twice about using corticosteroids when treating patients with sickle cell disease," Walter said in a journal news release.
Walter noted that the study found that corticosteroids are often prescribed for conditions unrelated to a patient's underlying sickle cell disease.
"Vaso-occlusive events and related hospitalization appear to follow corticosteroid prescription fairly quickly," Walter said. "This evidence suggests corticosteroids may be contributing to the events and should be avoided as much as possible in these patients."
Hsu explained that VOE pain episodes usually occur when steroids are stopped abruptly, so when they are necessary, he slowly weans patients off the meds over several days.
"I would give them three or five days and then from that full dose go down and down and down and down with a taper over another five days or seven days," he said.
Hsu said doctors should avoid prescribing steroids to sickle cell patients.
"For asthma, it would be to use more inhaled steroids, which do not give you this problem of pain, and other anti-inflammatories for treating autoimmune conditions that don't involve steroids at all," Hsu said.
Dr. Banu Aygun, a pediatric hematology oncology specialist at Cohen Children's Medical Center in New Hyde Park, N.Y., agreed.
"This study confirms what we have been doing to avoid using steroids in sickle cell disease," she said. "If you have to use it for a medical reason, you have to be careful and use some caution."
Because some doctors may not be aware of the dangers of using steroids with sickle cell patients, Aygun thinks patients need to be proactive.
"Patients should be informed about this, and if they're going to be put on steroids they either have to let their hematologist know or they have to let the treatment team know that steroids shouldn't be stopped abruptly, but they need to be tapered off," she said.
Sickle cell disease affects an estimated 100,000 Americans. One in every 365 Black infants and 1 in every 16,300 Hispanic infants are affected, according to the U.S. Centers for Disease Control and Prevention.
For more on sickle cell disease, see the U.S. National Heart, Lung, and Blood Institute.
SOURCES: Lewis Hsu, MD, pediatric hematologist, chief medical officer, Sickle Cell Disease Association of America, and director, Sickle Cell Center, University of Illinois, Chicago; Banu Aygun, pediatric hematology oncology specialist, Cohen Children's Medical Center, New Hyde Park, N.Y.; Blood, study and news release, April 26, 2022