Welcome to Study of the Week from Patient Worthy. In this segment, we select a study we posted about from the previous week that we think is of particular interest or importance and go more in-depth. In this story we will talk about the details of the study and explain why it’s important, who will be impacted, and more.

If you read our short form research stories and find yourself wanting to learn more, you’ve come to the right place.

This week’s study is…

Risk of hospitalization for vaso-occlusive episode in patients with sickle cell disease after out-patient exposure to systemic corticosteroids

We previously published about this research in a story titled “Corticosteroids May Increase SCD Hospitalization Risk” which can be found here. This study was first published in the scientific journal Blood. You can view the abstract of the study here.

What Happened?

Sickle cell disease affects around 300,000 newborns annually, with people of African descent seeing the highest rates. A hallmark of sickle cell disease are episodes variously described as pain crises, sickle cell crises, or vaso-occlusive episodes in which the abnormally shaped cells cause blockages in the small blood vessels. This can lead to severe pain and more serious complications, such as organ damage. Some risk factors for these episodes include stress, lung disease, dehydration, cold temperatures, and infection. In several case studies, exposure to systemic corticosteroids, such as prednisone, has been suggested to increase risk of vaso-occlusive episodes. Regardless, the relationship between use of these drugs and the risk of episodes has not been settled. The goal of this study was to evaluate the risk of hospitalization for vaso-occlusive episodes in sickle cell disease patients that were using systemic corticosteroids outside of the hospital setting.

This study used data from the French national health insurance system database and included a total of 5,151 patients who had experience one vaso-occlusive episode from 2010 to 2018. Of this group 317 patients were exposed to systemic corticosteroids in the case period. The study found that patients who had been exposed to corticosteroids in the month leading up to the vaso-occlusive episode were more likely to need hospitalization during the episode.

Remarkably, hospitalization due to a vaso-occlusive event tended to take place soon after the use of corticosteroids began; in fact, the median time was just five days. Another interesting finding was that 46 percent of patients had been treated with corticosteroids at some point during the study period.

Younger patients, male patients, and patients that were taking hydroxyurea, a medicine prescribed in sickle cell disease to reduce the risk of episodes and the need for blood transfusions, were at lower risk, even if they were using corticosteroids. Overall, these findings demonstrate an association between the use of systemic corticosteroids and hospitalization for vaso-occlusive episodes, but do not demonstrate a causative relationship.

About Sickle Cell Disease (SCD)

Sickle cell disease is a genetic, inherited disorder of the blood. This disorder is characterized by an abnormality affecting hemoglobin, which is found in red blood cells and is responsible for carrying oxygen. The abnormality causes blood cells to lose their typical circular shape and instead take on an elongated, sickle-like appearance. This is caused by a genetic mutation that may have arisen as a defense against malaria, although this benefit only occurs in people with sickle cell trait, not the disease. Symptoms begin to appear at around six months old and include swelling of the hands and feet, stroke, bacterial infections, and acute episodes of severe pain termed sickle cell crisis. Severity of disease varies, but these attacks can result in serious declines in health and organ damage. Treatment is mostly symptomatic, but bone marrow transplant has been curative in children. The disease most frequently affects people of African ancestry. Life expectancy is between 40 and 60. To learn more about sickle cell disease, click here.

Why Does it Matter?

Vaso-occlusive episodes are one of the most serious concerns in the management of sickle cell disease. Aside from impacting quality of life through the severe pain that they can cause, they also seriously endanger the patient and can even lead to organ failure. Therefore, identifying potential risk factors for these episodes is important and can help improve the lives of people affected by the illness.

“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.” – Ondine Walter, MD, Toulouse University Hospital

The scientists found that most of the patients that were being prescribed corticosteroids were using them for something other than sickle cell disease. The fact that so many patients were using them indicates that physicians may not be aware of the possible risks:

“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.”