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COVID-19: Sickle Cell Disease Poses New Challenges For Higher Risk Patients, Says Study

According to a study published in the journal Blood Advances, examines the need for COVID-19 risk reduction strategies and vaccination.


For people with sickle cell disease, certain characteristics, including a history of intense pain episodes and concurrent organ disorders, have been observed to enhance the likelihood of severe COVID-19 illness, including hospitalisation (SCD).

COVID-19 risk reduction methods and vaccination for this medically susceptible population are urgently needed, according to a study published in the journal Blood Advances.

In the United States, SCD is the most common inherited red blood cell condition, affecting an estimated 100,000 people.

SCD affects one out of every 365 Black or African American newborns and one out of every 16,300 Hispanic American births, according to the Centers for Disease Control and Prevention.

SCD can result in severe pain, joint and organ damage, and stroke; these factors make people with SCD more susceptible to infections, including COVID-19 infection. Patients with COVID-19 and SCD are more likely to be hospitalised than Black people without SCD who become infected, according to a previous study.

The current study uses information from SECURE-SCD, an international registry that gathers data on COVID-19 infections in people with SCD, including hospitalisation, severity, management options, and consequences.

Between March 2020 and March 2021, researchers assessed information on 750 children and adults who had submitted reports to the registry. Half of the patients they looked at were youngsters under the age of 18, and the other half were adults with a median age of 31. Ninety per cent of the participants were black, while seven per cent were Hispanic or Latino.

Children with SCD who had previously experienced more than two pain events needing acute care were 2.2 times more likely to be hospitalised for COVID-19 and more than 3 times more likely to suffer severe COVID-19 illness, according to the study.

Adults with more than two past acute care visits for pain were 1.8 times more likely to be hospitalised with COVID-19 and 1.9 times more likely to suffer severe COVID-19 disease, according to the study.

In children, SCD-related heart, lung, and kidney issues were linked to a higher risk of severe illness, whereas SCD-related heart and lung conditions were linked to a higher risk of hospitalization. Adults, on the other hand, were not affected in the same way.

Lana Mucalo, MD, of the Medical College of Wisconsin, said, "This study reminds us that all persons with sickle cell disease are not at comparable levels of risk." “Patients with a history of pain, as well as those with concurrent organ diseases, must be considerably more cautious to avoid COVID-19 infection than those who do not have any comorbidities,” Dr Mucalo stated.

Dr Mucalo also stated that now that COVID-19 vaccines are available, physicians and patients alike must evaluate these risk factors while deciding whether or not to vaccinate. “Providers that care for individuals living with sickle cell disease should recommend vaccination, particularly for those with these comorbidities that put them at greater risk.”

The most prevalent complication of SCD is pain, and severe pain is the major cause of emergency room visits and hospitalizations among SCD patients. Pain was also the most common presenting symptom during COVID-19 sickness in both children and adults with SCD, according to this study, and many patients solely had pain as their presenting COVID-19 symptom.

“This means individuals with sickle cell disease who come to the hospital presenting with pain should also be tested for COVID-19,” Dr Mucalo noted.

Hydroxyurea for SCD

The researchers also wanted to know how hydroxyurea, a medicine typically provided to people with SCD to help them have fewer pain episodes, affected COVID-19. Approximately half of the 750 individuals studied were using hydroxyurea, and the researchers discovered that hydroxyurea use was linked to a decreased incidence of discomfort during COVID-19 in adults with SCD.

In children and adults, however, hydroxyurea had no effect on whether they developed a significant case of COVID-19 or needed to be hospitalised.

Dr Mucalo said, “Early in the COVID-19 pandemic, physicians were worried about whether to use hydroxyurea simply because we did not yet know the effects. Now we can see that while it does not affect COVID-19 severity, it does help to lower the incidence of pain episodes in adults with sickle cell disease, so those who are using it for treatment of their sickle cell conditions should not stop using it.”

The study was confined to patient data submitted to SECURE-SCD; due to the registry's voluntary reporting system, it does not include all known cases of sickle cell disease and COVID-19 infection.

(With ANI inputs)

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