15 July 2021

Certain risk factors have been identified that increase the chances of people with sickle cell disease developing severe COVID-19.

Newly-published research in Blood Advances suggests there is a need for COVID-19 risk reduction strategies and vaccination for this medically vulnerable population.

Previous research has shown people with SCD who develop COVID-19 have an increased chance of being hospitalised, compared with Black individuals without SCD who become infected. This is because the condition can cause severe pain, joint and organ damage, and stroke, which predispose people with SCD to worse outcomes with COVID-19, the researchers say.

The new US study analysed data from the SECURE-SCD international registry, which collects information about COVID-19 infections in individuals with SCD and includes details on hospitalisation, severity, management strategies, and complications.

Researchers assessed the records of 750 children and adults who were added to the registry between March 2020 and March 2021. Half the patients they studied were aged 18 and under, and half were adults with a median age of 31 years, while 90% were Black and 7% were Hispanic or Latino.

The team found that under 18s who had previously suffered more than two SCD pain events that needed acute care were 2.2 times more likely to be hospitalised for COVID-19 and over three times more likely to suffer severe COVID-19.

A history of pain events was also found to be a risk factor for adults. Those who had more than two prior acute care visits for pain were 1.8 times more likely to be hospitalised with the virus and 1.9 times more likely to suffer severe illness.

SCD-related heart, lung, and kidney conditions were associated with higher risk of severe illness in children, while SCD-related heart and lung conditions were also associated with higher risk of hospitalisation. However, these conditions did not have the same effect in adults.

The study also found that pain was the most common presenting symptom during COVID-19 illness in both children and adults living with SCD, and that many patients only had pain as their presenting COVID-19 symptom.

Study author Dr Lana Mucalo, of the Medical College of Wisconsin, said: “This means individuals with sickle cell disease who come to the hospital presenting with pain should also be tested for COVID-19.”

The researchers found that hydroxyurea, which was used by about half of the 750 patients studied, was associated with lower risk of presenting with pain during COVID-19 in adults with SCD. However, hydroxyurea did not affect the risk of developing a serious case of COVID-19 or having to seek hospital treatment.

“Early in the COVID-19 pandemic, physicians were worried about whether to use hydroxyurea simply because we did not yet know the effects,” said Dr Mucalo.

“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.”

She added that the study reveals that people with sickle cell disease are not at equal levels of risk.

“Patients with a history of pain, as well as individuals with coexisting organ conditions, need to be even more careful to avoid COVID-19 infection than those without any comorbidities,” she said.

Dr Mucalo added medical teams looking after individuals with SCD should recommend COVID-19 vaccination, particularly for those with these comorbidities that put them at greater risk.


Mucalo L, Brandow AM, Dasgupta M, Mason SF, Simpson PM, Singh A, Taylor BW, Woods KJ, Yusuf FI, Panepinto JA. (2021) “Comorbidities are risk factors for hospitalization and serious COVID-19 illness in children and adults with sickle cell disease.” Blood Advances, doi: 10.1182/bloodadvances.2021004288

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