People with sickle cell disease, who have a history of severe pain episodes and coexisting organ conditions, have an increased risk for severe COVID-19 illness, a new study suggests. Sickle cell disease is the most common inherited red blood cell disorder in the United States. It affects about 100,000 people, most of them Black or Hispanic. The disease can cause severe pain, joint and organ damage and stroke.
"This study tells us that all individuals with sickle cell disease are not at equal levels of risk," study author Dr. Lana Mucalo, of the Medical College of Wisconsin says. "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 co-morbidities."
"This means individuals with sickle cell disease who come to the hospital with pain should also be tested for COVID-19," Mucalo says.
Previous research has shown sickle cell patients with COVID-19 are at greater risk for hospitalization than Black individuals without the disease who are infected with COVID.
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The new study draws data from SECURE-SCD, an international registry that collects information about COVID infections in people with sickle cell disease.
Researchers assessed reports on 750 children and adults submitted to the registry between March 2020 and March 2021. About half were children. Adults had a median age of 31.
A history of pain events was found to be a risk factor for hospitalization. Kids who had previously experienced more than two pain events requiring acute care were more than twice as likely to be hospitalized for COVID-19. They were more than three times likely to have severe COVID illness.
Adults with more than two prior acute care visits for pain were about two times more likely to be hospitalized with COVID-19 and to have severe COVID-19 illness.
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Heart, lung or kidney conditions related to sickle cell in children were associated with a higher risk of severe illness. Heart and lung conditions in children were also associated with a higher risk of hospitalization.
These conditions did not have the same effect in adults, according to the study, published in the journal Blood Advances.
About half of the patients studied were taking the medication hydroxyurea. In adults, this was associated with a lower risk of presenting with pain during COVID-19. But it did not affect whether an individual would develop a serious case of COVID-19 or need to be hospitalized in children or adults.
"Early in the COVID-19 pandemic, physicians were worried about whether to use hydroxyurea simply because we did not yet know the effects," Mucalo says in a journal news release. "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."
What problems does COVID cause for Sickle Cell patients?
If someone with sickle cell contracts COVID, it can trigger complications because they already have the following underlying conditions:
- Hyperinflammation: When a body’s immune response goes out of control and begins attacking healthy cells.
- Deep vein thrombosis: A blood clot, usually in the legs, but can also happen in the lungs and other veins, that can cause pain and/or swelling.
- Immunocompromised: Having a weakened or impaired immune system to the point where your body cannot effectively fight infection.
- Life-threatening organ damage
Additonally, children with sickle cell have normal symptoms when they come down with the flu or other illnesses. However, if they come down with COVID they can experience life-threatening acute chest syndrome, pain crises or pneumonia. The physiological processes of both COVID and sickle cell overlap. Because of that, patients with kidney or lung damage from sickle cell disease can experience more damage if they contract COVID.
Avoiding COVID
You can avoid what may be a serious case of COVID by trying the following:
- Drink plenty of fluids: Being dehydrated can increase your risk of a sickle crisis. You should be drinking about eight glasses of water a day, more if you are exercising or in hot water.
- Get plenty of rest
- Eat healthy: Make sure you have a diet that consists of lots of fruits, vegetables, whole grains, and protein.
- Exercise: You can aim for two and a half hours of exercise a week. You don't want to overdo it, so you should consult with your doctor about the best routine for you.
- Take your medicine: Make sure you are taking your prescribed medicine as directed by your doctor. You may also talk to your doctor about taking other vitamins that can boost your immune system if appropriate.
- Vaccines: Being up to date on all vaccines (annual flu shot, and pneumococcal and meningococcal vaccines, etc.) can be more crucial for someone living with sickle cell because even a common cold can be really dangerous.
