COVID-19 Posing Grim Challenge for Sickle Cell Disease Patients

The coronavirus pandemic has caused tragedy and turmoil for millions of people around the world, but it has been particularly unkind to one group whose health journey is often overlooked: Those with sickle cell disease.

A growing number of studies now suggest that people with this painful genetic blood disorder who also are infected with SARS-CoV-2, the virus that causes COVID-19 disease, are more vulnerable to severe health complications than the rest of the population. They also have higher death rates. It is why more than ever, health experts say, people with sickle cell disease need to be vigilant about protecting themselves.

To underscore the point, one group of U.S. researchers recently established an international registry, called the SECURE-SCD Registry (https://covidsicklecell.org/), to collect data on COVID-19 cases among people with sickle cell disease. The researchers are using it to assess the impact of factors such as genotype, prior sickle cell disease complications, or sickle cell disease related medications, on health outcomes. They say they hope the project will ultimately save lives.

In a recent study, the researchers analyzed March-May 2020 data from 178 people from 22 states who were enrolled in the registry. The researchers showed that 69% of people with sickle cell disease who were infected with coronavirus were hospitalized, 11% were admitted to the intensive care unit, and 7% died, rates much worse than those in the general population who have COVID-19.

“These findings are alarming,” said Julie Panepinto, M.D., the study’s principal investigator and a professor of pediatrics at the Medical College of Wisconsin in Milwaukee. “We’re seeing a much higher death rate than the population at large—perhaps 3 to 4 times higher. It’s becoming clear that if you do have sickle cell and get COVID, you’re likely to have a more severe course for the illness. One of our goals is to find out why.”

The study appeared in the CDC’s journal Emerging Infectious Diseases.  It was supported in part by the National Center for Advancing Translational Sciences, part of the National Institutes of Health.

Sickle cell disease is a group of inherited disorders caused by an abnormal protein in red blood cells. It affects about 100,000 people in the United States and millions worldwide, particularly people of African descent. Complications of the disease include severe pain, infections, organ damage, stroke, and premature death.

Previous studies have shown that influenza symptoms are more severe and hospitalization rates higher among persons with sickle cell disease than those who do not have it. But the virus that causes COVID is not like the virus that causes influenza and appears to have a much more harmful effect on the sickle cell disease population.

In their efforts to understand why, researchers already are noting common trends. For example, over half of the registry patients who are sick with COVID had a history of frequent acute pain crises in the prior three years. About a third report having had a history of acute chest syndrome in the prior three years, a complication marked by fever, chest pain, or difficulty breathing. Whether these factors are simply more common in the sickle cell population in general or represent a key to understanding their risk of having severe COVID complications is something the researchers are studying, Panepinto said.

Also, among the 13 people in the registry who died, the researchers found that, as a group, they had high proportions of certain medical conditions in the previous three years—including pulmonary hypertension, decreased kidney function, and stroke. Of these 13 deaths, eight occurred in those with severe or critical COVID-19. But the remainder surprisingly occurred in those with mild or moderate COVID-19, they said.

“We’re just starting to learn more about the link between sickle cell and COVID and more data is being added each week from healthcare providers from around the world,” said Panepinto, who is an NHLBI grantee and member of the NHLBI Advisory Council. “The more data that gets added to the registry, the more we can learn about this important topic.”

She strongly suggests that people with sickle cell disease closely follow public health guidelines for preventing COVID—wear a mask, wash hands regularly, and observe physical distancing guidelines—to minimize their risk. Avoiding crowds and staying home, if possible, is another good strategy, she said.