COVID-19: What you should know if you have sickle cell disease

If you have sickle cell disease, you are at higher risk than the general population for severe complications from COVID-19.

"Many patients experience a pain crisis, also known as a vaso-occlusive crisis, early in COVID-19 infection before respiratory symptoms develop," says Jennie Hart, M.D., clinical director of the Department of Pediatric Hematology/Oncology at The Herman and Walter Samuelson Children's Hospital at Sinai.

Dr. Hart explains how COVID-19 can further complicate sickle cell disease, and offers additional measures people with sickle cell disease can take to keep themselves healthy in these times:

Possible effects

A novel virus, COVID-19 can cause severe inflammation and injury to the lungs. This can take a greater toll if you have sickle cell disease, a group of inherited red blood cell disorders.

Symptoms and complications of sickle cell disease are different for each person and can range from mild to severe. However, people with the condition inherited a mutation that causes red blood cells to transform from a round shape into a sickle shape (a process called "sickling"). Healthy, round red blood cells are able to move easily through the blood vessels to carry oxygen to all parts of the body. Sickle-shaped red blood cells, however, are not able to flow through blood vessels as easily: They can get stuck and clog blood flow, which can inhibit oxygen delivery, damage blood vessels and lead to inflammation.

Certain conditions can make sickling worse. Specifically, respiratory infections such as COVID-19 ramp up the formation of sickle-shaped cells because infections in the lungs lead to lower oxygen levels and worse inflammation.

"This increase in sickled red blood cells makes patients with sickle cell disease and COVID-19 infection particularly vulnerable to developing acute chest syndrome, a rapid and deadly lung injury," Hart says.

What you can do

People with sickle cell disease should be more cautious than the general population in adhering to social distancing guidelines, Hart says. The Sickle Cell Disease Association of America advises patients to stay at home unless travel is essential. "Patients who can work from home are strongly encouraged to do so. Your physician can provide a letter to your employer," Hart says.

If you have sickle cell disease, you should call your doctor immediately if you develop a fever or chest pain, have difficulty breathing, or are experiencing a pain crisis. "Close follow-up, either by telephone or frequent office visits, is important," Hart says.

You should take all medications as prescribed, especially if you're taking hydroxyurea, which minimizes certain complications from sickle cell disease. "If you are not taking hydroxyurea and you are interested in learning more about it, schedule an appointment to discuss it with your physician," Hart says.

Be sure to stay well hydrated, wash your hands frequently and avoid close contact with other people who have symptoms of a respiratory infection.

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