We are facing a global pandemic as cases of COVID-19, the disease caused by the coronavirus, rise around the world. More severe disease, including respiratory failure and the need for mechanical ventilation, is seen in patients with co-existing illnesses.
Individuals with sickle cell trait or sickle cell disease (SCD) are a particularly vulnerable group of patients, with a higher risk of severe complications than the general public.
Sickle cell disease (SCD) is a group of inherited red blood cell disorders in which the red blood cells become inflexible and sticky and are C-shaped, similar to the farm tool commonly called a “sickle.”
SCD is an inherited disease that most commonly affects people of African descent as well as people from South America, South Asia, Southern Europe and the Middle East. In the United States, the vast majority of those with SCD are African Americans.
Covid-19 pandemic barriers
The COVID-19 pandemic has created barriers that greatly impacted the health and well-being of all children and families. People with sickle sell trait or sickle cell disease are at increased risk for severe complications from COVID-19, an expert says.
Individuals with SCD have a weakened immune system and are more prone to heart and lung co-morbidities, increasing the likelihood of complications from COVID-19.
Respiratory viruses cause more severe complications in people with sickle cell disease, including higher rates of hospitalizations, acute chest syndrome, and need for mechanical ventilation. This is true every year for seasonal influenza and was even more pronounced during the H1N1 outbreak.
We have every reason to believe that the novel coronavirus, which has been shown to cause respiratory failure especially in people with underlying illnesses, will be even more serious for people with sickle cell disease.
Patients with sickle cell disease are at increased risk because they are in more frequent contact with the hospital than other individuals. But some patients still need to be seen for preventative visits or acute crises, thus putting them at higher risk of contracting the virus.
COVID-19 has also negatively impacted the ability of those living with SCD to get regular or emergency care for symptoms and treatment of the disease.
March 2020 was especially concerning, as the systems of care that children, parents and adult patients depended on for acute care, such as hospitals, clinics and emergency department care were affected or changed to serve the surge of patients with COVID-19 complications.
Even when patients were able to access care, the risk of exposure to COVID-19 and fear of getting infected may have prevented or deterred many from getting the care they needed.
COVID-19 can cause severe inflammation and lung injury. And that can have a greater impact on people with sickle cell disease, a group of inherited red blood cell disorders.
People with the disease, which most commonly affects Black people, have misshapen red cells that can get stuck and clog blood flow, inhibiting oxygen delivery, damaging blood vessels and causing inflammation.
Some conditions — including respiratory infections such as COVID-19 — increase 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 said in a news release.
COVID-19 and Sickle Cell: Take These Precautions
People with sickle cell disease should stay home unless travel is essential, according to the Sickle Cell Disease Association of America.
“Patients who can work from home are strongly encouraged to do so. Your physician can provide a letter to your employer,” Hart said. Social distancing is a must, she added.
If you have sickle cell disease, call your doctor immediately if you develop a fever or chest pain, have difficulty breathing, or are experiencing a pain crisis, she advised.
Take all your medications as prescribed, especially if you’re taking hydroxyurea, which reduces certain complications from sickle cell disease, she added.
Stay well hydrated, wash your hands often and avoid close contact with people who have symptoms of a respiratory infection, Hart said.
Family members and caregivers of people with sickle cell disease should take appropriate precautions and extra care to avoid bringing COVID-19 home. They should constantly monitor patients.
They should wear gloves when coming in contact with blood or body fluids of patients with a COVID-19 infection and thoroughly wash clothes and highly-touched surfaces such as counters, doorknobs, bathroom fixtures, and toilets.
If family members and caregivers show symptoms of COVID-19 themselves, they should avoid coming in contact with the patient until the self-isolation period is complete.