What to Know About Thalassemia and COVID-19

Thalassemia is a hereditary disorder that affects the production of hemoglobin. Hemoglobin is the component of red blood cells that carries oxygen to the tissues of the body. There are many types of thalassemia. Symptoms can range from none at all to severe anemia that requires frequent blood transfusions.

At this time, scientists are just beginning to learn how a diagnosis of thalassemia may affect the risk of COVID-19, potential complications, and how the pandemic may affect treatment, but preliminary research has revealed some trends.

Learn what having thalassemia may mean for you during the COVID-19 pandemic.

What Is Thalassemia?

Thalassemia is an inherited disorder that impairs the production of hemoglobin. Hemoglobin normally is made of four chains—two alpha chains and two beta chains. In thalassemia, the body is unable to make either alpha or beta chains in adequate amounts. This means red blood cells aren't made properly or they are destroyed.

There are four types of alpha thalassemia, and two main types of beta thalassemia. Within each of these types, the severity varies, from causing moderate to severe anemia to requiring frequent blood transfusions.

Thalassemia and COVID-19 Risk

When looking into thalassemia and COVID-19 risk, it's important to identify both the risk of potential exposure and susceptibility.

There are a number of factors that might increase the chances of a person with thalassemia being exposed to SARS-CoV-2, the virus causing COVID-19. The need for frequent blood transfusions and complications of the disease that result in more clinic visits and hospitalizations could raise the odds that someone would come into contact with the virus.

While the research is early and there is much left to be learned, people who have thalassemia (at least some types) may be more susceptible to contracting coronavirus and having a serious outcome.

In a large Iranian study, the prevalence of COVID-19 in people with non-transfusion dependent thalassemia (those with the disease not dependent on transfusions) was much higher than in the general population (45 vs. 22.3 infections per 10,000 people). The prevalence among those who were transfusion dependent was roughly the same as people without thalassemia.

However, some limited evidence suggests that certain types of thalassemia may have some protective effect against COVID-19. Globally, the prevalence of COVID-19 in people with beta-thalassemia specifically appears to be lower than the general population.

Risk of Severe Outcomes From COVID-19 for People With Thalassemia

There are a number of reasons why people with thalassemia might be at risk for more severe outcomes with COVID-19. For example, some of the common complications and coexisting conditions caused by thalassemia may also worsen COVID-19.

Some of these complications include:

• Iron overload: Frequent transfusions due to thalassemia can cause a build up of iron in the blood, which can, in turn, lead to heart disease, liver disease, diabetes, adrenal insufficiency, and other issues. These conditions are linked to poorer COVID-19 outcomes.
• Splenectomy: When a person has a spleen removed for thalassemia, they are more likely to develop a secondary bacterial infection while fighting COVID-19.
• Reduced immune response: Excess iron can build up in regions of the body such as the hypothalamus and adrenal glands. This can cause damage that impairs production of certain hormones that stimulate the immune system to fight infections. In addition, those who are on corticosteroids may not be able to clear the COVID-19 virus from the respiratory tract as rapidly as someone who is not taking these medications.
• Hypercoagulability: Thalassemia increases one's tendency to form blood clots. This raises the risk of forming dangerous blood clots when infected with COVID-19.

Thus far, studies have been mixed as to whether thalassemia patients are more likely to experience serious outcomes of COVID-19 (such as hospitalization or death).

The study from Iran found that having a diagnosis of thalassemia was associated with poorer outcomes, particularly for people who have complications of thalassemia.

Looking at a small sample group early on in the pandemic, the overall mortality rate of those with thalassemia from a COVID-19 infection was 18.6%, compared with a mortality rate of 4.71% in the general population. (Note: The overall mortality rate for COVID-19 is currently less than 1%.).

It's important to note that complications of thalassemia likely played a role in the severity of outcomes. Among people aged 36 or older, an average of 60% of thalassemia patients had at least one coexisting condition (100% in the non-transfusion dependent group and 41.7% in the transfusion-dependent group).

Examples of coexisting conditions included insulin-dependent diabetes, heart disease, pulmonary hypertension, kidney disease, osteoporosis, hepatitis C, liver disease, and asthma.

Complications of Thalassemia and COVID-19

People with thalassemia often experience similar COVID-19 complications as those without thalassemia, but they may be at greater risk for certain problems. Some of these include:

Pneumonia and Respiratory Failure

Secondary infections (bacterial infections that develop in people with a viral infection such as COVID-19) may be more common in people with thalassemia, especially those who have had their spleen removed. It's recommended that antibiotics be started immediately if people develop a fever or other signs of an infection.

Respiratory failure may occur just as with people who do not have thalassemia. Common COVID-19 treatments, including prone positioning (turning a patient on their stomach), non-invasive ventilation (oxygen delivered via a face mask), and intubation and mechanical ventilation (where a tube is inserted down the throat and a machine pumps air in and out of the lungs), may be needed depending on the severity.

Blood Clots

Blood clots are fairly common with COVID-19, and many physicians view it as a disease of blood vessels. Thalassemia also increases the likelihood to form blood clots.

It's not known whether blood clots with COVID-19 are more common among people with thalassemia, but healthcare providers should be aware of this potential risk. It's currently recommended that people with thalassemia who have severe COVID-19 be given blood thinners, though this could change as more is learned.

Heart-Related Complications

Some people with thalassemia have cardiomyopathy (heart muscle disease). This complication should be monitored and treated as it is for people with other forms of preexisting heart disease.

Adrenal insufficiency (a lack of sufficient hormone production by the adrenal glands) can occur with thalassemia. This condition can cause abrupt changes in blood pressure or heart rate that may worsen a person's condition during a COVID-19 infection. Treatment is with corticosteroids.

Long-Term Complications

"Long COVID" is a term meaning symptoms that persist after the infection has cleared. Among people with thalassemia, little is known about long-term complications from COVID-19 at this time.

Thalassemia Treatments and COVID-19

The COVID-19 pandemic may affect both the treatment of thalassemia in general and how COVID-19 is treated in people with thalassemia who become infected.

There are very few medications that are contraindicated (should not be used) in people who may be exposed to SARS-CoV-2 or who have developed a COVID-19 infection. In fact, some medications used for thalassemia may actually be helpful.

Managing Anemia

Blood transfusions are a mainstay of treatment for some people with thalassemia. Current recommendations are that a person's usual regimen of transfusions should be continued during the pandemic.

That said, the shortage of the blood supply has proven challenging. Some blood donation programs were stopped (or decreased) worldwide, and hesitancy of blood donors to venture out and risk exposure to COVID-19 has also contributed to shortages.

There has also been a shortage at other steps in the process, such as personnel available to perform collections, as well as process and deliver donated blood. As an example, on March 17, 2020, the Red Cross announced that their blood reserves were reduced by around 80% in the United States due to canceled donations and reduced distribution.

This shortage has been of even greater concern in South Asia (due to a high incidence of beta-thalassemia), where it's been estimated that 200,000 people depend on regular blood transfusions to manage their condition.

Reducing the Need for Transfusions

Sadly, the reduction in the blood supply has led many people with thalassemia to receive fewer transfusions than usual, prompting healthcare providers caring for people with the condition to look for options that may reduce the need for or frequency of transfusions. A few options include:

• Reblozyl (luspatercept): The medication Rebozy is one option for people with beta thalassemia, and has been found to reduce the need for blood transfusions by roughly one-third in people with thalassemia. Unfortunately, the medication is very expensive and not available in some countries where thalassemia is most common. The medication is given by injection.
• Hydroxyurea: The medication Hydrea (hydroxyurea), approved for people with non-transfusion-dependent beta thalassemia as well as sickle cell disease, is thought to improve hemoglobin levels with relatively few side effects. In June 2020, the Thalassemia International Federation released a position paper on COVID-19 recommending the use of hydroxyurea in people with thalassemia who require regular transfusions to help safeguard the blood supply reserves.
• Zynteglo (betibeglogene autotemcel): Zynteglo is the first cell-based gene therapy for beta-thalassemia approved for the treatment of patients with beta-thalassemia who need regular red blood cell transfusions. Zynteglo is an individualized single-dose treatment created by modifying the patient's own bone marrow stem cells to produce functional beta-globin. In one clinical study of Zynteglo, 89% of the patients achieved transfusion independence, meaning they did not need a transfusion for at least 12 months.

Current recommendations are that for people who have been treated with Reblozyl, the medication should be continued even if a person develops COVID-19 (there is no evidence that it should be stopped). For those who have not yet started the medication, delaying the use of the medication should be considered.

Monitoring Anemia with COVID-19

It's been found that, in general, people who have COVID-19 often have significantly reduced hemoglobin levels, especially with severe disease. Since hemoglobin levels already run low for many people with thalassemia, the combination of factors has raised some concerns.

While there are no specific guidelines at this time, some researchers believe that hemoglobin levels should be monitored closely in people with thalassemia if they develop COVID-19, even when symptoms are only mild.

Managing Iron Overload

Iron overload due to repeated transfusions is the cause of many complications seen with thalassemia, and therefore many people are treated with iron chelation therapy. These medications bind to iron so that it can be expelled from the body.

These drugs can reduce inflammation in blood vessels (endothelial inflammation) during viral infections. They also may have antiviral effects and positive effects on the immune system, particularly with RNA viruses (viruses that have RNA as its genetic material, such as the virus causing COVID-19).

It's not known whether this therapy has any effect on the outcomes of COVID-19 in people with thalassemia. But some researchers believe it may, based on the primarily mild symptoms of COVID-19 in people with thalassemia found in a study out of Southeast Asia.

Current recommendations for those in the United States is that iron chelation therapy be continued unless symptoms of COVID-19 develop. In that event, it's advisable that the therapy be stopped until the infection has cleared.

People Who Have Had a Splenectomy and Transplant Recipients

Special consideration is needed for both people who have had their spleens removed and for those who have had bone marrow/stem cell transplants to treat their thalassemia.

People who have had splenectomies have a greater risk of developing bacterial infections as well as sepsis. For those who develop COVID-19, doctors should be aware of this increased risk of a secondary bacterial infection so that antibiotics can be started early with any signs, such as a fever.

It’s considered safer to wait to begin gene therapy or prepare for a stem-cell transplant for thalassemia in communities currently affected by COVID-19. An exception is for people who have already begun the conditioning process for a stem-cell transplant. These transplants should proceed.

Other Considerations

The use of corticosteroid medications (such as prednisone) can have both positive and negative effects and must be carefully weighed in people with thalassemia. On one side of the equation, glucocorticoids can slow the clearance of the virus from the respiratory tract. On the other, people with thalassemia may be at risk of adrenal insufficiency (adrenal crisis), which can worsen a person's condition with severe COVID-19.

Summary

Thalassemia may increase the risk of serious outcomes of COVID-19. Additionally, COVID-19 has complicated some treatments for thalassemia, such as the availability of lifesaving blood transfusions. Working with your healthcare team to keep up with thalassemia treatments, getting vaccinated, and monitoring and treating for complications early if COVID-19 symptoms do develop are the recommended strategies to staying healthy through the pandemic.

A Word From Verywell

If you're living with thalassemia, you may be at greater risk for serious outcomes of COVID-19. Prevention measures are very important. The most important thing you can do is make sure you are vaccinated and stay up to date on when boosters are needed.

If you become infected, it's important to talk to a doctor knowledgeable about thalassemia regarding any special precautions you should take or changes to your medical plan.

The pandemic has been hard on everyone, especially those with chronic illnesses. Yet, no matter how difficult the trial, there are often positive points. Try taking a few moments to list a few of the positive areas in your life. To begin, think of something you've had time to do that you'd otherwise not have time for, or something you learned. It's hard to focus on positives at this time, but sometimes making an effort can help you find a sense of gratitude that would otherwise be elusive.

The information in this article is current as of the date listed. As new research becomes available, we’ll update this article. For the latest on COVID-19, visit our coronavirus news page.