Infections and Sickle Cell Disease

Sickle cell disease (SCD) often causes spleen damage and other immune system problems. This makes people with SCD more likely to experience complications from infections.

People with SCD have a higher risk of infection from certain types of bacteria, especially pneumococcus. This can cause infections in many parts of the body, such as the lungs, brain tissue, ears, and joints. It can also trigger acute pain crises and acute chest syndrome.

The rate and severity of infections have decreased with the use of preventive antibiotics and vaccines. However, infections are still life-threatening for people with SCD. If you have symptoms of an infection, call your doctor immediately.

What types of infections do people with sickle cell disease experience?

People with SCD, especially infants and children, have a high risk for infections. Until the 1990s, up to 30 percent of children with sickle cell anemia in the United States died from infections. Infections can lead to life-threatening complications, such as pneumonia, acute pain crisis, and acute chest syndrome. Here are some infectious diseases that people with SCD commonly experience:^1-3

Pneumococcal disease

This is any infection caused by bacteria called Streptococcus pneumoniae, or pneumococcus. Before preventive measures were common, SCD made children 30 to 600 times more likely to develop pneumococcal disease. It is a common cause of lung infection (pneumonia). It can also cause ear and sinus infections, and infections of the tissue covering the brain and spinal cord (meningitis).^2,4,5

Respiratory syncytial virus (RSV)

RSV is a common cause of lung and respiratory tract infections. Most children are infected with RSV by age 2. In children without SCD, symptoms usually mimic the common cold. However, in children with SCD, RSV infection is linked to episodes of acute chest syndrome.^6-8

Haemophilus influenzae type B

This is a type of bacteria that can cause pneumonia and meningitis, as well as infections of the bloodstream, skin, and joints. Despite its name, it is not the cause of influenza or “the flu”.^2,9

Parvovirus B19

This is a common infection that is also called fifth disease or slapped cheek syndrome. In people with SCD, it can cause a complication called aplastic crisis. It happens when the bone marrow stops making new red blood cells. This leads to severe, potentially life-threatening anemia.¹⁰

Other infections

People with SCD also have a higher risk of infections of the urinary tract, gallbladder, and other organs from other types of bacteria. In resource-poor countries, salmonella infection is a common cause of pneumonia, meningitis, and infant mortality.^2,5

Why does sickle cell disease increase the risk of infection?

SCD increases the risk for infection because of damage to the spleen. The spleen is an organ that filters blood and destroys old red blood cells. The spleen also contains the only cells in our body that can destroy certain types of pathogens (organisms that can cause disease and infection), such as encapsulated bacteria.^2,3

People with SCD often have non-working spleens. This is called functional asplenia. When red blood cells become sickled in the spleen, they can block blood from flowing through the normal filtering pathway.²

This makes people with SCD less able to clear certain bacteria, especially encapsulated types. Other immune system deficits, such as zinc deficiency, also increase infection risk in SCD.^2,11

What are some symptoms of infections?

Symptoms of infection depend on the part of the body that is infected. Some symptoms of lung infection (pneumonia) and blood infections include:⁴

• Fever and chills
• Cough
• Rapid breathing or difficulty breathing
• Chest pain
• Low alertness

Some symptoms of brain tissue infection (meningitis) include:⁴

• Stiff neck
• Headache
• Light sensitivity (photophobia)
• Confusion
• Nausea/vomiting
• Fever

Some symptoms of ear infections include:⁴

• Ear pain
• Swollen, red ear drum
• Sleepiness

See a doctor immediately if you notice any symptoms of an infection. It is a medical emergency for people with SCD, and early treatment is important to prevent problems.

How are they prevented and treated?

Preventive antibiotics, especially penicillin, have decreased the rate of pneumococcal disease in children with SCD by 85 percent. Penicillin is safe and beneficial for children up to 5 years old. However, there are some concerns about long-term penicillin treatment.^1,12

Universal use of pneumococcal and other standard vaccines has also reduced mortality from infectious diseases. The first pneumococcal vaccine reduced the rate of pneumococcal disease by 93 percent.

Infections may be prevented in people with SCD by:^3,8

• Washing hands often
• Making sure food is prepared safely
• Avoiding contact with people who have fevers or colds
• Avoiding exposure to tobacco smoke
• Taking penicillin daily until 5 years old, or as prescribed by your doctor
• Regularly getting the flu and pneumococcal vaccines, and any others recommended by your doctor

Infections are usually treated with antibiotics. Sometimes, blood transfusions will be used, especially to treat an aplastic crisis.³