People withsickle cell diseaseare immunocompromised, which means they have an increased risk of infection. For this reason, immunizations are an important part of complete sickle cell care.This article looks at the importance of immunizations for people with sickle cell disease.Pamela Moore / Getty ImagesWhy People With Sickle Cell Disease Are at Risk for InfectionsSickle cell disease starts damaging the organs in the first year of life. One of the first organs affected is the spleen. The spleen is a small organ on the left side of the abdomen underneath the ribcage. The spleen filters the blood and removes old, damaged red blood cells.The spleen is also an important part of the immune system. It has two immune functions. It filters out bacteria, particularly a type of bacteria that has an outer covering that helps protect it from the body’s immune system. It also produces lymphocytes, white blood cells that help make antibodies to infections or in response to immunizations.People with sickle cell disease have sickle-shaped blood cells. These cells can stick together and pool in the spleen, which damages it. The damage occurs repeatedly and spleen function is lost before the age of 5. This loss of function significantly increases the risk of infection.Potentially Harmful BacteriaPeople with sickle cell disease are at higher risk of developing serious bacterial illnesses. For example, people with sickle cell disease are roughly 300 times more likely to develop bacterialmeningitisthan people without the condition.Bacterial meningitis is a serious infection of the lining of the brain.Sickle cell disease also raises the risk ofbacteremia, a bacterial infection of the blood. Without antibiotics, unvaccinated children with sickle cell disease who develop a fever have a 3% to 5% risk of developing bacteremia.Additionally, people with sickle cell are more likely to developsepsis, a blood infection that causes a major inflammatory reaction.Bacteria that can cause these infections include:Streptococcus pneumoniae:These bacteria are a common cause of pneumonia in children and adults. They can also cause meningitis, bacteremia, and sepsis.Neisseria meningitidis:This species is the number one cause of bacterial meningitis in children and young adults. It can also cause bacteremia or sepsis.Haemophilus influenzae type b:Prior to routine vaccination, this used to be the leading cause of meningitis in children. It should not be confused with the influenza virus.In addition,influenzais a virus that causes respiratory infections. People with sickle cell disease who develop an infection with influenza are more likely to be admitted to the hospital. Influenza infection can also trigger lung complications like acute chest syndrome.Recommended ImmunizationsImmunization can help protect people with sickle cell from developing serious infections. Children with sickle cell should receive all recommended childhood vaccines as well as an annual flu shot. Adults should make sure their vaccinations are up to date. Special vaccine schedules are recommended for certain vaccines, including:Haemophilus influenzae type b (Hib)Pneumococcal vaccinesMeningococcal vaccinesPneumococcal ImmunizationsThese vaccines protect againstStreptococcus pneumoniainfection. Vaxneuvance (PCV15) or Prevnar 20 (PCV20) is given to all infants as a four-dose series at 2, 4, 6, and 12-15 months of age. Children with sickle cell disease who completed a PCV series other than PCV20 should receive one dose of PCV20 or Pneumovax 23 (or PPSV23), starting at 2 years of age.Adults 19 and older with sickle cell disease who have not received a pneumococcal conjugate vaccine (PCV) should receive a single dose of either Vaxneuvance (PCV15), Prevnar 20 (PCV20), or Capvaxive (PCV21). Those who get PCV15 should get one dose of PPSV23 at least one year later.Meningococcal ImmunizationsAll children need to be immunized againstNeisseria meningitidis,but children with sickle cell disease receive theseimmunizationsin infancy or early childhood. Two vaccines are required.The first is called Menveo or Menquadfi, and it protects against meningococcal types (called serogroups) A, C, W, and Y. Depending on the vaccine used and when the immunization series is started, it involves two to four shots spaced weeks apart. Boosters are then needed throughout life.The second vaccine, called Bexero or Trumemba, protects against meningococcus type B. It is given as a two- or three-dose series starting at age 10.In 2023, another vaccine option was approved that protects against all these meningococcus types. It is called Penbraya and can given after age 10 for children with sickle cell disease. It is an option to replace the type B vaccine and ACWY boosters.Haemophilus Influenzae Type BImmunization forHaemophilusis part of the routine immunizations in the first year of life (four doses). This immunization is called Hib and can be given in combination with other immunizations. It is recommended at 2, 4, 6, and 12-15 months of age.InfluenzaImmunization with the yearly influenza vaccine is recommended for most people 6 months and older, including everyone with sickle cell disease.The first time someone under the age of 9 receives the influenza vaccine, two doses are required (at least four weeks apart). Afterward, only one dose is required each year. Because the vaccine changes, it must be given annually.Flu season runs from about October to March. If you receive your influenza vaccine in January, you would need another one—as soon as it becomes available—to cover the new influenza season beginning in October.SummaryPeople with sickle cell disease are at higher risk for infection. This is because the disease causes early damage to the spleen, which plays an important role in the body’s immune response.Immunization can help protect children and adults from developing serious infections. The CDC recommends all people with sickle cell disease receive recommended vaccines. Some vaccines should be given on a different schedule.A Word From VerywellThese infections sound worrisome, but fortunately, immunizations have significantly decreased the risk. Healthcare providers also give penicillin to children with sickle cell disease from birth to age 5. This can help preventbacterial infections, too.If you have questions or concerns about these immunizations, please discuss them with your healthcare provider.
People withsickle cell diseaseare immunocompromised, which means they have an increased risk of infection. For this reason, immunizations are an important part of complete sickle cell care.
This article looks at the importance of immunizations for people with sickle cell disease.
Pamela Moore / Getty Images
Why People With Sickle Cell Disease Are at Risk for Infections
Sickle cell disease starts damaging the organs in the first year of life. One of the first organs affected is the spleen. The spleen is a small organ on the left side of the abdomen underneath the ribcage. The spleen filters the blood and removes old, damaged red blood cells.
The spleen is also an important part of the immune system. It has two immune functions. It filters out bacteria, particularly a type of bacteria that has an outer covering that helps protect it from the body’s immune system. It also produces lymphocytes, white blood cells that help make antibodies to infections or in response to immunizations.
People with sickle cell disease have sickle-shaped blood cells. These cells can stick together and pool in the spleen, which damages it. The damage occurs repeatedly and spleen function is lost before the age of 5. This loss of function significantly increases the risk of infection.
Potentially Harmful Bacteria
People with sickle cell disease are at higher risk of developing serious bacterial illnesses. For example, people with sickle cell disease are roughly 300 times more likely to develop bacterialmeningitisthan people without the condition.Bacterial meningitis is a serious infection of the lining of the brain.
Sickle cell disease also raises the risk ofbacteremia, a bacterial infection of the blood. Without antibiotics, unvaccinated children with sickle cell disease who develop a fever have a 3% to 5% risk of developing bacteremia.
Additionally, people with sickle cell are more likely to developsepsis, a blood infection that causes a major inflammatory reaction.
Bacteria that can cause these infections include:
In addition,influenzais a virus that causes respiratory infections. People with sickle cell disease who develop an infection with influenza are more likely to be admitted to the hospital. Influenza infection can also trigger lung complications like acute chest syndrome.
Recommended Immunizations
Immunization can help protect people with sickle cell from developing serious infections. Children with sickle cell should receive all recommended childhood vaccines as well as an annual flu shot. Adults should make sure their vaccinations are up to date. Special vaccine schedules are recommended for certain vaccines, including:
Pneumococcal Immunizations
These vaccines protect againstStreptococcus pneumoniainfection. Vaxneuvance (PCV15) or Prevnar 20 (PCV20) is given to all infants as a four-dose series at 2, 4, 6, and 12-15 months of age. Children with sickle cell disease who completed a PCV series other than PCV20 should receive one dose of PCV20 or Pneumovax 23 (or PPSV23), starting at 2 years of age.
Adults 19 and older with sickle cell disease who have not received a pneumococcal conjugate vaccine (PCV) should receive a single dose of either Vaxneuvance (PCV15), Prevnar 20 (PCV20), or Capvaxive (PCV21). Those who get PCV15 should get one dose of PPSV23 at least one year later.
Meningococcal Immunizations
All children need to be immunized againstNeisseria meningitidis,but children with sickle cell disease receive theseimmunizationsin infancy or early childhood. Two vaccines are required.
The first is called Menveo or Menquadfi, and it protects against meningococcal types (called serogroups) A, C, W, and Y. Depending on the vaccine used and when the immunization series is started, it involves two to four shots spaced weeks apart. Boosters are then needed throughout life.
The second vaccine, called Bexero or Trumemba, protects against meningococcus type B. It is given as a two- or three-dose series starting at age 10.
In 2023, another vaccine option was approved that protects against all these meningococcus types. It is called Penbraya and can given after age 10 for children with sickle cell disease. It is an option to replace the type B vaccine and ACWY boosters.
Haemophilus Influenzae Type B
Immunization forHaemophilusis part of the routine immunizations in the first year of life (four doses). This immunization is called Hib and can be given in combination with other immunizations. It is recommended at 2, 4, 6, and 12-15 months of age.
Influenza
Immunization with the yearly influenza vaccine is recommended for most people 6 months and older, including everyone with sickle cell disease.
The first time someone under the age of 9 receives the influenza vaccine, two doses are required (at least four weeks apart). Afterward, only one dose is required each year. Because the vaccine changes, it must be given annually.
Flu season runs from about October to March. If you receive your influenza vaccine in January, you would need another one—as soon as it becomes available—to cover the new influenza season beginning in October.
Summary
People with sickle cell disease are at higher risk for infection. This is because the disease causes early damage to the spleen, which plays an important role in the body’s immune response.
Immunization can help protect children and adults from developing serious infections. The CDC recommends all people with sickle cell disease receive recommended vaccines. Some vaccines should be given on a different schedule.
A Word From Verywell
These infections sound worrisome, but fortunately, immunizations have significantly decreased the risk. Healthcare providers also give penicillin to children with sickle cell disease from birth to age 5. This can help preventbacterial infections, too.
If you have questions or concerns about these immunizations, please discuss them with your healthcare provider.
6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Chenou F, Azevedo J, Leal HF, Gonçalves MD, Reis JN.Bacterial meningitis in patients with sickle cell anemia in Salvador, Bahia, Brazil: a report on ten cases.Hematol Transfus Cell Ther. 2020;42:139-44. doi:10.1016/j.htct.2019.06.006
Baskin MN, Goh XL, Heeney MM, Harper MB.Bacteremia risk and outpatient management of febrile patients with sickle cell disease.Pediatrics. 2013;131(6):1035-41. doi:10.1542/peds.2012-2139
Centers for Disease Control and Prevention.Infections and sickle cell disease.
Centers for Disease Control and Prevention.Pneumococcal vaccination: Summary of who and when to vaccinate.
Food and Drug Administration.October 20, 2023 Approval Letter - PENBRAYA.
Centers for Disease Control and Prevention.Immunization schedules.Field JJ, Vichinsky EP.Overview of the management and prognosis of sickle cell disease. In: DeBaun MR, Tirnauer JS, eds. UpToDate. Waltham, Mass: UpToDate; 2022.
Centers for Disease Control and Prevention.Immunization schedules.
Field JJ, Vichinsky EP.Overview of the management and prognosis of sickle cell disease. In: DeBaun MR, Tirnauer JS, eds. UpToDate. Waltham, Mass: UpToDate; 2022.
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