Immunizations and Sickle Cell Disease

Reviewed by: HU Medical Review Board | Last reviewed: February 2023

Children with sickle cell disease (SCD) are more vulnerable to severe infections because of damage to the spleen. The spleen is an immune system organ that protects against certain types of bacteria, such as Streptococcus pneumoniae (pneumococcus). Pneumococcus infections quickly become life-threatening in children with SCD.1

Children and adults with SCD should receive all routine vaccinations, including the annual flu shot. They should follow special vaccine schedules for pneumococcus, meningococcus, and Haemophilus influenzae type B.

Your doctor will help make sure your child gets the right vaccinations at the right time. This depends on your child’s age, timing of previous vaccinations, and any additional risk factors.

Why are immunizations so important for children with sickle cell disease?

Childhood survival rates have greatly increased with universal use of pneumococcal and other routine vaccines. The first pneumococcal vaccine decreased the rate of pneumococcus infection in children under 3 years old by more than 90 percent.2,3

Vaccinations work by helping the immune system develop protection from a specific disease. They are safe and effective ways to prevent many infectious diseases for everyone. They are even more important for people with increased vulnerability to infections, such as people with SCD.

Vaccination schedules are also easier to follow than twice-daily penicillin, but are not a replacement for penicillin. Children with SCD should receive all routine immunizations, as well as special schedules for additional immunizations.3,4

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The Centers for Disease Control and Prevention (CDC) recommends an immunization schedule based on how children’s immune systems respond to vaccines and when they may be exposed to diseases. It also adapts the schedule for people with chronic conditions. Continued education helps parents and caregivers get children with SCD their vaccinations on time.5,6

What routine immunizations are necessary?

Children with SCD should receive all routine immunizations. This includes vaccines for:7

  • Hepatitis B
  • Rotavirus
  • Diphtheria, tetanus, and pertussis (whooping cough)
  • Polio
  • Measles, mumps, and rubella
  • Varicella (Chickenpox)
  • Hepatitis A
  • Human papillomavirus (HPV)

Children and adults with SCD should also receive the annual flu vaccine, starting after 6 months old. People with SCD should usually take a version called the inactivated influenza vaccine (IIV).7

What additional immunizations do children need?

SCD increases the risk of severe infections from certain bacteria because of damage to the spleen. This includes pneumococcus, meningococcus, and haemophilus influenzae type B.

Pneumococcus

Pneumococcus is the most common and severe cause of infections for children with SCD. There are 2 types of vaccines that prevent pneumococcal infections:8,9

  • Pneumococcal conjugate vaccines, including Prevnar (PCV13) and Vaxneuvance (PCV15)
  • Pneumococcal polysaccharide vaccines, including Pneumovax (PPSV23)

PCV13 or PCV15 are recommended for all children younger than 5 years old, including those with SCD. Pneumovax is usually given to adults older than 65 years old. However, children with SCD should also receive Pneumovax. Your doctor will help give these vaccines at the right time.7,8

Meningococcus

People with SCD are also vulnerable to infection from bacteria called Neisseria meningitidis (meningococcus). There are 2 types of vaccines used to prevent meningococcal infections:10

  • Meningococcal conjugate (MenACWY)
  • Serogroup B meningococcal (MenB)

MenACWY and MenB protect against different types of meningococcus. MenAWCY is usually given in 2 doses at ages 11 or 12 and then at 16 years old. However, children with SCD receive more doses at a younger age, starting any time after 2 months old. MenB is not routinely given, but children with SCD should receive multiple doses after 10 years old. Your doctor will help give these vaccines at the right time.7

Haemophilus Influenzae Type B

SCD also increases the risk of infections from Haemophilus influenzae type b (Hib). The Hib vaccine is usually given as 3 or 4 doses from 2 months old to 1 year old. Children with SCD may receive extra doses.7

What are the risks associated with immunizations?

Immunizations are safe and effective at preventing specific diseases. However, like all medicines, they can have side effects. Side effects usually do not last long and can include:10,11

  • Redness, swelling, or pain where the shot was given
  • Fever or chills
  • Loss of appetite
  • Irritability or fatigue
  • Feeling tired
  • Headache

Allergic reactions to vaccines are very rare. These types of reactions happen within a few minutes or hours after the vaccination. Get emergency help if you notice signs of an allergic reaction, such as:11

  • Hives
  • Swollen face or throat
  • Difficulty breathing
  • Fast heartbeat
  • Dizziness or weakness

What about immunizations for adults with sickle cell disease?

Adults with SCD should make sure they are up-to-date on all routine vaccinations, including a yearly flu vaccine. Your doctor can help you catch up on any vaccinations that may have been missed. You also may need boosters of vaccinations received during childhood.12

For additional immunizations, adults with SCD may receive:12

  • MenAWCY vaccinations every 5 years
  • MenB vaccinations every 2 to 3 years
  • Hib vaccine if you have not already had it, or before spleen removal surgery

Talk to your doctor to determine when you need to receive these vaccinations.