Immunisation in immunodeficiency
This is a complicated area to give guidance on because suitability for an individual is dependent on the particular immunodeficiency and on the vaccine involved.
There are, however, two golden rules.
- Advice should always be sought from your specialist immunologist/medical team before you have an immunisation.
- No person with an immunodeficiency should receive ‘live’ vaccines unless they have been explicitly told its OK by their immunologist.
Types of vaccines and how they work
Vaccines are used to provoke the immune system into making particular antibodies or T-lymphocytes that will fight or prevent the associated infection. To do this, vaccines have to reflect the virus or bacterium that they are designed to protect against.
Live vaccines use bacteria or viruses that have been altered (attenuated) so they resemble the wild bacterium or virus but no longer cause disease. Other types of vaccines consist of a bacterium or virus that has been killed or parts of these germs. These are known as dead or ‘killed’ vaccines.
Why are live vaccines a problem?
Live vaccines can cause problems in people whose immune systems are not working normally because they grow for a while unhindered after vaccination; this is why they are so effective. This can result in symptoms that may be similar to those the vaccine was intended to prevent.
For all immunisations it is important to weigh up the risks and benefits: to analyse the potential damage against the gain to an individual. For patients with an immunodeficiency where they are unable to make antibodies, the risk often outweighs the potential benefit.
Test immunisations with different vaccines may be given to make a precise diagnosis of immunodeficiency, with the response checked with a blood test three to four weeks later.
Vaccination and immunoglobulin therapy
In adults and children receiving replacement immunoglobulin therapy, certain vaccines are not useful because the immunoglobulin already provides adequate cover. These include vaccines for tetanus, polio and diphtheria.
Common vaccines
Here we provide specific information on the considerations involved in giving some common vaccines.
Influenza
A question commonly asked by people affected by a primary or secondary immunodeficiency immunisation is whether it is appropriate for them to have a ‘flu jab’. Some people with primary and secondary antibody deficiencies may benefit from having a flu jab as a precaution against developing flu complications, such as chest infections, which are common even in healthy people. This is beneficial even in patients with antibody deficiency, because the vaccine should prime T-cells and help shorten the duration of any influenza infection.
Most immunology centres recommend that patients, carers and close family members have a flu jab.
The children’s flu vaccine
This vaccine comes as a nasal spray and is called Fluenz. It is a live vaccine so it should NOT be given to children with an immunodeficiency without getting individual, specific advice from your immunology medical team. centre. Other killed, injectable flu vaccines may be recommended.
BCG
BCG is an attenuated form of live mycobacteria that is related to tuberculosis (TB). Where there is a high risk of TB infection in your local area, BCG may be given near birth. However, in children with T-cell problems, such as severe combined immune deficiency, BCG can cause severe widespread infection that is difficult to treat, so BCG must NOT be given to any baby with a suspected immunodeficiency.
It is also vital that babies with a family history of immunodeficiency of any kind should NOT be given BCG until carefully assessed by an immunologist.
BCG appears to be safe in those with antibody deficiency alone, but again, only after careful assessment by an immunologist.
In children and adults who have been exposed to TB, a skin test may be used to check the response to TB before BCG is given. This approach, however, may cause problems of interpretation in patients with immunodeficiency, including chronic granulomatous disease, because a ‘false negative’ test can result. A blood test called interferon-gamma release assay (IGRA) can now be used instead, and this caveat about interpretation will apply. So a careful history of unusual infections should be sought before BCG is given to anyone.
Combined diphtheria, pertussis, tetanus and polio vaccine
This is a mixture of dead bacteria or parts of the toxins secreted by bacteria. There is no particular risk of harm from these vaccines in immunodeficiency, but they will not work in patients with known immunodeficiency. However, it may be used for test immunisation in order to make a precise diagnosis.
Haemophilus
This is a dead, bacterial vaccine using bacterial components only and does not cause side effects in immunodeficiency, though it will not usually provide protection either. It is routinely given in childhood and protects against meningitis and life-threatening throat infections (epiglottis) in children with healthy immune systems. Haemophilus vaccination is particularly important if the spleen has been removed, as these patients are prone to this type of infection.
Hepatitis B
This vaccine is mainly required in people at risk from their work or through sexual activity. It is a safe vaccine but may not always work in immunodeficiency or in older individuals.
Human papilloma virus (HPV)
All girls aged 12 to 13 are offered a HPV vaccination as part of the NHS childhood vaccination programme. The vaccine protects against cervical cancer. It’s usually given to girls in year eight at schools in England.
The vaccines available are recombinant protein vaccines – virus like particles, with no live virus potential they are safe for girls who have a PID.
Measles, mumps, rubella (MMR)
These are live vaccines containing attenuated viruses and are given in a combination (called MMR) during early childhood and adolescence. These disease-causing modified viruses pose a real potential risk of infection in immunodeficient patients, so these vaccines are avoided in patients with immunodeficiency. However, replacement immunoglobulin contains high levels of antibodies, due to collections from healthy immunised donors, so protection is provided by this treatment. Unlike polio, there is no risk of person-to-person spread of the weakened viruses used in MMR and contacts of a child with a PID should be vaccinated with MMR in the normal way.
Meningitis
Meningitis is inflammation of the membranes surrounding the brain and is caused by a number of bacteria. There are vaccines available against the different types of meningitis. Haemophilus, pneumococcus and meningococcus are probably the most important.
Meningococcus
There are several strains of meningococci; fortunately there are now vaccines against most of the common circulating strains in the UK. The oldest vaccine is against strains that are common outside the UK (A, C, Y and W135; known as the ACWY vaccine) and is required for travel to some areas. It is a safe vaccine comprising bacterial components but does not work in young children. This immunisation is especially important in people with complement deficiencies, due to a particular susceptibility of such patients to these bugs.
Since risk of meningococcal infection is highest in infants and young children, with a second peak of risk occurring in 15 to 19 year olds (particularly in communities, e.g. barracks and student halls), it is important to protect these groups. In order to make these vaccines more effective in young children, a new form of vaccine (conjugated vaccine) against type C was produced and universal immunisation with this safe vaccine, made of bacterial components only, was introduced in 1999. Since then, the incidence of type C infection has fallen and longer-term protection achieved by a booster jab in adolescence.
New vaccine against meningitis B
Meningitis B remains the leading cause of invasive meningococcal disease in the UK. In December 2013 a new protective vaccine for meningitis B was made available for the first time for children and adults, with primary immunodeficiency patients at high risk of getting this infection. This includes patients who are currently recommended to receive the ACWY vaccine – namely those who do not have a spleen, or have a spleen that does not work properly, or with complement deficiency, or those whose immunologist or relevant specialist believes there is an increased risk. If you think you may benefit from this vaccine, do talk to your GP or your immunologist.
Pneumococcus
Streptococcus pneumoniae (pneumococcus) is a common bacterium. Infections range from uncomplicated ear infections (otitis media) to meningitis, blood poisoning (septicaemia) or pneumonia. Serious infections are more common in young children, the elderly, patients who have had their spleen removed and in PID patients.
Pneumococcal vaccine
There are two types of vaccines against pneumococci. Pneumovax is a vaccine prepared from a mixture of 23 bacterial polysaccharide (sugar) components which is effective in older children and adults. This is used to protect those that have sickle cell disease or have had their spleen removed, in order to prevent chest and sinus infections. This safe vaccine may be used to test for antibody production as part of the investigation of a suspected immunodeficiency.
Children under the age of two do not respond to the polysaccharide vaccines. In order to protect this group of high-risk individuals, conjugated pneumococcal vaccines were introduced universally in the UK in 2006. These vaccines are also composed of bacterial components and contain no live organisms; they are safe in immunodeficient patients and may be used to investigate suspected immunodeficiency.
Rotavirus
Rotavirus is the most common cause of severe diarrhoea among infants and young children. This live vaccine is given to very young children at two and three months, so most children unless diagnosed very early (e.g. because of an affected sibling) will often have already received this live vaccine. Most immunology centres are still forming a policy on rotavirus vaccination in primary immunodeficiency and would avoid giving this live vaccine to children whose immune systems are severely compromised and those yet to receive treatment. However, doctors may consider giving this vaccine to children with milder immune deficiencies and the decision would be made on a case-by-case basis. As with all immunisations, specific advice should always be sought from your specialist immunology centre.
Shingles vaccine
Shingles, also known as herpes zoster, is a painful skin rash caused by the chickenpox virus (varicella-zoster virus). An NHS vaccine is available to prevent shingles for people aged 70, 78 and 79. This live vaccine may be suitable for some people with PID and decisions as to its use would be made on a case-by-case basis. As with all immunisations, specific advice should always be sought from your specialist immunology centre.
Polio
Live polio vaccine (the type given as drops in sugar) is no longer available in the UK. Killed vaccine (Salk) is used instead and is part of the routine immunisations given to infants (see combined diphtheria, pertussis, tetanus and polio vaccine, above).
In the past the live polio vaccine caused disease in immunodeficient children; as part of the World Health Organization’s drive to eliminate polio worldwide, this vaccine is no longer produced.
Typhoid
This vaccine is required for travel to some areas. A live oral vaccine exists and this must not be used in people with immunodeficiency or their contacts. A safe, dead, injected version must be given if immunisation is mandatory, though other precautions against infection are essential since there will be no protective antibody response in PID patients.
Yellow fever
This is a live vaccine given to people travelling to parts of South America and Africa. It is known to cause problems in people with PID and should not be given. Patients will require a certificate stating the reason they have not been vaccinated. Extra precautions should be taken to avoid insect bites when this vaccine has not been given.
Frequently asked questions
There is no concern about you putting your mum at risk because you had the BCG vaccine, she cannot catch TB from it. Take a look at this link for more information.
There are two Shingles vaccine, and one of these (Zostavax) is a live vaccine. Transmission to susceptible household contacts has been reported but is rare. The vaccine is however recommended for healthy susceptible contacts of immunocompromised patients where continuing close contact is unavoidable (e.g. siblings of a leukaemic child, or a child whose parent is undergoing chemotherapy). If in doubt check with your centre if they would be happy for you to be vaccinated, if the answer is yes, you need not worry about household contact transmission.
Please discuss with your health team whether the vaccine is suitable or not, as this depends on different factors such as your T cell function as well as whether or not you are on immunosuppressive therapy. You can find information on the shingles vaccine at Shingles vaccine overview – NHS (www.nhs.uk)
All girls aged 12 to 13 are offered HPV vaccination as part of the NHS childhood vaccination programme. The vaccine protects against cervical cancer. It’s usually given to girls in year eight at schools in England.
The vaccines available are recombinant protein vaccines – virus-like particles, with no live virus potential so they are safe for girls who have a PID.
I have just started university and have a PID should I have the meningitis and septicaemia vaccines?
Both the meningitis and septicaemia vaccines are killed vaccines so PID patients should definitely have the vaccination. PID patients may not make the most effective response to the vaccine but it’s better to have some protection.
Many but not all patients are suitable for pneumococcal vaccine. Patients on immunoglobulin (Ig) replacement therapy would not normally receive it but may do if their Ig is stopped. Other patients may have specific contraindications and this would be best discussed with their specialist health team.
Pneumococcal vaccine is not usually given whilst on Ig therapy. This is because there is a good spread of anti-pneumococcal antibodies in Ig, and this is part of its value. Pneumococcal vaccine can be “neutralised” by the antibodies present in the Ig. There is a limited useful T-cell response that would help provide further protection to these organisms, so there is no great utility in vaccinating. This is unlike the killed flu vaccine, which can provide useful T-cell protection even if there is no antibody response.
It is very difficult to give blanket advice on vaccination for people with immunodeficiency. We advise that you discuss this with your specialist team as they can provide advice specific to you and your condition. Some patients will be given pneumococcal vaccine as part of their work up, patients on immunoglobulin therapy would not normally be offered it since they get the protection from this treatment.
There are two types of vaccines for the ‘pneumonia jab’ Streptococcus Pneumoniae (pneumococcus). Pneumovax is a vaccine prepared from a mixture of 23 bacterial polysaccharide (sugar) components which is effective in older children and adults. This is used to protect those that have sickle cell disease or have had their spleen removed, to prevent chest and sinus infections. This safe vaccine may be used to test for antibody production as part of the investigation of a suspected immunodeficiency.
Children under the age of two do not respond to the polysaccharide vaccines. To protect this group of high-risk individuals, conjugated pneumococcal vaccines were introduced universally in the UK in 2006. These vaccines are also composed of bacterial components and contain no live organisms; they are safe in immunodeficient patients and may be used to investigate suspected immunodeficiency.
You may also find this IPOPI booklet on vaccines useful.
To date no cases of transmission have been reported. Children well enough to attend mainstream school do not need to avoid other children who have received the vaccine, but PID patients should not receive the live virus as a direct inoculation. Children undergoing or recovering from bone marrow transplant or with SCID (untreated) are advised that household contacts should have killed, not live, vaccines.
Although vaccinated children are known to shed virus a few days after vaccination, the vaccine virus that is shed is less able to spread from person to person than the natural infection. The amount of virus shed is normally below the levels needed to pass on infection (transmit) to others and the virus does not survive for long outside of the body. This is in contrast to natural flu infection, which spreads easily during the flu season.
It is more likely that you had naturally acquired flu and that you will hopefully have had less severe infection than if you were not vaccinated.
Although vaccinated children are known to shed the virus a few days after vaccination, the vaccine virus that is shed is less able to spread from person to person than the natural infection. The amount of virus shed is normally below the levels needed to pass on infection (transmit) to others and the virus does not survive for long outside of the body. This contrasts with a natural flu infection, which spreads easily during the flu season. In schools using vaccines, therefore, the overall risk of influenza transmission is massively reduced by having a large number of children vaccinated. In the US, where there has been extensive use of the nasal spray flu vaccine (live attenuated influenza vaccine; LAIV) for many years, serious illness amongst immunocompromised contacts who are inadvertently exposed to the vaccine virus has never been observed. Expert doctors at Great Ormond Street Hospital, who deal with many children with very serious immune problems, do not recommend keeping such children off school purely because of vaccination.
All pregnant women are advised to have flu vaccine to protect them and their newborn child. If you have a PID then live vaccine should not be used (but these are not licensed for adults so should not be an issue).
Vaccines reduce the likelihood of getting an infection and often reduce how badly a person will be affected if infection still occurs. Some people with immunodeficiency will be able to be vaccinated, but their responses may not be as good as individuals with fully functioning immune systems. Vaccinating family members or close household contacts with the injectable killed flu vaccine is recommended to help reduce the likelihood that someone brings influenza into the house and will therefore help protect the individual indirectly.
There is a theoretical potential for transmission of live attenuated influenza virus in Fluenz Tetra® to immunocompromised contacts for one to two weeks following vaccination. In the US, where there has been extensive use of the Live Attenuated Influenza Vaccine, there have been no reported instances of illness or infections from the vaccine virus among immunocompromised patients inadvertently exposed. Where close contact with very severely immunocompromised patients (e.g. bone marrow transplant patients requiring isolation) is likely or unavoidable (for example, household members), however, appropriate alternative inactivated influenza vaccines should be considered. For the majority of PID patients this means that household contact with a live vaccine is fine, but if in doubt check with your immunologist.
No Fluenz is a live nasal vaccine and should not be given to children with a PID. Other killed; injectable flu vaccines may be recommended. You should speak to your immunology team for advice.
In general, there is no contraindication between having immunoglobulin therapy and killed vaccines, but if the two are given close together and there are high levels of antibody in the Ig to the vaccine then the efficacy is likely to be impaired. Please check with your immunology team about the appropriate timing.
This page was updated April 2023.
