Immunisation in PID

This is a complicated area to give guidance on because suitability for an individual is dependent on the particular primary immunodeficiency (PID) and on the vaccine involved.

There are, however, two golden rules.

  • Advice should always be sought from your specialist immunologist before you have an immunisation.
  • No person with an immunodeficiency should receive ‘live’ vaccines unless particularly indicated in a special case.

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 PID is whether it is appropriate for them to have a ‘flu jab'. Some people with primary 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.

This is a fragmented (subunit) vaccine, which is safe but may not work well in patients with immunodeficiency. However, it is safe and useful in that antiviral T-cells will become activated and so lessen the disease should the individual develop symptoms. Swine flu (H1N1 influenza) is a relatively new strain of influenza (flu) responsible for an outbreak in 20092010. The seasonal influenza vaccine protects against this strain of flu. 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 a primary immune deficiency without getting individual, specific advice from your immunology 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 (e.g. in Asian and African families), 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 native 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 bacterium. There are vaccines available against the different types of meningitis. Haemophilus 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.

This page has been reviewed by the Medical Advisory Panel, April 2013 and updated in December 2013.