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Excerpted from the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases - Fifth Edition

Perhaps the greatest advances to improve general heath over the past two centuries have been the introduction of vaccines that effectively protect individuals from many of the worst microbial threats in our environment. Since primary immunodeficiency diseases interfere with the ability of the body’s immune system to respond appropriately, does it make any sense or do any good to give vaccines to patients with primary immunodeficiency diseases? Like so many things in life, it depends.

As discussed in the Patient & Family Handbook for Primary Immunodeficiency Diseases - Fifth Edition, our immune systems consist of two major categories of defense: the innate immune system and the adaptive immune system. The innate system is the first line of defense with its various components ready-to-go immediately when they encounter a microbe threatening our bodies. This is a critically important defense mechanism, but the innate system is not pre-programmed against every potential threat. It is the adaptive immune system that has the capacity to ramp-up a protective response to new threats. It does this by the generation of specific immune T-cells and B-cells, and the production of antibodies that are specifically designed to combat the new threat. The adaptive immune system takes several days before it reaches full power, but once it is fully activated, it remains on duty for a long time. If the body encounters the same threat again sometime in the future, this system has memory and is therefore able to respond much faster.

Vaccines are designed to activate the adaptive immune system to respond to specific microbes that innate immunity alone is not capable of controlling. We are familiar with the usual “childhood vaccines” that have greatly reduced the incidence of serious infectious diseases that in earlier generations sickened or killed millions of people. Polio, measles, whooping cough, mumps, Rubella, HPV, chickenpox, HiB, tetanus, meningococcus, diphtheria, rotavirus and influenza are the vaccines most commonly given today, and most communities require that children be up-to-date on their immunizations before being able to attend public school. So, what about people with primary immunodeficiency diseases?

First, it is important to recognize that many types of primary immunodeficiency diseases are fully capable of making a normal response to vaccines. Those with innate system defects like CGD and other phagocytic cell defects, individuals with complement deficiency, and even some with significant adaptive system deficiencies can produce antibodies to many vaccines and thus benefit from immunization. However, there are many others with primary immunodeficiency diseases that will be unable to develop protective immunity following vaccination, and in some cases the vaccine itself may represent a threat to the recipient.

Since vaccines for chicken pox, measles, mumps, smallpox, rubella, rotavirus, BCG, yellow fever, oral polio and the influenza nasal spray are live attenuated vaccines, individuals with primary immunodeficiency diseases could potentially contract infections if they receive these immunizations. In practice, infants with Severe Combined Immune Deficiency (SCID) are at greatest risk and it is the general recommendation that others with defects in adaptive immunity also avoid receiving any live agent vaccines. (See “Severe Combined Immune Deficiency and Combined Immune Deficiency.”) Since some of these live vaccine viruses (oral polio, rotavirus) can be found in the some body fluids and stools for up to two weeks following vaccination, it may be necessary to limit contact between any recently immunized individual and infants with SCID until the period of viral shedding has passed. For children and adults with primary immunodeficiency diseases who are receiving immunoglobulin (Ig) replacement treatment, the infused antibodies should give them adequate protection from any secondary spread of vaccine virus.

The usefulness of vaccination during treatment with intravenous or subcutaneous Ig therapy is not fully understood, in part due to complexity of the range of underlying immune defects treated with Ig therapy. Assessing patients’ antibody responses to vaccine is confounded by antibody in infused Ig. Vaccines can also stimulate T-cell responses, which may have antibody-independent (such as cellular immune) protective effects, but those are harder to measure and even less well understood with respect to their role in protection independent of immune globulins.

Some patients with milder forms of immunodeficiency (such as selective IgA deficiency, mild hypogammaglobulinemia, partial DiGeorge syndrome) can receive live virus vaccines at the discretion of their doctor.

Purified protein, polysaccharide or non-viable whole-agent vaccines pose no infectious risk to patients. However, for most vaccines, the patient’s antibody response is likely to be inferior to what is provided by Ig therapy. New vaccine agents may be exceptions to this general rule. For example, antibody to new strains of influenza may not be found yet in therapeutic Ig, and consideration should be given to administering such a vaccine to patients receiving Ig treatment. Although there may be theoretical benefit to inducing T-cell responses in patients on Ig, clinical benefit is unproven, and this practice may not be cost-effective, particularly for expensive vaccines such as HPV.

For families with a member who has a primary immunodeficiency disease, we recommend that all members of the family group, including the patient, should keep their immunizations up-to-date. This is particularly important for readily communicable diseases like influenza with many different strains circulating that change from year to year. Why do we recommend that everyone be immunized to influenza? First, some patients with a primary immunodeficiency may respond and benefit directly from the influenza vaccine. Even if they do not, there is little down side to receiving the killed vaccine. Family members who are able to respond to a vaccine will be protected. Even if the patient with a primary immunodeficiency disease does not respond to the immunization, they will benefit from having everyone else in the home protected from infection and thus not susceptible to bringing the virus home with them. This is particularly important if there are other school-aged children in the home. We want to create a “protective cocoon” of immunized persons surrounding patients with primary immunodeficiency diseases so that they have less chance of being exposed to a potentially serious infection like influenza.

Excerpted from the General Care chapter of the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases FIFTH EDITION. Copyright 2013 by Immune Deficiency Foundation, USA. This page contains general medical information which cannot be applied safely to any individual case. Medical knowledge and practice can change rapidly. Therefore, this page should not be used as a substitute for professional medical advice.