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Among the greatest scientific advances in the past two centuries is the development of effective vaccines. Many diseases that used to be epidemic, causing illness and death are now able to be prevented. Despite their proven efficacy in the population at large, vaccines can be problematic for people with primary immunodeficiency diseases (PI). Since some types of PI interfere with the body’s ability to make antibodies in response to a vaccination, many would ask if it makes any sense or does any good to give vaccines to patients with PI. As part of the PI community, it’s important for you to have the facts about vaccines.

What Vaccines Patients with PI Can Safely Receive

In April 2014, the IDF Medical Advisory Committee (MAC) published an article in The Journal of Allergy and Clinical Immunology called “Recommendations for live viral and bacterial vaccines in immunodeficient patients and their close contacts,” addressing the uncertainty regarding which vaccines can be given to patients with PI. The article also addressed the growing neglect of societal adherence to routine immunizations. IDF acknowledges the tremendous importance of the article and is grateful for the efforts of the MAC. Not all people with PI have problems with vaccines. Those with phagocytic cell disorders, such as Chronic Granulomatous Disease (CGD) or with Complement Deficiencies, benefit from immunization. Patients with these types of innate immune disorders should absolutely receive vaccines to protect them. However, people with T and B cell immunodeficiencies, such as Common Variable Immune Deficiency (CVID), Severe Combined Immune Deficiency (SCID) or Bruton’s agammaglobulinemia, are unable to develop protective immunity following vaccination, so the vaccines would not do them any good. Moreover, some vaccines may threaten the recipient. Live vaccines, such as the chicken pox vaccine (Varivax), measles, mumps, rubella (MMR), rotavirus, BCG, yellow fever, oral polio and the influenza nasal spray, could actually cause the disease it is supposed to prevent in individuals with these types of PI. Infants with Severe Combined Immune Deficiency (SCID) are at the greatest risk for problems with live viral vaccines (LVV). Some live vaccine viruses can be found in the body fluids and stools of vaccinated normal individuals for up to two weeks following vaccination. Because of this, families should limit contact between individuals recently immunized with LVV’s and infants with SCID during that time. For people with antibody deficiencies, immunoglobulin (Ig) replacement therapy is the standard of care. Ig provides these people with the antibodies that their own bodies cannot make, protecting them from vaccine preventable diseases.

The IDF Medical Advisory Committee recommends:
  1. Education about the critical need for maintenance of herd immunity (community immunity) in the population at large. Herd immunity offers valuable protection to patients with PI who are unable to mount protective antibody responses. It is particularly important for family members of patients with defective T and B lymphocyte–mediated immunity to receive all of the available standard immunizations in order to protect their family member with these types of PI.
  2. Avoidance of live viral and bacterial vaccines in all patients with significant T- and B-cell deficiencies. Early diagnosis of these conditions is critical, particularly in infants. Standard immunizations given to infants at 2, 4 and 6 months of age include the rotavirus vaccine which is a LLV. In those states with newborn screening for SCID, via the T-cell receptor excision circle (TREC), infants with low T-cell numbers can be identified shortly after birth. Live viral and bacterial vaccines should be deferred in these infants until they are evaluated by an immunologist. Similarly, any infant born into an extended family with a history of SCID should also have these vaccines held until he/she is evaluated by an immunologist. This latter precaution is particularly important for high-risk families living in states that do not have TREC–based newborn screening.
  3. Determination of the degree of immune reconstitution in patients treated with stem cell/bone marrow transplant, enzyme therapy, or gene therapy before live vaccines are given. These individuals should only be vaccinated after consultation with a clinical immunologist proficient in the diagnosis and management of PI who can explain the risk/benefit ratio for parents or patients.
  4. Parents need to balance the need of the immunoreconstituted child (post-transplant SCID) to be protected from exposure to infection from live vaccines and close contact–transmitted vaccine-derived infection with the need of the child to integrate into society and develop social and learning skills in group environments.

Click here to download the chart "Specific Recommendations for Immunization of Children and Adolescents with Different Types of PI." For a person with PI, vaccines are an important weapon in the arsenal to prevent infection and stay healthy. People with PI, their parents and family members should consult their immunologist and primary care providers to determine the need for vaccination. In all cases, parents and patients with PI should consult their healthcare providers for immunization recommendations and further information. For more information about the flu vaccine, CLICK HERE.