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Shot or Not:
Should Children with PI Receive Immunizations?

Understanding the risks and benefits

Since the 1950s, vaccines have been used to ward off many childhood illnesses, including polio, measles and mumps. The immune system, a complex network of organs, tissues, cells and proteins, normally responds to vaccines by fighting off the weakened or killed form of the germ used to make the vaccine. Immunity is the end result.

In some children with a primary immune (PI) deficiency, however, their immune systems are unable to fight the germs used to create certain vaccines—especially those that use a live virus. While some vaccines may be risky for children with PI, others may be beneficial.

How do you know if a vaccine will help or harm your child? According to physicians, it depends upon the type of immunization, the type of treatment your child is receiving, and the specific PI disease.

How Vaccines Work. Vaccines use a harmless form of a germ to 'trick' the immune system into believing that the body is being invaded by disease. Vaccines stimulate the immune system into producing antibodies, a protein in the blood that helps to strengthen immunity. Immune cells such as the B and T cells play an important role in helping the body to fight disease. Each type of immune cell has a specific role. For example, B cells form plasma cells that secrete antibodies, which help to:

  • Neutralize toxins,
  • Aid white cells in destroying germs,
  • Group viruses together,
  • Activate the complement system, comprised of 20 proteins important in immunity and inflammation,
  • Produce memory cells to help the body fight future infection.

T cells secrete powerful chemicals called cytokines that in turn stimulate B cells and control inflammation and healing. Some T cells turn into killer cells, directly attacking cells that have been infected by virus.

Different Immune Diseases. For each of the over 100 types of immune disease, the response to vaccinations may be different. For example, children with B cell problems cannot respond to vaccines by making an antibody. A child with Severe Combined Immune Deficiency will not benefit from any vaccines and could be harmed if given a live virus or bacterial vaccine. However, a child with Partial DiGeorge Syndrome will respond to most vaccines. Children with defects in other parts of the immune system, such as Chronic Granulomatous Disease, can respond normally and should be immunized. Be sure to check about benefits and risks of each vaccine for your child's specific immune disease.

No Live Vaccines. Children with PI lack the immune defenses necessary to fight certain vaccines.
They should not, for example, receive any form of live virus or bacteria vaccine, including measles,
mumps, BCG, chicken pox (varicella) 1- 2 and the nasal vaccine for influenza, FluMist®. In addition,
family members or caregivers of PI patients should not receive live virus vaccines because they
might spread the virus to the child with PI. It's also important for children with PI to avoid contact
with others who may have received a live vaccine.

Unlike live vaccines, vaccines that have been killed do not pose as high a risk to children with PI.
For example, the killed influenza vaccine is a safe alternative to the live flu vaccine, as are other
forms of killed vaccines.

Role of IVIG Therapy. If your child is receiving IVIG, there is a risk that it may interfere with the effectiveness of certain vaccines, even causing the vaccine to fail. In general, this is not a problem with tetanus or influenza vaccines, but it may be problematic with measles and varicella vaccines. Physicians recommend waiting at least three months after the child has completed IVIG before vaccinating the child with most vaccines, and one year after IVIG before vaccinating the child with the measles vaccine.

Talk to Your Healthcare Professional. Researchers are constantly developing vaccines to prevent new and emerging diseases. Use of these and other vaccines in children with PI must be determined on a case-by-case basis.

For more information about vaccines and primary immune deficiency, talk to your physician.

1. National Institutes of Child Health and Human Development. National Institutes of Health. When the Body's Defenses are Missing: Primary Immunodeficiency. NIH Pub No. 99-4149: p17.
2. Winklestein JA, Winklestein ML, editors. Patient and Family Handbook For the Primary Immune Deficiency Diseases, Third Edition. Towson, Md.: Immune Deficiency Foundation: 2002: pp27-28, 39.

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