About Primary Immunodeficiency Diseases

What are Primary Immunodeficiency Diseases?

Nearly everyone has suffered from colds, the flu, or sinus and ear infections. Just as many have been affected by cuts, scrapes and abrasions that become infected. Even in the case of more severe infections – such as pneumonia – we expect the cough and congestion to ‘run its course’, aided by prescription antibiotics, over-the-counter symptom remedies, and our body’s own immune system.

Recovery times vary, but the human body can usually rid itself of the infection-causing germs, and work to defend against future “bugs” and viruses. There are, however, instances in which the body cannot recover, and some of these apply to individuals with a primary immunodeficiency disease.

Primary immunodeficiency diseases occur in persons born with an immune system that is either absent or hampered in its ability to function. While not contagious, these diseases are caused by hereditary or genetic defects and can affect anyone, regardless of age or sex. The World Health Organization recognizes 185 primary immunodeficiency diseases – some are relatively common, others are quite rare. Some affect a single cell within the immune system; others may affect one or more components of the system.

And while the diseases may differ, they all share one common feature: each results from a defect in one of the functions of the body’s normal immune system. Because one of the most important functions of the normal immune system is to protect us against infection, patients with primary immunodeficiency diseases commonly have an increased susceptibility to infection.

The infections may be in the skin, the sinuses, the throat, the ears, the lungs, the brain or spinal cord, or in the urinary or intestinal tracts, and the increased vulnerability to infection may include repeated infections, infections that won’t clear up or unusually severe infections. People with primary immunodeficiency diseases live their entire lives more susceptible to infections–enduring recurrent health problems and often developing serious and debilitating illnesses. Fortunately, with proper medical care, many patients live full and independent lives.

Is it just an infection?

Do the same symptoms keep coming back or never seem to completely clear? Is the infection severe enough to require hospitalization or intravenous antibiotics? Is there a family history of early infant death or of susceptibility to infection?

If you answered yes to any of these questions it is critical to get an early diagnosis and proper medical care – don’t hesitate to ask your physician to check for the possibility of a primary immunodeficiency disease.

Please contact the Immune Deficiency Foundation with any questions you may have about primary immunodeficiency disease through Ask IDF or call 1-800-296-4433.

Click Here to See IDF Publications with More Information on Primary Immunodeficiency Diseases

Click Here to Read More About Specific Types of Primary Immunodeficiency Diseases