WHAT IS PRIMARY IMMUNODEFICIENCY DISEASE?
Primary Immunodeficiency Diseases are disorders in which part of the body's immune system is missing or does not function properly. In contrast to secondary immune deficiency disease in which the immune system is compromised by factors outside the immune system, such as viruses or chemotherapy, the primary immune deficiency diseases are caused by intrinsic or genetic defects in the immune system.
Primary Immunodeficiency Disease takes so many forms. Early diagnosis and treatment is key in order to prevent permanent organ damage to individuals affected by this disease. The World Health Organization recognizes over 150 primary immune deficiency diseases including X-linked Agammaglobulinemia (Bruton's Disease), Common Variable Immune Deficiency, Selective IgA Deficiency, and Severe Combined Immune Deficiency (boy-in-the-bubble disease). Some disorders, such as Selective IgA Deficiency can be quite common, occurring as often as 1/500 to 1/1000 individuals. Others, such as Severe Combined Immune Deficiency, may be as rare as one individual affected per million. Untreated primary immune deficiencies may be characterized by frequent life-threatening infections and debilitating illnesses.
Because of advances in our medical understanding and treatment of primary immune deficiency diseases, individuals who in the past would not have survived childhood are now able to live nearly normal lives. Many individuals affected by primary immune deficiency diseases require life long therapies including intravenous gamma globulin infusions, antibiotic therapies, or bone marrow transplantation. The Immune Deficiency Foundation is dedicated to improving the diagnosis and treatment of primary immune deficiency diseases through research and education.
TOP INDICATORS PIDD MAY BE PRESENT
If you have infections that are:
Recurrent - keep coming back
Persistent - Won't completely clear or are slow to clear
Severe - Require hospitalization or IV antibiotics
Unusual - Caused by an uncommon organism
If you have a family history of PIDD
If any of these describe your infections, or if you have a family history paired with recurrent, persistent, severe or unusual infections, ask your physician to check for the possibility of a Primary Immunodeficiency Disease.
FACTS ABOUT PIDD
PIDD is NOT contagious!
PIDD is considered a life-threatening illness
There are currently over 150 different types of PIDD recognized by the World Health Organization (WHO).
DISEASE SPECIFIC PATIENT ORGANIZATIONS
non-profit organization dedicated to raising money for Shwachman-Diamond Syndrome research and education.
Ataxia Telangiectasia (A-T) Children's Project
A non-profit organization dedicated to accelerating the research toward a cure for Ataxia Telangiectasia. They also provide educational literature on AT.
Chronic Granulomatous Disease (CGD)
A web site designed to provide information about Chronic Granulomatous Disease. Periodic chat sessions are held for members of the group.
Hereditary Angioedema Association
An organization dedicated to providing education about Hereditary Angioedema or C1 Inhibitor Deficiency Disorder.
Severe Combined Immunodeficiency (SCID)
A web site designed to provide information about Severe Combined Immunodeficiency Disease. The site includes an email listserve for parents to talk with others about this disorder.
IMMUNE DEFICIENCY FOUNDATION
The IDF is vigorously working to raise awareness so the time between onset of disease and diagnosis is significantly reduced. Please continue to support the IDF and their mission of raising awareness and advocacy!
For more information, please contact the IDF at www.primaryimmune.org or call them at 1-800-296-443.
All information viewed on this page is courtesy of the Immune Deficiency Foundation.