Primary immunodeficiency diseases (PI) are a group of more than 300 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly. While not contagious, these diseases are caused by hereditary or genetic defects, and, although some disorders present at birth or in early childhood, the disorders can affect anyone, regardless of age or gender. Some affect a single part of 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 PI 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 PI 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 (IDF).
- CVID, the most common immunodeficiency is found in about 1 in 25,000
- It takes an average of 9-15 years after onset of symptoms to receive diagnosis
- A batch of Ig replacement takes 10,000-50,000 plasma donors
- Approximately 25,000 Americans have been diagnosed with PI
- An estimated 25,000 more have not yet been diagnosed
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