When part of the immune system is either absent or not functioning properly, it can result in an immune deficiency disease. When the cause of this deficiency is hereditary or genetic, it is called a primary immunodeficiency disease (PIDD).
The immune system is composed of white blood cells. These cells are made in the bone marrow and travel through the bloodstream and lymph nodes. They protect and defend against attacks by "foreign" invaders such as germs, bacteria and fungi.
Important signs that may indicate a PIDD include:
• Recurrent, unusual or difficult to treat infections
• Poor growth or loss of weight
• Recurrent pneumonia, ear infections or sinusitis
• Multiple courses of antibiotics or IV antibiotics necessary to clear infections
• Recurrent deep abscesses of the organs or skin
• A family history of PIDD
• Swollen lymph glands or an enlarged spleen
• Autoimmune disease
Some immunodeficiency disorders are not primary (hereditary or genetic).
A secondary immune deficiency disease occurs when the immune system is compromised due to an environmental factor. Examples of these external causes include infection (HIV), medications (chemotherapy or systemic steroids), severe burns or malnutrition.
Primary Immunodeficiency Disease Treatment & Management
Research in primary immunodeficiency is making great strides, improving treatment options and enhancing the quality of life for most people with these complex conditions. Treatment options are targeted toward the specific immune defects and can include transplantation (bone marrow, stem cell, thymus), immunoglobulin (antibody) replacement, preventative antibiotics and strategies to manage autoimmune disease. Gene therapy has been successful in specific types of PIDD.
If you or your child have symptoms of these sometimes critical conditions, you want the best care available.An allergist /immunologist or a clinical immunologist has specialized training and expertise to accurately diagnose and coordinate a treatment plan for PIDD.