Did you or a family member struggle through winter with one illness after another such as an ear or sinus infection, pneumonia or a skin condition that would not heal? You took antibiotics but saw little or no improvement? If so, you could have a Primary Immunodeficiency Disease (PIDD).
PIDD causes children and adults to have infections that come back frequently or the conditions are unusually hard to cure. 1:500 persons are affected by one of the known PIDDs.
Immunology experts explain it this way. 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). Researchers have identified more than 150* different kinds of PIDD.
The immune system is composed of white blood cells, immunoglobulin and other proteins which assist white blood cells in defending against attacks by "foreign" invaders such as viruses, bacteria and fungi. The most common PIDDs involve deficiencies in immunoglobulin or antibody production. A pattern of repeated infections, severe infections and/or infections that are unusually hard to cure is suggestive of PIDD. Most often these infections involve include the skin, respiratory tract (including ears, sinuses and lungs), and the gastrointestinal system.
If you or someone you know is affected by two or more of the following symptoms, speak to your physician about the possible presence of an underlying Primary Immunodeficiency.
- Four or more new ear infections within 1 year.
- Two or more serious sinus infections within 1 year.
- Two or more months on antibiotics with little effect.
- Two or more pneumonias within 1 year.
- Failure of an infant to gain weight or grow normally.
- Recurrent, deep skin or organ abscesses.
- Persistent thrush in mouth or fungal infection on skin.
- Need for intravenous antibiotics to clear infections.
- Two or more deep-seated infections including septicemia.
- A family history of PIDD.
Primary Immunodeficiency Disease is inherited. This means that a person is born with altered genes received from his or her parents. People with PIDD are vulnerable to viral and bacterial infections, such as colds or sinusitis, and may “give” such an infection to another person, but the PIDD condition itself is not contagious. You cannot catch a primary immune deficiency disorder.
Immunoglobulin (IgG) is the part of blood plasma that contains antibodies. People suffering from immunodeficiency diseases involving poor IgG levels and/or function often benefit from a medical treatment called immunoglobulin replacement therapy, also known as IVIg or SCIg.
Immunoglobulin is prepared from the plasma collected from a large number of normal individuals, usually between 10,000-50,000, who have been carefully screened to make sure they are healthy and do not harbor certain infectious diseases. The plasma contains a broad range of specific antibodies to many different types of bacteria and viruses. Each plasma donor must be acceptable as a blood donor according to the strict rules enforced by the American Association of Blood Banks and the U.S. Food and Drug Administration (FDA).
IgG supplementation therapy involves giving antibodies that the body needs to fight infections. . Because antibodies provided by IgG infusions only last for 3-4 weeks and the infusions do not help the patient’s own immune system make more, regular dosing is required to maintain healthy levels of IgG.
PIDD can show up during the early months of life or manifest much later as an adult. The important thing is to detect, diagnose and treat PIDD before serious infections cause permanent damage.
Thanks to dedicated medical research, there are treatments and doctors who know how to help patients with PDD. When PIDD is properly managed, patients can live normal productive lives. While Primary Immunodeficiency Disease is a relatively new field for medical study, impressive advances continue to be made thanks to the commitment of dedicated doctors and researchers.