What are immune deficiency disorders?
Primary immunodeficiency disorders, also called immune deficiency disorders, are conditions due to defects in the body’s immune system, allowing infections and other health problems to occur more easily. Many people that are born with immunodeficiency are born missing some of the body’s immune defenses or with the immune system not working properly, which leaves them more at risk.
What are causes of immune deficiency disorders?
Many immunodeficiency disorders are genetic, passed down from one or both parents. Problems in the genetic code are the main cause of these disorders. While there are more than 300 types of immunodeficiency disorders, they can be classified into six groups based on the part of the immune system that is affected:
- B cell (antibody) deficiencies
- T cell deficiencies
- Combination B and T cell deficiencies
- Defective phagocytes
- Complement deficiencies.
- Unknown deficiencies
Secondary immunodeficiency disorders result from various conditions, including cancer therapies, bone marrow transplants, and immune-modulating medications that are used to treat a variety of autoimmune disorders.
What are symptoms of an immune deficiency disorder?
One of the most common signs of an immune deficiency disorder is having infections that are more frequent, longer lasting, or harder to treat than those of someone with a typical immune system. You may also notice that you get infections that a person with a healthy immune system would not get.
Signs and symptoms will differ from person to person and can be different depending on part of the immune system that is affected. Signs that you or your child might have a primary immunodeficiency 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
How are immune deficiency disorders diagnosed?
Immunodeficiency disorders are diagnosed via blood testing that looks at specific aspects of your immune system. It is important to have this evaluation through a board-certified allergist/immunologist.
How are immune deficiency disorders treated?
- Treating infections – some infections require rapid and aggressive treatment with antibiotics. This may require a longer course than prescribed depending on the infection.
- Preventing infections – long-term antibiotics can be a strategy to prevent respiratory infections and permanent damage to the lungs and ears.
- Immunoglobulin therapy – Immunoglobulin replacement therapy contains proteins that your immune system uses to fight infections (immunoglobulins and antibodies). Replenishing these back to a normal level can help prevent further infections. Immunoglobulin replacement can be done in the vein (IV) or under the skin (subcutaneous).
- Treating underlying causes – in some cases transplant or gene therapy is needed. These are targeted toward the specific immune defect.
