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What is Immune System?

Immune system is a part of the body that is involved in defending against foreign material (antigens) i.e. bacteria, viruses, fungi of all kind, allergens, as well as keeping in check unwanted cells or tissues like cancer or transplanted tissue like donor kidney, liver, heart etc.

If one or more pieces of immune system are missing due to whatever reason, patient will experience severe, repeated, persistent and unusual infections. Patients will often say “I am sick all the time” to summarize their conditions. Apart from infections, patients with defective immune experience bread and butter allergy issues as well as higher risk of cancers. They tend to experience autoimmune disorder more often i.e. lupus, rheumatoid arthritis, I.T.P., inflammatory bowel disease etc. You should suspect immunodeficiency if you are experiencing a lot of infections.

How Frequent are Primary Immune Deficiency?

There are more than 200 individual disorders affecting as few as one patient to thousands of patients. Selective IgA immunodeficiency, most common of primary immunodeficiency affects 1 in 500-700 people worldwide. Approximately 0.5 million in U.S. have this disorder.

Most people are aware of HIV infections or AIDS (Acquired Immunodeficiency Disease) but not primary immunodeficiency. Primary immunodeficiency cases are found worldwide and can affect anyone. Many are inherited but not all.

How Can We Help?

Allergists and immunologists are trained to diagnose and find help with the treatment of some relatively benign disorders to life threatening diseases, which may require bone marrow transplant. If diagnosed early, these patients can have healthy lives.

Treatment Options

Depending upon what component of immune system is missing or defective, here are some of the treatment options available:

• Patient who are predisposed to infection with pneumocystis carinii will benefit from bactrim prophylaxis. Many patients will repeat pulmonary and sinus infections need prophylactic antibiotics.
• Intravenous and subcutaneous immunoglobulin therapy in patients with low or non functioning IgG. However, patient with low IgA and low IgM don’t benefit from replacement therapy.
• Many patients benefit from bone marrow transplant, gene therapy and replacement deficient protein i.e. PEG-ADA in patients with AD Adeficiency.
• There may be no treatment available to fix the problem i.e. patients with complement defects may benefit from early diagnose and treatment of infections.

Most importantly, finding what is wrong with the patient can put an end to frustrating search and allow healing to begin.