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Immune deficiency diseases
PrintSelect any question to learn more about immune deficiency diseases below:
What are primary and secondary immune deficiency diseases?
How does the immune system work?
How are primary immune deficiency diseases diagnosed?
What treatments are available for primary immune deficiency?
What should I do while on immuneglobulin therapy?
Where can I find more information?
What are primary and secondary immune deficiency diseases?
There are two types of immune deficiency diseases: primary and secondary. Primary immune deficiency is when the body's immune defense system is missing a part or does not work correctly. This condition is caused by a genetic problem in the body's defense system. There are over 150 different primary immune deficiency diseases. Some occur with great frequency (Selective IgA deficiency), while others are extremely rare (ADA deficiency). If a primary immune deficiency is not treated, it can cause serious illness and life-threatening infections.
Today, thanks to enhanced technology, doctors can recognize the signs of primary immune deficiency even earlier, so people can live close to normal lives with treatment. Before, treatment options were limited, and life expectancy was shorter for some of these individuals.
Secondary immune deficiency disease is when the body's defense system is changed due to an external factor outside the body. Some examples include dialysis (a treatment that does some of the things done by healthy kidneys), burns, chemotherapy, exposure to toxic chemicals or UV radiation, or a viral, bacterial or parasitic infection.
Our focus will be on primary immune deficiency disease since secondary is treated in so many different ways, depending on the external agent that enters the person's body.
How does the immune system work?
The immune system is very complicated. B-cells (B-lymphocytes) develop from cells inside the bone marrow, lymph nodes, and spleen. The blood carries the cells and proteins of the immune system from one part of the body to another. Foreign materials (antigens), such as germs, cause the B-cells to develop. B-cells grow and mature into plasma cells that produce antibodies. Antibodies and antigens fit together like a lock and key and attach to each other, which activates the immune system to destroy the foreign material. Gamma globulins are the protein fraction of the blood that contains immunoglobulins or antibodies.
There are four major classes of antibody proteins: Immunoglobin G (IgG); Immuniglobin A (IgA); Immuniglobin M (IgM); and Immuniglobin E (IgE). Each class has special characteristics. For example, IgG is formed in large quantities, lives for a month, and travels from the bloodstream to the body’s tissues; even crossing the placenta. IgA is produced near mucous membranes and finds its way into the secretions of tears, bile, saliva, and mucus while protecting the respiratory tract and intestines from infection. IgM are the first antibodies to be formed in response to any infection. And the IgE class is responsible for allergic reactions.
How are primary immune deficiency diseases diagnosed?
A careful medical and family history and a thorough physical exam by your doctor are completed before any laboratory evaluation is initiated. Usually screening tests are completed before the more sophisticated and costly tests are ordered. The range of laboratory testing available today is impressive and continues to expand. Currently, there are four major categories of tests designed to screen for immune deficiencies.
What treatments are available for primary immune deficiency?
Many patients with primary immune deficiency will need treatment with intravenous immunoglobulin (IVIG) and antibiotic medications. Gamma globulin is extracted from healthy people's donation of plasma and is purified through a manufacturing process before administering. Other patients may need more invasive therapies.
What should I do while on immuneglobulin therapy?
- Avoid infection:
- Establish good hand washing habits.
- Avoid individuals who are ill or large crowds during cold or flu season.
- Do not share water bottles or drinking glasses.
- Maintain a healthy lifestyle:
- Visit your dentist every six months and brush and floss regularly.
- Participate in appropriate physical activities approved by your doctor.
- Get an appropriate amount of sleep each night.
- Keep your stress level manageable.
- Be aware of the signs of infection and report them right away to your doctor.
- Learn how to manage your therapy side effects, including knowing when to contact your doctor.
- Ask your doctor if you should be on a special diet if you have an infection (avoid taking any herbs or nutritional supplements until you consult your doctor).
Where can I find more information?
- Learning about your treatment is important, so that you can take an active role in your everyday life. To learn more about immune deficiency, please visit: www.primaryimmune.org, www.niaid.nih.gov/final/immds/immdef.htm or www.immunedisease.com.
Inclusion of resources on this list does not imply endorsement by Walgreen Co. or any of its subsidiaries or affiliates. These resources should be used for general information and educational purposes only. Information provided by these resources should not replace necessary medical consultations with a qualified health or medical professional.
Information provided here does not constitute professional medical advice. Although it is intended to be accurate, neither Walgreen Co., its subsidiaries or affiliates, nor any other party assumes liability for loss or damage due to reliance on this material. If you have a medical question, consult your medical professional.