Primary Immune Deficiency


Most people don’t consciously think about all the day-to-day exposure we come into contact with, as we typically rely unknowingly on our immune system to fight off colds, infections, viruses and other disease-causing microbes. The immune system works efficiently to protect our bodies. Therefore, people who suffer from immune deficiencies are at a much higher risk of infection, because they lack an immune system capable of fighting these infections and viruses.

Primary immune deficiency diseases impact people who are born without a properly functioning immune system, or one that is nonexistent. These diseases are not contagious, but unfortunately, they are inherited from family members. Genetic defects can lead to an immunodeficiency disease as well. The World Health Organization (WHO) identifies more than 150 primary immunodeficiency diseases. The increased vulnerability to infection for those with primary immune deficiency diseases sometimes results in repeated, long-term or unusually severe infections.

Symptoms:

Symptoms of primary immune deficiency diseases can vary wildly form person to person, from severe to cases so mild they are undetected for years. What’s more, symptoms can differ depending on the particular type of disorder that infects a person. Infections and viruses can appear in many parts of the body, including the skin, brain, ears, throat, sinuses, lungs, spinal cord, and the urinary and intestinal tracts.

Among the many signs and symptoms of primary immune deficiency diseases are:
• Frequent, recurring ear infections, pneumonia, meningitis, bronchitis, sinus infections or skin
infections
• Blood infections
• Inflammation and infection of internal organs, such as the liver
• Autoimmune disorders, such as lupus, arthritis or type 1 diabetes
• Blood disorders, such as low platelet counts or anemia
• Digestive problems, such as cramping, loss of appetite, nausea and diarrhea
• Delayed growth and development

Treatment:

Since people suffering with primary immune deficiency are more susceptible to infections due to the lack of antibodies in their system, they typically need immunoglobulin, which helps the body to kill germs that could cause infections. Intravenous therapy can help the body generate this vital immunoglobulin. Most side effects of this treatment are mild and can be by slowing down the rate of infusion instead of discontinuing therapy.

People with primary immune deficiency diseases live their entire lives more susceptible to infections, and if their conditions are not treated, it can lead to recurring or long-term health problems, including permanent damage to organs such as the ears or lungs, or physical disability. Fortunately, with proper medical care, many patients live full and independent lives.

Sources:

http://www.primaryimmune.org/about_pi/about_pi.htm

http://www.mayoclinic.com/health/primary-immunodeficiency/DS01006/DSECTION=symptoms

http://www.immunedisease.com/patients-and-families/Ig-therapy/

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