April is Primary Immunodeficiency Disease (PIDD) Awareness Month in the USA. The governor issued a proclamation declaring April Primary Immunodeficiency Awareness Month in Louisiana.
PIDD affects people of all ages and can vary in severity and morbidity. While some people are born with PIDD, others develop PIDD as children or adults.
Because of lack of awareness and delayed screening, the lag between onset of symptoms and making a definite diagnosis may be over 10 years. The prevalence of PIDD is about 1 in 500 for Selective Ig A deficiency; to 1 in 10,000 for Common Variable Immunodeficiency; to 1 in 50,000 for Severe Combined Immunodeficiency (David Vetter, the famous Bubble Boy).
Symptoms can range from commonly recognized and repeated infections involving the ear, sinus, throat and bronchial tree or pneumonia, abscess formation involving different parts of the body, meningitis and infections of the gastrointestinal tract. Patients can present with recurrent, protracted and at times severe, difficult-to-treat infections with uncommon or unusual bacteria identified only by cultures.
Complications of the PIDD can include unregulated fever, allergies, autoimmune diseases and malignancies.
If you or your children get recurrent infections, think of PIDD and get screened. Physicians, health-care providers and pharmacists may refer their patients with recurrent infections, requiring multiple courses of oral or IV antibiotics for PIDD screening.
Diagnosis of PIDD is made by specific blood tests. Once diagnosed, the patients are treated with antibiotics and monthly IV or weekly subcutaneous immunoglobulin infusions. Certain PIDD are successfully treated with bone marrow transplant and gene therapy.
Please visit http://www.primaryimmune.org.
Dr. Prem Menon
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