Primary Immune Deficiency Diseases

If the school nurse has you on speed dial and you find yourself blazing a path to your child’s school due to repeated infections, you may be dealing with something other than the average cold or allergies.

“In children, infections are very common, especially when they go to school or daycare,” says Prem Menon, MD, founder of the Asthma, Allergy and Immunology Center in Baton Rouge. Repeat infections that don’t clear up may point to something more, such as Primary Immune Deficiency Diseases, or PIDD. 

PIDD are a group of more than 300 conditions caused by defects in the immune system. Most PIDD are inherited and present at birth, but they can be diagnosed at any time of life. PIDD can affect the immune system in every part of the body, Dr. Menon says. And infections can be anywhere, including skin, the respiratory system, ears, brain or spinal cord, or urinary or gastrointestinal tract.

At least 250,000 people in the U.S. have PIDD, Dr. Menon says. “It’s common, but it is not commonly diagnosed.” Getting a diagnosis can take years for patients if they don’t see an allergist or immunologist. 

PIDD can be diagnosed by measuring antibody levels. Treatments vary, where the first may be additional immunizations or preventive antibiotics. Stem cell transplants would also be considered for some patients, and certain immune deficiencies can be treated by gene therapy. 

Intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG) infusions are other PIDD treatment options. IVIG infusions need to be repeated every three or four weeks, and SCIG can vary, depending on the patient.

Immunoglobulin treatments can work alongside children’s maturing immune systems, and some patients get better. Dr. Menon says, “Sometimes, if a two-year-old patient is diagnosed with PIDD, by first grade, I’ll be able to stop the IVIG because they won’t need it.”

If you or your child have PIDD symptoms, an allergist or immunologist can ensure an accurate diagnosis and treatment plan.

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20 Jan 2020

By Mari Walker

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