Primary Immunodeficiency. What exactly is it? Well, that’s really hard to say because it’s not EXACTLY anything in particular. Primary Immunodeficiency refers to any of over 400 rare, chronic disorders in which the immune system is missing or has improperly functioning components. With over 400 conditions under the umbrella, it’s impossible to point out one feature and say “That’s primary immunodeficiency.” The thing all these conditions have in common is an impaired immune system, to some degree or another.
The stress of having a disorder of any sort is multiplied by the rarity of the disorder. The total population of people in the US diagnosed by any of the 400 conditions is about 270,000. To put that into perspective, the entire country has about as many people diagnosed with PI as would equal 1/6th the population of Philadelphia, or about 30,000 less than the population of Pittsburgh. That’s 1 out of 1,200 people in the United States.
Now I’m no expert on PI. In fact I have known next to nothing about PI until I started researching this article. I do have a rare condition though, so I understand a little about that dynamic. I know that I know much less than anybody who is affected by this group of conditions. But perhaps pulling information from multiple sources and presenting it in a layman’s terms can be helpful to even those who are experts, the affected individuals themselves. If not, at least some others who are like me, novices in the PI world, may learn something about these disorders.
Perhaps first we’ll talk about some of the general groups of conditions that fall under the PI umbrella. Antibody deficiencies include Agammagloublinemia, Common Variable Immune Deficiency and Hyper IgM. Common Variable Immune Deficiency may also be associated with low T cell counts so that condition actually may act like it falls into the next category. Significant T cell deficiencies include Wiskott-Aldrich Syndrome, Combined Immune Deficiency and DiGeorge Syndrome. Phagocytic defects include Chronic Granulomatous Disease and Neutropenia.
These categories all react a little differently to the COVID 19 infection. The first of these categories, antibody deficiencies, seem to be not affected by SARS_CoV_2, the virus that is responsible for COVID 19, but may play into the functioning of a potential vaccine. The second category, those conditions related to low T cell counts, can be very susceptible to any infection and so the COVID 19 infection must be avoided, if at all possible. The third of these categories may not be directly affected by COVID 19, however with any viral infection the chances increase for an infection with a super bacteria. Phagocytic defects generally make a person much more susceptible to bacterial infections.
I think that we’ll explore the vaccines that are in development more in the next blog entry. Also, we’ll look at how HIV infection interacts with the SARS-CoV-2 virus and COVID 19 infection. Until next time!
Please note: I am not an expert on PI, COVID 19 or SARS-CoV-2. I would encourage you to read or view the information in the following source documents and videos:
- Day 100 of the Pandemic: COVID-19 for Antibody Geeks, video, Immune Deficiency Foundation, 6/18/2020
- What is primary immunodeficiency (PI)?, webpage, my Ig source
- Coronavirus (COVID-19), website, Centers for Disease Control and Prevention
- Coronavirus Resource Center, website, Johns Hopkins University & Medicine
- Primary Immune Deficiency Diseases, website, NIH National Institute of Allergy and Infectious Diseases