Primary immunodeficiencies (PIDs) are a group of over 300 different conditions that affect how the body’s immune system works.
People affected by PIDs have parts of their immune systems missing or not working. This leaves them with reduced or no natural defence against germs such as bacteria, fungi and viruses, which surround us everyday.
The consequences are that people with PIDs get infections more often than is normal; they can take longer to get better when they have antibiotic treatment and even then the infections can keep coming back.
How do people get PIDs?
PIDs are caused by errors in the genes of the cells that make up the immune system. Most people with PIDs are born with the condition. PIDs are mainly genetic disorders, meaning they are inherited and can be passed on from one generation to the next.
In PIDs some of the DNA building blocks that make up the genes involved in the workings of the immune system are either not there or altered. This results in either missing or faulty genes, causing essential parts of the body’s defence against infection not to be made or work properly.
In some types of PID, for example, common variable immune deficiency (CVID) and other antibody deficiencies, the link to specific faulty genes is not yet so clear-cut, but this is likely to change as the technology for the screening of genes improves and more research is done.
How serious are these conditions?
The effects of a PID can be immense. For an adult, frequent debilitating illnesses can make it more difficult to pursue a career or enjoy family life. There can be psychological problems, too. Feeling isolated, the fear of losing one’s income, or the anguish of caring for a sick child, can take its toll.
http://www.immunodeficiencyuk.org
Immunodeficiency UK is an organisation supporting individuals and families affected by a primary immunodeficiency in the UK. It is the national member organisation of the International Patient Organisation for Primary Immunodeficiencies. You can learn more about PIDs on Immunodeficiency UK’s website
My Condition
After years of my doctors thinking I had Common Variable Immunodeficiency (CVID) examinations a few years ago confirmed that this was not the case. My current diagnosis is that of Primary Immune Dysregulatory Disorder.
My doctors have not been able to identify exactly which genes have caused my immunodeficiency but I do know that without my weekly infusion of immunoglobulin I’d be seriously ill or worse.
Basically my immunoglobulin saves me life. It is invaluable to me and it’s value is why a lot of patients call it “liquid gold”.
Immunoglobulin is made from donated blood-derived plasma. I’m at a greater risk of infections than other people and the immunoglobulin means I get fewer infections and the ones I do get can be less less serious than if I didn’t receive this treatment.
So every week I sit for an hour to infuse my immunoglobulin into my stomach (subcutaneously). Aside from a slight issue with needles I don’t remember ever having a big issue with my infusions and having done it since I was a baby it’s part of my routine, although I’m sure my mum would say differently.
How Rare? 