IgA-Deficiency

I always got sick as a kid, always had bad infections and it took more than one course of antibiotics to treat them.  As I got older, the infections started to get worse, and harder to control.  I had sinus infections twice a month, I had double ear infections for 5 months straight (I almost had to have tubes as an adult).  Then, I ended up with double pneumonia for 6 months straight, I was put on every antibiotic known to man, and it seemed to just be getting worse.  Finally, after they found out what was wrong, I was able to recover.  I was seen by an immunologist, and he ran some tests on my immune system, because it obviously wasn't working correctly.  I found out I have an IgA-deficiency, an anti-antibody to immonoglobulin A and an IgG subclass 2 deficiency.  What the heck does all of that mean?  Well, I'll explain, and this is what my blog is on today.

First off, what exactly is an antibody?  Antibodies or immunoglobulins are proteins in the body that guard against invading organisms or substances.  Basically, they attack viruses and illnesses to help them go away, our very own antibiotics, so to speak.  There are five types (classes) of immunoglobulins or antibodies in the blood: IgG, IgA, IgM, IgD, and IgE. The immunoglobulin class present in the largest amount in blood is IgG, followed by IgM and IgA. IgD is much lower, and IgE is present in only minute amounts in the blood.  Out of these classes, it is primarily IgG and IgM that protect us from infection. 

One of the most important jobs of protecting the body, is protecting the mucous membranes from the environment.  Mucous membranes are openings in our body that are easily exposed to the environment, they include: mouth, ears, sinuses and nose, throat, airways within the lung, gastrointestinal tract, eyes, and genitalia.  IgA is secreted to those areas, and it is the A antibodies job to protect those areas, other immunoglobulin classes are also found in these areas, but not in nearly the same amount as IgA.   If you were to take our A antibodies that are present and protective in these locations, they would equal one-and-a-half tennis courts.  So the importance of the IgA is very important.

A person with an IgA-deficiency, like me, has no immunoglobulin A to speak of.  In fact, in my case, my body has built up a resistance to A, known as an anti-A-antibody.  An antibody against another antibody, yes that's true, hopefully not too complicated to understand.  My body sees IgA as a foreign substance, if I were to get any, like in a blood transfusion, I'd go into anaphylactic shock.  There is no way to get these antibodies back, and leaves one immune system at risk.  Most doctors don't understand why this happens, there is no known cause.  My doctors believe it could be from years of being on immuno-suppressant medicines for my Rheumatoid Arthritis. 

What are symptoms of having an immune deficiency or how do you even know if you're immune system is missing some of it's important antibodies?  Most people don't experience any symptoms at all, and can go through life having no idea.  My doctors said that I could have had this deficiency for years, there's no way to pin-point when it occurred first.  Other than that,  the most common way to tell is recurrent infections or infections that seem to take more than one course of antibiotics to treat it, both of these happening very often.  The infections that may ocur often are: Bronchitis, recurrent diarrhea, conjunctivitis, mouth infection, ear infection, pneumonia, sinus infection, skin infections, or an upper respiratory infection.  Also, an IgA deficiency can be hereditary, so if it runs in the family, it's a good idea to get tested. 

How do you know if you have an immune deficiency?  If you are experiencing repeat infections, that never seem to go away, it's a good idea to talk to your doctor about it, especially if you're on immuno-suppressant medications.  To test for an immune deficiency is actually very, very simple.  The only way to test to see if a patient has an immune deficiency, is a blood test.  It's just normal blood tests that are taken, and they measure the levels of antibodies in your blood.  Next, they'll give you a vaccine like the flu or pneumonia vaccine and then re-test your blood in due time to see if your body responds to the vaccine as it would to a virus or foreign substance in your body.  If your body is working correctly, your anti-bodies will be elevated after the vaccine, to ward off the bacteria or virus present in the body.  That's how they tested mine, I found out vaccine's don't work on me, since my body doesn't have the antibodies to build up against a virus.  It's as simple as that!

How do you treat something like this? There is no specific treatment, it depends on the person and varies case by case.  Most people just have to go on extra courses of antibiotics when sick.  However if those with selective IgA deficiency who also have IgG subclass deficiencies (like myself) can benefit from immunoglobulin treatments.  My doctor has gone with two different courses of treatment.  I take the antibiotic Azithromycin 500mg, once a week and I also take something called Hizentra.  Hizentra (immune globulin) is a sterilized solution made from human plasma, that contain antibodies (but not A).  It is given subcutaneously, once a week.  As you can see in the photo, there are two needles that look like IVs in my tummy.  Yes, that's me, giving myself my Hizentra.

There is no cure, but some people who just have a low amount of a certain antibody, are able to get them back with medication, or time.  In rare cases, like mine, the body builds an immunity to those antibodies, making the immune system all the more vulnerable.  It puts your body at even more of a risk if you have to be on immuno-suppressant medications for illnesses like Rheumatoid Arthritis.  I have to be extra careful with germs, and have to be on the medicine regime of the Hizentra and antibiotics for life.  It makes going out into public, and seeing friends very difficult, but it's another lifestyle you just have to learn to adapt to.   This is something I had never heard of before being diagnosed.  I think it was important to do a blog on this topic, because you never know who could be experiencing this and have no idea that they could possibly get some help.  As always, I hope my blog was useful and can help others learn a bit more of rare or different things out there.