Living with Primary Immunodeficiency

Ever since my second child was born in 2013, I haven’t been the same.

My pregnancy, delivery, and recovery seemed uneventful enough (apart from a short bout of postpartum anxiety), so I had no reason to believe I would have lasting complications. But there have always been these nagging symptoms, and after four years I was determined to get to the bottom of it.

Every 4-8 weeks I develop what I have simply named “the virus” which begins as a sore throat and presents itself as at least 48 hours of extreme exhaustion. I spend about two days sleeping and barely able to lift my head. Unable to diagnose this, I have simply accepted it as how my body reacts when presented with any kind of contagion. I rarely have the normal symptoms of a common cold – I just get this. All. The. Time.

I also experience frequent chronic sinus and respiratory infections and daily exhaustion that forces me into bed well before sunset on summer nights.

My quest to solve this medical mystery was kicked into high gear in May 2016 when I experienced the pinnacle of enigmas – I almost died of septic shock. The sepsis was caused by random infectious colitis which has never been traced. All the bacteria tests were negative and I swear I was not food poisoned. I am fully convinced that my gut bacteria had been shot to hell from all the antibiotics and steroids I was taking to try to kick my chronic infections. A random pathogen entered and my body reacted hard.

Bloodwork a couple months after Sepsis showed that I had Selective IgA Deficiency (also known as primary immunodeficiency or PI), an immune disease that makes me prone to infections and allergies. At the time I just sort of shrugged and figured that probably explained a lot but didn’t give it much more thought.

But after sinus surgery didn’t really improve my frequent infections and the frequency of the “virus” seemed to pick up speed, I started thinking more about that bloodwork. I went back to have it tested again and found that not only was my IgA level even lower, I was also now showing low levels of IgG and freakishly high levels of IgE. This means I have a type of PI called Common Variable Immune Deficiency (CVID)

Basically, the immunoglobin in our blood is comprised of five different letters. Three of mine are totally out of whack, leaving me susceptible to every gnarly germ on a door handle. And, since I refuse to wear rubber gloves or live in a bubble, I never know what to expect.

Primary immunodeficiency diseases (PI) are a group of more than 300 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly. While not contagious, these diseases are caused by hereditary or genetic defects, and, although some disorders present at birth or in early childhood, the disorders can affect anyone, regardless of age or gender. Some affect a single part of the immune system; others may affect one or more components of the system. And while the diseases may differ, they all share one common feature: each results from a defect in one of the functions of the body’s normal immune system.

Because one of the most important functions of the normal immune system is to protect us against infection, patients with PI commonly have an increased susceptibility to infection. The infections may be in the skin, the sinuses, the throat, the ears, the lungs, the brain or spinal cord, or in the urinary or intestinal tracts, and the increased vulnerability to infection may include repeated infections, infections that won’t clear up or unusually severe infections. People with PI live their entire lives more susceptible to infections–enduring recurrent health problems and often developing serious and debilitating illnesses. Fortunately, with proper medical care, many patients live full, independent lives.

Unfortunately, there is no cure – only “symptom management” – which basically means sleeping way more than I’d like and being a bit high maintenance when it comes to self-care. I never know when I will have to cancel plans last minute or have to find someone to pick up my kids from school. I can’t share hotel rooms with friends because of my demanding sleep schedule, I can’t consume alcohol, and I, by no means, can attend a dinner or party that ends after 9 p.m.

In college, I drank every night and stayed up until dawn. In my 20s I was the life of the party. I don’t miss or long for those days – I’m glad I had them! It would just be nice to not have to be the actual worst all the time.

But here’s the thing: some days I feel pretty great. I can do a seriously hardcore workout and manage to juggle work, kids, and home for the rest of the day. Some nights I can even get dinner on the table. And I have been able to drag myself to evening events as long as they don’t run too late.

Invisible illness can look like this.

In fact, people probably see me climbing a rope and wonder what the hell I’m complaining about! That’s the thing with invisible illnesses – you can’t always see them but they are always there.

This is how I visualize primary immunodeficiency.

Sometimes you wake up with all the hearts. Sometimes you wake up with none. Usually, if you start out with full hearts you are down to half by dinnertime. On a great day, you can rock full hearts really hard for a morning workout. Unfortunately, there is no magic potion for when the hearts are down. You just have to rest really, really hard.

I will be going to an immunologist in a few weeks to see if there is anything that might be helpful. I know that some people with PI get regular blood transfusions, but I have no idea if I would be a candidate or what fresh hell I would have to go through with my insurance company.

A little over a year ago when I was recovering from Sepsis I wrote a blog post about how I want for nothing in life apart from a lemon tree. I was taking stock of my life in the way that one does after a near-death experience and realized I have everything I need. Yesterday was an awful day. My PI left me unable to lift my head and our power randomly went out for 7 hours in 90-degree heat. So what did my family do? They went out and bought me a lemon tree.

May it bare fruit.

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