IgA and ABCs

As much as I've missed writing here, I've been feeling lost about where to begin.

I'll start with the fact that Cora's test results came back over a week ago. She was diagnosed with an immunodeficiency called Selective IgA Deficiency. According to our doctor, about 1 in 700 people have immunoglobulin A (IgA) deficiency, making it the most common immunodeficiency. Her tests weren't able to detect IgA, the antibody produced by B cells to ward off infection at mucousal sites throughout the body. Basically it means that Cora's nose, eyes, ears, throat, GI and urogenital systems are more susceptible to disease than in people with normal levels of IgA. IgA plays an important role in fighting infection because it is the first antibody to "rise to the occasion" and start battling an invader. It reaches peak levels before IgM and IgG (blood components) take over. Cora's IgG and IgM counts appear to be normal.

(Note: If you're reading this entry, you might be interested in reading this post as well.)

Research shows that 25-50% of IgA deficient people develop an autoimmune disorder (I've read 40% as the average in other sources). Lupus, rheumatoid arthritis, and other immune disorders seem to be more common in IgA deficient people over time. They are also more susceptible to pneumonia and HIV, can't receive live viral immunizations because they could actually contract the disease, might not produce appropriate antibodies to "take" a vaccination properly, and are susceptible to anaphylactic death from blood transfusions due to a violent reaction to IgA in donor blood. (If necessary, they are often able to receive "cleaned" blood from which IgA has been removed.) Although many people spend most of their lives asymptomatic, they generally just get a lot more runny noses, colds, sinus infections, ear infections, etc., than the common person. Currently, there is no treatment for this deficiency. Antibiotics have been shown to reduce IgA levels.

Concurrent with Cora's test results, we were pretty affected by the H1N1 flu headlines during its initial outbreak. Some people have commented that they didn't pay any attention to the news because it just seemed like a bunch of hype about the typical flu, but I think that if they had read any of the articles they might have felt differently. Avian flu, which is one of the viruses in this strain, has a 60% mortality rate, and the WHO currently predicts that if the H1N1 virus continues to mutate, we might see two billion people get sick, and a number of them die.

These scary statistics unravelled me for a few days. I worried intensely about Cora on several levels; not just about her health, but about her development and childhood happiness, too. It doesn't seem fair to be relegated to a life of careful hand washing and hand sanitizer, to a stream of colds and flus or missed school days, sled days, or sunny adventures.

After a tense week of trying to hold myself together, I took a cue from my daughter: I had a good, long cry. I felt so much better afterward. After it's all out there, it's easier to turn to a more reasonable perspective, to see how tiny our diagnosis is in comparison to what it could have been.

The good news is that Cora is only 16 months old, and the immune system isn't fully developed until age 2, which means there's a chance her body will still develop IgA. Every night as we put Cora to bed, we imagine her whole body lit up with B cells busily creating IgA. We are hoping that she will have a dramatic turnaround and will produce IgA in beautiful amounts in time to retest her after her 2nd birthday.

(If you'd like to imagine Cora's immune system totally intact, feel free to send your wonderfully positive thoughts her way.)

This news has left me frankly bewildered about a few things. I was not familiar with the components of an immune system until I began researching it and talking with Cora's doctor. One major question is why don't doctors regularly test immune system components at an earlier age? If Cora can't tolerate a live vaccine, then why is every child instructed to have the MMR vaccine at 12 months? Or the live polio virus at an even younger age? Or even the live flu vaccine? Why is it OK to proceed with one set protocol when all children are not created equal? I am thankful that we took a slower approach to vaccinations and weren't planning to vaccinate with MMR until 5 years or even older, and chose not to immunize against polio yet, but it's not a universally-accepted approach. Even though vaccinations in general have certainly gained a lot more attention in the past few years, it's a lot easier to feel fine about the whole thing if your kid gets through their vaccinations without any issues, but if you take a good hard look at the possible complications, the vaccine injury hotline, and the potential that your child might have an immune disorder (or, according to some sources, might develop one after an immunization), it starts to feel very clearly like something that should generally be approached with more caution that it currently is.

Update: The US Dept of Health and Human Services's Vaccine Injury Table lists polio and measles infection due to administration of certain vaccinations to an immune-compromised person. See 5B and 6A and 6B. I asked Cora's former doctor to look into the effects of MMR on an immune-compromised (in this case, IgA deficient) child. She followed up with a top immunologist at Seattle Children's Hospital who said that while there isn't any data to show that an IgA deficient person shouldn't receive the MMR vaccine, he said to 'proceed with great caution'. I said to Cora's doctor, "So don't get it." She responded, "right."

I don't care if it's costly, or even unnecessary to test 699 kids, it's the 700th one that I worry about. (Some sources say IgA deficiency is as common as 1 in every 400, making it even more alarming to consider the number of kids who shouldn't receive a live vaccine.)

I am a huge proponent of public health initiatives that protect vulnerable populations, reduce deadly diseases, and result in a healthier population over time. I understand the herd mentality of vaccinations, and in general I believe in their stated benefits. What I have a hard time with is learning about the fact that my child can't tolerate live vaccines, or viral vaccines very well in general, after the age when she normally would have received such an injection. We got lucky. I feel that there is a lot more study and thought that should go into the administration of vaccinations. Who should receive them? Who shouldn't?

How on earth do parents deal with a diagnosis of leukemia? Brain cancer? AIDS? It must feel as if the earth comes to a complete halt, that you are fighting through fog, that all you want to do is run to the highest mountain and hide your child away in a meadow feeding her nothing but the purest air and water and whole foods while searching for the absolute best medical care in the world. I send those parents my heart.

Cora is doing really well, she hasn't been sick for over two weeks and she runs (never walks) full-tilt through the hallways, pitter-pattering her way laughingly through her days. She is obsessed with a big ABC book that we read everyday, and she points to labels now and says "ABCs" in recognition of the words there. She's back to her animated, lively little self.

The summer is ahead of us. We can't wait. We're looking forward to beach combing, swimming in outdoor saltwater pools, having picnics, going hiking, running through the sprinkler, and exploring other classic summer joys.