Angry Zebra

Yesterday started out great. Then it all went to shit. Here's how...I'll start from the beginning, which was almost week ago.

As I've written about here, the dude began infusion therapy for his immune deficiency this month. All was going well for the first two weeks, except for some hyperactivity. No big deal; I got this. Then came dose #3. After his infusion  last Monday, Jack was a little drowsy, took a nice long nap, and woke up...high. He was yelling like a crazy person and running in circles laughing. It was kind of funny, but definitely worse than the week before. He seemed to be back to normal by the next morning. Thursday morning, he intentionally broke 3 toys within less than two hours. My child has purposefully broken a toy maybe once (?) in his entire life. Things just got worse from there--he wasn't just hyper, he was aggressive. He yelled at me and the hubs, he frequently swung at us; he even tried to push me down when I wouldn't let him have his way. His behavior was back to normal Friday and Saturday morning.

Practicing infusions on his therapy buddies, Iggy & ZeeZee

Saturday afternoon, we left the dude with my mom for an adults-only date night. His behavior while we were gone, as well as all day Sunday and some of Monday, was the stuff toddler-mom nightmares are made of. More screaming, aggression, throwing punches, and just being mean. I called his immunologist--she'd never heard of such a side effect. I talked to several experienced moms in online support groups who said their kids had reacted similarly to this particular drug, although no such effect is listed in the drug insert (or online). We needed a plan.

I decided to take him to the new indoor play area at the mall and meet up with a mom-friend, thereby accomplishing two goals: keeping the dude active and getting a little adult conversation. The morning could not have gone any better, I tell ya! He played so well, alone and with other kids. He shared, he used his manners--I was such a proud momma! We bought new shoes (such a good sale!!), had a mother-son lunch, and even got cookies as a special treat. 

Enjoying a fruit cup while I shopped...

Then we went home, and all hell broke loose. It started with a simple request to check his temperature, and well...

My sweet baby had a complete and utter meltdown. He yelled, he clenched his fists, he threw anything he could lift over his head across the room. He screamed at the top of his lungs until his face was purple. And then this momma called the doctor and had a little "Come to Jesus" meeting with the nurse, and then a very similar meeting with the pharmacist. We cancelled the next shipment and decided to give him a few weeks off before deciding if we will resume treatment with an alternative drug. And then we both sat on the floor and cried. No more Hizentra for this boy.

This post was truly difficult to write. It was hard to relive the meltdowns that broke my mommy heart. Watching your child lose control of his behavior to such an extent is awful; the pitiful, confused look in his eyes tore me to pieces. He didn't understand what was happening, and I felt useless because I had no answers; no solutions. It also gave me a new understanding, a new empathy, for parents and caregivers of children who struggle with these types of meltdowns daily. I chose to post our story because this information was not available to us. Had we known this was a potential side effect of that drug, we likely would have chosen a different one.

Zebras

This is a long story, so I'll just jump right in. If you've already heard it, feel free to skip to the next post. :)

In July 2013, Jack starting running a fever with no other symptoms. After running temp everyday for a week, we went to our pediatrician. She assumed it was something viral, and sent us home. Two weeks later, we were back again; still running fever. She gave him a full check-up and still couldn't find a source. After six weeks of still running fever, we starting running labs. SO MUCH LABWORK! I swear if it's possible to detect via blood test, we tested for it. Still nothing. After nine weeks of daily fever, it finally broke and was then recurring about 5 out of 7 days. Finally, on October 30th, we saw a pediatric rheumatologist at Children's Hospital in New Orleans. Jack's pediatrician was trying to rule this out because I have an autoimmune disease that manifests as moderate to severe joint pain, which doesn't show up neatly on labs.

The rheumatologist was very thorough and decided to run more labs. His initial opinion was not rheumatological, but instead related to his immune system. These new tests came back two weeks later; the doc was right. We were referred to an immunologist closer to home. In December, Jack was diagnosed with Specific Antibody Deficiency (of IgG). In my own non-medically-educated words...the immune system has several different types of immunoglobulin, which function as antibodies to fight different types of infections. IgG is the type of immunoglobulin needed to fight off viral and bacterial infections. A healthy immune system has 14 different IgG antibodies; Jack's body was only producing 3, and the amount of those three in his blood was barely traceable. His only physical symptoms were persistent, recurrent fever and little to no appetite, with a history of being resistant to antibiotics If you have no experience in Primary Immune Deficiencies, which we did not, you've probably never heard this expression. In medical school, doctors are typically taught "If you hear hooves, think horses," meaning, look for an obvious source first. Immunologists practice by the idea of thinking zebras instead of horses. Kind of cute, huh? Anyway, patients with P.I.D. call themselves zebras.

The first step of treatment for Jack was a booster shot call Pneumovax, which boots production of 23 different antibodies. He initially had a great response to the shot, but after 6 weeks was going back down. We decided to start immunoglobulin replacement therapy. We opted to have the medicine administered via subcutaneous infusions, which we can do on our own at home, versus IV therapy in a infusion suite at a hospital. Jack started treatment on June 9th, and gets an infusion once a week. We'll be rechecking labwork in another month to see if it's working. If it isn't, we'll be back at square one and will be referred to another specialist. If it is working, we'll continue treatment for up to 6 months, then take him off to "see what happens." From what we've been told, in some cases a few rounds of replacement therapy seems to clear up the condition, and no further treatments are needed. More commonly, the condition is life-long, but thankfully not life-threatening.

For more information about Primary Immune Deficiencies, please visit http://primaryimmune.org/about-primary-immunodeficiencies/.