titers and vials and more

Yesterday, my sweet little man had his quarterly follow-up with genetics. Since I don't feel like getting all fired up again, here's the annotated version:

After almost 45 minutes in waiting room with screaming toddler, our brazillant nurse crossed my path (not her best move) and I wanted to drop-kick her for her craptastic attitude... fast-forward another 45 minutes, doctor finally joins my terroristic toddler... he immediately apologizes for the "unacceptable wait time" (his words, my thoughts --- someone double booked his 9am slot - mind you, our appointment was scheduled for 3 months... sigh)... I express my dissatisfaction with his nurse... he acknowledges that "changing the system is hard, but he's diligently working on it."... we move on to the real reason for our visit - sweet Pierce. I tell him about our ST's concern with his decrease in words - he says we are probably seeing some regression but the positive thing is that he will progress back up. Mitochondrial disease has it's ups and downs - like a roller-coaster. I told him that we saw allergy/immunology and that Pierce's IgG levels came back low. We discussed Pierce's history of pneumonia, (several cases of) bronchiolitis, wheezing, neb'ing, etc... he thinks we need to add Pulmonology to our (ever-growing) list of specialists. He also said that Pierce is anemic - his levels aren't critically low. We are adding Poly-Vi-Sol to his daily regimen of medications/supplements. If that doesn't help, he may need elemental iron. We waited forever and a day for his labwork (due to miscommunication... again)... his left arm pooped out after {barely} two vials (I knew it would as soon as they stuck him because of the way his blood was flowing...)... his right arm was such a champ... 8 vials of blood later, one teal bandaid, one red bandaid one orange and one grape lollipop, two glittery Elmo stickers and we were FINALLY out of there... a whole 3 hours later.

Stats --- 23lbs 4oz (16th% weight) and 78.9cm (20th% height)

I received a phone call today from his allergist/immunologist team. they received his titers back... not so great news.

Pierce received his Prevnar (PCV7 - now it's PCV13) vaccine at 2 months, 4 months and 6 months of age; at 15 months, a child receives a Prevnar booster. A child's immune system is typically where it should be against the 14 most invasive strains after the first 3 Prevnar vaccines... Pierce's is not. 

They want to see at least 7 of these serotypes greater than 1.29 --- as you can see with Pierce, only 2 of his are... and just barely. Because of his low titers and low IgG levels, they are going to give Pierce a vaccine called Pneumovax 23. They said his body will (or should) mount a different response to this vaccine. Approximately 6 weeks after, his titers will be re-checked. If his levels are still low, we will discuss our next step (possibly close monitoring of his infection levels or straight on to IVIG). [side-note: I am slightly nervous about this vaccine seeing as how it has not been established in the under 2 population... Pneumovax23]

It's so hard seeing this sweet, smiley-faced boy that looks a-okay on the outside but really isn't on the inside... that's all for now... I feel depleted... but in 30 or so minutes, that sweet smiley-faced, blonde curly haired boy will be calling my name... and showering me with wet open-mouthed kisses... and that my friends replenishes my spirit.

{just breathe}

When it rains, it pours {at times}...

I am feeling very overwhelmed...

I know it's said to "count your blessings, not your problems" - but on some days, just thinking about that idea is tough...

As far as George, myself and the boys go, we're fine. But outside of that, my family (specifically my immediate family) could use all of the prayers, thoughts, wishes you have to offer. My heart is broken and heavy. For now, please respect our wishes that this stays a private matter.

Now onto other stuff...

Yesterday, I spoke with one of Pierce's doctors (new to his team of doctors) - I was concerned that some cold-like symptoms were a result of his allergy skin testing (performed on Wednesday). She said that they were not. She was also happy to hear from me because she was going to call me. They did some bloodwork on Pierce on Wednesday - to look at his immunoglobulin levels (basically his immune function) - IgG, IgA, IgM, IgE, IgD (if you've read my blog for any length of time, then this will sound slightly familiar as we've {kinda, sorta} traveled this path with Brice). For Pierce, his:

• IgE levels were low - this is a good thing. IgE is related to allergens. If your body meets a particular offender (that you're allergic to), it will build IgE antibodies in response to it. Even with this finding, Pierce is still labeled as "Milk Allergy."
• IgA levels were normal
• IgM levels were normal
• IgG levels were low (not a good thing) - about 20 points below normal. "IgG antibodies are found in all body fluids. They are the smallest but most common antibody (75% to 80%) of all the antibodies in the body. IgG antibodies are very important in fighting bacterial and viral infections. IgG antibodies are the only type of antibody that can cross the placenta in a pregnant woman to help protect her baby (fetus)." source

We may be dealing with an immunodeficiency, specifically an IgG Deficiency (with the subclass undefined at this moment). We're in a wait-and-see boat at the moment... we're waiting to see what his blood titers look like. She anticipates having those results around the week of June 6th. Depending on those results, we may begin treatment soon. If those results buy us some time and he were to get pneumonia, treatment would begin immediately. If those results buy us some time and he doesn't get pneumonia, we will be watching him for any signs of infection over the next 2 months (ear infection, sinus, etc).

Treatment (IVIG) for this is with blood product (purified plasma) that has Ig antibodies in it. We are looking at once a month treatments for 1-2 years. From what I've gathered, it is done through IV infusion.

I spoke to his pediatrician yesterday about the results and she said the IVIG would probably be beneficial to him.

So we sit and wait...

But in the meantime, Brice will be seeing the same doctor. While reviewing Pierce's medical history and going over his family history, I mentioned Brice's bloodwork (if you didn't read the above link, Brice had similar testing done in August 2009 - his IgM levels were low, IgG and IgA levels were borderline low). She asked if his levels were ever re-tested and encouraged having his levels redrawn. Further into our conversation, I mentioned that Brice is presently being treated for pan-sinusitis (infection of all sinus cavities - it was incidentally noted during his May 6 brain MRI). She looked at me and said "he really needs to be seen again." The next available appointment was June 28.

Between now and June 28, Pierce has an eye specialist appointment, a genetics appointment (w/bloodwork), his 18 month well-visit (w/immunizations), an orthopedics appointment (w/x-rays) and Brice has a GI appointment.