Sunday night post

My head can barely keep everything straight - I've asked so many questions and received so much information...I usually use this blog to digest and record it all. I will save it for another time as it is just too late...

The short story is this: 

1. Belly Button Infection: His redness around his belly button has improved minutely, but we'll take it. That's something. Also, just to be clear, it is for sure an infection, we finally got a doctor to explain they never thought otherwise (for example just simply irritation of the skin). Apparently it is what an infection looks like sans neutrophils (which I will sometimes refer to as "newts" from here on out, which is my completely non-medical, short-hand, terminology). It is your neutrophils that cause puss and the like, which makes an infection look like we'd expect it to. So in his case the deep red color plus the fact that he has basically no neutrophils equals infection. Also, from my own online scouring I've learned skin infections and belly button infections are common in cases of Alloimmune neutropenia.

He will remain on the two antibiotics that are given through iv. until his newts are at a "respectable level" - which is only 400-500  (2,000 is ideal). At that point, if they remain at that level we can switch to oral antibiotics and be given our discharge papers.

2. Neutrophils: Today's ANC (actual neutrophil count) was 80. So the stimulant (known as "G-SCF" for short) is either not boosting them or he is using what he does have too quickly and they are going out of circulation before the count (could mean the small number of newts he has are busy working on the infection while the rest our suffering at the hand of my antibodies in his blood). But it could mean he is just not responding.

Therefore, we are now moving to the next course of action - a treatment called IVIg (intravenous immune globulin). This "is a plasma product formed by taking antibodies from about 20,000 donors and mixing them together." Will it work? Is it without some risk? Between the docs and my research - it is kinda a shot in the dark and there are some risks. There simply are not enough cases to gauge anything - it's essentially uncharted territory (alloimmune neutropenia is rare 0.1–0.2% of live births). Gwen received both G-CSF and IVIg, however we have no way of knowing which one "did the trick" since they did both at the same time in her case. I now know both were only temporary treatments for her (no, I'm not okay with that statement - and yes, that is correct, when I was at CHOP and when I left CHOP I did not know Gwen's neutrophil numbers could drop again...but as I said, this post is the short version of what it is my head...).

I found this little chart to help give a sense of where his numbers need to be:

• greater than 1000. Normal protection against infection
• 500-1000. Some increased risk of infection
• 200-500. Great risk of severe infection
• lower than 200. Risk of overwhelming infection; requires hospital treatment with antibiotics

        (source)
 

That said, he needs to get his newts up and they need to see that they are staying up for several days. Then he will be monitored and when and if they drop again, he will receive a treatment again. And again, once the newts are up then we can talk about going on oral antibiotics.

3. A Test and CHOP Records: They drew blood for an antibody test. I need to ask them more questions about what exactly they are looking for. I know it is to show there are antibodies and help confirm the diagnosis, but from what I read there are different kinds of antibodies and I wonder if they are interested in "type" as well and then why they are. Problem with this test is that it has only a 50% chance of being "correct" - so it may not show antibodies when their actually are. At the same time they are getting Gwen's records from CHOP to go over. My doctor still believes it has to be the same thing Gwen had and the bone marrow biopsy, she explained, is a black and white test - so if CHOP said the test revealed that her bone marrow was fine and it was when the newts left the blood that they were being destroyed, then it is correct. There are other types and causes for neutropenia, but it would be an astronomically low probability that I would have two children with two rare and separate forms of neutropenia. But believe me, I'm terrified they will either tell me Gewn's diagnosis may have been wrong or that well, I did manage to win the wrong lottery, again and that this is something different that what Gwen had.

Again, that is the short version.

Tomorrow morning (Monday) we'll see if his latest G-CSF shot boosted his numbers anymore. If not then the next treatment is up, the IVIg. And then we wait. 

Tomorrow we will also see if any more improvements happened with his infection. 

I am praying.

I am scared. I am so scared. I don't like that the G-CSF really didn't do anything.

Here are some pictures from today's visit with Aunt Sarah and Lil. Aunt Becca needed to give her little boy a quiet day at home, so sadly we didn't have their company. We all had a good time being together. But, now I won't see Lil till Wednesday -it is hard to swallow that - however I know she is being well cared for and that gives me peace of mind.