Numbers In and Up!

We made our regular trip to Sick Kid's this morning so that Parker may receive his PEG-ADA injection. We normally go a little earlier so that Parker may give some blood so that a CBC (complete blood count) can be done on him, but that was not going to be the case today. We were told last week that he was not going to have to give blood every week, and that every other week would suffice. This was because he seemed to be doing so well, and the weekly blood work always seemed to be yielding the same results.

Last week, Parker gave enough blood so that another T and B-cell marker could test could be done using flow cytometry. The last time that this test was performed was on June 6th and informed us that his thymus gland was still not functioning appropriately yet. The marker test gives us an absolute count of the different lymphocytes Parker has. Recall that there are three different types of T-cells that are matured in the thymus gland. For the first time ever, we finally had some evidence that Parker has significant numbers of these types of cells. On June 6th Parker had 121 cells /uL of CD3+/CD4+ T-cells, today the test informed us that he now has 592 cells/uL. On June 6th, the test revealed that Parker had 13 cells/uL of CD3+/CD8+ T-cells
, today the test revealed he had 232 cells/uL. On average, his mature T-cells have increased from 168 cells/uL to 903 cells/uL.

To say that my wife and I were happy about the results is definately an understatement. Now, these numbers are still below what is considered normal for a child his age without an ADA deficiency but they are very good for a child with ADA deficiency.

Parker is currently receiving ADA enzyme replacement therapy to replace the missing enzyme that his body is incapable of producing. As a result, the enzyme has mopped up the toxins in his body that is causing his lymphocytes to be killed off. I had mentioned in numerous articles dating back to the time we had to decide on a treatment for Parker in April that long term enzyme replacement therapy is not a successful long term treatment for ADA Deficient Severe Combined Immunodeficiency and for that reason we disregarded it as his treatment. That said, he must remain on it for six months to take part in the gene therapy trial in Milan, Italy. The fact that he is capable of producing the different forms of T-cells lets me know that his thymus is capable of working. This is important because his thymus will need to do the same thing when it comes to gene therapy; it will have to mature T-cells.

Parker currently has an absolute count of 468 cells /uL of his natural killer(CD56+) cells and 1117 cells/uL for his B-cells (CD20+). His high number of B-cells means that there will not be any more IgG troubles for a long while.