And The Winner Is ...

Source:  And The Winner Is ...    Tag:  neutrophil production
Parker Joseph DesLauriers.

We received confirmation today that Parker was the Grand Prize Winner for the April Photo Contest on BabiesOnline. We all knew that he was the cutest, but with everyone's support everyone now knows it. Thank-you so much to everyone for voting for Parker each day during the final round.

A day has passed since Parker had his first injection on PEG-ADA. It went very well. There were no major side affects that we noticed. He was in some pain afterwards as it was a large injecton in his right inner thigh. I can only imagine how much that would have hurt. But my boy is tough. Very tough.

For those of you that are unsure of what PEG-ADA is and why he is taking it I will explain. First, to be considered in the Italian study Parker must be on PEG-ADA for six months. The idea behind getting PEG-ADA injections is that Parker's gene that regulates the amount of ADA his body produces does not work correctly. Well, it does not work at all. So he does not produce any adenosine deaminase (ADA). The purpose of ADA is to remove a specific compound in his body. Deoxyadenosine is a natural substance that is made during the break down and synthesis of DNA in the body; however it is toxic. ADA normally binds to it and converts it to deoxyinosine a non-toxic compound. Obviously, if the body does not produce ADA then it cannot convert the toxic deoxyadenosine to the non-toxic deoxyinosine. As a result, these toxins build up in the body. The toxins kill off his lymphocytes, and we hope have also hindered his neutrophil production.

The idea behind ADA enzyme injection therapy is to give the body the ADA it needs to convert the toxic compound to the non-toxic compound. PEG is just a compound that the ADA is attached to so that it lasts longer in the body than it normally would; as it usually removes itself quite quickly. As a result, the bodies T-cells are able to survive, and in some cases, so do the B-cells. We are hoping to see these effects in the next four to six weeks. If we do we will be able to bring him home before we go to Italy.

Some of you who have not read my previous articles may be wondering why we not just do that forever. Unfortunately, for some unexplained reason, the T-cells production and function decreases dramatically after a few years. As a result, we do not feel that long term PEG-ADA injections is a solution to Severe Combined Immunodeficiency caused by an ADA deficiency.