Infusion!

Last week was a very busy one. Sebastian had his bone marrow extracted from his hips. He then had chemotherapy taken through an IV (called Busulfan). He reacted fine to the chemo drug but was a little out of it and easily agitated due to some anti-seizure meds (which is common). The dose was reduced a bit and all was well. The reason he is given chemo is so his immune system, now propped up by Adagen, will be compromised so the gene therapy administration does not have to compete against any naturally occurring immune system reactions to fight the vector (the virus that delivers the corrected gene). While this was going on, the talented scientists were taking his bone marrow, preparing the cells and incorporating the vector for injection. This was all orchestrated like clockwork. The staff of doctors, nurses, etc. have all been wonderful. They are an inspiration to me—there are many ill children around this part of the hospital and they are doing amazing work! I hope Sebastian will go into the medical field, it is the most noble of professions in my opinion.

On Friday at 4:30PM Sebastian received back the corrected gene. Dr. Aiuti said the cell counts, etc. were of good quantity and was optimistic. It was a day of excitement, relief and stress for us. We are excited and relieved that he has had his gene therapy treatment administered. Next, as I think I mentioned in an earlier post, is a thirty day waiting period in order to discern if the treatment is working. This is where the stress comes in. The mind tends to play scenario games in quiet hours thinking of all the variables that could go both right and wrong. Only time will tell.

The fireman drill: When Sebastian has to go pee (he has a potty seat in the box) we have to put on full surgical regalia (hair net, mask, robe and gloves) in order to lend a hand. This is not ideal by any means, but necessary. I joke to everyone it is like being called to go put out a fire the way a fireman must adorn all their protective gear and get on the truck—and if you are not quick there is going to be a big mess to clean. Our bodies, when functioning correctly, are truly amazing machines and this multi-step safety procedure reminds me every time I go into his area.

A few notes of thanks: Two of Lynette's co-workers gave Sebastian a few games for his PSP device. One of the games, after trying to appease him with several others, is a hit! It is a Lego meets Star Wars game in which there are miscellaneous tasks and one can run around in different "worlds". This bit of escapism has kept him entertained for hours when coloring, stories, etc. is of no interest. Thank you! Secondly, we met a Swiss family who has undergone the same procedure in Milano. The boy's grandma gave Sebastian a toffee that he took a liking to, she then gave him the rest of the box. Last week as we entered isolation a package came from them. Inside was a lovely note and beautiful drawing from their son, two boxes of toffees, and a guardian angel ornament. Thank you—Danke! Lastly, when Sebastian was undergoing his first bone marrow extraction procedure our Canadian friends gave Sebastian a cool soccer ball of the Milano team and brought us all some pastries. This was wonderful and much appreciated. We look forward to playing with the ball soon. No matter where you go there are great people in this world!

That's about it for now. Thank you all for your help in getting us here, as well as your thoughts and prayers. It is greatly appreciated. When Lynette and I are going through some of the lower points, we think of all the gestures of kindness.