One day when we were back home in Arizona, we were being interviewed by a reporter who asked to describe the gene therapy treatment we were hoping to get for Keira. Dave said it best when he began to answer with “It’s something out of a science fiction novel…”
The process by which they do all of this is remarkable. But simply put, they extract stem cells, put them into vectors, take out the bad genes, replace them with good ones and then reinsert the stem cells into the body via a virus (without the bad parts of said virus) so that it quickly spreads throughout the body. 😳
Step 1 is leukapheresis, the removal of stem cells. In order to do that, Keira had to have a central line surgically placed into her femoral artery. That is where they would hook up the machine that would process her blood and remove stem cells.
It was a quick surgery and as with all IVs she gets, once they are placed she doesn’t even care they are there and just wants to continue playing like normal (fun for Mom who gets to watch her every move and make sure nothing gets tugged on or twisted).
Straight from surgery she went to the leukapheresis room to get hooked up to the machine. Each session would require her laying (optimal position for extraction) as still as possible for 4 hours. No easy feat for an almost 8 month old who is on the move.
The first session was a little easier as Keira was coming out of sedation from the surgery and was tired. The second day I had to pull out some distractions. Thankfully, we did not need to do a third session as they got the maximum amount of stem cells needed for the procedure.
They generally hope to get around 30 million stem cells per kilo (which I hadn’t known prior). So when they told me on the first day they got 10 million stem cells per kilo I thought ‘Wow, great! We’re done in one session.’ Not so much. Thankfully they were able to double her dose of the medication used to stimulate the stem cells’ movement throughout the body so on the second day they were able to extract 32 million stem cells per kilo! Of the 42 million total, 5 million will be used as back up and the rest will be used in the gene therapy, giving her the maximum dose for the procedure.
It was really crazy to see all of this happening in one machine and Keira laying there happy as a clam like nothing was going on.
Around us were three other beds with people also receiving stem cell removal. They were all older men and women who got a kick out of Keira smiling at them, and blabbing on and on. “Bella!” or “Bellisima” they would all say. 🥰 Hopefully she brightened their days a little as they brightened ours!
Next step for Keira: surgery on Monday to have a permanent central line placed for the upcoming gene therapy.