It’s a little hard to grasp that we are finally here and moving forward. Strangely enough I am not nervous. I can’t say that I am excited but it’s nice to be at the point where we are changing her destiny, for better or worse. Hopefully we can eradicate her Epidermolysis Bullosa. At the very least, I hope we can let her have a less severe form of the disease. Most importantly, at 4am on Wednesday, 5 hours from now, there is no turning back. Rafi will get her first dose of busulfan in the early morning hours of day -9. Busulfan is a chemotherapy drug that kills a cell’s ability to divide. There are a host of potential side effects (vomiting, hair loss, mouth sores, etc) and we hope that Rafi will whether the four days of the drug well. It is our understanding that the side effects take a few days to manifest themselves, but we will see.

Rafi is having her first blood transfusion as I type. Her hemoglobin dropped to 7.7, and they give a blood transfusion when you dip below 8. This is the first of many transfusions in the coming months. We are not sure why she has such a low count but this was going to happen anyway from the conditioning regimen (chemotherapy and immunosuppressants). She’s also receiving some other drugs at this point. In preparation for the busulfan, they gave Rafi Keppra, an anticonvulsant. She also got her first dose of Caspofungin, an antifungal drug. From what I understand there are relatively few side effects from this drug, so that’s good, and a welcome change.

Thank you to everyone who wrote us emails, text messages and left comments on this site. Jackie and I really appreciate the support, the kind words, the thoughts and the prayers. We know this is going to be a long and possibly painful journey, for us and particularly for Rafi, and we really feel lucky to have so many people on our side.

One of the things I want to happen during this journey is to educate people about this disease and how it really hurts everyone involved. It’s my hope that each of you will pass this site along to your friends and family, and ask them to do the same, so that we can raise some awareness about this horrible disease.