IGG - Missing Puzzle Piece

Day + 344.... Sam is near her one year post BMT marker. This is such fabulous news in so many ways. She continues to show strong engraftment. Her chimerism (measure of Kaeden's cells vs. her cells) at her most recent draw were +98%. Some labs will say 100% but for some reason at Lurie's lab 98% is the strongest number they report so we'll take that for now! We will monitor this number as we continue to decrease her cyclosporine dose. Cyclosporine is the drug that actively suppresses Sam's immune system to limit GVHD (graft vs. host disease) symptoms and to help decrease changes of engraftment rejection. After one year, engraftment rejection chances decrease again and are very rare (although with Sam's disease being literally one in a million these numbers don't alwas provide the comfort they should.) Cyclosporine actively suppresses T and B cells though so the longer one has to remain on cyclosporine the longer one is at increased risks for infections and developing certain cancers. This is one of those "balancing acts." We hope to have Sam off of cyclosporine in April or early May. As we decrease her dose, she is showing some mild skin GVHD symptoms with rashes etc. We are working to find a skin care routine to help alleviate these and hope they stay in the "mild" zone so we can successfully wean completely. On Monday at Sam's most recent clinic visit, her WBC count, hemoglobin, and platelets all looked pretty good. The remaining number as we have discussed in previous posts that is not showing adequate recovery is her IGG level. Again I will oversimplify based on my understanding, but there are almost countless different types of WBC that each have different jobs. For a "strong" immune system one does not just need high numbers in one area, one also needs the right "balance" and types of cells to work together. Sam's WBC that fight fungal and bacterial infections are looking pretty good. IGG is needed as the first line defense for viral infections. Since Sam's IGG remains low, this means she has a less than optimal response against viral infections. Over the last year, when her number has dropped below a 400 threshold, we have been transfusing her with IVIG to boost her IGG number and help keep her protected. Now that we are through the cold/flu infectious season, the doctor wants to let Sam's number "go" a little bit and see if this will force her body to step in and produce more actively on its own. We will not let her bottom out to 0 and she said if at her next appointment in April, she is below 100 we will need to give her IVIG no matter what. We also can not start any re-vaccination process until this IGG line shows recovery because her body will not be strong enough to produce an adequate antibody response to make the vaccine effective. Typically post transplant patients start slowly revaccinating with inactive vaccines at one year post transplant and live vaccines at two years post transplant. SO.... the IGG mystery is like the last big "missing piece of the puzzle" right now. We REALLY hope that in the next month or so her T cell and B cell line will "wake up" and start producing IGG. Like so many other parts of this process, we'll wait, we'll hope, and we'll see!

Overall though for a transplant patient, Sam has done remarkably well and we are so incredibly thankful for where we stand right now. It is almost unfathomable at times to look at her and realize how far she has come in one year. Despite these overwhelming feelings of joy and relief we feel daily though, we also long for more for her. We really hope she will have more opportunities with other children her age soon. We hope she can attend some sort of preschool option in a year or so. We hope she can go swimming or that we can take a trip on a plane together somewhere as a family. We  really can't feel comfortable with any of these scenarios though until this missing puzzle piece falls into place. It's such an internal struggle though because as soon as I start to feel frustrated by these limitations, I feel guilty.  I know so many others have had less fortunate outcomes post transplant and would give anything to be sitting where we are right now. So we are going to try to focus on what we can control over the next month which is to acknowledge Kaeden and Sam's strength and love for each other. That bond has gotten us where we are today and that is something truly remarkable to celebrate. GO Sammy strong! Go IGG!!! Go! Go! Go!:)