Day 0: Today is transplant day!! Miia, Kiira’s sister went in to the OR around 8:30 AM. I was able to be with her while they put her to sleep and though she was crying in that moment, she has been incredibly brave and goes along with whatever we ask of her (except drinking her Tylenol this morning). The procedure took about an hour. She came out of sedation well, but because they had to intubate her, her voice is hoarse and they knocked out her loose front tooth. She was up walking around, eating and drinking within an hour. Her recovery is quick and fun with a movie, Popsicles and a new toy. She said she had no pain either and didn’t need a blood transfusion later today, so she is now home. We are beyond proud of Miia and so happy she was able to sacrifice herself in this way for Kiira.
The other positive is that her blood was so rich with stem cells–about 4x the amount they hope for. The doctors were pleasantly surprised and we hope that helps Kiira even more.
Kiira received those cells at 2:30 pm today. Miia was here to watch and got to unclamp the line to start the flow. It was…beautiful. I don’t know how to describe it, but we are so happy to be at this point. Kiira slept through it all, but has been so happy and energetic tonight. Last time, she started getting sick at this point, so it’s really fun to see both girls having fun, celebrating such a monumental day.
The point of chemo and radiation is to lower Kiira’s own immune system, so now her white blood cell (wbc) counts are very low. She is extremely susceptible to infection until Miia’s cells start to grow. Kiira still has 2 doses of chemo over this weekend too.
As Miia left tonight, she asked if Kiira’s owies will be gone tomorrow. Such a sweet, innocent question, and one that comes up often as we talk about doing a BMT for EB. A BMT is not a cure and is a very long term, complicated process. Over the upcoming YEARS it should help Kiira to not get injured as easily and help her heal faster, but it is not designed to completely take away EB–only gene editing can do that. However, we are hopeful that as Miia’s cells grow in Kiira, they will go to injured areas of skin and heal them with the corrected gene, which would then give her Collagen VII in those areas and make her skin stronger. The outcome would be to slow the progression of EB while we wait for a cure. Damage to the skin is irreversible and scars are deep, so minimizing the damage now will help Kiira in the long-run.
So in short, we need Miia’s cells to grow steadily and become stronger than Kiira’s.
Thank you so much for all your prayers. We know today went so well as a result.
