It has been 14 days since Paige was admitted to the hospital. Since our last post on transplant day (last Wednesday) there hasn’t been much happening. They call this the ‘honeymoon phase’ since the chemo drugs have all been administered and her spirits pretty high. Paige has started to experience some of the side effects of the chemotherapy. She has had some vomiting, but her toughest days will start any day now. Her tongue has started to be sore, most likely from mucositis beginning to set in. She will begin to develop painful sores on her throat and mouth, making it difficult to eat. She is currently getting the majority of her nutrition through her gtube, thankful another BMT family suggested that to us. Her team will continue to administer pain meds to help alleviate some of her discomfort. The mucositis will also begin to cause much and frequent nausea and vomiting. Please continue praying that this next phase is minimal for her. Along with mucositis her hair will begin to fall out any day. Child Life services at the hospital have been fantastic in helping walk Paige through the changes she is going to experience and answer any of her questions. And yet, despite all Paige has experienced in here, she still continues to find joy throughout her day. I hope I’m more like her when I grow up! Her team continues to emphasize that this week—to week and a half are going to be the toughest on her. We plan on taking one hard day at a time, keep reminding ourselves this is temporary and continue to find our strength through God. God is able to do hard things!

 

We have some people curious as to why Paige’s BMT requires her to be so isolated on the floor for 6 weeks compared to others they know who’ve had a BMT. In case you were curious as well, our understanding is that all BMT’s depending on diagnosis require different conditioning. For children/adults with leukemia as example, chemo is used to target and kill only the cancer cells and donor cells are used for transplant. Neuroblastoma for example also targets the cancer cells, but the patient uses their own cells for transplant.

 

With LRBA deficiency and many other autoimmune diseases, a BMT requires donor cells and chemo to target ALL immune cells. As her doctor has explained it to us, think of her immune system as a factory, the chemo she is getting is poisoning and slowly killing every factory worker inside, not just the poor performers.

 

Each day we receive counts of important key markers they monitor; her hemoglobin‘s, platelets, and ANC (neutrophils). The ANC tells us how many of Paige’s cells are still waiting for the poison to take full effect on them and evict them. As of this morning her ANC was at 190 (she started at 13,870). Essentially once it hits 0 all workers/old cells have been killed. It is possible for it to remain at 0 for several days as we wait for Hannah’s donor cells to begin to take over and engraft into Paige’s new immune system. Once her ANC begin to increase, she will slowly start feeling better. At one point she will need to receive both blood and platelets transfusions, all which they anticipate as part of this process. We are going to continue trusting this process and trusting God for this opportunity to heal our girl.

 

It’s been a long 2 weeks with many more weeks and months to go. We are so thankful for the supportive families we have; they have prayed with us and over us, they have stepped in to help care for our other two girls, mikes parents who in his absence have kept the business running, my mom who has graciously spent a week quarantining in order to stay in Philly the last 2 weeks, my sisters who have tirelessly organized COTA fundraisers to help with a lifetime of transplant-related expenses, and for the two greatest friends (who are like family) who drop anything to be by our side—time and time again. No way we’d be able to face any of our life challenges without any of you. We love you all!