Planning for a Bone Marrow Transplant

"It's empty!" Those words, the words of our doctor when referring to Nathan's bone marrow, were not at all unexpected. We are five weeks from his ATG treatment and he should be showing some early signs of growth given all of the white cell stimulant (both g-csf and gm-csf) he has been given each day. But thus far nothing has changed in his daily labs, no growth, nothing indicating to us that he is showing even a minor response to the treatments. His bone marrow is just "empty", officially having less than 5% (essentially immeasurable) cellularity, so his lack of progress makes sense given that there is effectively nothing to build from. On the plus side, nothing out of the ordinary was seen on his biopsy, no histiocites, nothing growing that shouldn't be there. And the ATG worked a small miracle on his liver inflammation. And he is still very happy and experiencing minimal complications or discomfort. And these are all really good things. Now it very much feels like the time to plan for the next steps... a bone marrow transplant. I'm going to go into detail regarding some of the preparation that we are looking at in the next couple of days.

Tomorrow Nathan will begin all of the preparation to clear him for transplant. This includes a series of exams to ensure that all of his organs are healthy which includes a CT scan, a GFR test for his kidneys, an echocardiogram, a pulmonary exam, a comprehensive series of blood tests and a dental exam. He will also need another PICC line in his other arm as he will need four access points for all of his conditioning. And of course he will also have a psychological and physical therapy evaluation. And we will switch to a different room that is specially cleaned and prepared for a bone marrow transplant patient. I asked for a room with a sunrise view since Nathan just loves to watch the sun rise; if we are going to be stuck in our room again while waiting for his immune system to grow, we may as well have something beautiful to wake up to!

Camryn is all but cleared to be his donor, and we are just waiting for a few minor pieces of paperwork to be completed. She has had several physicals, two huge blood draws (22 vials in total), and a psychological evaluation. She is of course anxious and wishes he would get better without going through the transplant, but don't we all? She is also very willing to overcome her fears and anxieties to help her brother get better knowing that this is his best chance at recovering and getting back home again. But this is a big deal for her and we are hoping as much as we can to make it a positive experience for her so she remembers her courage first and foremost.

Once both of them are cleared, then the real work will begin. He will start a four-day round of chemotherapy using cytoxin, fludarabine and R-ATG likely before the end of the week. This is considered reduced intensity conditioning for a bone marrow transplant. However, while most regimens for an identical HLA matched sibling would not include fludarabine, Nathan has enough abnormalities in his illness and has had a huge number of transfusions so it makes sense to include this additional medication to ensure that engraftment occurs. After the chemotherapy is complete, we have two rest days and then the transplant occurs. The actual bone marrow harvest from Camryn will essentially be very similar to a bone marrow biopsy except the needle is different and more fluid will be extracted; Camryn will be sedated and then a weight-appropriate amount of her bone marrow aspirate will be removed with the whole process taking less than an hour. After the procedure, her aspirate will be processed and prepared for the transplant to occur most likely the same day. I am told that transplant day will be pretty uneventful for him as he is simply getting a bag of his sister's bone marrow aspirate which will look like blood and it will effectively go into his system through his PICC line just like a blood transfusion. Then we wait... and hope that chemotherapy side effects will be minimal as the doctor suspects given his "reduced intensity" regimen. However, bacterial and fungal infections, viruses and graft vs. host disease are huge risks in the initial weeks following transplant and there seems to be a very rigorous protocol to reduce these risks. He will get a lot of medications to control complications during this time. Then hopefully within a few weeks, we will see his blood counts increase and we can begin planning for the future. But, during this time, he will be very severely immunocompromised and we have made decision to not allow any visitors until he has at least a few white blood cells and neutrophils to protect him. This could take days or weeks to even begin happening... there's no way to know right now exactly how it will go. 

I have felt for a long time that this is where we were headed but I really appreciate the doctors doing their due diligence because it really feels like everything is coming together at just the right time. Getting genetic testing back that rule out major genetic triggers like HLH or a GATA2 defect, our primary and favorite BMT doctor being on staff the majority of the month, Camryn feeling truly ready to do this major thing for her brother, his liver healing, and even small things like getting a visit from our "Make a Wish" wish granters all within the past week... it seems like everything is falling into place to ensure his successful recovery and that is the most that any of us can hope for. 

At the same time, reading through the paperwork outlining his regimen is very sobering. Effectively it states that his disease will result in his death but that the treatment could also result in his death... thats a huge pill for a mom to swallow. But it's not as though we have a real choice in the matter either. And his doctor, if I haven't said so already, is incredible. It is rare to have a doctor who will sit and really talk with you, collaborate, think outside the box, consider protocol but really look at the child in front of him first and foremost. I feel very fortunate that we have one of those doctors and I think I may be his biggest fan right now. I have to continue to trust that Nathan is in great hands and surrounded with the right team of people to help him get well again because he truly is, and the rest is completely out of our hands. We just have to hope that all will be well. 

So I'm going back to a poem that I have read many times through this journey...

"Hope" is the thing with feathers— 
That perches in the soul— 
And sings the tune without the words— 
And never stops—at all— 

And sweetest—in the Gale—is heard— 
And sore must be the storm— 
That could abash the little Bird 
That kept so many warm— 

I've heard it in the chillest land— 
And on the strangest Sea— 
Yet, never, in Extremity, 
It asked a crumb—of Me.

~Emily Dickinson