Today, Adahlia received her 18th blood transfusion.

As far as time goes, the day was one of our worst, but as far as events go, it was perhaps one of our best. The time delay was due to a problem at the blood bank. It took them 5 hrs to deliver the blood after it was ordered, and since transfusions take about 4 hrs, we didn’t leave the hospital until 7:30 pm. Apparently, the first batch of blood had something wrong with it — it started clotting, or reacted with her blood sample, and they tried to fix it (?) but couldn’t. (The nurse wasn’t sure what had actually happened so her explanation was unclear.) Bottom line was that they had to start over after about 3 hours with a new batch of blood.

Contrary to what you might expect, what I learned from the experience has actually made me more confident in the transfusion process. Not only does the blood bank screen to match blood type, check for antibodies, viruses, and diseases, and irradiate the blood (to kill undiscovered pathogens and donor white cells, which could cause problems in her blood stream), they also actually mix a little of the donor blood with a sample of her blood to see what happens. If there is no reaction, excellent. If there is a reaction – antibody, clotting or otherwise – the transfusion is halted and they find a different donor.

This whole time, I thought that it was all a matter of checking each blood sample separately. You know, matching type O to O and doing separate screens for antibodies and viruses. I didn’t know they took the extra step of actually combining samples of the blood to check for compatability. Apparently, they do this – as well as the irradiation of donor blood – on a routine basis for all kiddos. (Adults don’t routinely get such deluxe treatment.)

Today’s IV was placed with just one poke, and Adahlia screamed and sobbed and struggled, but the tech did an excellent job and I was grateful for his steady hand and keen eye. Over the many hours that followed, Adahlia did remarkably, incredibly well. We read books, looked at plants in the attached outdoor garden, did laps through the halls, lounged around on her bed with her, and played with various toys. Not once did we let her crawl – we couldn’t, because of the IV in her hand and the armboard holding her wrist in line with her forearm. (If and when she bent her wrist it would occlude the IV.) Since shes not walking yet, no crawling basically meant she spent the whole day being held all day in a bed or in our arms. For her to be okay with that was no small miracle… and she really was okay with it. When it finally came time to remove the IV, she did not whimper or so much as give a peep of protest. She just sat on my lap and let me hold her arm out, watching the nurse peel back the layers of tape and remove the tube. She was ready to be done I suppose – but it was remarkable, even to the nurse, and quite a relief.

Perhaps my favorite moment was when Joe was carrying Adahlia while pushing her IV stand, and I was chasing them, growling and reaching like a tiger, and she was filling the whole hematology/oncology ward with laughter and delighted shrieks. Or maybe it was after reading her beautifully illustrated Animal ABC book together, when she kept cuddling up and collapsing into me, kissing me and touching her forehead to mine, rubbing her head against mine, like an affectionate house cat. It’s hard to say.

As expected, the doctors talked to us about steroids again, but I told them that we are simply not ready yet. First, because she just started her vaccines, and I don’t want to do them simultaneously. Second, because we are only willing to put her through the ordeal of steroids and all their potential physical-mental-emotional-cognitive side effects if it is her best chance at health and if the timing is right.

Today marked the 5th week since her last transfusion, with a Hb of 7.3 at the time of transfusion. It seems she is trending towards 5 weeks between transfusions now, instead of 4.

DBA is not a matter of simple genetics, if such a thing exists. You can have the gene(s) associated with DBA and express no symptoms. You can have full-blown DBA and have no genetic mutation.

That means there’s something else – or a lack of something else – triggering it. Like a cofactor. Or a hormone. Or a pathogen or toxin. Maybe its a combination, a perfect storm sort of situation. There are many reasons why a complex system, like bone marrow, might not be getting the proper signal to make adequate RBCs. Despite all our incredible medical advances, no one actually understands the complexities of genetic expression and intercellular communication.

What is known? Steroids help kids make an adequate number of their own RBCs so that they no longer need regular blood transfusions in 80% of cases. (Although no one knows why, because technically, if DBA is a genetic disease, steroids shouldn’t be able to make a difference.) Steroids are like hormones and hormones are like steroids. The preponderance of kids who experience a spontaneous remission from DBA are boys going through puberty. There are many plant, animal, and mineral herbal compounds that can act like steroids or have steroidal properties, and do not carry the mental-emotional, cognitive, and growth impairment risks of pharmaceutical steroids. Yet, an herbal approach is not without risk. The wrong herbal supplements (or the wrong dose) can actually worsen DBA (shortening the time between transfusions).

To my knowledge, we are the first to try to use Chinese herbal medicine for a child with DBA in an educated, controlled, and scientific manner based on traditional, classical chinese theory, while also testing the herbs every 4-6 weeks to make sure they are the right match to Adahlia’s current condition before we administer them (much like testing the donor blood to the recipient blood before transfusing).

I dont know if the Chinese herbs have the capability of healing a genetic disorder. I do know that Adahlia is an incredibly and increasingly bright, active, happy, and healthy child even when she is anemic, and that she is one of very few kids with transfusion-dependent DBA who can go 5 weeks between transfusions.

I think the Chinese herbs might be at play, here. I think we are not yet ready to gamble with steroids yet. I think we are on to something.