I am very sad to say that we received some troubling news about Reese last night.
The team came to the door a second time, late in the day, and told me they wanted to talk to me about her marrow. Remember, she had a bone marrow aspiration yesterday. My heart stopped.
As they were talking, I was only partially with them, which is often what happens in these troubling meetings. I am outside of myself, I am normal Jen who has a normal life, watching scared and confused Jen, trying to make sense of what is going on. The doctors told me Reese now has TA-TMA (Transplant-associated thrombotic microangiopathy.) This is the trifecta- there are three rare, big things things that can go wrong in transplant. VOD, severe GVHD, and TMA. You may remember Reese beat VOD in May, we narrowly escaped TMA a couple months ago, and now here we are. Facing the third beast while still trying trying to conquer the GVHD.
TMA is a rare but serious medical disease. It is a pattern of damage that occurs in the smallest blood vessels inside many of the body’s vital organs – most commonly the kidney and brain. TA-TMA occurs because of treatment-related endothelial damage after transplant. The risk factors include high-dose chemotherapy, radiation therapy, unrelated donor, HLA mismatch, exposure to CNIs with or without concomitant exposure to sirolimus, GVHD, and infections.
Of these risk factors, Reese has high-dose chemo, unrelated donor, exposure to CNIs, GVHD, and infections (CMV reactivation.) As I’ve mentioned, JMML is a beast and requires the most aggressive conditioning before transplant. This puts JMML kiddos at extra risk for transplant associated problems. But why Reese got all three, we do not understand. I am scared and frustrated.
The doctors are immediately weaning her from Tacrolimus. This is known to be a cause of TA-TMA. Tac is used to prevent graft failure and keep GVHD in check. I know what you are thinking, Reese has GVHD. Tac is being replaced with Jakafi, a new-ish drug that is promising. The catch, Jakafi needs to be absorbed by the gut. It does not come in IV form, so Reese’s healing gut has a big job to do. It needs to absorb the Jakafi to get her off Tac. Come on Reese!! This matters.
As you know, Reese has been in an incredible amount of pain the past three months. Her narcotic intake to keep her (not actually) comfortable is high. They sort of work… she seems in pain with or without them, leading me to not fully trust they are doing the right job in this situation. Today we met with the pain team to brainstorm ways to help treat Reese’s pain. Everyone knows that Reese is very attuned to her care. She asks pointed and intelligent questions and truly understands more than a 4-year-old should. (This little girl even likes to squeeze the blood out of her finger for her glucose check- she finds it fascinating.) For this reason, I was looking for a way to connect her to her pain management. We have come up with two non-narcotic ways to help Reese. One is her TENS, or “Buzzette” as she named her (pictured.) TENS is used to help suppress pain signals in the brain with electric pulses. Reese controls the little computer to help control her pain. Next, she chose “orange” as the essential oil that “takes away pain”. I just bought her a diffuser necklace online, to smell when pain hits. I’ll let you know how it goes and if you should run out and buy orange essential oils for your own needs right away.
Thankfully, our battle with GVHD is on the right path again. Yesterday, she weaned down to a 1.2 (again.) This appears to be going well and she is enjoying a lot of food off her little “menu” each day. Now it’s time for her gut to show us what it can do!