Reese had an exciting week both inside of clinic and at home with her family. We welcomed the 4th of July with a two day celebration… it was beautiful. It was not lost on me how dramatically different this was than last year. My little girl had already lost the ability to sit up on her own, and the pictures I have from this day are her laying in various parts of the room, helpless. Yesterday she went to the parade followed by the pool, running and swimming with her sisters. Independence Day indeed. She doesn’t remember anything about the 4th of July last year, this is a gift, and a result of the incredible amount of drugs she was on for pain control. The process of healing has been truly a wonder to observe, she is incredible.
Reese went to clinic at Lurie this week and a battery of tests were run to see just how her immune system is functioning. Specifically, her B cells and T cells. Reese’s B cells were wiped out last fall by a very powerful drug called Rituximab. This was to combat a viral reactivation that has been connected to secondary cancers, so it is taken very seriously. She had two rounds of this medicine. We were told it would eliminate her B cells and open her up to a host of terrible viruses (and also, sometimes, B cells never come back.) We did NOT want to do this, but as we learned in the bone marrow transplant world, often the best choice is the only choice, or the least awful. So we did it and then we watched her B cells last fall, winter, and spring. Nothing. It was March since we looked at them (a very long time, we were checking every two weeks at UCSF.) This sub panel was run and B CELLS ARE IN THE HOUSE BABY! That’s right, they are back, in big numbers. And with them, her ability to make all these different immunoglobulins that matter. PHEW! By the way, I never knew any of this existed and I hope the only time in your life you have to read about B cells is here in this blog. On to the T cells. (These also hadn’t been checked since March, so I have been greatly anticipating this test, having had a bit of withdrawal from test result info!!) Her T cells are looking as solid as they ever did, with the same problem they have always had. They are coming in strong and growing well. But, there are more helpers than bosses. Which is fine if everyone is behaving… as they have been. Reese is not experiencing any GVHD, so we know that her T cells are getting along with her body. But, at some point, this ratio should really flip flop and get the administration back in charge. Why it doesn’t, we don’t know. But it doesn’t seem to matter as long as she is well. (That is the second grade version, as I understand it!!)
Another important test that Reese underwent this week is Adrenal Function. The adrenal glands are best known for secreting the hormone adrenaline, which rapidly prepares your body to spring into action in a stressful situation (fight or flight.) When a person has been on high dose steroids, adrenal function shuts down. Basically, the adrenal glands stop producing steroids because… well, they don’t have to. And sometimes they never wake up. This can happen if high dose steroids are used for two weeks or longer. As you know, Reese started the highest dose steroids the first week of June ’18. A rapid taper was attempted mid-July, when her GVHD was believed to be steroid refractory, because it continued to progress on steroids. The team believed that the steroids weren’t helping, so it was important to get them off because they are really bad. Well, a couple weeks into this taper was her awful flare, the worst moments of her gut GVHD. As we know, she prevailed, thank God, but we learned that her GVHD was just so aggressive that the steroids WERE in fact helping, but not controlling. So her taper was painfully slow. From 2.0 to 1.8 to 1.6… you get the idea. She was on a scary, high dose steroid taper for June, July, August, September, October, November, December, and she switched to hydrocortisone at some point in January… and reached physiologic (I think) in March. Physiologic dose means that the hydrocortisone we give her is meant to replicate exactly what her body would be making naturally, if her adrenal glands worked. Reese receives this important dose of medicine 3x a day (and a stress dose should her body need it to respond to “stress”.) So, the doctors tested her adrenal function (last tested in March) and she failed. This means that currently, she still has adrenal insufficiency. I don’t know what happens going forward, we meet with endocrinology in two weeks, and they are in charge. I pray that we get good answers, a thoughtful plan, and hope. The earliest recovery can be seen is after 4 weeks… and it can take 6-12 months. What I don’t understand is exactly when we start counting. A question for endo.
Reese lost a friend last week named Ella. Her first friend at the Family House, they met as this little girl was coming out of transplant and Reese was going in. They suffered a similar course, wrought with GVHD and complications. Sadly, Reese’s friend lost her battle on June 24th. I can’t pretend to understand what this mother is going through, though I do understand the journey and she is on my mind often. Two similar courses with different outcomes. I don’t have to write that I am reminded on a minute to minute basis how truly blessed we are and how fragile life really is. RIP Ella, please say a prayer for this little girl.
Reese- “Can I wish upon the sun?”
Pictured: I took thousands of pictures on this journey. So when I set out to pick head shots that mark important steps, it took hours. I chose about twenty. I am sharing four of them here. A BMT journey: just before, during transplant, during GVHD, and after. The last picture is Reese this week. And she is getting better and more “Reese” every day. God bless my little fighter.