Yesterday, as you saw in my last post, Reese’s TPN went down with a bang! TPN stands for total parenteral nutition, IV food. This is a glorious thing, the ability to sustain yourself with oral intake. Reese’s gut was severely damaged by severe GVHD, and now here she is. It was 21 weeks ago that GVHD wrecked total havoc and devastation on her entire digestive track. For months she was screaming “potty!!!” 40+ times a day, no less than 20x throughout the night. The pain, the horror. Now my little girl’s gut is put back together again AND functioning!! This is a miracle, and I thank God for this gift. We are not out of the woods, yet. Reese is still experiencing devastating pain and nausea, which we pray will continue to get better with time. The next question is, how well is her GI track actually absorbing? The doctors need to determine if her gut is absorbing at a level that is safe enough to move steroids from IV to oral. This matters, a lot, because her steroid taper is being done safely and carefully at a rate that her body can handle. If her gut isn’t absorbing as the doctors would expect, the results could be catastrophic to her progress. So we need to test her absorption with something else. Our only choice is posaconazole, her anti-fungal medicine. These blood levels are able to be checked (unlike steroids and her other meds).) It is so important to keep posa levels therapeutic. BMT kids are at very high risk of fungal infections… things in the environment that you breathe in everyday are very dangerous when you lack an immune system, and this is one of the medicines that protects Reese. So, when the doctors decide that they believe her gut is absorbing appropriately, they will “test” it by switching posa to oral, then see what happens. There is talk of doing this in the next week. Amazing. Speaking of steroids, we tapered again today!! Reese now sits at 0.7. This is incredible, a feat I dreamed of for months! PHEW!
TA-TMA. As you know, we began eculizumab two days ago. This morning her hemoglobin stabilized and so did her platelets. This is a good sign that this drug will work!! Celebrating, we need this! The renal team (deals with Reese’s kidneys and high blood pressure) was here tonight and they are changing one of her blood pressure meds around a bit. You may recall that Reese was on THREE high blood pressure meds during her high dose steroid months (with an additional spot dose of a 4th now and then.) Then, suddenly, a couple weeks ago, her blood pressure made a dramatic shift and went very low. So we quickly removed all three blood pressure meds immediately. A few days after that we added her clonidine patch and enalapril back on (not the amlodipine) in much smaller amounts. Then things were fine. Reese’s blood pressure has been high again the past week, 130s, with the TMA diagnosis. She has needed the nifedipine (quick acting spot dose) the past few mornings. Instead of adding amlodipine back on, which they don’t want to do, we are changing the elalapril to twice a day, a teensy bit higher dose, to spread the dose out. We are hoping this works since her high BP have been in the mornings. It’s important to have excellent blood pressure control with TMA. The renal team also confirmed my fears, this TMA process is going to be long, like everything else.
Anyone who has been following my posts long enough knows that I like to have goals. Goals that usually revolve around having my family back at our yellow house, together. My long standing goal has been Christmas. I dream of our Christmas tree and stockings by the fireplace. This has gone away. I know it isn’t going to happen, and I accepted this fact when I asked my parents to bring our elves to San Francisco. Luckily, they travel well, and Santa texted my girls to tell them he has no problem sending Susie, Kokie, and Dottie (yes, we have three elves) to San Francisco. So now I need a new goal. I pray that Reese will be out of the hospital for the Christmas season. And because I know she can’t be at home, I pray that my family will be together, here. Near the hospital, but under one roof.