It’s hard to imagine that we’ve been in this hospital room for 15 days already. Actually, I take that back, it’s easy to imagine, the clock on the wall can run backwards at times. Especially considering, I’ve watched Shrek at least 200 times, Bob the Builder and Pooh Bear more times than I can count, and let’s not even mention the little red fuzzy monster. Conventional wisdom states that we are now in the really difficult phase of the transplant. I am not sure what is worse, the current manifestations on my daughter, the anticipation of things yet to come or the things yet to come. What I do know is that it is exhausting and that’s how I know it’s been 15 days.

For all intents and purposes, Rafi is doing exceptionally well. She is a sick little girl, but, honestly, it could be a lot worse. Her internal organs are in great shape, her numbers are all within acceptable limits, she breathes
well, her coloring is good. She’s been running fevers above 104 daily and that takes it’s toll on her body. Her skin is taking a beating and that takes a toll on us. At this point the nature of a blister is changing so we are trying to figure out the best way to address each new one. For instance, the blisters now tend to be blood blisters that just don’t drain. I find that counter-intuitive given that her platelet count is so low. I would assume that they drain faster and more easily with smaller holes. It is what it is. The problem is that the blisters can just get that much bigger because of the thicker, heavier fluid build up. It is what it is. One of the worst things now is she is more itchy than we’ve ever experienced. That means more self inflicted damage. Dr. Wagner tells us that the itchiness is one of the first things to dissipate. That will be a wonderful milestone.

Other than that, Rafi has had a platelet infusion and is getting her second one now. She as also given more blood earlier because her hemoglobin was low. Our new nightly ritual starts at 12:30 am, when she wakes up with a very hoarse cry. I have to change her clothing and some bandages at this point, because they’ve given her lasix to make sure she doesn’t retain too much fluid, and well, diapers without the elastic borders are a tad useless. The mucositis has had time to build up so she is in a little pain and this scares her. Add to those facts that she is once again running a fever, due for Tylenol, and it all makes for a perfect storm. I feel bad for her and we try to talk it out. We try to breathe through it, deep breaths in and slow breaths out and I bounce around the room a little. So, after a little ativan and a bump in fentanyl, and about 45 minutes on my shoulder, she’s back to sleep.

Jackie came down with a touch of food poisoning so I’ve been in the hospital for the past three nights. I swear I think it is harder for her to not be in the hospital than to be here. The three nights here alone just makes me respect my predecessors all the more. How you all had the strength and fortitude to live with no sleep and deal with the stress by yourselves for such extended periods amazes me. It makes me proud of not only the EB kids, and I am very proud of them, but also of you. I know I’ve said this before but your strength, courage and determination is inspirational. I am lucky Jackie is here and that she is as strong as she is.

Here’s a small world story for you before I finally lay my head down on a pillow. My cousin, who is a junior in college, interviewed a young woman for what I think is a student leadership council position. On this woman’s application, she wrote that she had a rare disorder. My cousin, immediately knew the look of EB, and emailed her after the interviews. Jackie, my cousin, told her the story of Rafi, and asked if she also had EB. Turns out this young woman has EB, has a father that sits on the board of DEBRA with me, and was willing to talk to our family about the ways to lead a normal life with EB. Pretty amazing.