It’s almost been a year since we first went into the hospital to begin this journey. Now we are back home in New York City, in a new apartment, and still trying to stay one step ahead of EB, not often successfully either. We have been so incredibly busy since we left Minnesota that we are lucky if we get to sit down as a family for a dinner. It’s been a long two and a half months since I last wrote. I do apologize for not updating, so many have written emails asking if Rafi is doing alright. Is she doing alright? The answer is complicated.
I think I will work backwards on this one this time. We just got back yesterday from Cincinnati where Rafi had an esophageal dilatation. It was the first time we met with Dr. Richard Azizkahn and he is just wonderful. He exudes confidence in an unassuming way. Anyway, Rafi needed to have her third dilatation in a year. Turns out her esophagus was about 1.5 millimeters wide. The average size of an esophagus at her age is 14 millimeters, so it was quite a stricture. We drove the 650 miles on Tuesday, got caught in the huge storm and had to pull off the road about 70 miles from Cincinnati. It was one of the first times I was ever really afraid of driving. Rafi had the procedure on Thursday and we drove back to NY on Friday. About 24 hours in a car for an hour and a half in the operating room. As of today, Rafi is still not swallowing. Because steroids, an anti-inflammatory, are also an immunosuppressant, Rafi can’t really have them. So instead of the normal 5 doses given to those who get this done, Rafi only got one dose. Everyone is hopeful that Rafi will start swallowing in the next day or two.
Otherwise, the past two months have been about low grade fevers, being on and off some strong antibiotics, which reek havoc on her skin, and always being on the verge of going to the emergency room. She has had a lot of open wounds which get infected quickly. I think we could bath this kid in just bleach or vinegar alone and it wouldn’t help. So now on top of the staph, Rafi is also growing out klebsiella and pseudomonas. These are the first fungal issues she’s had in a long time, probably 18 months.
Although in the past week and a half, Rafi’s wounds have been healing up nicely. It’s been six months since the rituximab and they say that is what it takes to recover from it. Hopefully, that’s true and we can get back to where we where before the Epstein Barr Virus and the 4 infusions of Rituximab. We are hopeful that Rafi will now be on the upswing and will show all the positive effects of the transplant that we were seeing 8 months ago. The past month has been hard because she hasn’t been able to swallow, and therefore she can’t talk very well. This is a kid who loves to talk. So in the brief moments when she can, she is all smiles and animated. Before all this happened, she was clearly not feeling well as her behavior was different. She was difficult and moody, and this is beyond the normal 3 year old fun.
To top all of this off, I have gone back to work full time and this means I am out of the house most of the time. Unfortunately, that leaves the burden of taking care of Rafi square on Jackie’s shoulders. I feel terrible about it, but I guess there isn’t really much else I can do. I have been working from home since Rafi was born but I had to go back to an office. Jackie is strong and I have become rather useless in helping with the bandages.
I know that many of you know that I have become the new Executive Director of the Debra organization. I hope I can due some good for those that suffer from EB. There is a lot to do, so if I don’t post as often as I should, I apologize.