Halloween, Day +254

There has been a lot going on here in Minneapolis with little Rafi. The little one has needed IV medications, she’s had some low grade fevers, and one high one. We’ve had a trip to the ER and a few sprints to the BMT clinic. The trip to the ER was completely and utterly my fault and that botch led to a little surgery, but I’ll get there in good time.

Since we got out of the hospital last month from a bout of sepsis brought on by staph infections in her lines, Rafi has been up and down. She still has no B cells so anything can cause a fever and another trip into the hospital. For two weeks after our last hospital visit, Rafi was on a strong IV antibiotic that we had to give her every 6 hours. About a week after we finished that round of drugs, Rafi started to sweat at night and her daily temperatures started to increase. She also started throwing up again. It was only a matter of time before we ended up back in the hospital or here at home on IV meds. So, after a temperature spike of 102.9, and two days of IV vanco, the incident occurred.

First some other information for you EB families that still follow this at all. For two years, Jackie and I have been mixing bleach with soap and water for Rafi’s daily bath. We were never really able to control the staph infections, only keep them at bay. One day in clinic with Dr. Tolar one of the two visiting physicians from Toronto said we should try pool salt in our bath water to better control the infections. In doing some research about pool salt, I came upon some information that should have been given to us years ago. It’s a simple chemistry lesson, and one that every pool owner knows. In order for bleach to be effective you need to monitor the PH level of the water. If the water is too alkaline, chlorine loses its effectiveness. By adding soap to the water we are changing the PH levels. So, we stopped adding soap to the water, and guess what happened, we got the infections under control. Why couldn’t a dermatologist tell us this? Why couldn’t anyone tell us this? Some times this is so frustrating.

Excuse the digression. Last Wednesday, at the end of our bath and bandage change, I really screwed up. I had just cleaned her central line site, and put on the appropriate dressings and then it happened. In trying to trim one of the bandages, I cut of one of the lumens of the central line. For those that don’t know what a central line is, it’s a plastic tube that is inserted into the chest that travels into the heart. On the outside of the body, the tube splits into two lines or lumens (at least in Rafi’s case it’s called a double lumen hickman). Anyway, I cut one of the lines completely. Within seconds, both Jackie and I are running in separate directions grabbing the special clamps in case one of the lines is ruptured. It’s important to clamp off the line, because now you have a direct line to the heart that is open. Blood would start pouring out of that line shortly. We had to get to the ER quickly to get the line removed. It was 5:30pm, and we thought we would need emergency surgery on an EB kid. Not a great thought to say the least.

I am on the phone with the BMT fellow, commanding her to get in touch with a specific surgeon and one of two anesthesiologists, as well as Dr. Tolar. We get to the ER, and as it turns out, the surgeon we wanted is on call. He tells us that we can create a temporary fix to the line, he’ll make sure all EB friendly people are in the OR, and we’ll get it done in 24 to 48 hours. This was the surgeon who put in the line and Rafi’s Gtube back in January. He did both procedures and there was no collateral skin damage. None, on an EB kid. A severely affected one as well. I was amazed. This guy is truly a great surgeon so both Jackie and trust him implicitly.

The question was whether to replace the central line or give Rafi a PICC line in her arm. She still gets some blood tests and it’s likely she will need some medications in the near future. I mean she just had a high fever and was on IV meds and she has no B cells. In discussing this ad nauseum with Dr. Tolar, we decided that we would just pull the line and if she needed a PICC line later, we would throw it in. We weren’t really using the line anymore anyway. Rafi’s been off TPN for two weeks and everyone thought that the line was the reason for the fevers anyway. So, on Friday morning, we once again brought Rafi to the hospital for yet another procedure, albeit one we planned on soon enough. It went great, the procedure was completed quickly and once again, there was no skin damage whatsoever. Her line is gone. It’s yet another huge milestone that Rafi has achieved.

Since the surgery, Rafi hasn’t had a fever and she’s stopped sweating at night. Her skin is healing up nicely after a good two months of incredibly fragile skin. I think we have once again turned a corner and I hope we have some straight road ahead of us. I am really tired of the twists and turns.

In dealing with all of this, we decided to stay in Minneapolis for another month. Maybe we’ll leave in the middle of December, maybe we’ll leave toward the end of the month. All I know is that I don’t have to clean those damn lines again.

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