Sarah and I were pushing for the doctors to classify this as an emergency and pull the staff together to get this done. Our reasoning was that if this infection actually does stem from the granuloma in his lungs, then it is an emergency and we need to address it, but we won't know without that CT scan.
Dr. Tolar said that yes, he thinks this is as important as we do and is as anxious to get the CT scan done. However, looking at all of the data and the available options, it doesn't make much difference one way or another whether we do it today or Monday.
In assessing Ethan's condition, Dr. Tolar said that if this were the granuloma, he would expect Ethan's condition to be much worse than it is. Specifically, he said that Ethan likely would have already gone over to the intensive care unit with specific respiratory problems, and that the level of infection would be so high that the fungal cultures they took would have come back positive by now.
He said that with our consent, he's going to start Ethan on Voriconazole today anyway, just until the CT scan comes back negative and we're sure. He said this is the same thing that he would do if the CT scan comes back positive. Further, it's the only option available if the CT scan should come back positive, so in effect we're doing all we can irrespective of the CT scan. Dr. Tolar also said that he estimates the likelihood that this is from the granuloma to be about 10%.
He asked if we agreed to this course of action. As is so often the case, I explained that neither Sarah nor I are doctors and while we try to educate ourselves about Ethan's condition and treatment, we don't have the expertise to weigh his condition against the potential benefit or side effects of a particular medication. As such, the only thing we can do is trust his judgement.
Dr. Tolar says that Voriconazole is an effective and reasonably safe drug. I expect that it is certainly effective. But, as to the safety of the drug, I think if the side effects weren't significant then Ethan would have been on it before now. The reality is that we're starting him on a drug with serious side effects because the hospital isn't staffed with a pediatric technician today who can do a CT scan. But, we'll get the results from the CT scan by mid-day tomorrow and can take him off of the drug if the scan is negative. All things considered, I suppose it's a reasonable risk, if for crappy reasons.
Looking at Ethan's fever and assuming that it is a bacterial infection unrelated to the granuloma, Dr. Tolar said that he's fairly happy with how Ethan is doing. Ideally, he said, the antibiotics would have knocked out the fever by now. It's worrisome that they haven't done so. But to paraphrase Dr, Tolar, I think he considers that the infection is partially under control, because, he said, if it were not he would expect that Ethan would have developed sepsis by now. (Gee, isn't that comforting?)
In other news today, Ethan's white blood count is 0.2 (x 10^9) today. That's very good news. White blood cells are, of course, the body's healing cells. I asked Dr. Tolar if 0.2 actually meant anything (Normal is 5.5 - 15.5 (x 10^9)) if such a low level really makes any difference? He said yes, 0.2 does make a difference. He said it's important to note that this number is simply the number of white cells in the blood and does not count the white blood cells that have been absorbed into Ethan's tissues and are helping to heal his mucositis, etc. So that was good news,.
Another recent change is that they seem to have his insulin dosage dialed in. So that they don't need to do hourly blood draws to check his glucose level. Sarah and Ethan had a long night Friday night and a long day Saturday with ERT, hourly insulin checks and the abortive CT scan. So it's nice to have one less reason to prod him at the moment.
Unfortunately, we've got to change the dressing on his central line today. This involves peeling off the 4"x3" dressing that's adhered to his chest, cleaning the skin and applying a new dressing. Dressing changes are painful for him under normal circumstances. Today, with his energy low, his nerves raw, and his pain threshold pretty well maxed out, it's not going to be any fun for anyone--but especially for him.
Oh damn. I just remembered that I forgot to ask Dr. Tolar if it's okay if Ethan gets a tattoo to go along with his newly shaved head. I think he'd look super cool with a black leather biker jacket and an "MPS Sucks!" tattoo on his bicep. Maybe tomorrow.
Thanks for checking in all. We're taking it one day at a time. Everyday here without a crisis is a day of rest and healing.