What now?

6:00 a.m. James spiked a fever…



"Do not be anxious about anything, but in everything, by prayer and petition, with thanksgiving, present your request to God. And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus. Philippians 4: 6, 7



After six days of being fever free, we have taken another step backwards…a very big, heart aching step backwards. After a couple days of being able to tolerate slightly lower oxygen levels of around 1.5L, we're back up to the 2-2.5L area.

The question if whether this came because we dropped the Gentamicin medication on Wednesday arises. It's very well possible, but we won't ever know. The initial plan had been to administer Gentamicin for 10 days, but after some discussion between the Team and Infectious Control it was decided to change this to 5 days, which they say is still a normal treatment length for this medication. I don't know all the reasoning behind why they dropped it, for they did blood work often enough that there shouldn't have been an issue with the levels (Gentamicin is difficult on the kidney's). Maybe five more days would have done the trick, maybe the same thing would have happened at the end of ten days, maybe the fevers aren't from infection after all ... we really have no idea what we're dealing with. If only they had kept him on the Gentamicin, we wouldn’t face the as many question marks as we do now.

If only….

Whatever the reason, it's done now and we're back to fevers.

Yesterday we were told we would wait for the full 10 days of his remaining antibiotic to be completed (Sunday) and then wait to see if the fevers reappear. The idea was to be confident that we had gotten rid of the infection we assume he has. Once they’re confident that we're rid of that, they would be better able to evaluate and determine what's going on in his lungs to cause the increased oxygen levels again. So they admitted that they don't really think an infection is the issue here, they just want that out of the way before they try get to the heart of the issue. With this morning’s fever, everything changes again and now they have decided to go ahead and have a closer look at what's going on in his lungs. Originally I had thought they were thinking along the lines of doing a bronchoscopy – (a common procedure used to look at the lungs, do small biopsies, test fluids in the lungs, etc). But they have advised they are thinking of doing a WLL lavage again. They want to get more samples to test for infections again, and they feel that if they are going to go in, they might as well go in completely and do a “therapeutic" treatment as they called it. In some ways this seems logical, as a bronchoscopy isn't really going to help, it's just going to give them a chance to look, whereas they in the WLL they can look and treat at the same time. But we can't ignore the risks involved with a WLL. We've had two "successful" lavages in that we had no unexpected emergencies, but that doesn't mean the dangers are not there. At the same time since we're again at a (never ending) dead end road, there really isn't any other choices.

The words of Team Doctor when he left today were "we're trying". Those words were probably meant to encourage, but it also was said in such a way that it felt like they were admitting that we're really out of options, but we'll keep going as long as we have the opportunity to. More and more so lately I've been feeling the same way. Is this a losing battle? The WLL did seem to work, but hours later everything turned around. So maybe it's possible a WLL will work, but if his PAP is going to return so quickly and drastically anytime he has an infection then what hope do we have? I know the LORD can make anything possible, and we, with many others, keep praying that some day James will come home with healthier lungs. But at the same time we are fully aware that the LORD’s ways are higher than our ways, and He will answer our prayers, even if it is not how we would like them to be answered. We are prepared to accept that James may never come home, or if he does it may only be after they have said there is no more they can do for him. These are not easy things to accept, but they are not unrealistic.

This is not to say the doctors have truly given up though. I spoke to my Genetic Doctor yesterday who was very impressed with the work that has been going here, and with what the scientists are trying to do to learn more about PAP and how to treat it. They are currently experimenting in the lab how a cholesterol reducing medicine might inhibit the production of surfactant (the fluid which they washed out of James’ lungs), and from there they will decide whether to try it on James or not. How this will be administered, how long it will take, side effects, etc. we don’t know yet, since we have not sat down to thoroughly discuss it.

To understand what they are trying to achieve by the GM-CSF treatment and the cholesterol reducing treatment, think of what happens when taking a shower. You place a strainer in the drain so that a little bit of water stays in the bottom of the tub to keep your feet warm, but no more than that. But after a while, hair clogs the drain, and the water level rises. So you administer drain-o (GM-CSF) to help get rid of the water. This doesn’t work, so you reach down and lift the strainer so that all the water flushes away (WLL). But now the water is rising again, so you close the tap a little (cholesterol reducing medicine), and hopefully you’ll reach homeostasis. Whether James will ever reach that or not, well, that has yet to be seen. If it doesn’t, God will take him out of the tub, safely in His arms.

After all, He will take all of us out eventually.

However, for the majority it’s not until their skin is all wrinkly.


"So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand." Isaiah 41:10