Eight Weeks!

After five doses of MethylPrednisolone James has not shown any significant improvement. Last time they gave him a Pulse Dose they gave him 10mg/kg once a day, this time they doubled the dose to 10mg/kg twice a day. So at this point he has already had more then the last Pulse Dose and the only real improvement we seen is that he is no longer having fevers and there’s a slight decline in his heartrate.

Our doctors has been in discussion for the last weeks with the doctors in Finland who are more familiar with LPI and LPI PAP due to the fact that 50% of the LPI cases are in Finland (about 50 LPI cases in Finland). Unfortunately even though they've tried many things they have not had any more sucess then here when it's children that have PAP. It seems that when developed as adults PAP is more of a minor "side-effect" of LPI, but with children it's progresses quickly, effecting the inflammation system and there seems to be no answers.

Over the last few days James has spent most of his time sleeping, or resting. A lot of the time he sleeps with his eye part open in order to watch in his sleep what is going on and wakes as soon as a nurse or doctors gets too close. This is a habit he developed right from his first hospitalizations. Eventually he does fall into a deep enough sleep that then you are able to examine him and he doesn't even wake.

It seems this excessive sleeping is simply because he takes so much energy when he is awake. We have tried to play with him or entertain him, or even just "chat" with him, but this can only be very draining and he really rather us just sit in the chair beside the bed and be there. The other day the clown came to visit him, and even though he enjoyed it, it drained him and made him deSAT. He is most content just to be left in his bed watching TV with the occasional chat.

Realizing what lies ahead the doctors have now removed the ICU restrictions. We are now allowed more then two "visitors" (we are classify as visitors, so that meant whenever someone visited one of us stayed in the waiting room and the other took visitors in one at a time) in the room at a time. The girls are also now allowed to come and visit whenever they want. As well we no longer have to call in to ask permission to come in each time and are allowed to come and go as we want, even during shift changes.

Yesterday was the girls came to visit for the first time. They are aware of what is to come, or at least we have discussed it, although we're not always sure how much is truly sinking in or how they are cooping. Rebecca seems to be doing fine. Before coming in her most important question was whether she would be allowed to give James a kiss and hug. Words cannot explain the smile on her face when we said she could. True to her question, the first thing she did was climb up on the bed and give him a great big kiss, or two, or three :) James and Rebecca have always had a special bond and we could see that yesterday when he allowed her to lay on the bed beside him, no one else is allow on the bed. Marietta is much more reserved, not saying anything and not asking anything .... and for anyone who knows Marietta you realize that this is not normal and does cause us some concern. We hope we can break through this and she will feel free to speak about what is going on in her head. Today the girls had an enjoyable time again, this time together with James they attempted to make a mold of their hands. It is currently waiting to dry and we’ll see how it turns out tomorrow.

For the past three weeks they have been restricting James fluid intake as well as medicating him in order to make him pee. The idea behind this is to keep him as dry as possible in order to keep his lungs dry. Realizing that he does not have a pneumonia or anything of that assort we have not really been in agreement with this continual treatment as we can see how much he wants water. Because he is intubated he cannot drink as it could end up in his lungs. He has become dependent on sponge suckers, which we are only suppose to moisten enough to wet his lips or mouth. We have been wetting them generously so that he can get some fluid out of them and he keeps them in his mouth pretty much around the clock. Today they stopped medicating him to pee and allowed him to have freezies. He was too tired to complete a whole one, but quite excited to have something moist and flavourful in his mouth.

We have been bombarded by doctors, child life specialists, therapists, whatever you want to call them all, in the last few days. They mean well, but I think we've gone over everything often enough now. On the other hand they are very good at making sure the siblings are kept in mind. They let us know of activities going on, even take them to the activities if we are busy with doctors meetings. They bring things for them to do when they come to see James and also try to help with any questions that we may have with regards to helping the girls through this. I think we have this all under control (as best we can given the circumstance), we're just a bit concerned about Marietta's quietness, but she may just need time and pouncing on her is not going to help things. Although she is quiet she is still enjoying herself immensely and both girls are loving all the attention, craft things, baking, games, etc that they get to do now.