Pulmonary Alveolar Proteinosis (PAP)

This afternoon we received the results of the bronchoscopy. The stains "suggest" that James does have PAP. By suggest we mean that there is evidence of PAP but it is not clear enough to give a confirmed diagnosis of PAP. To us this is a good as saying he has PAP, the symptoms lean towards it and now the evidence leans towards it. It is sort of like when he was diagnosed with LPI, the symptoms agreed with the findings in the urine but until the DNA results came back it could only be "assumed" he had LPI but not confirmed. In the case of his LPI it was obvious enough that they didn't doubt they had the right diagnosis ... in this case ... time will tell, but we see how the doctors all seemed to have thought PAP was a factor and now we have more proof that it is. From a doctors perspective we can under stand that he cannot truly label James as having PAP without having a very clear confirmed result.

Once again standing our ground has paid off, once again we were able to find something the doctors did not. I feel for those parents or even adults who believe that if the doctor says the doctor knows and so the doctor is right. We have learned over and over that if we pay close attention, evaluate things ourselves, do our own research, we can be just as helpful or more helpful in the treatment of our child. Sometimes it is hard to stand our ground when the doctors oppose what we say, but after several experiences of being right in the end we now are more confident and do not let the doctors intimidate us. If they truly believe we are wrong in our opinion then they will need to prove we are wrong, until they do we will not give up.

So what is Pulmonary Alveolar Proteinosis (PAP)?

PAP is a rare lung disorder in which the tiny air sacs of the lungs (alveoli) fill up with a secreted mateiral known as surfactant. Surfactant is normally necessary for the air sacs to remain open, however, patients with PAP accumulate excessive surfactant lipids (fat molecules) and proteins in the lungs, which impairs absorption of oxygen from the air and causes a feeling of breathlessness.

Treatment of PAP is whole lung lavage. In this procedure they wash the excess surfactant with large volumes of salt water. Usually this is done in two steps, first they wash the one lung, then allow a few days of recuperation, then they wash the other lung. How often this needs to be done is very individual to each case, but it usually has to be done repeatedly. James has already had two lavages, these were not the same as a whole lung lavage, but they were a washing of the lungs ... the second one more then the first. This was in a three month span and is probably why the doctor suggested that he would need it done every three months, considering the amount of fluid they found in each lung the second time. Research tells us everyone is different and some only need it a few times and then for no explained reason are okay, while other need it repeatly and can still progress and develop respitory failure or go as far as needing a lung transplant. With treatment the survival rate is approximately 95%.

Although our Lung Specialist has not been very useful at showing his face during this admission, I must admit that he does seem to be "coming around", being a bit more down to earth and more willing to listen and answer our questions ... he's learning :) Today he showed up for the first time since the bronchoscopy to see how things were going, he had not looked if there were any results yet, so our discussion was more "what if's" The one thing he said that was opposite to others we have talked to is that if we see no improvement with the J tube it was more reason to have the fundoplication done ... whereas other see it as reason not to have it done since it gives evidence that food is not coming back up from his stomach since the J doesn't put food in the stomach. I figured I wouldn't argue that one out all too much at this point, but deal with it as we see what the J tube does ... just another question we can ask in Toronto.

The other thing I questioned is if he were to show PAP would we need to do a whole lung lavage right away ... and if so, we want that coordinated with the J tube insertion. He said he could certainly arrange another lavage but the impression I get is that it would not be to the extent of what I read about the whole lung lavages. He feels that a whole lung lavage can also be risky because you are cleaning out all the surfactant, and surfactant isn't a bad thing, it just is when you have too much. In James' case we see trouble in the bottom of his lungs, but the tops areas are okay, so he would focus on just those parts, and by the sounds of it do both lungs at the same time. But he had no issue with doing a lavage again, even though we just did two weeks ago and also said they would send stains away again hoping to get a more definite answer ... we're seeing more cooperation on his part!!

After our conversation he left to check if there were any results and came back a few minutes later to tell us what they had found. Interesting enough he did say that the only way to get a definite answer is by a lung biopsy, but this time he did not push to have it done, instead he suggested a watch and see approach.

At 5:30 this afternoon we found out they hope to put the J tube in on Monday, but yet they have no time or confirmation of that ... this just before a weekend. So James has to be prepared for this (no food again) Monday morning. It's nice to have things done, but we're feeling a little rushed now. You can't get answers on the weekend and they haven't told us what time Monday ... are we talking 9am or 3pm kind of idea ... 9am does not allow time for answers, 3pm does. Radiology did not show up to discuss tube options today ... nobodies doing anything until we've had a clear discussion about the options. The nurse who specializes in the tubes was not in today and was asked to speak to us Monday ... will she be in before we go down? So I could be left trying to make a last minute decision or not sure that I have gotten all the options because I haven't talked to everyone.

Then there is the PAP issue, so the nurse went back to find out about that and came back to say that they'd make it a go ... all sounds like too many promising words last minute. Once again this will require a transfer under anesthetic, and the Lung Specialist had said he'd likely do it in PICU again. He also said it's possible James will be required to stay in PICU afterwards, both meaning a bed needs to be available. We have a better answer now and our research shows that the only treatment for PAP is lavage, so we don't have too many issues with this, but at the same time it would have been nice to have gone to Toronto firs ... just in case they bring any more insight. We're also not sure if too many lavages is in a row is good either. Just lots of questions that we hope to have answers in a "short" period of time (short - when we're talking the way hospitals operate).

So Monday could possibly be a busy day and I will admit I'm much more nervous about this anesthetic then ever in the past ... also knowing his lungs have not been great lately and wondering how long it will take to recuperate. We leave these things in the Lord's hand and pray for that he gives the doctors and James all they stand in need of. In the meantime we hope to enjoy a weekend at home as a family as James has been given day passes ... meaning he can leave when he wakes and come back at bedtime ... no waiting around for doctors rounds. It will be challenging for James tho as he will have to be hooked up to his feeds all day, but we'll make the best of it.

Two long updates in one day ... hopefully I'll have nothing more to report until Monday (or likely Tuesday by time I get around to it). We wish you all a good weekend.