Almost There

James oxygen is down to 50ml now, just a wee bit more to go and he should be oxygen free ... hopefully tomorrow. Character wise James is James again, active, energetic, playful and ready to go. Thankfully he easily accepts his many cords and wires and plays well around them. He also has accept that he can't leave the room and is content to play on the cot, in the high chair or look out the window, with some occasional TV time. A few times he has expressed his desire to go home, knowing that he needs to get rid of the nose prong to do so. He point to the nose prong, declares "out" and then says "go home". But with a few distractions he's okay again.

We are once again at a standstill with the Team ... our continual frustrations with hospitalizations comes out again. The Team is quite content to just get him off oxygen and send him home and we are quite adamant that we need to know the root of the problem or we'll be back again in a week or so. James has three more days on his current antibiotic, previous experience dictates that shortly after coming off the antibiotics the troubles start again. So while the Team sits on there hands, buying time and hoping to get rid of us without having to dive too deeply into the issue we have been doing our research. We have presented our information to the Team and have told them we would like further investigations into the information we found ... as well, we want a 2nd opinion from Toronto Sick Kids. The last we heard they are trying to arrange a Meeting of the Minds of the various doctors for tomorrow to review and discuss how to proceed.

Thankfully we have our Genetic Doctor on our side ... unfortunately he just left today and will be gone for two weeks. We know we'd get cooperation from our Pediatrician, but when in hospital that is no help and once an outpatient things take far too long. Our Genetic Doctor also informed us that Toronto has an LPI case as well, that makes a grand total of 2 in the province (not sure about Canada), and if we go there he'd wouldn't mind us getting together with the Metabolic/Genetic Team there.

We know that aspiration is a big part of James' problems, and likely the reason for this hospitalization, but yet they seem unwilling to do anything to be sure that we are giving James the maximum help we can in this way. They are pushing us towards changing James from a G-tube to a GJ (gastrojejunal tube) Tube. James' G tube is inserted into his stomach, the GJ tube is extended past the stomach and into the small bowel/intestines. The biggest different ... continous feeds. The intestines can not handle "large" volumes of food at once so you have to be fed slowly and around the clock in order to get all that you need. We have not had much time to research this change yet as we've been spending our few spare moments working on researching some lung issues. The GJ tube is even less favourable then having a G tube because it has to be placed through Radiology each time and more easily becomes dislodged. For now we have changed James' to continous feeds while on the G-tube, with the hope that the small amounts in his stomach will work, instead of having four large feeds put in each day. We started him on around the clock, 24 hour feedings, and are now working to see if he can do it in 20 hours ... giving him a grand total of 4 hours each day where he doesn't have to be hooked up to a tube. Now how realistic is that for a busy active 2 year old?? We thought that the continous feeds was helping his gut issues as he no longer had diarhea, but he has since proved us wrong here ... so at this point we're not sure how we want to proceed in this area.

Even though we think aspiration is an issue it seems that there may be more to the situation, and after we told our Genetic Doctor what we thought could possibily be the problem we found out that he was thinking along the same line. From the information we have received on LPI, we know that lung problems are an issue and have resulted in deaths in LPI. How the lungs are related they do not know, but the common LPI lung problem that has arisen is another rare disorder called Pulmonary Alveolar Proteinosis (PAP). Our limited reading of various sites tells us that James' falls under the symptoms/descriptions ... right down to the unexplained nighttime sweating that he gets. The only symptom he doesn't display is the coughing, and of course I can't vouch for all of the CT findings or blood works info, but some I know he does fall under. An interesting fact about PAP is that not only does prednisone NOT help but it usually increases the risk of secondary infections ... so could that be the reason to our recent admission ... will we ever know?

So we have sent the doctors off to go find what they can about PAP and what they think ... we'll see what comes of this meeting of the minds ... if the lung specialist doesn't show up for the meeting we'll be no further ahead ... and considering the fact that we've been in hospital for a week and half now and have not seen him or anyone from his office ... well ...lets just say we're not to hopeful we'll get anywhere with this meeting.

But I am once again out of time and must return to my post.