We saw a geneticist in the morning to confirm the diagnosis of Jeune’s with the new group of doctor’s…it was confirmed (no big surprise). They are perplexed by the Neutropenia (where his bone marrow doesn’t work correctly and doesn’t produce white cells) and are contacting Cedar Sinai hospital where there is some research going on about Jeune’s to see if there is any new information about the syndrome. From there we went to see the anesthesiologist who will be putting Michael under for the procedure (they call it a “scope fest” as there are three doctors who will be scoping him in different ways and for different reasons). She wasn’t able to hear any breath sounds at all from the right lung (the side that the airways are collapsed on) and said he was working on one lung alone. This isn’t surprising but we weren’t sure if he was getting some use out of it or not, apparently not–although we’ll have that confirmed with results from tests done this afternoon and tomorrow (Tuesday) morning. That makes some concerns for putting him under, but she said that if his other lung is working pretty fully that she thinks it could go fine–there is some possiblity that he wouldn’t come right off the vent and have to keep it in over night but from what she could hear of the left, she felt that there was a good chance that wouldn’t happen.

Then he went to get a lung function test that was an hour and a half of exhaustion for him, I wanted to lay on the floor and I have two good lungs and was just holding my breath and blowing along with him! He’s had pulmonary function tests before, but what he had was the very beginning of this version–poor guy was wiped out. We’ll find out the results tomorrow afternoon when we have consultations with the main three doctors.

Tomorrow morning EARLY he has some chest scans and a 3d reconstruction of of his lungs which will (combined with the pulmonary function tests) give us a very clear picture of where things stand for his lungs and whether or not for sure they will go ahead with putting him under on Wed.