Thursday was rather uneventful, his labwork from the morning testing his TPN related levels were fine, a couple levels were low but nothing that was out of the ordinary for him. They’ll being doing a full lab workup including some regarding bone health in the morning (Friday). We had a whole group of GI/nutrition related people come in the room, which Michael wasn’t too fond of as he said he felt like a monkey in a cage. The attending rattled off his history including the fact that there was no air sounds in his lower right, and very little in the upper, and very little movement in the airway itself. The head doctor quizzed the interns on how to spell Shwachman Diamond, one looked like  a deer in the headlights and the other confidently said S.C.H.W….and was stopped and informed there was no initial C and that he expected all the paperwork to reflect that. They both looked at each other and said they had some changing to do. I remember all while Michael was growing up under the equally rare diagnosis of Jeune’s Syndrome that his head pulmonologist would always bring in an intern, command him/her to go over his symptoms and give him the correct diagnosis. Of course they couldn’t, and likely he wouldn’t have either had it not been told to him, and there was always a panicked look…or the over confident cocky look…in their eyes. Once he was done I always told them that very few doctors have ever heard of it and not to worry. It’s kind of ironic that all those panicked interns could actually give a sigh of relief as none of his doctors had gotten it right in the end!

Anyway, that was a rabbit trail! We saw endo who are doing some lab work to determine osteoporosis vs osteopenia (less severe) and then what they want to do about it. We also saw a nutritionist who is going to be working in the background following his oral eating in conjunction with the TPN. She sent up an Instant Breakfast shake which he declared much better than mine…I cannot win

UPDATE, FRIDAY AM: I was pulled out in the hallway for rounds this morning (maybe someone over heard his “monkey in a cage” comments all morning as they didn’t crowd into his room today). They are doing some adjusting of the TPN to balance out his alk phos and sodium and are going up a bit more on lipids but he’s pretty well topped out at his protein and calories (I think) intake and tolerating it well. They are going to get renal panels for the next couple days to make sure that is evened out before we go home. It is much easier for them to make daily adjustments here, versus at home when we get a week’s supply so they want things set before releasing him. His CBC was normal for him, still the downward drop of the platelets, we’re at 42,000 now, his white count was at 3.1, anc at 2, lymphs still low. The earliest we are going to be leaving will be Monday now. Endo came in and said puberty issues are moving along fine for his body size and though his bone mass is low, it is not extremely so when you take into account his physical size matches his bone age–his vitamin D levels are low as well as calcium so their first thing is to get that up, and then they do see a call for him taking bisphosphourous (I know I am slaughtering that) to help increase his bone mass once his calcium and vit D levels are stabilized. 

Michael was frustrated at having to stay but he’s doing better about it so we’re trying to just roll with it, he slept a lot better last night.

NOTE: Latest update is that we will be staying until around next Friday. Monday I’ll be trained in PICC line care, Tuesday refreshed and tested on PICC line care and some TPN & lipids and wed. through fri. on TPN & lipids. I’m hoping it will go quicker than that, but that is the latest word.