Results...

We ended up not having to wait that long. The results were sent to a special pathologist in Texas who specialized in certain types of lung disease and she was waiting for Alana’s lung to get to her. It came back and now it seemed as though we’d have a diagnosis after all this time.

After we got home the wait for the test results was crazy long (okay it wasn’t as long as it was suppose to be but it seemed like it). Alana seemed to be doing really well. She was healing nicely and was in a phase that makes us think that she’s not sick at all, that we are just mistaking a bad cold for something else. We can dream right!

Dr. McArthur called as soon as the results came in and it appeared to be what Dr. R felt it was; Hypersensitivity Pneumonitis. What they found in her lung tissue was not good. You have to remember now that this isn’t like her bronchoscopy; they were not looking at matter in her lungs like fluid, cells, inflammation, etc. They were actually looking AT her lung, on the cellular level, in the dissection sort of way. This isn’t stuff floating around in her lungs, this is her lungs. What they found was this:

1. Small necrotizing and non-necrotizing granulomas.
2. Interstitial lymphocytic inflammation, patchy mild, with focal organizing pneumonia.
3. Non-specific airway injury.
A. Airway subepithelial fibrosis, minimal, focal.
B. Lymphocytic Bronchiolitis, minimal, focal.
C. Lymphoid hyperplasia, mild.
D. Alveolar distention, focal, suggestive or air-trapping.
E. Focal mucus stasis and alveolar macrophage accumulation in bronchioles.
4. Increased tissue mast cells.
5. Pleural lymph node, no pathologic abnormality.

Now that all the medical talk is out of the way, what that all means is that her lung biopsy was bad, very bad. There was a note in the pathology report though that struck a chord with me, there were things that are not “typical” of hypersensitivity pneumonitis (HP). This was important to me because as I read and learned more about HP it didn’t seem to really fit her. There were things that did, truly did fit but there were more that didn’t. Part of the problem that I had was the fact that when her tissue was sent for pathology it was sent along with the fact that they were looking for HP. So basically we are sending you this lung biopsy and we want you to confirm that she has HP. To me that set up a bit of a bias on what to look for, especially given the fact that she didn’t really fit anything 100% and even Dr. R was in agreement with the fact that she was a difficult case.

Now if things were not already stressful they get even more so. We openly disagree with the diagnosis of HP. There are a few reasons for this, one big one being that testing of the house over and over again shows no antigen (thing that she could be reacting to), and her blood itself isn’t revealing anything that it’s responding to. Multiple tests are done and then they are done again with triggers that are unheard of with HP just to be sure. It’s just not adding up, there is no time frame that she always gets sick, say every time it warms up or gets cold or every time we go to the grocery store. With HP there has to be some pattern of exposer that leads to the acute reaction.

We have a care conference with Dr. R, Dr. McArthur, and Dr. Demain to discuss what to do and how to do it. This gets nowhere to be honest. I respect Dr. R and that has come grudgingly. It’s not that we don’t think he’s a great doctor, we just think he’s not a great doctor for us. There are things with Alana that are so beyond the scope of normal that it makes it hard for doctors to accept that their knowledge isn’t working and that they have to think outside of their comfort zone. Not all doctors can do this and it doesn’t make them bad or incompetent, it just makes them wrong.

After the care conference the search goes on to find what would fit her. With the biopsy results in hand and staying focused on her lung issues only we find a doctor in Cincinnati who seems to be very knowledgeable about rare lung diseases in children. Dr. Lisa Young at Cincinnati Childrens Hospital, one of the top 5 for children hospitals, a mecca as 1 doctor here put it. After talking with Dr. McArthur about it we decide to contact Dr. Young and ask her if she’d take a look at Alana’s case and consider giving it fresh eyes. Within days of sending out that email she responds back and is more than willing to look at Alana’s case and all that she is. She already has some ideas and is very interested in her case but the best thing of all is that she says she may not have the answers that we are seeking but that she won’t give up helping us. That to us is amazing news!

There is what I like to think of as a phenomenon with Alana and her medical issues, we get all these doctors and residents who want to know everything, every little detail. They labor for a day or two racking their brains with what it could be, promising to give us answers and help and be there for the long haul. What ends up happening is the opposite of that. She’s “interesting”, “complicated”, “intriguing”, and “confusing” all at the same time but as soon as it moves to “frustrating”, “complicated” and “unknown” they move on. That excitement that is there when you are a doctor or anybody for that matter, faced with a problem that you feel you can have the answer to leaves you when you start to just get annoyed and frustrated. Most pack it up and move on to another problem. Some hang around and tackle the challenge head on like Dr. McArthur, our own saving grace, and she’s going to help us get to Cincinnati because there are things amiss with Alana’s lungs and we are not the only ones who would like to know what to do.