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.

It's time for the biopsy...

Alright so we continue. So we make the choice to NOT do the biopsy at that time. While getting a diagnosis is something that we need, we don’t need it bad enough to put her in anymore danger than she is already in. We do decide though that she will need it soon. On that note it is scheduled for the second week of January. We leave the hospital with the weight that we will be returning in 2 weeks to have the procedure done.

It’s amazing how fast some days can go while others drag on like the sun forgot to set. She started to finally show signs of getting better and we started to prepare for the surgery. Dr. J oozed confidence in her preop appointments and honestly that really helped calm our nerves.

The day of surgery finally arrives and we head of to Providence knowing that this is going to be a bit of a trip. After all the usual pomp and circumstance we are alone with her in the preop area. I don’t know how I managed to keep it together while they started the sedation on her because inside I was screaming and crying and angry and sad and everything else you can think of. There were no words that can fully describe how useless I felt that day. No matter how this turned out it was going to be hard on her. Mom & dad came as did Janine and we waited the long 3 hours for Dr. J to let us know that she was okay.

Dr. J came out and told us that she did fine. Everything went as he had expected and he removed a 2 x 3 inch section of her right lower lobe. She was asleep and hooked up to all sorts of monitors but he’d take me to see her. Now I had looked at everything I could get my hands on regarding lung biopsy’s but nothing could have prepared me for what I saw when I saw her. If they didn’t have a chair for me there I would have fainted. She had on “No-No’s” which are restraints for childrens arms so they don’t hurt themselves by pulling out lines or tubes, she was moaning but not awake but then so quite that it was scary. She had a tube in her nose (NG), a bloody tube in her chest, a catheter, an IV, and her oxygen on. The amount of disorientation that she was going through was heart breaking in a way that I will never be able to explain.

After the surgery she was moved to the PICU to spend a day, 2 days, 3 days; however long it would take for her chest tube to stop draining. She didn’t wake up for a few hours and when she did it was clear she was in pain. They had a morphine drip going to help and it didn’t take but a day to realize that she was allergic to it. That and the silk tape that made her face swell up like a balloon. That was no fun either! She had to stay in the PICU for 3 days until her chest tube was able to be removed. She also the NG tube in (which goes in through the nose and into the stomach to help suction out blood that may have drained in there). Luckily that only had to stay in for a day and a half and she actually pulled it out herself before the nurse was ready too. Fun times, fun times.

Once the chest tube was removed she was able to be moved to the regular floor. From there it became what is typical of Alana and a battle to drink and eat. She ended up needing to stay another 3 days to heal and get to the point that she was eating and drinking. She was still sore but she was up and moving and started to get better. We were told that they wait for the biopsy results may be up to 6 weeks. Wait, wait, and some more waiting, isn’t that the name of the game with Alana.

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.