The results are in...

“My sense is that if this is an immunological mediated disorder, it originates at the level of the TLR system or is closely related to this. As such, she has trouble sensing and dealing with danger signals that she encounters through her mucosal systems (GI and lungs)…”

“Now, how to make this less fantasy and more fact will be very challenging.”

Those two quotes came from Dr. Bleesing, the immunologist that we saw in Cinci, and were sent in a letter to Dr. Demain, Alana’s immunologist here, and myself. That’s what it boiled down to from their best guesses and from the tests that they ran. What they were telling us is that while Alana clearly has a disease or syndrome, it’s not one that they can diagnosis right now. “As medicine catches up with her then we can try again to come up with a diagnosis and treatment plan.” In the meantime, we’d have to just figure out how to treat each system and symptom when problems arise within or with them with what we could.
I try to remember that everything that we rule out is something that does make a difference; it’s something that we didn’t know beforehand even though it feels like it was for naught. What makes it that much harder is as we’ve taken this journey each thing ruled out moved us farther down the list of things that are known, things that generally have some type of protocol or treatment. Each time we knock one of them off the list it adds something stranger and more complex. What we ended up getting in the form of a diagnosis, and this was mainly for insurance paperwork, was:

1. Chronic Lung disease SECONDARY to immune dysregulation.
2. Interstitial lung disease syndrome.
3. (Already diagnosed) Eosinophilic gastroenteritis.

The thought was, from all of her doctors, that what is going on with her lungs, the fevers, the eye pain, the whole shebang is being caused by her immune system not knowing how to shut itself off. In layman’s terms-- her immune system does not catch colds or bugs any more than other children but what her immune system does with it is the problem. A cold that will cost Shaylin 3 days of misery with a cough and a day or so of fever will cause Alana’s immune system to think that’s it’s under attack 10 fold. It takes her 4-9 weeks to get over a cold but by that time her body has been so weakened that NOW she’s unable to fight off what’s going on inside her and we just keep going and going.

They did recommend things like not taking her out during the peak of flu season, keeping her away from stores around Christmas time when there are a lot of people out, being notified of outbreaks at Noelani and Shaylins schools and keeping her away from places like Chuckie Cheese always. Keeping her O2 at night and when needed would help her sleep better and maybe make it through illnesses faster. They did talk about doing some different drugs with her but we opted not to as some of the side effects presented as many problems as what was going to be eased.

I’d like to say that I was just content with what they told us, that this wasn’t so much an undiagnosed case but an unknown case, but of course I was not. There were certain things that they put into place for when she did get very sick again and those included using O2 regularly, having special labs drawn to measure the levels of things like interleukins and cytokynes, and putting her in the hospital when the fevers had reached the 3 day mark instead of the 10 day mark. All of these things where suppose to offer their own brand of relief so to speak and they did help to some degree, I just wanted more of a fix, more of a clear protocol to help her.

I just ended up making myself crazy, that I can tell you now. As the summer moved forward Alana was a lot sicker then she’d ever been before. The cough was so bad that she would barely be able to speak a sentence without having to pause and catch her breath. On top of that the fatigue that had been plaguing her was getting worse; she was requiring 2-3 naps a day and still seemed tired after that. By this point we are going into Dr. McArthur almost weekly trying to find something to help her. She was having so many ear infections, fevers, diarrhea, coughing fits, muscle pain, poor eating and drinking episodes, and was just miserable. Nothing ended up making this any better besides upping her O2 when the cough was very acute and doubling Motrin and Tylenol without missing a dose.

August rolls around and it’s to the point that we think she’s going to be admitted soon with how sick she’s been. One day she’d be bouncing around and playing but the next she’d be laying on the couch crying and not eating because something hurts or she’s coughing so badly when she moves that she has no energy. Nothing can make those days better and looking back on last summer it is so very apparent that she was in the best shape of 2008 when we were in Cincinnati for testing. No, it wasn’t being away from home as much as it was true to Alana’s course she had had 2 major flair ups a couple of months before we went there and then again so close to our trip that we were almost not able to make it there.

She does end up making it a bit longer then I’d anticipated though. It was one of those times that in hindsight, I should not have waited till she was as sick as she was to take her in. I did though and by October the inevitable came, she was admitted after 4 days of 103+ fever, weight loss of 6%, and being so lethargic that when they placed the IV she just let them. Don’t cry, just breathe.

Glenn Allen, Craig, Cooper Landing

You may have noticed—or not, that I have mentioned Glenn Allen & Ketchikan in regards to my parents homes. My parents have had 3 homes, all in different locations in Alaska, over the past 5 years. Glenn Allen, Craig, on Prince of Wales Island (POW) in Southeast Alaska and now Cooper Landing. The reason I mention this is so you can get a feeling of what my parents have done for us and the girls. My folks were living up in Fairbanks for my father’s job and when we told them I was pregnant with Alana they applied for a transfer of sorts and moved to Glenn Allen so they could be closer. While things were still very different for Alana than other children things were still not as bad as they are now and when they had been in Glenn Allen for a few years they were given the chance to head on down to POW and live a life more their liking; the ocean, small town, business owners, & just mellow moving that is hard to find in a lot of places in Alaska (mostly the ocean part!).

A couple of things are related to this move and when Larry and I went to go get the girls after the Seattle trip we fell in love with it as well, the ocean and catching fresh shrimp and crab every day, magic forests, the smell of the cedar trees which are so massive that you cannot get your arms around them and so many other things. A factor of living was a job though. My parents brought a small café, 1 of 3 restaurants in Craig and they wanted Larry to help them run it and be a part of it. We considered it so fully that in October of 2007 Larry headed down to Craig for 7 weeks to see if he would like it and want to devote all the time and energy that it would take to make it not only work, but be great. Turns out that Larry could do all those things and he worked with my mom and the town like a well oiled machine. He was a favorite of the more mature crowd; aka my mom’s friends who thought he could do no wrong ;-) and he loved them just as much. When he came home we made decision that we’d move down there and bring the girls up somewhere that was smaller, safer, and had more of the things we were looking for.

Now you have to remember a couple of things. This was way back in 2007 and we were trying to focus on Alana getting better not worse. Of course the fact that there was only a clinic on the island made us nervous but we talked with the doctors down there about here and they assured us that a medi vac flight from the island to Ketchikan was less than 60 minutes round trip and she’d be at Ketchikan General which had a good track record. While I remained guarded about the idea I also was being reminded that we could not allow fear of Alana’s illness to rule our lives, we had to keep on aiming high and dreaming big (or small).

As we prepared to set things in motion for the move Alana started to get very sick again. Now if you remember in December of 2007 I had a procedure and then she quickly followed behind for almost a 2 week stay in the hospital with lung collapses and 105 degree fevers. This changed everything because this is when the interstitial lung disease came up, the lung biopsy, and the problems that she was facing were going to be long term. What ended up happening is that instead of us moving to Craig and continuing Larry and my mom’s dream of small café ownership my parents asked for a special transfer to be closer to us and TCHAP to help with Alana’s care and the care of her sisters. After all the juggling was said and done, with great thanks and appreciation to my father’s commanders, they were able to move to Cooper Landing, 90 minutes away by car, in August of 2008, to help us take care of all those things that we were struggling with. Now Noelani and Shaylin could go to Grandma and Grandpas house for the weekend when they needed a break, when Alana and I were in the hospital my mom could come up and help Larry with school and shuffling to and fro; all those things that are really hard when half of the house is missing.

My parents shelved so many things that the loved to help us; a home they finally felt was perfect for them, my mom's thriving and amazing quilt store, the ocean, the people, the café, the fishing, and the serenity of Craig all for us. How can you beat that eh? I can’t think of a way at the moment, to beat it I mean, but maybe someday I will.

I'm leaving on a jet plane...

So we make the flight to Cincinnati in May of 2008 and the whole flight went like a charm, we landed on time in Seattle, made our connection, and arrived ahead of schedule. HA-HA got you right! So we do leave on time, that much is correct but weeee here comes the fun.

Seattle is in the middle of a storm and it’s so foggy that we cannot land. We have to be diverted to Portland and we will be cutting it close to make our connection to Cinci and on top of that we have a big problem. Alana has to fly with O2 and O2 takes 24 hours to get together. Alaska Airlines won’t let us remove the tank that we are using to take it onto another flight to get back to Seattle to make our connection. We originally had a 40 minute layover in Seattle but it looks like we are going to miss that and we have to scramble to get another flight out of there as well as call the DME (durable medical equipment provider) in Cinci to let them know b/c they have a O2 concentrator waiting for Alana at the hotel that we have to physically be there to get. What ends up happening is that we meet a wonderful Alaska Airlines employee named Jared and he ends up being a godsend. He pushes us up the list of people trying to make it back to Seattle and he overrides the regular rules and lets us take the tank onto the new flight back to Seattle. Thank god for the small favors, they are the ones that make all the differences. Once we had that squared away we ended up having a 5 hour layover in Seattle and again, Jared has come through-we can hold onto the tank until our connection flight with Delta is ready to leave. Now we can let Alana take a much needed nap without worrying about her Sats or the nap causing her more exhaustion than not taking a nap due to working so hard to breathe. All and all we make it to Cinci in one piece and the DME was fine and understood that things happen in a kooky way sometime.

The next morning we are up and ready to go meet Dr. Young and her team of gurus. Talk about an experience! Getting there was fun to say the least (you’d think we’d have learned that after getting lost from the airport to the hotel for 1 hour in pitch black downtown Cinci!! Keep smiling, don’t cry…) but we make it and it was amazing. We love TCHAP dearly but after seeing what Cinci has I have no doubts why they are such an amazing hospital. Everything down to the floors are geared towards children and a regular day for them is like a record day for TCHAP but again, we’d rather have Alana here, at our home hospital then lost in the motions of a giant like Cinci. I am getting sidetracked again huh…

We make it to Alana’s first appointment of the week and Dr. Young is just how we’ve pictured her. She’s kind, loving, intelligent, and is listening. Our first appointment with her is just a touch base kinda one. We meet with her for about an hour before heading down to have a high resolution cat scan, HRCT, under general anesthesia, GA, because they do it a little different down there. What happens is under GA the can pretend that the child is holding their breath for x amount of time and they are super still. Hard to do with a 4 year old that is already having lung problems with regular breaths. The other reason is because they flip them over onto their stomachs so there is no compression on the lungs which could lead to fake reports of atelectasis. The HRCT goes great and we head back upstairs to have our real appointment with Dr. Young and go over the test results of the scan and some labs that had been drawn.

We do talk about everything that has and is going on and while we are up there the results of the scan make it and we go over them. It again ends up being one of those bittersweet moments that I’ve talked about in the past. The scan was relatively okay because Alana had such an acute flair up before we left Anchorage that she's in her "well phase" when we actually need her to be in her acute phase (say that 10 times fast). She had mild air trapping (abnormal retention of air in the lungs after expiration. The cause is obstruction such that the patient is unable to expel air completely), thickening of the lung walls, and bronchial damage. All in all an improvement from her last CT though. This of course makes things a bit more confusing. If she had NEHI (see last post) or LIP (again, last post) then those things would not have shown any improvement. Do you remember where I mentioned the kid and the labs and the labs and the kid not matching? This was one of those times; Dr. Young said that she was unsure how to proceed with the lung portion because she was expecting to tell us what she thought and that would be backed up by the HRCT staying the same or worsening. Now that didn’t happen so she has to re-think what would account for her lungs having a period of relative wellness; not normal lung but better then the last CT. She tells us she has to get more information from the other doctors that we are going to be seeing and when we have our finally conference we will have the game plan. That’s alright with us because we:
1. Don’t want Alana on any meds that are not needed.
2. We don’t want a diagnosis that is incorrect.

That ends that day and the following day goes by sorta the same, we meet GI who really doesn’t have much to offer us and that’s alright, we are happy with Dr. Demain who handles her EGE here but adds that he doesn’t think it’s EGE related. The next day we meet with Immunology who we do have high hopes for. There has been this underlying thought that Alana’s immune system is goofed up on such an innate level that it’s unable to be detected by the means that we’ve had available to us. Cinci has some ground breaking workups going on and getting her into their hands will be very helpful. Our immunology appointment ends up being a 6 hour adventure. Unfortunately it ends with no clear outcome. Dr. Bleesing thinks that there is a malfunction with her Toll-like receptors (if you are interested go here: http://en.wikipedia.org/wiki/Toll-like_receptor to read about them b/c they are complicated) because there are parts of her immune system that are activated at the same time that should not be and for no reason. We are promised a complete workup from the 13, yes 13 vials of blood that they draw but those won’t be in until we are home due to the complexity of them.

That night she had a full sleep study done and talk about weird. She hated every minute of it and I would have too. She was hooked up to heart monitors, a pulse ox, neurological leads, etc. it took over an hour to get every attachment on her but at least by that point she was so tired she went right to sleep. The sleep study didn’t show us anything new so that was okay to. She did great though from our stand point with all the leads and glue and aggravation. We did get a break that day and took her to the Cinci Zoo and it was nice to see her smile and be happy for at least once that week.

The week went on and we had our appointment with Rheumatology to get to, and again, nice doctor, just not a lot of ideas. He did test her for a disease called TRAPS but did tell us that he thought she had about a 10% of having it. Par for the course results would takes up to 8 weeks to come in.

The next big thing we had coming up was another bronchoscopy as well as a nasal cillia biopsy (to look for another lead, Primary Ciliary Dyskinesia) and this does have me more than a lot worried. Her last bronchoscopy was scary due to many reasons and again, it’s another procedure with GA which we are realizing that she has a problem with. It does end up going well though; she shows us why she is such an amazing, strong and brave little girl. She does take about 5 hours to wake up from GA and this starts to solidify that there is something with GA that is abnormal with her and GA but for now we are just happy that it’s done and she’s alright.

We go back to the hotel later that night and get some much needed sleep and prepare to go home. We can’t leave until she’s cleared which will be in a day or two so we can make sure she really did have a good procedure. In the meantime it’s back to the same ole’ game of waiting on labs and results, nothing new in that respect but there is something renewed…hope. Hope that this is going to be figured out and she is going to be alright in the end. We get that it might take some help along the way but that’s something we can deal with as long as it gets her better, it brings back more smiles instead of more tears.

She gets the clearance to go home a couple of days later and we do. It’s a long, long flight home because we opt for the straight shot but yea, that’s an 8 hour flight with a 4 year old. One thing I didn’t mention about the flight thing was that when we went through security at Ted Stevens we (as in all of 3 of us) were all patted down, swabbed, the whole 9 yards. We didn’t think anything of it because it happens, but when we checked into our flight home we noticed that the agent again marked all of our tickets. We asked why and he told us it was because we were flying with O2. Are you kidding me?!? Alana, our 4 year old was being patted down because she needed O2? Again we had to go through the special line at security and this time we had to go through the “puffer” that checks for explosives. Funny how times are that a 4 year old can really be considered a terrorist. Okay, yea, I am soap boxing again.

Long and short we make it home in one piece and start a long wait. Dr. Young assures us that as soon as all the labs are in she will call us to go over what the thoughts are, the game plan, the everything. We do trust her so we are again, okay with that. Home sweet home and speaking of sweet, I’ve so missed Noelani and Shaylin. They are two of the brightest lights in this dark tunnel.

Cincinnati

Time seems to fly by rather quickly for the next few months. It’s now February of 2008 and as is now par for the course Alana is rather sick again. In the meantime we’ve been working on getting a sleep study done to see if shes de-sating at night and if that’s what’s causing her to wake up at night at such random intervals. There isn’t a pediatric sleep center here so Dr. Young, who we are now working with, suggests that when we make the trip to Cincinnati we have it done there. That brings me to my next thought is that yes, we are planning a trip to Cincinnati Childrens to meet with a team of doctors. Dr. Young has spent countless hours with me on the phone, emailing us off and on for weeks with pages of questions about Alana. She’s contacted all of Alana’s doctors here and requested records and x-rays, and CTs. Dr. McArthur is just amazed at how involved Dr. Young has become to Alana’s care and how much she’s trying to help. For the first time in a really long time it feels like we may have an end in sight for the diagnostic phase.

By the time that April rolls around tho the news isn’t very good. Alana is so sick that we may have to wait on the trip. At this point we are planning on being in Cincinnati in May to meet with Dr. Young (Pulmonology), Dr. Bleesing (Immunology), Dr. Putnam (GI), Dr. Graham (Rheumatology), and Dr. Boesch (Pulmonology for another bronchoscopy). To have to reschedule all those appointments is going to be very hard. The suggestion is made that we go ahead and do a small sleep study at home using a monitor that will be provided by the local oxygen supplier and it will record her SATs while she’s sleeping. If she’s coughing so bad maybe we can get her on O2 and that will help her heal a bit so we can make the trip down there. We already know that sh'es gong to need O2 on the plane so trying to figure out that is such a pain in the ass.

We do the mini sleep study and wait to hear the results of it from the O2 Company. At the time there was a wonderful gal working there named Aurora and she was very good at keeping us updated with how things were moving along. As I waited for her to print out the results, the whole time with Alana coughing and gagging and looking like hammered crap I couldn’t help but think that there wasn’t going to be anything. It’s par for the course with Alana that her labs don’t match the kid and the kid doesn’t match the labs. What I mean by that is when doctors take a look at her CTs, labs, x-rays, history they picture this very frail and sickly looking child before they’ve even seen her. Then they see her and notice that she’s smiling, small for her age but otherwise the quintessential “She doesn’t look sick” child they start to overlook all those labs and tests that they were so concerned about. Anyway—So Aurora comes out and she looks unhappy. Great, it didn’t show anything was what I was thinking. I was so, so wrong. It showed in a 4 hour period she had de-sats down to 84% more than 20 times. The highest that she got up to was 96% and that wasn’t for very long, her baseline was close to 89% for most of the time she was hooked up to the machine. Aurora had the tech’s make sure there wasn’t a problem with the machine because it was so awful and then they told us that everything was in order and the machine was working properly.

So all in all it turns out there was something we were missing; Alana needed to be on O2 at night even when she wasn’t acute. It’s now only a few weeks away from her appointments in Cincinnati and things are starting to look up. She’s sleeping a bit better with the O2 on and she’s not even fighting putting on the canula (prong looking thing that delivers the O2 up her nose) and tells us that it makes her feel better when she has it on. WHOOO HOOOO something seems to be helping her. Dr. Young is staying in touch and letting us know how things are looking for appointments. She’s set us up with some of her colleagues whom she thinks will be best suited to help Alana. We are scheduled to have a high resolution CT scan (HRCT), many, many labs, sleep study, bronchoscopy, and anything else they deem needed while we are there. We are looking at a solid week there and the hope that finally we are going to have a diagnosis for Alana. Dr. Young has mentioned things like Neuroendocrine Hyperplasia of Infancy (NEHI), Lymphocytic interstitial pneumonia (LIP), Surfactant disorders, and anything else that she can think of that “fits” Alana. It is such a relief to feel like the end might be in sight! That while it sucks the fact that Alana has to deal with having 2 rare diseases, Eosinophilic Gastroenteritis (EGE) and now a probable interstitial lung disease on top of that, there is hope that for some of the lung diseases there are treatment plans that can be put into effect to help her. There is some light at the end of the tunnel and all we have to do is make it across the country, an 8 hour flight, to Cincinnati Childrens hospital to meet the person who’s going to help us and it can't come a moment to soon as things are changing with Alana, she's getting sicker faster then ever before and she's just getting very tired very fast. Here we come Dr. Young, hope you are ready for us.

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.

The summer is here...

The summer ended up being a long one. Alana spent even more time sick and in and out of the doctors’ office as the summer continued. Dr. McArthur tried to make things easy. She ran tests. She asked for consults and then she ran more tests when the first ones didn’t yield what we were looking for. It is now May of 2007 and she has been sick for all most 3 years straight.

The rest of the summer is pretty much the same as the start of the year was. Noelani and Shaylin are trying to be brave and remain little girls. Shaylin seems to have the hardest time with all of this. Noelani seems to cope better and that may be due to the fact that she’s older, more mature and able to talk through her emotions. Alana keeps us from a lot of things. We don’t go camping at all because she can turn at the drop of a hat. She can be smiling, eating, drinking, and coughing all time than WHAM! She is dehydrated, lethargic, and struggling to breathe.

Because we are not getting anywhere when it comes to her coughing we try and focus on the diarrhea. What if we can at least get that under control? Maybe other things might fall into place as well. We meet with the GI doctor again and yes again he tells us that there really isn’t anything that he can think of to do. It was a blessing that her immunologist, the wonderful Dr. Demain wrote another referral stating that she did need the GI doctors’ help.

We meet with Dr. P and he tells us that doing an EGD (esophagogastroduodenoscopy) and colonoscopy might give us answers about the chronic diarrhea. After thinking about it for a few days we decided that while it is another procedure we need to do it if we hope for any answers. Early November we go back to the hospital and have the scopes done. I get to stay with her this time until she is fully under. That was an experience in itself. She was usually sedated when they took her but never fully under. She did great though. It did take her hours to wake up in recovery which seems to be her M.O., but we were overall pleased with how she handled it.

The results came back with some results (say that 5 times fast)! In her duodenum there were eosinophilic cells which are a type of white blood cell that is related to allergic reactions (among other things). Dr. P tells us to go ahead and go back to Dr. Demain and speak with him about the results because they might mean that we are missing some of her allergies (remember from above that she has Eosinophilic Gastroenteritis). It isn’t too long before we have our appointment with Dr. Demain and he has his thinking cap on.

We end up deciding to re-patch test her. I don’t remember and I don’t feel like looking back to see if I explained what that is but here it is again. When you are patch tested for allergies compared to scratch testing (where they place small scratches on your arm or back with the tips of the “needle” dipped in an allergen) they place small metal discs in your back filled with the perceived food threat and a special compound. The discs are left on your back, covered in tape, for 48 hours. No shower and no messing with them. After that time you go back in and the patches are removed. The nurse does a first impression and writes that down. After that you go home and the actual read is done the next day. With it being a “cell based” reaction, not the same as an oral reaction which may it turn cause an anaphylactic reaction, it takes time for the body to react.
She has her whole back filled up with patches including the ones that we all ready know of, beef, chicken, pork. Now I have seen enough of these to know that something wasn’t right with her back the night that they were taken off. In the past she’d never really complained about them once the tape was off. Now she was hysterical and scratching against anything she could find. *sigh*
She comes back positive for beef, chicken, pork, turkey, corn, carrots, rice, oats, and eggs. I started to cry. I just could not help it. I know in the scheme of things it wasn’t terrible. She had gone through so many things and we knew that she was having diarrhea for a reason so this shouldn’t be a huge surprise but it was. I was expecting maybe 1 or 2 new foods but not 6 more. To make things even more stressed out Dr. Demain decides that she also needs to be taken off all diary foods to boot. Sometimes even though things do not come back positive for milk he explains that it’s a tricky one. There is a thing called Heiner syndrome and she does fit it. That was exciting. The cure…NO MILK! It is a relatively unheard of disorder but as you can see here are some of the symptoms.

• Chronic airway disease
• Iron-deficiency anemia
• Recurring diarrhea
• Failure to thrive
• Cough
• Shortness of breath
• Wheezing
• Recurring respiratory infections
• Retarded growth
• Gastrointestinal disorders
• Pulmonary hemosiderosis
• Partial or complete lung collapse

Hello!! How many of those does she have! It was a mix of bittersweet about all the foods but maybe, just maybe there is another answer out there. The test is just to remove every speck of dairy from her diet. We meticulously remove all dairy from her diet. No one would feed her they were so afraid to mess it up! The goal is no milk or other dairy for 6 months and within 1-2 months she should show signs of improvement if it is Heiner syndrome. Fingers and toes crossed right…

As December rolls around with all its festiveness and warmth and Alana is going down for the count. By mid-December there is no use denying it; she is really sick. By the way, if that isn’t enough right now I end up having to have a surprise total abdominal hysterectomy at the start of the month. What the hell right!! So anyway-my hospital stay wasn’t going as smoothly as hoped and I had to spend a few extra days in there. All the while Larry is juggling the girls and work and yea, as mentioned Alana is getting visibly sicker each and every day. I leave the hospital sore but all right and 8 days later Alana enters the hospital again. I’d like to say it wasn’t something usual that she had a bad virus, that everyone in the house was sick with the flu but alas, nothing could go that right. She has atelectasis again and infiltrates. The fevers are just under 104° and we cannot get them under control (I could have cut and pasted that from above right…).

Dr. R comes in and we start discussing what should be done. He is now suggesting that she have a lung biopsy done. What they would need to do is make 3 incisions on her chest. One on the back where the biopsy specimen would pass through, one on her side where an instrument would go, and finally another on her chest, right below her nipple, where another instrument would go. He tells us this is the best way to get a “definitive” diagnosis for her (to confirm his diagnosis of hypersensitivity pneumonitis) and to actually see her lung under the microscope. We are listening and agree to meet with the surgeon. Dr. J comes in and we start talking. He is a gentle man and you can tell that he’s been around the block for awhile. This can be a good thing and a bad thing. Older doctors have the wisdom, experience, and proven track record to get things done. Both Dr. R and Dr. J fall into this category. The downside is that they are set in their ways and sometimes unwilling or incapable of exploring newer possibilities. Luckily, Dr. J was a wonderful combination of both old school wisdom and realization that the parent is running the show.

Dr. J does a complete exam on Alana and feels that she is too ill to do the biopsy. He tells us that she could end up on a ventilator and there is the possibility that she won’t come off of it. Yes, things were so very, very hard right then. I mean think about it. It was a no brainer that we did not want her in a worse state than she is all ready in but that small possibility that we could finally have a diagnosis! That we could without a doubt see what her lungs looked like and what they were “made” of and what if “it” isn’t there when she is well!?! Do we miss the window in which she is acute and is the best chance to see what is causing it or do we just let it go and hope that in time when she’s better but not better we do it then? Nothing is easy with Alana is it?

We are upset, Dr. McArthur is upset, Dr. R is mad that we are considering not doing the biopsy, and Dr. J was probably wondering what the hell we are all doing. I don’t think that I have cried that much in my life as I did that day. Oh and yes, it is the day before Christmas and we are not going anywhere. We spend Christmas in the hospital praying that Alana will make a turn for the better.