Our Christmas angel...

So yep, she had pneumonia again for sure. Her CRP was elevated, she had an infiltrate in her right lung and she was miserable to boot. Needless to say we did not get to do the EGE testing that time; instead we went on home with ‘scrips for steroids, antibiotics, and orders to check back in with Dr. McArthur the next day or sooner if she got worse overnight. We did end up taking her in the next day to see the doctor and as expected there was the “do we admit her or not” issue to be dealt with.

I know I said that I’d just say go ahead and let’s admit but it just isn’t that easy. There is a fine line that I feel I can walk with Alana in regards to when to admit. At that point I felt that if she could just get some fluids in that she’d feel better, ergo, be able to stay home longer. For the time being the doc agreed and what we settled on was doing an IV in the office. It wasn’t going to be a magic fix by any means but it might allow her the strength that she needs to make it through this. While they are getting things ready Dr. Tapple, whom she’s seen many times when Dr. McArthur isn’t around and admitted Alana for her October stint, sees that we are there again. He inquires as to why, is told, asks if she’s going to be admitted again and shakes his head. I think that the Alana frustration is rubbing off on him as well…

Once the line is placed she promptly falls sound asleep and we just wait for the bag to run through her. It’s amazing how much it can hurt to watch her have a line placed even though you know that she desperately needs it there. I’ve seen her have so many placed I couldn’t tell you a number, I can tell you it still hurts me; not as much as it hurts her, but in a very different way. Over time the line seems to be working and she’s sleeping a bit better and has some color to her cheeks. This is all great news at this point as it means that there is hope that we can go home!! It’s right around Christmas time and she really wants to be the angel in the play at school and I really want her to be that angel.

It does end up working out and she is allowed to go home on tight orders. If she gets worse, doesn’t eat or drink in ‘x’ amount of time, or has an even harder time breathing then we have to return, not only return but return with the knowledge that she will be admitted straight away. I can handle that and I do think that she is OK enough to be at home for the moment.

The play rolls around and she’s WONDERFUL! Thank goodness for such an amazing teacher (Mrs. C) who just takes Alana in stride; she knows when to push her, how much to push her, and when to just offer her a hug without any pushing involved! I am so very blessed with some of the people that are in Alana’s life. She does her lines wonderfully and is in great spirits for the duration of the play. Lucky for us it’s not very long and she’s able to make it through without coughing to bad.
We go back the next day for another check-in and there’s been no real change in Alana. We are given the go ahead to head down to Cooper Landing to spend Christmas with everyone again, conditional; she gets no worse, the hospital in Soldotna is within a short driving distance, and nothing new pops up. This is all doable in my book so off we go to grandma’s house.

Christmas was as well as could be expected. She did have problems with eating and drinking like I’d anticipated but not to the point of dehydration. She did lose more weight, a total of 12% of her body when it was all said and done, but she enjoyed Christmas at home, with her family…not in the hospital like the previous year. It took almost 3 and a half weeks for the pneumonia to finally clear up and things never went back to the ‘old’ way again. Her baseline had officially changed, not for the better.

There are things that I don’t remember to mention in here until I am at a point like I am now. These are the little things that no one can put their finger on but are like pink elephants starting to fill up a room. Alana has had new things going on that I’ve been, for lack of a better word, ignoring. I know that sounds ignorant, foolhardy, neglectful, you name it I’ve thought it. I wasn’t ignoring them because I thought that they were not worthy to be followed, I was ignoring them because I was worried that they needed to be followed. Of course this didn’t last long at all. As Goldfinger so put it, “the first time is happenstance, the second time is coincidence, and the third time is enemy action”. None of this was really going un-noticed by me; I just didn’t know what to make of it was the problem. Here’s a partial list of what I was noticing:

Extreme redness on her face
Hot ears
Hot and red hands and soles of her feet
Raw, painful patches on her tongue
Increase work in breathing
Decrease in her SATs even with the increase in work
A lot of rashes
Sleeping 2-3 hours a day
Increase in her heart rate as well as dips in her heart rate that last longer than they should

I didn’t know what to make of those things or even what to do about them so how the heck was I going to take care of them!? I did take heart that I was not the only one noticing them, Dr. McArthur noticed them as well and agreed that this indeed seem like a new baseline even though it was lower than her crappy baseline to begin with!

Things pretty much continue in that same manner for the next few months. Alana sleeps even more than usual, she is coughing more, she’s running fevers tighter together, not gaining back the weight that she lost in October and December, having frequent rashes and tongue issues, etc., etc. Amongst all those concerns I notice that she’s just really struggling to do daily activities that she used to be able to do, swimming for instance. Granted she still had some issues with them but she still was able to pull them off. Now she can’t even do that and it frightens me to see her literally and figuratively, sink. We are now in February of 2009 and there are changes on the horizon that shake up a lot of things.

The new way of things...

By now I feel like I should be used to this; the unknown of all of it and what it will bring this round. I am not though, nothing seems to make it easier or better or even okay. The same labs are run, the same doctors are contacted, and the same things happen. She holds onto the fever for 11 days and as soon as the fever starts getting better the cough picks up and holds on tight.

There are a few new things that have been creeping up on Alana lately though and here’s something that I feel holds true about her when it comes to new “things”: 1st time a fluke, 2nd time happenstance, 3rd time our enemy. She is now doing something that fits into that last category…she’s having periods of bradycardia (abnormally slow heart rate). Her sleeping heart rate is around 80-120 bpm and now she’s alarming because she’s getting down to 48-50 bpm and not bouncing back up unless she’s jostled. It’s enough of an issue that when it happened in the past the nurses came in and just hooked her up to the heart monitor on their own accord and watched her. When they do it again this time at 2 a.m. because she’s been alarming so much I realize that it’s time to ask the cardiologist what he thinks about it.

When the next morning rolls around and Dr. McArthur is making her rounds she mentions the heart issue and says that she is going to ask Dr. B (cardiologist) about it today and she will call and let us know what he says. Something isn’t right in the state of Denmark and it just cannot be ignored. Calls get made and guess what! Not a big deal at this time. As long as she DOES bounce back then it’s nothing to be worried about. This doesn’t sit well with me on so many levels but again, I am not a doctor right.

As Alana starts to heal from the inflammations attack on her but we are still having problems with eating and drinking. Dr. McArthur at this point is thinking that she might have to start TPN (nutrition through an IV which can cause its own mess of problems) in order to stop her from losing any more weight and not getting any nutrients and vitamins. She’s got 1 more day to stop losing weight before it has to be done. Wouldn’t you know it the next day rolls around and she’s not lost any more weight!! She is holding steady and there is hope that she is going to pull through it without needing any more support than shes already been given! Whew!

I’d like to say that we left the hospital and she was much, much better. If you’ve been reading then you may have picked up on the fact that this year has been like no other with Alana, she’s sicker more often, she’s having issues with gaining and holding onto weight, she’s getting dehydrated within a day of major illnesses, and she’s just miserable more often than not. When we leave she is better, just not better than she was a year ago. She is still so tired and fighting us on eating and drinking. She will eat 1 small meal and not want anything else for the rest of the day. She’s now starting to flux between diarrhea and constipation and that is bringing on stomach pains. There seems to be no end in sight as to what is going to happen with her the next day or even the next hour. Her O2 needs are creeping up and she is struggling with things that she could do almost with ease before. Things like swimming; she used to love to swim and was very good at it for her age. She could do stroke, stroke, breathe and she could dive off the edge of the pool and swim up to the surface by herself no problem. Now she can’t do either of those things even with help. I cannot figure out what is going on and why she’s having such a hard time with everything, is it her lungs? Is it inflammation we cannot see or find? Is she having another issue that we are not aware of? All these things are not things that I want to add to her unknown "in basket"!

Things go by really fast because it’s the holiday season. After Thanksgiving I start to gear up for Christmas and Alana is very excited about something, she’s the Christmas angel in the school play-again! Last year she was slated for the role but where was she last Christmas, oh that’s right…she was in the hospital again. :’( This year will be different though because according to the doctors this too will pass and she will be okay by the time the play comes around. I won’t mention that I’ve taken her into Dr. McArthur almost every week ‘cause her cough is so bad that she cannot make a single sentence without pausing for air and having a coughing fit. I won’t mention that Alana herself has asked her doctor how she’s suppose to talk for her part in the play without coughing. Nothing is making her cough better besides increasing her O2 and that’s a hindrance in itself.

A week or so before the play things start to change for Alana again. We have a big appointment coming up with Dr. Demain to redo her EGE testing and we are all looking forward/terrified of it! Last time we went from 3 foods up to 9 so it’s anyone’s guess how this is going to go. Her appointment is on a Monday morning but by Saturday night it’s not looking good-Alana has a fever of 102.8 and it’s climbing. By Sunday we are hanging steady at 103.2 and pushing Tylenol and Motrin to keep it in check before making her an appointment to go see Dr. McArthur on Monday. By Monday morning though things start to improve and she’s down to 100.4 and looking a little better. Not much mind you but enough that I take her to see Dr. Demain first (it’s a 6 month wait for these appointments and yes, been there done that with her fevers) and figure I will take her into the ped. after her visit with Dr. Demain. Well that goes over like a charm!

She’s lost more weight and after he listens to her lungs during the first part of the exam it all goes south. She has pneumonia. Go get a chest x-ray right away and walk the films back up with you to the office. Stop and get labs on your way as well so we can check her CRP, SED rate, WBC, and start cultures. What’s the motto? Don’t cry, just breathe.

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.