Seattle Childrens

Alright, so taking deep breaths and hoping against all hope that they have an answer for us off we go to Seattle. Larry takes Noelani and Shaylin to Ketchikan to meet my parents then he continues onto Seattle to meet me and Alana. We took an earlier flight so she wouldn’t be as tired the morning of her appointment and in hopes that she’d be able to settle a bit as well.

So we head to Seattle Childrens hospital bright and early. I am still chipper at this point because what’s the worst that can happen? They tell us she doesn’t have an immune issue and send us to rheumatology for the answers that we are sure to find. Alana is what, just over 3 now and it’s been a long and frustrating road thus far. Dr. McAurther had tried to gently warn me that if they had clear cut answers about Alana there’d be no need for them to actually see her. They’d be able to relay all the information on the phone. The hopes of this visit would be to gather more information, a fresh set of eyes if you will, and then come home and await the test results. With that in mind we meet with the fellow in immunology first, go over everything from bow to stern. He thinks a bit then tells us he is going to get the doctor and yes, the doctor comes in and wants us to go over it all…again. *sigh*

Now, before I go any further I hate to say it but…DOCTORS CAN BE THE BIGGEST JACKARSES ON THE PLANET!!!! Alright, I feel better now. Going on now.

After we tell him all there is to tell he sits back and thinks about it, takes it all in. Then he starts to tell us all the great news. Let’s start with #1- Alana is a “complicated” case. #2- There will be no easy answer. #3- We just will have to wait for medicine to catch up with her.

So yes, those are his tidbits of wisdom. He tells us that she’s had a rather in depth immune work-up. That what we are doing is all they can think to do at the moment. But, all in all this wasn’t something we didn’t expect. We are waiting anxiously for him to say the magic words (RHEUMATOLOGY) and he does!! What he says is…”We have another doctor who is both an immunologists and a rheumatologist, we will let him have her file and see what he thinks about it. Thanks for coming and as medicine catches up with her then we will be able to help her more. In the meantime what you are doing is great. Continue to manage her needs as they arise and stay in touch with all your doctors. See ya now!” (Remember to breath, don’t cry, remember to breath, don’t cry)

Through my frustration and tears I manage to speak. Why did we come here? Why can’t we see rheumatology? You are not giving us anything more than we came here with! Maybe it was my absolute desperation or maybe it was he just wanted this blubbering mess of a woman out of his office but he throws us what he considered a bone. Here, why don’t we send you to pulmonary and see what they think. First go ahead and get an x-ray so you can have that with you when you see them and they will get you in today before you have to be at the airport. What more can I say or do? He and his fellow are done; they are hand on the door, walking out D-O-N-E, done.

We leave his office, defeated, hurt, and feeling very, very alone. I call Dr. McAurther and she leaves a patient to take my call. I am amazed the woman could even understand a single thing I was saying (or trying to say). I tell her what just transpired and ask her, beg her, what we should do. I have to admit, that is the first and only time I’ve ever heard Dr. McAurther angry. It’s not her style from all we’ve seen of her but oh, she’s angry now. She tells us to go ahead and do the x-ray, go to the pulmonary appointment and she will call us back with a plan.

Alana does the x-ray like a super girl she is and it shows that she’s got an infiltrate in the LLL (left lower lobe) but that’s all. That’s not something totally unusual for her. Sometimes they are there, sometimes they are not. It’s nice to know as it means that we are just getting over another episode or we have one coming on.We take the x-ray up to pulmonary and meet with a Doctor. I can honestly say I don’t remember her name. One thing I do remember is that she made a point of telling us that she is a friend of Dr. R. (yes, if you’ve been paying attention like I know you are, Dr. R is her pulm. here. Go figure) and why are we there in Seattle? She listens carefully to us recant again the story of Alana. She looks at the chest x-ray and then she tells us what we have heartbreakingly come to expect. Alana is a complicated case. Stick with the status quo. We don’t feel that nothing is wrong with her; we just feel that we can’t figure it out right now. Did you enjoy Seattle? What time is your flight?

We leave Seattle Childrens at 9 pm that night. By the next morning Alana is coughing and running fevers again. We settle in for the long nights ahead and try to not fall apart.

You are joking right...

So now we have a diagnosis?? That’s the question we are left with. A time that should have been a strange sense of relief for us, a diagnosis, we were left with the feeling something that I equate to shoving a size 2 dress on a size 10 woman. Sure you can make it fit but should you?

Our life at this point is chaos. The older girls muddle through her hospital stays and endless visits to the doctors. We are searching harder now for an answer and finally it is very clear to everyone that what’s going on with Alana isn’t going to go away. It’s not getting better and this last stay was a wake-up call for all of us.
Time goes on yet again. It’s funny how that happens. When you least expect things to happen they do and when you expect them to happen they don’t. We spend month’s on pins and needles waiting for her next big flair up. Don’t get me wrong. She’s sick every time we turn around. Something to remember with Alana is this, when she’s “OK” it’s just that. She is always coughing. She is always having either fevers or diarrhea. The thing that makes it an OK day vs. a bad day is how she handles them. She can have a 100 degree fever and be laid out on the couch and needing to be watched at every breath or she can be 102 degrees and be up and playing and eating just fine. It really is a day to day on how she’s feeling. We can have weeks with no issues other than her normal self (slight fevers, 10-12 BM’s, slight coughing) or we can have weeks of bad days (high fevers every day, 12-15 BM’s, non-stop coughing). All is relative to us at this point.

As I said though things are not going unnoticed now and her symptoms are demanding that the doctors pay attention to her. Something that is in the works is sending her to see doctors in Seattle. The childrens hospital here is amazing. It truly is and for our location on this big world and being isolated from a lot of things it really makes a huge difference for the children that live here. That being said, there are some services that they cannot provide. There are some thoughts floating around now that Alana has a rheumatological condition that is causing all of her problems. This is a great idea and seeing as we’ve done such extensive immune work-ups and random things with no real results. This is one area that she does fit, and that we’ve not looked at to a great degree.
The process of getting her there isn’t a piece of cake though. I mean actually getting her there is as easy as getting on a plane. Getting her seen there is another issue unto itself. I can say one word and make it all very clear to you why that was an issue…insurance. Do you have any idea how flipping hard it is to get clearance to go on a “fishing expedition”? We don’t have a diagnosis, we don’t have a clearly defined pattern of illness (in their thoughts) and why can’t we just use the doctors here?

Now this is another area that Dr. McAurther shines like the supa star she is. She gets to work writing letters to the powers that be that Alana does need to see rheumatology that no, she cannot wait for them to do their every 6 month rotation up here and that she needs to go now while she’s well enough to travel. Long and short or it, Dr. McAurther rocks and she gets us going. We are so excited as the approval comes down because that also means they will pay for our tickets, well Alanas and mine, hotel, and food for the both of us. This is a huge, huge, relief for us. Now all we have to do is wait for Seattle to call and tell us when our appointment is going to be. Yea!

That call finally comes (it wasn’t really all that long, a week or so) and it’s from Immunology. Huh?? We don’t need to see Immunology seeing as we have a wonderful one (Dr. Demain) here who’s been in touch with the doctors in Seattle already about Alana. For that matter he’s contacted colleagues across the country regarding her and what tests should he add to her already impressive resume. Why are you making me appointments to see them when we are suppose to see rheumatology, they are suppose to be the ones that help us with her not sending her somewhere where they are going to redo the things we’ve already done!!

After some phone calls to and from the doctors in Seattle and here turns out that rheumatology doesn’t want to see Alana. They feel that she does have an immune issue and that if we see their fellows and doctors and they say that she doesn’t that have an immune issue then we can see them. ARE YOU KIDDING ME!!

Backtracking a bit...

Going back to that last hospital stay I mentioned that we met Dr. R. He had plans to diagnose her while we were there and had plenty of ideas simplest being that she had aspirated food when she was an infant and it was stuck in her lungs causing the inflammation and always negative lab results. Then he asked if she had been checked for CF and yes, Alana in fact had already been tested 2x via sweat test and then finally genetically as was I. Neither of us are carriers for CF so that effectively removed that from the equation. Anyway-we talked for hours about her past history and we mentioned that we had recently* brought 2 birds. Parakeets named Tweet Tweet and Opal. Wow. That opened his eyes to what was wrong with Alana. She had what is called Hypersensitivity Pneumonitis and it was from the birds. It’s an interstitial lung disease and it’s mostly seen in an on the job based scene i.e. coal miners lung, bird fanciers lung, & most commonly mold in walls. It is a hard disease to diagnosis and even harder to prove in some cases. There are things that are almost always seen with it and then there is a fine tooth comb that needs to be run over every aspect of one’s life. You have to have environmental studies done to make sure that you are not taking an already super sick person back into an environment that will cause them almost immediate distress. There are three overlapping versions of HP (Hypersensitivity Pneumonitis) Chronic, Sub-Acute, & Acute. If Alana indeed has HP she would have the chronic, which is the worst, because of how long her symptoms have been going on at this point. Chronic is low level exposures over days or weeks with acute flairs in between. Finding out what the antigen is a fundamental step in stopping the progression of the disease. Here is a medical definition of HP:

Hypersensitivity pneumonitis (HP) is a group of rare inflammatory interstitial lung diseases that result from repeated inhalation of certain fungal, bacterial, animal protein, or reactive chemical particles, called antigens. The body's immune reaction to these particles causes inflammation of the very small airways in the lungs, called alveoli. If exposure to the antigen is continuous, parts of the lungs may become scarred or fibrous.

Can you see why we would be so scared at the mention that she may have this? Is it something in our house? Is it something in the air that’s seasonal? Is it everywhere? Before we leave the hospital we set up with an environmental company to have our house tested for mold. Mold would be the most common reason a home would make someone sick and she’s lived in this house her whole life. The day she’s set to return home the house is tested and she’s started on steroids. The steroids are to make her lung stronger and if indeed it is a mold issue in the house than they can help protect her while we await the test results. It is going to take 2-3 weeks to get the results from the environmental company so in the mean time we just try and keep her well and return to normal routines.

Finally after what seems like forever the results come back and they are negative. The whole house was tested down to the crawl space and there aren’t enough molds to even slightly raise an eyebrow. During this time as well I have started on another search to learn about HP. The more I read and the more Dr. McAurther and I talk about it the more it doesn’t seem like Alana. Granted there are things about it that do, the fevers, the weight loss, and a few others but the main hang-up for me are two things,

1. She was so young when the coughing and fevers started, under/around 3 months of age, for HP that’s taking a rare thing and making it even rarer.

2. She’s able to have short period’s in-between that she’s OK. We have done 2 antigen panels by now (antigen panels are where they actually take her blood and test IT for HP triggers. They subject it to known and in her case, some newly discovered antigens, and get (-) or (+) results that way) and she’s tested (-) for everything including mold.

The more I learn the more I doubt that she has this. It’s neither the first time nor the last that she fits something but it isn’t her (Hyper IgD, Muckle Wells syndrome, Familial Mediterranean Fever, etc.) so we’ve learned how to be hopeful but skeptical at the same time. Nothing is ever black and white with Alana much to our dismay. On goes the testing and seeing Dr. R a few times. We have a stressed relationship with him at this point because what we were hoping was going to turn into her knight in shining armor with a DX has turned into someone who doesn’t appear to be listening to us and going with what doesn’t seem to fit her. It’s so frustrating. It’s so disappointing. It’s so usual.

We see Dr. R for what would turn out to be our last office appointment with him and he just tells us that she’s more than likely got HP to something that we don’t know about yet. That there are so many things that she could be responding too and medicine doesn’t know everything. Clearly that answer doesn’t work for us. Larry and I are not going to accept that she’s just got something because it’s a convenient DX. We start our search again and hope that she will make it there healthy.

13 days and nights...

So I think last time I ended up getting distracted. I was talking about her tonsils and Dr. P. Then I got sidetracked with all the other doctors and such. Let’s get back to that. So Dr. P thinks that she will do better (well I think he more or less hopes at this point) if we remove her tonsils that she will sleep better which in turn might help her eat better which in turn might help her get better.

Something that is important to mention at this point is that we have a new pediatrician! Dr. Erin McAurther and she is in the top 10 of the most amazing women in the world. She’s compassionate, brilliant, and just an amazing person to know. She’s taken Alana (and us) under her wings and is working to find out what is wrong with our baby. She’s great with the girls and she never ever makes us feel like we are asking dumb questions or to many questions for that matter.

It’s been a long road and we are desperate to find something that is going to be that magic *poof* that we’ve heard so much about. We agree to do the surgery and also given her history she is going to spend the night in the hospital to monitor her overnight. It’s funny that now days they have things down so ‘pat’ that even 10 years ago that type of surgery would have been a 2 day stay. Now it’s an outpatient deal and they don’t even kiss you on the way out the door. OK-so off track again. She has her tonsils removed in November of 2003. Dr. P is great and we have no doubts that he will take care of her and do his best to make this easy.

I didn’t mention another thing again that ties into this. When she was in the hospital last time one of the thoughts was she may have an infection in her tonsils or somewhere behind them. An abscess somewhere that wasn’t visible to just a tongue depressor. So they did a head CT and what they are looking for isn’t there but they do ask us this…”Are you sure she’s had her adenoids out? Not just tubes put in? You see her adenoids are huge…” We are most certain she’s had her adenoids out and then they explain to us in some cases they can grow back after they’ve been removed. It’s not common but hey, what about Alana is at this point.

So that ties into her latest surgery as Dr. P is going to look again to see if they have indeed grown back. If they have he is going to remove them again so that’s another possible surgery that she’s going to have to ‘feel’ again.

Again Alana proves to be amazing and makes it through the surgery no problem. Dr. P talks with us and no, her adenoids have not grown back. He isn’t quite sure what they saw on the CT and he’ll take a look at it but it wasn’t her adenoids. She is taken upstairs and oh can you hear her from down the hall!! Again we used versed as a sedative before surgery but this time we did it through an IV so maybe that will make her wake up better is what we are told. Yea again that’s not happening.

So she spends the night at the hospital and we go home the next morning and she’s doing fine. She’s isn’t eating anything yet but she is sucking on popsicle's and trying to drink small amounts. At this point that’s all we can ask for.

Life yet again goes on. Alana is sick a lot more than her sisters or anybody in the house for that matter. We still search for a reason and find all sorts of things that sound like her yet are not her. She still isn’t sleeping through the night which was one of my biggest hopes with the tonsillectomy. Sleep. She’s still waking 2-3 times a night sometimes more. Her snoring has improved but she is still coughing and there are days that I have to fight myself to stay awake because of the lack of sleep she’s having.

It’s only a matter of time and she’s getting very sick…again. Bags under her eyes, coughing fits, fevers, losing weight, and yes, all the same things she’s now been doing for years. This is the same path that she has gone down before and here we go again. For months she is perpetually sick. There’s no break and by February 2007 she’s admitted again for “Pneumonia”. This was one of her worst episodes. She was so very feverish that she couldn’t sit up. This is where we first heard that she was having “ocular swelling” because she would be in such pain from light and noise’s a black room and hands over her ears was all we could do to keep her from crying.

During this stay is where we finally, yes finally, after all this time meet the local pediatric pulmonologist. Now where we are there is only one. Yes, only 1, uno, ONE, and oh did I mention, #1. So I am not going to waste a whole lot of time explaining our relationship with Dr. R. There is no point in it because it can be boiled down to just a few words. He’s always right and we are always wrong. There you go that’s all you need to know to understand how this relationship plays out.

Alana in now getting sicker everyday we are in the hospital. It’s been a week and she’s not improving at all. In fact she’s having more fevers and her O2 issues are getting worse. Dr. R tells us that if she gets sick again he will consider doing a bronchoscopy on her to see the food that he’s sure she’s aspirated. It would explain the coughing, the fevers, everything according to him. By that night she’s gotten so sick that he decides that it can’t wait and they prep her for the surgery.

That was by far the worst night in our lives at that point. She was so frail and so sick. What seems like hours past by and we see the doctor talking with one of her nurses. We brace ourselves and he comes in and tells us that he didn’t find anything specific like an inhaled piece of food but that her lungs are “sticky and swollen” with “gunk” in them. Then he tells us that her left upper lobe has collapsed. He says that that’s what more than likely happened when we saw a very prominent increase in her breathing rate and her start to really struggle to breath. If there wasn’t a wall holding me up for the first time in my life I felt what it was like to feel faint. The room spun and I almost collapsed. It was terrifying and so very emotional that it makes me upset thinking about it.

We wait for the tests to come back and she just stays where she’s at. By the start of the 2nd week in the hospital she’s having such a hard time even sitting up because she’s so weak from not eating and being so feverish. Trying to get her up and moving and using her lungs is proving to be a battle that leaves her exhausted from crying, me exhausted from making her get up then holding her while she cried and us both exhausted from the emotional stand point. There are more tests done and we are waiting on her to start and get better. We spend 13 days in the hospital this time. It’s not good and we are quite honestly, pissed off.