She's not contagious...really

Why expect more...

2010-03-17T14:13:00.003-08:00

So lets just copy and paste the beginning of the last post onto here. I was so tired yesterday that no, I didn't get a chance to post how the day went.

The hospital called on the way out the door to tell me that the scanner was down so they'd have to move it to today's fun filled agenda. She had the ultrasound of her of her diaphragm done though and it was normal so that's good news. She did fantastic with her PFT but I guess she wasn't consistent enough and it cannot be used so yea, nothing learned from that.

Today we saw Dr. Young and you know, I love her and she tries but to hear her say that there's nothing that she can think of to make things better right now is so frustrating. She is frustrated and concerned with what's going on with Alana and the fact that she's been on O2 full time for a year now and it's not making sense. Is it her lungs? Her muscles? Her heart? Her metabolic system? Anyway--we go to do the HRCT which we hope is going to show us something and yep, it cannot be done b/c Alana cannot breath how they want her too so therefore it has yet again been rescheduled. AGHHHHH!

On Friday when she has her scopes done they are going to go ahead and do that as well. We spent 6 hours at 4 appointments today and left with not much. Dr. Young just called a bit ago is still frustrated and I can hear it in her voice as much as she's telling me it. No one knows what to do with Alana and that's getting old. Very, very old. Tomorrow we meet with GI and go over those issues so who knows, maybe this won't be another why-are-we-here trip :-/

I know when we get home and all the information from her HRCT, sleep study, scopes, etc. come back we may have some answers but I have learned to not get my hopes up. They are not able to give us anymore on the mitochondrial front aside from saying she needs to go on the cocktail when we return home. sigh.

Will update tomorrow if there's anything good to share and for sure when she's okay after the procedures on Friday.

1 down...

2010-03-15T13:53:00.003-08:00

So today we had the appointment with the neurologist and he was a good guy. Okay well that's all on that front. No seriously, there was nothing learned from that appointment as of right now. They do feel she needs to be put on the mito cocktail as soon as we return for a minimum of 6 months. He also told me, as I already knew, that a lot of children do not respond to the cocktail at all. They are going to add a shyt load of labs to her list for when she's under on Friday and be in touch while we are still here. The good news is though that he was kind and did appear interested in her case.

Tomorrow she will be having the ultrasound of her diaphragm and then her HRCT. Both important to see what's going on with these lungs of hers. It's scary and all that but needed to be done like yesterday. On Wed. we meet with Dr. Young again but before that she's having her first PFT done so we will be meeting with Dr. Young with all that information so that is a good thing fo-sur.

She was wired after sleeping on the plane yesterday night and didn't fall asleep till after 1 a.m. Cincy time and was a p.i.a. to wake up this morning. She's been moody all day but what else can you expect, it's a 4 hour time difference and it shows.

I will update tomorrow night unless something goes south.

We couldn't get the spoons to stick lol..we suck!

Tomorrow, tomorrow, I love ya tomorrow...

2010-03-13T14:17:00.003-09:00

Tomorrow is the fun filled flight day. I will do regular updates while we are there, promise. Here is our appointment list so far (not in any particular order b/c I cannot remember what is where at the moment):

New visit with mitochondrial neurologist (no one is sure what he is going to eant done yet so we go in the morning with the thought that the rest of the day will be done running tests)
HRCT
Ultrasound of the diaphragm
Sleep study
PFT
Stress test
Pulmonary visit
Colonoscopy/EGD
GI visit
Labs (of course)

I think I am missing something but for the life of me cannot remember what it is at the moment. I still have a lot to do to get ready but didn't want to not post that we are indeed going and let you know a basics of what's what with appointments. I will update each night time permitting of course!

Very overdue...

2010-03-08T16:12:00.005-09:00

So yes, it's been awhile my old friend. I know with you though I can leave you for awhile, have time to figure things out, and return and you won't judge; you won't hate.

Honestly I have come here, to this page many times and started this very entry and walked away from it. For the first time it all became to much to look at. To much pain, to much uncertainty, and mostly just to much. Today I will hit the publish button and I will stop hiding from the world.

First and foremost things with Alana are at an impasse of sorts. I am going to give you a bridged version of how things have been going as it will suffice. Since December she has had "pneumonia" every month. Her fevers have been out of control and shes had a significant turn in regards to her muscle strength. She falls...a lot. Her gait has not returned to her normal-learning-to-walk-been-walking-along-time pace. When she runs her feet and legs are spaced out and she's wobbly. She has taken to falling out of the blue and looks confused about it stating that her leg just didn't work right suddenly.

It's to the point that PT & OT are doing formal evaluations again, even though they were done not 6 months ago, to see where she's at now.

Her GI system has not been playing nice well either and we've already gone through 2 Mic-Key tubes due to balloons breaking. The feeds are still hit and miss and mostly miss. She is uncomfortable and cries a lot when we do them faster than 60 mL/hour. There has been no weight gain. She is still dependent on 3 med's to do what she needs to do and it's not easy on her stomach either way.

Due to the frequent issues with her lungs now she's been at 2 liters since December, up to 3 for PT & OT in order to maintain above 90. When she goes to school for a few hours it never fails that I get a call from the nurse telling me that she's at 89-90 and they had to have her rest and do some deep breathing for awhile in order to get her to 95. What should they do?

Things here have changed dramatically and anyone who tells you their other children and their marriage are "fine" and "have no issues" with having a chronically ill sibling/child are full of shyt. Shaylin resents everything about Alana's illness. Noelani tries to fix everything about her baby sister and just wants to hold her and make it better. Alana herself today asked me why I pick on her and do mean things to her like giving her a 2nd belly button and not making her lungs not hurt. I am sorry is all I can say to her. I am so sorry baby.

No matter how long I put off this post there's nothing I can do to make this a happy or easy to read. It's not easy for me to type, it's not easy for me to think about. There's so many things that are floating in my mind and while I've let you all into our family and our struggles with Alana, Shaylin and Noelani there are somethings that even I cannot wrap my head around and are going to have to remain unsaid for the moment. In time they will come out, trust me.

Before I go 2 things:

1. This is not a pity post. I don't want pity. My family does not need pity. Sometimes things that we say and feel are not meant to be taken as woes me but more along the lines of 'they have to come out of my head so I can move past them'.

2. We are headed back to Cincinnati on Sunday. My mom is coming with Alana and I and we will be there about 8 days if things go smoothly. I will update soon on all that's going to be done and who we are seeing. It has been a pushed trip, about a month in the making, due to her change in status.

Smile. We are at the end for now.

The big talk...

2009-12-02T18:21:00.007-09:00

(simple. pure. serenity. home.)
(The view from my parent's front yard)

Serenity is not freedom from the storm, but peace amid the storm...

What a super mad-busy day we had today. We arrived at the hospital at 11 a.m. and didn't leave until 3:40 p.m. and all this so she could have 2 doctor's appointments, an x-ray, and labs. I ended up canceling PT in order to accomplish all this so that was sorta a bummer as Alana had been looking forward to getting into the water.

Tomorrow McDr will call with the results of all that hub-bub and let us know if anything needs to be looked at further. We did the usual CRP, Sed rate, CBC, etc. but also added profiles for lactate, pyruvate, glucose, and a couple of other mito specific markers (for lack of a better word). Her x-ray was iffy to me. The streaking looked better and I didn't see anything that screamed bad, but I did see a lot of hazy areas. The side angle the whole lung looks hazy, but who knows when it comes to Alana's lungs.

Last night we, Larry & I, had a meeting with McDr to make sure we are all on the same page with Alana and how she's being cared for, what the thoughts on her immediate future are, and just all those things that I don't like to think about. It wasn't much in the way of surprises. She has dramatically progressed in the not a good way manner. She has many systems involved that cannot be ignored or even put on the back burner anymore, and yada-yada. It was hard to talk about, hard to hear in black and white but much needed. McDr has some hopes for starting her on the mito cocktail but is waiting to do so until she hears back from the doc's in Cincy (because she needs to see if they want to see Alana "as is" or with a powerhouse of vitamins on board that *could* change things). She did tell us though that if we didn't advocate for Alana as much as we have and keep things as good for her as we have she'd been in a very, very bad way, more so than she already is. She did tell us that she worries each time Alana get's sick now that it's going to be the one that she doesn't recover from. Her baseline changes each time she get's sick and we can never seem to get it back to where it was before. Like I said, nothing we already didn't know or see happening but to hear them laid down like that sucks.

So here's all this talk about mito...I am sure you didn't notice at all right? I am going to try and explain this as good as I can so here goes. The full reports on the muscle biopsy came back a bit ago and it showed things that are consistent with mitochondrial disease, somewhat. Dr. H in Seattle wanted to run another round of testing and 1 thing came back better, 2 came back the same as the original testing. So clear-cut answer? Of freakin' course not. So again, where does that leave things? It leaves them like everything else...confusing. So what we are doing now is that Alana is participating in a mitochondrial testing study that's going on at Seattle Childrens. I mentioned that Dr. H asked permission to include her when we were down there, but now she's in it to win it. The testing is looking for unknown (as of now) variants of mito in children who test borderline or atypical with traditional methods but clearly present with mito. Sooooo--based on all this, from what we know now it appears that Alana has an unknown variant of mitochondrial dysfunction. No one yet is willing to make that "official" call on her paperwork because once it's there, it is THERE but she is now being treated as such. I know that it's hard to understand how it can be and not be at the same time, but I think it boils down to this: Alana presents like a typical mitochondrial patient. She has progressed at an alarming rate over the last 2 years and is continuing to progress. She has multiple systems involved and new systems joining the party at a frightening clip. Nothing else has ever fit her like this does.

Larry and I had started to come to terms with this when the first round of testing came back a few months ago. When the rest started to come back and more talking was done and more thinking back to the start of this and how things have gone we started to come to a scary realization that this might be it. Now we are waiting for them to do what they always do--tell us it's not it. That sadly has not happened yet.

I think that's enough to digest for tonight. As always there's been more fun around here then a barrel of monkeys could provide but I'll give you a hint..Alana was chewing on her O2 tubing, it was yanked on by accident and because her teeth are weak and have no enamel on them to keep them OK, a tooth came out with said cord. Talked to the dentist and from her x-ray a month or so back dentist said, yep, that tooth had another good year before it was suppose to come out but it should be fine, she will be toothless there for an extra long time now.

Weeeeee!! Can I get off this ride now?

PS: On Friday Alana goes in for a change from this long tube to a Mic-Key button and also a brain MRI (don't ask). Will post after she's in.

(she's so a pissy teenager sometimes)

So done with this verse...

2009-11-20T16:36:00.003-09:00

Well the good news, the okay news, or the bad news?

Let's start with the okay news; the meeting to slap the PIA woman we were dealing with went well. Not great, not bad, just well. She will at least (so she says) respect my place as Alana's mother and NOT give me any more shyt about the fact that we do not have a clear-cut diagnosis for Alana (in regards to the overall picture). McDr has already told this woman that that does not negate a single thing that is going on with Alana. We ended up coming to an agreement that:

1. I do, not only legally, but on the because-I'm-her-freaking-mother, have full access to any information (that's done on paper) about Alana and she cannot deny me that.

2. She will no longer question us if we say something. Alana's care is not up for debate! Either take care of her of step the hell outta the way so someone else can.

3. The mediator that we had help us with this, Larry and I, and the "woman", will now be having biweekly meetings, only 20 minutes or so, to make sure we are all stil on the same page.

So like I said, it was okay.

On to the good news. McDr has been talking with Dr. Y (Cincy) and they'd like us to come back and have another round with the whole team. We've been aware that we were going to need to do this soon anyway but now that it's in the works it's kinda scary. How can that be good news? Well Cincy strongly believes in a team approach to care and even though we have McDr who, in my opinion is the greatest thing since sliced bread, having a pulmonologist, neurologist, gastro, immunologist, etc. all working together as 1 cohesive unit is great. McDr will be the leader of this band so that is even better. Dr. D that we work with here will still be our right hand man so that's icing! I don't know when we are going to go, I've requested not until March-ish so will let you know when I know.

Alright so I have some shocking news to share with you...I know it's going to be a huge surprise and all so here goes...remember how I told you she was getting sick?

Ear infection and pneumonia. Her g-tube is completely irritated again and we are back to caking medicine on it. She's gained NO weight in the 3 months that she's had the tube itself and we have to go back on Wed. to see if we can head to my parents house for Thanksgiving. There's no use crying over spilled milk right? It seems that this fall has been hard on even children with no underlying issues but really?! Didn't we just sing this song and dance a month ago?!?

It's a reindeer mama...

2009-11-18T19:39:00.005-09:00

Okay so not to be a whiner here, but I think she's getting an ear infection. Argh! She came up to me a bit ago saying that her ear was hurting her and like I said, shes been off anyway. Good thing McDr called today and we talked for awhile and she said (before any mention of ear pain) that she wanted to see her and get some labs done that Dr. Hahn (Seattle) wanted so on Friday we will go ahead and go in.

We've been dealing with a lot of peeling piggys :/ It's happened to her since birth but it still hurts to the point that she won't walk on her feet. We've tried OTC remedy's, 'script ones, etc. and nothing helps until they've completely skinned themselves and start to regrow. This usually coincides with other strange things like her need to have a lot of sugar to get moving. I am not talking about candy but more along the lines of woke up screaming>Larry and I restraining her>forcing her mouth open>dumping juice into it and then from there we do it again until she swallows it and it takes about 60 seconds for her to calm down and realize that she's alright. Then she will guzzle the juice down and be a completely different child than she was 10 minutes ago. ???? Anyone care to pipe up with an answer 'cause we've got none!

Both my brother and my mother are really sick with some wicked stomach virus crap that is going around and I have banned them from my house. My mom lives 90 minutes away so that's not a big deal and my brother lives 45 minutes away but still, unless they are in Hazmat suits they are banned until further notice. Larry thinks I am crazy...I happen to disagree, I think I am perfectly sane.

Tomorrow morning we are going to go and lay a smack down on some woman who thinks she knows our daughter better then we do and even better than McDr. This has been an ongoing battle that we've been dealing with and the proverbial straw was broken today and I am not sure what she thinks she is doing. McDr is frustrated with this person and I am thinking how much jail time I'd have to spend if I just did the V8 commercial to her forehead along with a heaping of "here's your sign" but again...Larry thinks I am crazy. If you've ever had a child or loved one with any sort of malfunctioning system then you know what I am talking about in this lady--she thinks that things are ALWAYS by the book and things ALWAYS follow a set path. She clearly is not a member of the "A" team.

Moving on...

I was taking some pictures of what it means to live in Alaska and some of Alana outside (in 0 weather. And yes, schools here go outside until it's -10..m i n u s 10) and as we go to leave when what to my wandering eyes should appear, a moose making a traffic jam with nary a care. (OOO I can totally quit my day job and be poet) so we got some nice shots of a pretty typical day around these parts, wildlife and all.

You all in the "lower 48" remember my frozen toes and cold fingers at -3 (current temp) when you are enjoying your +60s and 70s!!

1, 2, skip a few...months that is...

2009-11-11T15:16:00.007-09:00

It does really feel like that to me at least. This month, well actually last month, zoomed by at super speed. Things are holding steady here though. We had a wonderful birthday party, and yes, I was surprised with some gifts of my own as well! We had fun making our pumpkins this year although Noelani was being grouchy and wouldn't let me get any photos of her (I did sneak some but won't post them so she doesn't have a tissy-fit).

Alana's party was rocking and she was a skeleton-bat-girl and she was amazing. She did great overall and Noelani and Shaylin looked wonderful! Shaylin was a "glamor" witch and Noelani was a nerd. The funny thing with Alana was that she was actually an x-ray, not a skeleton. Go figure, my radioactive girl wanted to be an x-ray!

Halloween was a bit of a bust as Alana didn't feel well and then as we go to leave Shaylin threw up on the front porch...wonder-freaking-full lol. It was such a comedy of errors that I was ready to just go and hide under the bed myself. But being the fantastic parents that we were we went ahead and took them for a quick 30 minute trip to get some candy and at least try and have fun. It worked and they did have fun so all was right in the world of candy land. For Shaylin it seemed to be something she ate because she was fine later on that night and from then on. Alana has been in a perpetual state of unrest lately. Not bad but not good either. Confusing is more like it but again, if that wasn't the case around here then things would really be messed up right!

She is adjusting to her feeds, slowly. McDr cranked her down even more, she's now at 78 mL/hour for 2 hours. Oh and she is also on a pump now in order to do that. We had been pushing her 4 ounces via a bolus feed but she was having so much pain with that we had to come up with a better plan. We then tried 118 mL/hour and that still was hurting her so here we are. She's uncomfortable to some degree but it's better so we are going to try and just hand onto this and see if she can work through it.

We did also have her 6 year check up and even though we knew that she was FTT (failure to thrive) and was struggling with her height as well but here's the break down: On November 4 2008 she was 45.4 pounds and 42 inches tall. On November 3 2009 she was 43.8 pounds and 43 inches tall. Now if she was a 18 year old that might not be a cause for concern but being 5-6 it's a huge bummer. It's all good though, this is why she has a feeding tube now--we've just got to figure out her GI system and we should be golden!

There's been more going on, isn't there always, but for tonight I am going to wrap it up. On I will go over the rest of her appointment and what's been going on at PT & OT (increase hypotonia) and what we are doing in preparation for her care conference and what the deal-eo with the mitochondrial testing is. No I didn't forget to tell you about it, I've just been keeping it close for right now until we have some information we've been waiting on. I am getting sidetracked though so let me stop while I am ahead!

I'd also like to thank Rattles for her kind words and warm heart. She has honored Alana's site with an award and I wanted to sincerely thank her for that. She herself is an amazing young woman struggling with what life has challenged her with and she's doing this all with grace and laughter.

It's been so long...

2009-11-10T21:42:00.002-09:00

Okay--tried to upload the post and the pictures tonight but blogger is being a pain in the arse so I will upload tomorrow! *grumble*

Buckle your seat belts...

2009-10-22T16:56:00.007-08:00

'cause this is going to be a long one. So yes, we are alive around here, crazy, but alive none the less. Noelani is fine and by all accounts so far our house has been spared anymore outbreaks of H1N1.

We have had a LOT going on, here's the synopsis before I go into details; Noelani turned 14, Alana had a delayed gastric emptying scan, Alana's new 'chair' came in, Alana's new chair was not put together properly so she almost went out of it head-over-feet, Alana's tube stopped working and started causing a lot of pain, Alana started walking weird, Alana's autonomic system has decided it no longer wants to play nice, Alana had a g-tube barium study done, Alana asks me "Why do I sleep more than anyone mommy?" and the most important, most amazing thing is happening next week...Alana turns 6.

Did I mention that Noelani turned 14?!? Wow...how'd that happen? Here in Alaska that means she is old enough to drive now (with an adult) and she is studying to get her permit. She is so smart, beautiful and caring that it shocks me how all those things can fit into one package. She had a wonderful birthday and I think she got everything that she wanted, which wasn't even much. We had a family party and it was great to have everyone over to share with. She is such a young woman now that it makes me sad, yes sad, to watch her grow up so fast. I am also happy and awed by the fact that she is such a young woman.

*sigh*

Moving on to Alana...We had to do the DGE scan to see if that was causing the back-up of her tube. We've still been unable to get more than 4-5 ounces into it and that's taking awhile all in itself. It started with it just backing up and not moving anymore down the gravity feed, then it moved into pain and screaming with her asking/begging us to stop putting things into it. The thought was that she may have delayed emptying of her stomach and by us pushing things into it it was causing her stomach to be over inflated, i.e. stuffed. I've not gotten back the official results of it yet, just that our doc sent it out to be read by the GI and he has yet to respond to it. Go figure.

Super-duper cool is that fact that her chair came in. Not so super-duper cool is that the DME didn't put the straps on correctly and when she fell asleep in it the first time she tipped over (like she does all the time due to the hypotonia) and if little miss Shaylin wasn't paying attention she would have landed on her head in the store. I had questioned the straps when we picked it up and was told that it was suppose to be that way and once she was in it fully and all the buckles tightened down she'd be fine. Again, lesson learned...ppl who deal with medical things all day long can be the dumbest ppl to ask medical questions. As I am walking out of the store, trying to get all the pieces of the straps off the ground ('cause the are now completely falling apart as she's thrashing b/c we had to wake her up) I am telling the receptionist at the DME "I DON'T CARE THAT _____ IS BUSY, PUT HER ON THE PHONE NOW!!" and then calmly try to explain to her that the straps are NOT alright and that they are going to fix them first thing in the morning.

Whew. When I get home I am telling Larry this and he brings it inside and we start looking through the owners manual and going online trying to find pictures of how the straps are suppose to look. Low and behold they are not on right, you are shocked I am sure, and it takes us 2 hours and pulling apart the chassis and fixing all the straps. Woudln't you know it once they were on right she was golden! She could no longer get out and was comfortable. Now that that is fixed though it is all that we'd hoped it would be. It fits her great, she loves the tilt feature and her feet are braced in a comfortable position. It weighs a bit but it is so worth it! She picked out the colors and says it reminds her of spring so all is good.

Moving onto the next few issues all in one ball o'wax. We kept trying to push fluids through her g-tube and then we no longer could. We'd get 1/2 her meds in and then she'd be doubled over crying in pain and begging us to not touch it again. McDr is seeing her almost every other day at this point b/c we are still working on clearing the pneumonia and sinus infection and she tries to palpate her stomach and that brings on an autonomic bump with flushing, eye drooping, temp. dysregulation, etc. and she, McDr, decides that she will call Dr. Dipstick and ask him what we should be doing. Dr. Dipstick agrees that this is not right at all and now wants to check the placement of the tube, the tube itself, and her stomach in general again.

Today Alana had the g-tube barium study done and there's good news and not so good news to report there. The good news is that it is not the tube itself and it doesn't appear to be her stomach either. The not so good news is that after they were done with pushing the barium in (20 mL) they wanted to flush it with some water and the fun began. By this point they had turned off the machine and were all done, nurse was just pushing the water and I was getting my stuff done while telling them that 20 mL is usually fine, it's once you get to the 3-4 ounce that it starts to be a problem. As I am telling her this she is still slowly pushing the water and I notice that Alana is no longer talking but withering on the table and flushed. Now she starts to moan and say it hurts please stop. The nurse turns back on the machine and takes another look and whatever it "is" appears to be something to do with the small intestine. We couldn't do anymore b/c there was no order for a "small bowel follow through" so they took what they could picture wise and instructed me to call our doc and have them write an order for that, the small bowel thing, so we can see what about her small intestines is causing her pain. Round and round the mulberry bush the monkey chased the weasel...

Wrapping up here; pt has been hard. She had to stop 25 minutes early today and as the PT said, she was done 10 minutes into the session. We are hoping it's because her core is so out of whack but she is walking very odd. Alana's gait has never been a big noticeable problem, sure she falls down a lot and runs into things but it's mostly due to her proprioceptive problems vs. her gait. Well now she is having major gait issues. It's hard to describe but she's throwing her feet out, left, right, left, right, and walking like she's trying to completely flatten her foot down without using her heel. She is so incredibly fatigued that even after taking a 3 hour nap after pt she is yawning beside me and it's only 5:30.

I wish I could answer the question that she asked me...how I wish I could not only answer it, but make it better and not so. In the meantime though I am going to celebrate and grant her her wish that she be a princess bat for her birthday and we are going to play games and have pumpkin cupcakes and laugh and sing and be ever so grateful that Alana will be 6 in Tuesday.

P.S. If you saw "Alaska State Troopers" on NatGeo you have seen my dad!

The promised update...

2009-10-03T17:25:00.003-08:00

See, I didn't forget to update =) I can report that I am working my way back from my trip to the land of pity and woe's-me. That's a vacation I have been taking a lot lately and I am not sure why. I know that sounds dumb, the I am not sure why part, but still that's no reason to be getting all helpless and crap. But enough about me, moving on to "pumpkin" (her new official name is "pumpkin" and she will only answer to it, or so we've been told).

We are on plan b and she's on 2 antibiotics now. We didn't see any improvement by Friday so that's that. The good news though, she's not much worse; right now she's more pale and drawn then she is coughing. She's been pretty low-key for the last few days, which is to be expected I suppose. She's been having short, 5-10 minutes, bursts of energy every few hours and is up and wanting to play a board game or color.

She did have OT this morning (OT comes to the house) and they worked on massage and getting her to breathe better. I don't know if I have ever mentioned it but Alana does not breathe properly. Big surprise there I know, but combine inefficient breathing patterns and lung disease and on a regular day that leads to issues, throw in some pneumonia and you've got yourself a tricky situation. It was very relaxing for her I think, not to much movement aside from switching from back to belly, and a lot of massaging of her back and rib cage to engage the diaphragm (Alana 95% of the time does not use her diaphragm to breathe).

So overall we are home so that's the great news. I'd love to report that she's all better or at least on the mend but she's not there yet. Tomorrow is another day though and as long as she's not getting high fevers, coughing worse, or having overall anymore difficulties, then we can continue to remain at home. We are still not getting formula into her g-tube and I have only been able to get around 3-4 ounces of water into her plus her meds. It's at least 3-4 ounces more than she'd be getting if left to her own devices though.

I'll post again soon, sooner if anything changes either way. Thanks again, and again for all of your prayers, words of encouragement, and support; it truly means more than you will ever know.

Pack your bags...

2009-09-30T19:15:00.000-08:00

Sorry there won't be any pictures with tonight's posting. To be honest I wasn't even sure I was going to post anything at all. It's funny in a morbid way though, I have not been posting much on purpose and then the reason why I haven't been posting was posted in another blog, Eithene's, a little bit ago...If you have nothing nice to say don't say anything at all. It's something my mother used to tell me all the time. And yes, I mean all the time 'cause lord knows I had a mouth on me! It seems that there is a universal feeling of helplessness going around right now.

You know I like to mix up the good vs. bad news and which one I deliver first so here goes the good news tonight, the one that makes me breathe a sigh of relief, Noelani is recovered =) She went back to school on Friday and aside from a small cough she's golden. It was a very tough 4 days with the fevers but she pulled through it and no one *knock on wood* has shown any signs of contracting H1N1.

This is going to be short and sour. We are still having many problems with Alana's g-tube not working. We are only getting in about 4 ounces of water and her meds. On top of that she started coughing really bad over the weekend and was generally not feeling well. We tried to come up with a better plan on the g-tube and keeping her hydrated but nothing seems to be working. Today she needed to go into see McDr (on McDr's day off none the less) and the hope was that it was a small viral infection, the catch term of the decade, possibly in her sinuses. While this didn't account for why she was needing her O2 turned up it was a start. From there it was decided to do a sinus, chest, and abdominal x-ray.

I don't have a lot to say at this point for a few reasons, 1 is stated above, 2 my head hurts, and 3...please see #1 again.

Alana has a sinus infection, ear infection, walking pneumonia, and here's the kicker...her GI system is STILL completely backed up.

I promise to post more in the next day or so. I know that I left some of you hanging but I just am having a hard time dealing with these issues. I feel like I am failing her. Now see, this is where I have to stop because the self-doubt and pity party will get out of control and there's no helping it.

Till next time

If it walks like a piggy and oinks like a piggy...

2009-09-19T19:37:00.003-08:00

Last night around 8 p.m. I had to take Noelani into the ER b/c she had a fever of 104.2, could barely swallow, coughing, body aches, etc. After we made it into the quarantine room and she was swabbed it confirmed what we feared and what the doctor told me he thought it was...H1N1.

We made it home around 11 p.m. and shes was miserable even with the med's on board. It was a very, very long night between her and Alana so any hopes of getting sleep are gone. Bye-bye sleep, it was nice to know you!

Thank god for my moms friends; 1 brought dinner over and the other brought over bread, milk, eggs, and speggati O's so neither Larry nor I had to leave them. They even risked contamination by hugging us lol.

*sigh* don't expect much from me for the next few days though, it's the first time I've been by the computer for the most part today and I don't think it's going to get much better for awhile.

Hey...guess what...

2009-09-18T13:51:00.001-08:00

Just wanted to do a quick update...

WE ARE H-O-M-E NOW!

She's able to be managed at home now so here we be. She's still in a lot of pain BUT her BP is better, still on the high side but better, and same with her pulse. Lungs still have sticky-to-themselves issues but she is able to hold her sats up just fine. We are trying the thing we knew was going to come, managed care. There's no way around it anymore and even though it's hard to accept, this is how it needs to be, we can "manage" her care at home now.

We have to check in on Monday or sooner if anything goes south. Tonight we will do a feed, we've been giving her stomach time to rest & reduce some of the swelling and pain, so hopefully it will go smoothly. We are only going to do 60 mL so not to much at all. Later tonight or tomorrow morning I will post some new pictures so until them...

Nothing much to say...

2009-09-17T17:32:00.003-08:00

Maybe tomorrow...

No new news to report really. Stopped the g-tube medication b/c her belly was so swollen and distended that McDr could barely touch it. Lungs are holding their own still (great news) now we just need the GI system to fall in suite. Her BP is still high but getting better so McDr said to look at the trend and that's what's more important; the fact that it's going down. Also her pulse is following this trend as well.

The x-ray still showed a good amount of stool and I am waiting on the GI gal to come in and talk about it. I know tonight we are going back to seni. & m.o.m. so we will see if those will help get her to a good place.

Again, maybe tomorrow...

Not tonight honey...

2009-09-16T19:53:00.003-08:00

So we are still in la-la land. In the spirit of keeping this short and sweet tonight here's the short version:

1. The labs look good, in fact her B12 is on the tippy top of the high side so check one thing off the list. Her CRP was 1 point away from the high range, but again, not to uncommon with Alana so we are just leaving that at that. Check.

2. The chest x-ray was unchanged so that's both a good and bad thing. Good 'cause it's not worse, bad because she still has sticky-to-themselves lungs. The thing that trumps all of this and makes it a positive is that she's been able to keep her sats up and aside from a lot of coughing and a scratchy voice I think she's able to hold her own in that area--for now. Check, Check.

3. Her GI system refuses to cooperate. It is moving things, but not like it should. We are on the 2nd jug of Golytely, almost half way through it in fact. It's causing her to be swollen and uncomfortable and we keep having to turn it down, turn it up, turn it down, turn it up due to all of the issues it's causing. Not checked.

So, here I was thinking that 1 thing would not keep us here but gee, so much for that. It's also a little two-fold to be honest, her blood pressure has been high again all day today; 136/76. Her BP is usually a lot lower, even with all the things we deal with with Alana, high BP is not one of those.

What's left to do is finish cleaning out her GI system, have an abdominal x-ray, do a regular feed, and keep her BP within normal limits. Here's to tomorrow...

PS: Usually don't ask for things like this but there is another little girl, her name is Eithene, and her mom and I have talked about how she and Alana have a lot of similar issues, 1 main one being undiagnosed (for the most part) with the added bonus of "probable this, and probable that". Anyway--she's got a wicked infection and it really needs to get under control. I know that there are a lot of people out there praying for Alana so maybe while you are doing that you might send a few to Eithene in Mass.

Another day...

2009-09-16T06:47:00.004-08:00

This is what she said when she woke up this morning; "Mommy, are we still in the hospital?", "Yes chicken-butt we are still here". The morning started early around here, like 1 a.m. early. She was desatting again last night and was 2.75 ltrs for her O2 needs and then that gave away to a 4 a.m. wake up call for the bathroom. The medication finally started to work and it took about an hour to get things done and her back to bed. From there McDr came in at 7 a.m. so at this point I gave up on sleeping any longer.

As of this morning not only did her right lower lung sound worse, well actually no sounds, but the left lower lung as well! This prompted the outlook of another chest x-ray (the girl is radio-active I swear), labs to check her CBC, electrolytes, CRP, etc. and if we can get her to pee a clean catch. McDr feels that not only are we dealing with the surgery and her lungs but we may be dealing with something viral on top of it all. Tomorrow morning we will get all the goodies from these tests but the good news is, drum roll please, that she's been down to 1 ltr since noon and has been able to stay about 92% for her saturation's! It's not great, and her RR is at 37 right now as I type this, but it's a start in the right direction.

Tomorrow she will probably have yet another x-ray, this time of her abdomen to check and see how the clean out goes. After 40 hours we are starting to see good results and good lord would I love to be using her g-tube for FOOD and not laxative. She's barley drinking, and eating is very minimal. I want to put this tube to use for the reason she has it!

So our goals for tomorrow/go home are:
1. Lungs need to sound stable enough to go home, i.e. no more 2 ltr desats, good movement, better x-ray.
2. GI system needs to be sparkely clean and ready to accept her g-tube feeds.
3. Labs need to be good with no signs of infection/electrolyte imbalance, etc.

Now a lot of these things can and are open to overall wellness so we will go from there. If 2 out of 3 are good then we go, if 1 out of 3 are good then we stay.

I am beyond tired, hence the reason for no update yesterday as I fell asleep before I had a chance to. I am feeling positive that she is on the mend, at least for now, so let's just keep that going in the right direction. We know that things with Alana are never going to be the same as they were even a year ago and this has been and is hard to accept. I think one thing this admission has taught me is nothing with her is ever going to be easy-peasy-japanasy but that's what makes her so special!

*Wrote this last night but the server was having issues at the hospital, I will update again later when all the answers come back for these things*

And the beat goes on and on...

2009-09-13T21:42:00.003-08:00

So we are still in la-la land, aka Childrens, but that's alright. First the pain is much,much better. She's still guarding and very uncomfortable if the tube is touched, but she is moving around now and wanting to get up and move about the room. Today she managed to walk down the hallway to the art room and paint a wooden puppy but it certainly took a lot out of her. That's when her fever started to climb again as well as what we are dealing with now, high blood pressure. She then promptly fell asleep and while she was sleeping her face swelled up, turned bright red, had the pallor around her mouth and nose, and her whole body felt uncomfortable. Her site is healing nicely but she is incredibly distended from lack of "moving things along" so that's adding to her pain/uncomfortableness/swollen belly. Tomorrow we will be discussing what to do about this b/c she's 'gained' almost 2 pounds due to blockage. Just freaking peachy.

As for this morning her cough was worse again so they ordered another x-ray to make sure things are going in the right direction. At first the doc on call thought (when I told him) that she was coughing a lot more that it was a good thing, that she was opening that section of lung that is stuck together, as he was leaving the room she had another coughing fit (he had not heard the cough) and walked right back over, listened to her, saw her gasping for air (with O2 on) and then said "What is that?!". At this point I just shrugged because if he had been listening to me he'd have known THAT is what she had been doing all night. Needless to say he said maybe you can leave tomorrow but in the meantime we need another chest x-ray. It's still not back yet :/ so dunno what the deal is.

Her heart rate decided last night that it had had enough of being a speed demon and then took the pace of a Moped and we had a fun filled night of alarms from that! The nurse manually counted out her respiratory rate and it was in fact 10...this from the kid who lungs like to march to their own beat therefore she rolls at about 25-45. Needless to say it was yet again, another long night.

All in all though things seem to be on the mend. I am not sure what to make of her BP, RR, HR, fever, or facial swelling but at least I am not the only idiot in the club right now. We have a bunch of doctors right there with us!

PS: Remember those old TLC commercials about Merlot and Email not mixing? Well last night, yesterday nights post could be the same token...stress from a long day/night and Blogging might not mix! Sorry for the dour mood of it all, this "stuff" has just taken a bit of a toll this time.

And the beat goes on...

2009-09-12T21:32:00.002-08:00

So I know I was going to tell you all the things that were messed up yesterday but if I do that then I won't get to the update. On that note--here's the update.

Last night was very difficult and frustrating. She was running a fever most of the night and struggling to get any decent breaths. She did sleep finally and that helped enormously. This morning she was unwilling to get up and was very sore so we just took it slow all the way around. By noon she was drinking apple juice and was able to sit up on her own. We then set out to walk about 2 minutes down the hall and oh boy that was fun. She was so sore and unsteady that it was upsetting to see her even try. She did try, and she did make it, and she was okay.

The rest of the day was a lot of the same, tears, smiles, sitting up, sitting down, etc. By 5 p.m. she was able to eat some apple sauce and then she worked up to some mashed potatoes and that was enough for her. Tomorrow it sounds like we are going to try and start a feed through her new tube and see how it goes. I know the cleaning of it and rotation of it 2x today was torture. This had been by far the most painful thing she's ever had done. Yes, worse then her lung biopsy and chest tube.

Moving on though. The fevers have been waxing and waning today and we are in the waxing part right now. Yesterday was a lot of the high respiratory rate as well as the high heart rate, well tonight it is the opposite. Both those rates, RR & HR are scary low tonight. They've already called the doctor and there's really nothing we can do about it at this point besides keep a close eye on her. She is sleeping, soundly at that, so that's good news.

I don't know what tomorrow will bring, going home? Staying? Feeding? It all depends on if they think she can be managed at home I suppose. It's something that brings me no joy, no sense of security, that we are to the point that her set of kooky medical things are things that are okay to go home with. Things that no other "typical" child would be allowed to go home dealing with are things that we must. I know it's late, and I know that I should be in bed instead of typing this and having yet again another weak moment but it just is. It just really sucks.

Tomorrow will be better than today and tomorrow will also mean she's another day healed.

Post surgery...

2009-09-11T22:45:00.000-08:00

Sorry I didn't update earlier today but it's not been a good day around here. I am going to keep this one short, sweet and to the point and will give a more detailed account tomorrow.

The surgery went okay, not great, just okay. First they had to place the tube completely different than where they'd shown us and marked it. :( It's way closer to her ribs and completely mid-line but what can you do.

During the procedure she bled more then she should have from the site, enough that they drew labs to check clotting time but I haven't received word on those yet.

When she was brought up to her room (and we got to see her for the first time) she was taped up (IV, GTube, etc.) in BOTH tapes she's allergic too. After how things had already gone that morning, again more on that later, I was steaming and ended up getting the shift supervisor involved.

Within a few hours she was screaming in pain and then we started to have a lot of lung issues and for me to say Alana was having lung issues means that it was bad. We now know that she had a small section of her lungs get stuck together/co

llapse onto itself, therefore she's just shy of 3 ltrs of O2, not getting above 95, RR over 40-50, and miserable. After the stat x-ray this all came to light why she was having such issues so I just don't know. They hope by tomorrow she will be able to reinflate that area if we can get her pain under control and get her up and moving.

They've started bolus IV fluids b/c she's getting dehydrated so that's a whole nother round of fun.

She also has a fever close to 102 so we are just riding this all out and hoping it's just Alana being Alana and it's going to get better tomorrow.

I will update more tomorrow but for right now I am beyond tired/angry/scared/sick of hearing that she's a complex child and that some of these things are expected.

It's all about the hair...

2009-09-01T19:13:00.008-08:00

So we made it through the first week of school for Alana. Aside from the face + foot = smooching, the daily trips to the nurses office due to very cautious school staff (I am NOT complaining-better safe then sorry!), tears due to music class (just way to much noise and moving around), I think she's made it through the week relatively unscathed. We had a lot of carry over this weekend though, fevers, a major, major melt-down when OT was trying to get her to cooperate on Saturday morning, and a significant lack of eating and drinking.

Although none of this was expected it has still been a rough few days but hey, we go ahead and decide that because she showed some signs of perking up on Sunday night we'd go ahead and skip out on Monday and take the girls to the state fair. We are brainiacs I tell ya! There are those windows of insight that you only get AFTER you have done something pure genius and can sit back smugly and look out that window and gloat about how you tricked the man, how you pulled a fast one. This was not one of those times. *grin* IT did go well, the girls DID immensely enjoy it (well they did until Shaylin discovered that they toy that you get after paying $5 at a game is a P.O.S and breaks on the way home), and Alana has paid for it all day today. I am still glad that we went, and even though I am still going to be going to bed early tonight to try and recover some lost sleep from Alana's restless night, it was worth it.

On the medical front not a lot has changed. She wasn't able to go to school today because like I said, she's having a rough time since last night and the fever finally has started to ebb so that's great news. She did have to go in for another x-ray this morning and an impromptu meeting with the GI nurse at noon so that killed another 2 hours of the day right there. Unfortunately those things that I've been doing to try and help her in that area are not working and she's been experiencing a lot of pain in her stomach and back area. The x-ray showed that she hasn't made much progress, not much at all :-/ so we are now going to be "hitting it from the top", i.e. something really nasty that I've been told fruit punch loaded with sugar will help hide the taste of. Great. Now she's going to be hurting, mad at me for making her drink something nasty, and running around the house like she's on crack. Just great.

We check back in on Tuesday with GI and then again of course on Thursday (the 10th) for pre-op. Taking this picture of her this weekend really reminded me why we are doing this. The whole thing is making my head hurt so moving on. Backtracking though, Thursday (the 10th) is the big neurologist appointment so just pray. Just pray that even if it's not good new's it's an answer that we can move forward (?) with.

Here's a then and now for you as well--this was last year at the fair, within days of each other exactly 1 year ago. So many things change, not so many things change.

Yes, that is the same jacket that she wore last year. Normally not a big deal, Noelani is wearing the same winter jacket although Shaylin is not. I'd have hoped though from 4.10 to 5.10 (years.months) she would have outgrown something!

The big day...

2009-08-25T18:54:00.004-08:00

Noelani and Shaylin started school last week on Tuesday and have LOVED every minute of it. Noelani is thriving as an 8th grader and making even more friends then she already has, keeping me confused to death Algebra I and French B, and coming home with funny stories about her wacky teachers every day. She was frustrated that even on the first day there was homework and it's not let up at all! Now that's funny stuff!

Shaylin has let her teacher know that she cannot have homework because "I have to play with my sister, eat dinner, watch America's got Talent, take a shower, and read a book each night so homework won't fit anywhere" Wow. Talk about having a way with words! Thank goodness she has a fantastic teacher, Noelani's 4th grade in fact, and knows that Shaylin is one of those kids that really speaks what's on her mind and then sorts through the consequences. The teacher then explained that not having homework wasn't an option but she'd keep that in mind for future reference. Shaylin so rocks!

So the first day of school went off without any major hitches, thank god! So starting from the top she woke up in a fantastic mood! I am talking a big giant smile on her face from the moment she opened her eyes and asked me if I remembered what today was. I played along and told her no, I didn't. She then jumps up on the bed and yells it's the first day of "kindy-garden" and she get's to go! It was wonderful to see her so excited and happy over something that most children would groan about.

Once we got to the school we did a quick stop by the nurses office to drop off tanks and do a quick rundown on switching bags in case she wanted to tote her tank behind her instead of carry it (she won't take off the lil'bug now that she's had a chance to get it on). Once we were done with that we headed down the hall to her big girl classroom and she had a few trepidations about entering but got over it quickly and did her thing. She put up her lunch box, her backpack, her jacket, and moved her name over to the "in" box. She got to pick where she wanted to sit and sat next to another little girl and started drawing a picture and just kept holding onto our hands. We stayed for awhile, 15 minutes or so, then got ready to go while they were moving over to the reading corner. She asked us to stay a few more minutes and we did, but left within 5 minutes as she settled in and started smiling!

When I went to pick her up from school at 12:15 I stopped at the nurses office first and before I even got all the way in the TA for her class saw me and said "Oh did you already see her? That's a good fat lip she's got!"

----groan-----

The nurse leads right into that and tells me that Alana was around the monkey bars and somehow ended up saying hello to someones foot. *ouch* She cracked her lip open pretty good, has a raspberry on her chin, and a fat lip. I am happy though that she had an aide keeping an eye on her and saw it happen and was able to take her into the nurse right away because she apparently, and would good reason, was sobbing and heaving. The nurse also told me that the SPED teacher brought her down about mid-morning to have her vitals checked and have the nurse take a look at her because she was very quite, pale, tired, and not responding like she had been 10 minutes ago & it worried them. I of course had told them that she does this, e-v-e-r-y-d-a-y & frequently, but it still didn't prepare them for what a change it is in her. After the nurse checked her SPO2, her HR, RR, blood pressure, and temp. (all within Alana range) they took her back to class but the SPED teacher stayed with her on a beanbag and just talked to her about dogs and cats and rainbows to keep her relaxed but involved with the class. She recovered enough to be able to work on another project and participate with the class during lunch and then the now infamous first kindergarten casualty so that was good. When I got there right after recess she was done, capital D O N E, done. She had an icepack on her lip, was curled up in the book corner listening to the teacher read and asking to go home.

Want to hear the best part though? I am talking the icing on the cake, the Pièce de résistance...as she was falling asleep in the car on the way home she said something that made every doubt I now had swimming around in my head about her going to school dissipate in an instant:

"it was awesome mommy, it was awesome."

The good, the bad, and the fugly...

2009-08-24T21:12:00.004-08:00

What a day today has been! There's been some good, there's been some okay, and then there's been some bad. When the day makes you question if you are really here or on some bad alien game show--that should be telling you something. Like go home and get under the covers.

This morning I met with the school to get the process going to have Alana evaluated for an IEP instead of just going with a heavy 504. For those lucky enough to not know what those things are here's a quick breakdown:

IEP: Individualized Education Program (alternatively called an "Individualized Education Plan," "Individual Education Plan," or some combination thereof). This is a legally binding document that spells out exactly what special education services your child will receive and why. The individualized part of IEP means that the plan has to be tailored specifically to your child's special needs -- not to the needs of the teacher, or the school, or the district. Goals, modifications, accommodations, personnel, placement, all should be selected, enforced and maintained with the particular needs of your child in mind. "We don't do that," for example, is not an individualized response. If your school has never had a child like yours (and since your child is an individual, they haven't), and now they do, and a service is appropriate to his or her needs, then they do do that now.

504: The "504" in "504 plan" refers to Section 504 of the Rehabilitation Act and the Americans with Disabilities Act, which specifies that no one with a disability can be excluded from participating in federally funded programs or activities, including elementary, secondary or postsecondary schooling. "Disability" in this context refers to a "physical or mental impairment which substantially limits one or more major life activities." This can include physical impairments; illnesses or injuries; communicable diseases; chronic conditions like asthma, allergies and diabetes; and learning problems. A 504 plan spells out the modifications and accommodations that will be needed for these students to have an opportunity perform at the same level as their peers, and might include such things as wheelchair ramps, blood sugar monitoring, an extra set of textbooks, a peanut-free lunch environment, home instruction, or a tape.

So now that you are "in the know" you can join the adults at the adult table! So just from that you can see why a IEP would be a better fit then a 504, therefore something we'd like to see if she needs/qualifies for. Anyway--that meeting went well, long, but well. My only concern is that she has to go through this testing procedure and it can take up to 45 school days to complete, ergo, up to 45 days that she's not getting aides, recess help, etc. The school did say that they will unofficially watch her and have one of the SPED TA's keep an eye on her as well as the regular class room TA, and of course, Larry & I will be stopping in. For right now she will go 4 days a week for 3.5 hours each day. We will then adjust accordingly to how she does on any given week.

Then next appointment of the day was kinda a surprise but a surprise in such a rockin way!! Her new tanks came in! The one's that the DME ordered just for HER and just because she is going to school! The one photo is of the tank that we currently use; that's what's hidden in her ladybug. As you can see the new tanks...holy hockey sticks are they way smaller! So they yes, the next logical question is well what are they going to last?! 5 minutes?! Nope, notice that silver funny looking piece on the top? Well that's a conserver device and it makes those tanks last the same amount of time as her larger ones! Whhhoooooo Hooooo!! First thing she said when we put it in a backpack (Lady bug came with a small backpack that matches) was "Mom! Mom! I can use both my hands!!" She was so excited and it really rubbed off on all of us. It's so wonderful to see your child excited over an O2 tank lol

So onto the last appointment of the day, the long awaited GI appointment. God love McDr. I felt like I didn't need to do anything for the first time in a long time. McDr had spoken with them a lot about Alana laying the ground work and covering a lot of the background information that I would typically have to do. For a new doctor to see her and already have so much information was wonderful and exciting, and a relief. We had done another x-ray right before we went up there and it didn't go well. It's worse then the abdominal one we did a few weeks ago instead of better. Her GI system is just not sending the messages to the brain that it needs to any more so there was a lot of talk about the things we are going to try over the next 4-6 months to rewire those pathways. I am not going to go into what some of those entail because they are invasive and uncomfortable and frankly, as Alana would say, "It's my privacy!". There is a plan in place and we shall see how it goes. We have who we need to contact next week to see how she's doing and we will go from there.

Her next official appointment with GI is on September 10th. This will be her pre-op appointment for her colonoscopy, endoscopy, & tube placement. At this point she is scheduled for all that on the 11th. It will be okay and even her PT's think this will make a difference for her overall. So repeat, it will be okay. It will be okay. It will be okay. Now that the choice has been made I honestly think it will be okay, not having to worry about which way this was going to go, for me, helps.

So that was the day. It was a long one and I still have things to prepare because guess what tomorrow is...Alana's first day of kindergarten. Wow. My baby is going to kindergarten. She's bright, smart, witty, stubborn, thoughtful, strong, and amazing and now she get's the chance to shine and share all of those things with her teachers and peers. If I ever could make a book about endurance and strength it would have all 3 of our daughters in it leading the way and rocking.

Where does time go while you are waiting for it?

EEG Results...

2009-08-15T16:50:00.003-08:00

I know I didn't post right after she did the EEG but I needed a break from thinking about things, even though that didn't happen. In fact the opposite did and I've spoke with McDr no less than 4 times this week!

The EEG went well, in fact Alana did fantastic. She only had one episode of tears and hell, who wouldn't want to cry when it feels like your face is being licked off by an angry kitten! She did not like it when the tech. was cleaning her skin to attach the electrodes and with her "sensitivities" to things touching her skin I was so proud of her that she held out that long. Once things were underway and the tech. told her she could go to sleep she did and she did it with a quickness! I secretly was jealous that she was so willing to listen to the tech. when it takes me bribing her with my ear (she has rubbed my ear since her first hospital stay as a small toddler for comfort) to get her to sleep or she cries herself to sleep!

Once she was asleep they let her sleep for about 40 minutes then woke her up for the strobe light portion of the test. After that, it was only around 10 minutes, they did the part where she had to blow on a pinwheel for 2-3 minutes straight with big, deep breaths. This was by far the hardest thing for her to do. She was only able to do it for 10-15 seconds before she was gasping and trying to re-group. During this time she had one of these blank stares that she's been doing, it was similar but not at the same time. The tech was standing there by this point trying to get her to blow on the pinwheel and noted it but felt it was due more to the fact that she was struggling with her lungs and not her brain. Anyway--once that part was over we got to head home and she slept for the rest of the day, well into the evening hours when I woke her up for eat some dinner.

The results came in yesterday mid-morning and it showed little and a lot. McDr started it off with "essentially normal". So basically first they were not able to elicit the episodes of absence, or staring spell. This does not mean that she isn't having them, it just means she wasn't having one then. What it did show was an "abnormal response" to the strobe light portion of the test. Now I don't know what that means exactly, McDr was just reading the report from the neurologist to me. She did say he would clarify all this to us on her appointment with him...September 10th. She at least told me that he didn't feel it was a "big seizure", just an abnormality and the neurologist noted this abnormality consistent with mitochondrial disease. What's odd was she was the most relaxed while awake for that part of the test. She was fully awake, had her eyes closed, and was just at ease. Go figure. Alana is nothing if untypical right!

Right now we are just in the fun loving holding pattern. Results are starting to come in from Seattle and once the whole shebang is in I will break those down best I can and share them.

School starts here for the 2 rocking ladies on Wednesday (sad and happy at the same time!) and this week I will be meeting with the school team to go over Alana's needs and requirements. On Monday we find out who her teacher is going to be but I am confidant that it's all going to be alright. We did get some wonderful, wonderful, news though! Our DME finally (okay I told them we were leaving them to go with another company that would get us these) agreed to purchase the super small tanks for Alana!! They are the size of a water bottle and weigh in at only 2.2 lbs and 3 inches in diameter & will last on a conserver device for 3.5 hours! Wayyy better than the 10 lbs she drags in her ladybug! She will still need a rolling case for them because it's still a lot for a peanut to carry all day but it will make it so she can participate as often as she's able in recess and such!

I can't believe I am going to have an 8th grader, a 4th grader and a Kindergartener! it makes me so proud, sad, joyful, teary, and old feeling all at the same time! All 3 of the girls have come such a long way and I pray for the strength to keep all of us heading foward through this.

PS: We went to grandma's and grandpa's for some more downtime. If only we all could live in a place where the only sound is that of the water.

Back to school haircut

qrst...followup from yesterday

2009-08-05T16:14:00.002-08:00

McDr called and first and foremost the x-ray showed that her left side of her GI system was cleaning out well, but her right side hasn't. On that note though we are going to give it another week, the right side. What McDr is thinking is since the left side is looking well that we wait and see if the right can follow suit. Maybe now that there's some more room in there the rest that's full will work it's way out without having to undergo another procedure. Check back in a week on that one is the game plan.

On another note though she, McDr, said she spoke with the neurologist here about Alana again and gave him an update from Seattle and what's been going on, the increase GI, tone, eye drooping, etc., issues and he wants to see her back in his office for a "long" appointment and he expressed to McDr, who then expressed to us, that he feels she has mito. Now I've sat here for a few minutes trying to think about what to follow that up with. It's not that like we are not actively looking for that right now but to hear the doc here say "I think she has it, regardless of what the muscle biopsy says..." was a lot harder then I thought it would be. Maybe I am just tired. Maybe. This of course is just his opinion at this point, nothing more.

On a totally happy note, and yes, going backwards from last night with the bad/good news. Guess who's registered for kindergarten!! She is SOOO happy and telling everyone today that she's going to kindergarten and dancing around! I on the other hand am terrified! We still have a lot to do and get in order but we can handle that.

Also quick note here...EEG is on the 12th at 9 a.m. nothing more I can say on that one right now...I am still working through what they are looking for.

Love to everyone of you, thanks for all your support...even if I don't know your names.

IEP, EEG, LMNOP...

2009-08-04T19:13:00.004-08:00

So this post won't be long, unlike the last one. PT finished up the muscle testing report and it was what we had come to expect, she's got issues in the balance/coordination area as well as the muscle strength arena. The official grade on it is as follows;
Alana's overall balance/coordination score was 8%. This means that she is more coordinated/balanced then 8% of children in her age group or 91% of children her age scored better in these tasks. In regards to her muscle strength she scored 24%. The range is the same as above for interpretation. Basically for many of the balance/coordination tasks she falls under what a 4 year old could do and around what a 4.8-5 year old could do for strength and she's almost 6. This doesn't seem like a big deal, I know this, but when you look back at what she USED to do this is again, a big change.

I know, I know, I need to stop focusing on the past and focus on the future. Nothing will come of fretting over what was, everything to gain from what can be, but it's hard and I won't deny that. When you watch things that used to be easy for your child turn into things that are now hard it's hard on everyone and so much harder on her.

Moving forward to today she had her recheck with McDr and some things went good, others went bad (go figure!). So do you want the good news or the bad news first? How do people usually want the news presented to them? For me personally I like to have the good news first, wait a few minutes, then get the bad news. That way I can have a few blissful moments of joy without interference caused by those pesky bad feelings so lets go that way: Good news...her ears are responding well to the anitbiotic drops and are showing signs of improvment! They are no longer bulging and red, just a little bit of fliud behind them still but none the less, getting better!

So just sit...sit still and take a nice deep breath. That's what I did--just breathe.

So to get right to it, she's lost more weight. In fact she's lost 1 pound 10 ounces in exactly 1 week. The MiraLax & Milk of magnesia is not working well enough to keep her GI system moving in the right direction therefor she is still having pain and backed-up. We ended up having to do another x-ray of her abdomen to check and see exactly how far backed-up she is. McDr will be calling tomorrow with those results. There is going to be another procedure (not going to go into that unless it comes to that) done if it's really bad so let's just hope that it's not too bad at this point. The plan is to change her from the magnesia to something else that I cannot pronounce right now and keep up with the MiraLax. She, McDr, was going to call Dr. P, the GI doc agian and make sure everything is inline for our appointment on the 24th. Honestly, this is getting freaking ridiculous! That's all I can think of at the moment to describe this!!!

Other then those major things we talked about school, tomorrow is registration, and what she's going to do. McDr thinks she for sure is going to need an IEP (individualized education plan) along with her 504 (to cover her O2 use). Our goal is something like 3-4 days a week for at least half the day and we shall go from there. Her pre-K teacher/adopted grandma, whom we adore, said she'd be willing to take her back lol!

We also went and looked at Alana's soon to be new ride tonight, it's an adaptaive chair. Think of it as a stroller on 'roids! It's a more supportive seating system for her and made for children with disablities. This isn't the one we are going to get now though, it was and that's why she's in it, we just decided to go with the step down model because it can be tweaked to fit her needs without being sooo overkill.

Anyway, that was how the last 2 days have broken down and I hope the rest of the week will go smoothly. Tomorrow doc's office is suppose to be calling me with the date and time of the EEG so we can get that done and over with but I don't suspect that will happen this week. If things look bad with her x-ray I will update sooner rather then later or if anything else changes.

"Wondrous is the strength of cheerfulness, and its power of endurance - the cheerful man will do more in the same time, will do it better, will preserve it longer, than the sad or sullen."

This is going to be a long one...

2009-07-28T21:28:00.005-08:00

I can't seem to decide where to start. I was thinking of going with today's events and then backtracking into yesterdays but then that seemed goofy and ack-wards, so in the interest keeping things fluent here's yesterday's breakdown--BUT before we get to that keep this in mind please. It's not very often that I feel so overwhelmed and upset that my "keep things positive" attitude starts to falter. Tonight is one of those nights though. Honestly, it's been one of those weeks so please bear with me while I work (write) through this and get it out so I can tuck it away in a little box and leave it there.

Yesterday was our big PT meeting to do the MMT (manual muscle testing) and a whole new evaluation on her balance and progression. Some things went OK, others did not. Her muscle testing showed some positive things (good strength which equates to a 5 for a score) in her arms although her right arm was weaker then the left. Then moving onto her legs she had some problems overall. Her score here was a -4. Now, that might not seem like a huge difference or problem but it has a lot of implications in regards to her overall strength. A 3, from how it was explained to me, means that she could lay on the floor and move her leg from side to side but could not do it with any force applied to it, i.e. someone pushing on it while she pushed against them. Now remember a 5 is normal, and a 3 is bad and a -4 is pretty darn close to that so that was very concerning to her PT. In regards to her strength the overall picture of her so far, is that her right side is weaker then her left, throughout her whole body. We still have some more testing to finish with that test and we will finish that on Monday.

So you might be thinking well, hell, that wasn't that bad. What's the malfunction here? Well I haven't talked about the balance and coordination part yet or, her lack thereof. The video that I have below this is just a small portion of the testing. What she was suppose to do was just stand there with her feet toe-to-heel for as long as she could. You can watch the video to see how that turned out. I was trying to not cry during these tests, watching her struggle and try and try again. She was being so cooperative, listening, and getting what was said. Unfortunately she could not get her body to behave. Some other things that we did was try and get her to do a jumping jack, which she could not. She also could not close her eyes and touch her nose. Overall they've realized that her proprioception is way off. What that means in a more in depth view is this:

"Proprioception" is a word we probably don't hear very often. But it refers to body placement, and the ability to sense where our bodies are in space. For most of us, through the many receptors in our muscles, ligaments, tendons, and joints, we are able to sense what our body is doing, and whether we are crouched, standing, turning, sitting, or moving. We can tell how close we are to something else, and can sense our bodies, through the pull of gravity, even when sitting still. But for some children with special needs, this "body position sense" is weak. Children with proprioceptive dysfunction can have a lot of problems managing themselves out in the world, because they tend to be clumsy, and invade other people's space. Since they don't have a good feel for where their body parts end and the air and objects around them begin, they tend to have poor motor control and poor motor planning.

Here are some symptoms you might observe in a child with proprioceptive dysfunction:

Grasps things so hard that they break or tear.
Falls and trips often, and has difficulty with team sports.
Has some trouble climbing up and down stairs.
Moves awkwardly-might have an unusual gait or keeps arms in a peculiar position.
Frequently bumps into other people or the objects around him. Gets into other people's personal space and is clumsy.
Might chew, twist, or pull on his hair or clothing.
May be overly involved with her own body...i.e. making noisy sounds with her mouth, slapping her thighs as she walks, putting her hands on her face, wringing her hands, etc.
Can't do physically complex tasks without help, and may need lots of visual or auditory clues.

Many of the awkward behaviors these children exhibit are due to their own attempts at self-therapy. They may roll around on the floor in a blanket, or bump into walls as they walk, in an effort to gain more sensory feedback.

She does.....every.....single.....one.....of those things up there. Watching her try and perform these tasks and then watching her just keep getting up and pulling through it was hard. Again, why is this a big deal? She's got much bigger issues going on then that right? What makes this even more of a big deal to me, the reason I want to curl up and make this all go away even more, is because this is new to her. This wasn't something she did 2 years ago. This is something that started happening and has gotten worse every month. It's another chalk mark on the board of things that Alana struggles with when she should be running around and struggling with what color backpack she wants for school, or what is her favorite candy flavor this week; fun, frivolous, 5 year old things.

As I mentioned that testing isn't done. We didn't get it completed because she was having such a hard time and, goodness do I love her PT, she just kept giving her more tries, more examples, more "You are so strong you can do this" talks. When we have the final report and grade I will let you know. It will all work out in the end, it always does.

On to today, did I mention somewhere that this was going to be a long one? PT started off good with her full of smiles and signing along the pool deck. After about 5 minutes though she was so tired and those lovely black/blue circles under her eyes where taking over her whole face. She was okay 1 minute, doing her puppets (a type of balance/muscle coordination exercise) and the next her speech was slurred, she couldn't move her muscles in any coordinated way, and she was unable to count to 10. It was enough that the PT asked if we should end the session or just let her rest. We opted to let her rest, horrible mom that I am, to try and see if she would be able to work through it after a few minutes of sitting still. She did get back to her normal tiredness but the whole session was tough. She was tired, and barley capable of thinking straight. We ended early after all and just let her sit at the edge of the hot tub while PT rubbed her feet for her.

After this I did make the call and take her into McDr, yes, I've been putting that off for a few weeks (hey, we've been keeping touch via phone and she didn't say I HAD to bring her in...just that it might be a good idea) so I bring her in after they say they want to see her even though it's McDr's lunch break (LOVE HER) and come to find out Alana has a double ear infection (ANOTHER INFECTION, no regard that she's on her 2nd set of tubes), a viral infection (she's having more rashes and has 3 large lymph nodes inflamed in her neck--again), and all this exasperating her normal crappy baseline. Sigh. We talked about the PT report, the pool episode, the eye drooping, the fact that the freaking room had to be 100 degrees and she was under a blanket and had mottled skin, oh and the chest and stomach pain that she's been having all weekend (forgot to mention that one eh...) is most likely from the fact that her GI system has stopped moving things on down the line. It was so backed up again, despite MiraLax and milk of magnesia, that she was most likely having reflux from literally being filled up to the top. We left with antibiotic ear drops, a new test that McDr wants done now, an EEG to check brain activity, and an already scheduled appointment for next week.

Well after a day like that guess what happened? She slept from 1-5:15 pm. It's alright though because she clearly needed it and it made her feel a bit better, as in she wasn't screaming at everything and being inconsolable. We woke her up for dinner and then off to Noelani and Shaylins soccer games. Fantastic news on that front, Shaylins team beat a very hard team and knocked them out of trophy contention and they get to move on!! She was jumping for joy and it was a fantastic game!! Noelanis team though was eliminated but that's still alright, they had a wonderful season and it's tough being 13 and playing with 18-19 year olds!

So that's the end of the pity party for now. I am still in a funk and having those ridiculous moments of crying at lame commercials and sad songs but it will pass and I will move back over into la-la land where there's nothing we cannot tackle. Tomorrow maybe, tonight I'd kill for a brownie ala mode and someone to cry with.

“Strength does not come from physical capacity. It comes from an indomitable will.”

River days...

2009-07-20T18:51:00.004-08:00

So we thought we'd get away for the weekend and go to Grandma's and Grandpa's house to relax and see if it would pick up Alana's spirit, give Noelani and Shaylin a break from appointments, and have some 1:1 time with Larry and I while Alana hung out with G-pa's. It went well I have to say.

Before we get into all that fun though on Friday we talked with McDr about the now weekly episodes of freezing cold, agitated, in pain, staring spell, night's she's been having. I asked what more I should be doing to make her comfortable and there really wasn't anything she could think that I hadn't done already. She did talk about the g-tube again and Larry and I had a long talk about it at 1 a.m. (yes 1 o'clock in the morning) as we drove down to my parents house and I didn't realize how for it he was. He is usually the one that is resistant, more so then I am, to things with Alana and for him to say that it will help her overall and if it's something we can finally do to help something then she needs to have it done. Talking with him put some of my fears and trepidation about it at ease and from what McDr said that's a good thing because it's almost time. On the other side of things she has patches on her tounge again that are bugging her as well as a new rash (go figure!) and after PT today she slept until 6ish tonight. Her PT did some offical testing of her hypermobility and that's going on the list now so I need to see if that ties into anything more then her hypotonia issues. Someone mentioned that those tend to go hand in hand so who knows, if it's nothing out of the ordinary from what we've already got going on then that's a good thing, right? Of course this wouldn't be Alana if she didn't add something new to the mix and she did on Sunday. It's called ptosis (droopy eyelid) and I have no idea where it came from! It was rather sudden, looked at her when she got out of the car and it wasn't there, went to change her over to her concentrator and there it was. Going to ask McDr about it later this week when we see her though, prolly going to be another FYI in the end though.

So onto the fun stuff! This is just a typical weekend at my parents house so enjoy the photos!

Throwing rocks in the river!

Fishing with Grandpa Alaskan style lol

My great fisherman who took my pole when he was suppose to be taking photos...

Shaylin and my brother with the dolly they caught together.

I am too cool to wear hip boots, being 13 and all...:P

She got to go on the Life Flight helicopter during the EMS open house.

Shaylin showing off her drawing from a firefighter!

Again, look how cool I am!

Speaking of Seattle...

2009-07-13T19:06:00.003-08:00

First off let me say how much I really, really like Dr. Hahn over there at biochemical genetics in Seattle. I called and left a message for his nurse this morning to see if the results of the Muckle-Wells and Familial dysautonomia gene testing (and was going to throw in there they whole, hey...any word on the mitochondrial testing lol) where back but no more then 45 minutes later HE calls back and goes over the results with me. He is high on my top 5 doctors that have tried to help Alana!

So onto the results though, and no answers here, both came back negative so we are just waiting on the mito testing. He said that he has been thinking about her and what else he could check for but was at a loss. She's had such extensive workup's and from a metabolic stand point, aside from the mito, there was nothing else at this time he can think to check her for, again, not sure how to feel about that. He promised to call as soon as the mito testing came back and he's anticipating that being some time in August. I really hope so and I don't even know what I am hoping for, yes or no.

Today's PT session sucked, as have the last 2 weeks. She's crying all the time, dropping her SATs at a ridiculous rate even with her O2 on, literally falling asleep in the pool or crying and refusing to participate. Tomorrow morning's session is cancelled and I hope that she will feel better for the next session but it's so frustrating right now. She's got some crazy rash that I've never seen before, she's sleeping from 4-5 pm until 9-10 am and still tired, eating well for 1 meal then nothing for the rest of the day and just so very pale. I don't want it to be mito but I don't want this to be another dead end and have no idea what is going to go wrong next. Her HR is so crazy and erratic right now as is her GI system that whatever "this" is is taking a toll on her in so many ways.She feel asleep right when we got home (3:45) and we just woke her up (7:15) to eat some dinner and she's picking at it so that's good hopefully she will manage to get some of that in her mouth.

No pictures today, sorry. Just didn't have time and honestly, she looks no better then the last one so we are going to go on a trip through memory lane...enjoy.

Fun times, fun times...

2009-07-10T17:27:00.004-08:00

Well things are clipping along on our normal route around here. We have been enjoying some fantastic weather here, I am talking 80 degree weather for almost a week. It's cooled down to high 70's and expected to stay this way for another week and I can't tell you how nice it is to have some heat this summer. On a cruddy note there are over 70 fires burning now within Alaska and it's created smoke inhalation advisory's on certain days so we are keeping a close eye on that and making sure Alana is wearing her O2 (and not making it "fall" off) and just being super aware of the air out there.

(have ladybug will travel)

We had a blast on Wednesday and Thursday though! On Wednesday we went to one of the local creeks with a park and had a picnic with our dear friend Carolyn. It was an amazing day, amazing time, and she's one amazing woman. She was Alana's preschool teacher and has not only melded into our family seamlessly, but she accepts Alana for who she is, illness, hysterics, laughs and all. I think sometimes it's hard for adults, particularly teacher figures, who want her to conform and relax when she's having a break down for being so over tired, over done, over being sick.

On Thursday we went to a lake and wow! it was warm! Talk about strange going to a lake in Alaska and it being warm water. One of the wonderful things about this beach, yes beach, is that they bring in sand so it's not only warm but you can truly make sand castles. Now all those that live in the lower 48 are prolly snickering at this but hey, at least we don't have to share hospital rooms LOL! We played on the beach from 11 am until 2 pm and yes, due to all the running around from the last 2 days today we are paying a dear price in the form of Alana screaming and crying, running a fever, refusing to eat much of anything and just very, very tired. She slept for hours after each day but it's still not enough to compensate for doing those many activities. I think it's going to be alright, she needs to be able to have some "regular" kid activities as do her sisters. She possibly won't remember being mad today but I think she will remember being happy and having fun in the creek and at the beach. It's all about balance and to help with that we've stayed home all day today and I've just let her lead the way in regards to her naps and everything.

On the medical front we did get the GI study back and it was all within normal limits. The nurse that called didn't give me much else to go on but she did say that there is *gasp* no reflux. Go figure. It's all good though because maybe now that can be put to bed finally...she does NOT have reflux. This should also put any thoughts of doing the fundo procedure out of the GI's head. I hope.

Right now her fever is over a 100 and she's uncomfortable and to be honest for awhile I've felt she's been more "off" then usual so tonight's going to be a long one keeping an eye on her. It's hard to explain the feeling, but other parents out there who deal with chronically ill children know the feeling (Linda!) that you get when you just can't put your finger on it but there's all those little signs that something wicked this way comes. Here's to being wrong, I am all for it!!

(Yes...she has taken "it" off again and I am working on getting "it" back on)

Happy 4th of July!

2009-07-04T10:26:00.004-08:00

HAPPY 4TH OF JULY!
To everyone who has served and everyone who currently is; to their families and their friends who
support and sacrifice each and every day,
thank you for letting freedom ring!

On the home front here things have pretty much stayed the same. Today we are going to go to a 4th of July BBQ with some friends and hope that Alana will either take a nap during so we can skip or minimize the meltdowns or stop trying to take her O2 off because she want's to be "normal like the other kids". This is becoming more and more a statement with her. She has this innate ability to make me smile and want to cry at the same time. This morning she slept in until after 10, this is another sign that she's still not feeling very well of course. After she woke up and came downstairs she told me that she was a normal girl today. I told her she was normal everyday why was today different? She then told me that she hasn't coughed yet this morning and her feet didn't hurt so that means that she's normal and isn't different then her sissy's and she liked being like them. While it makes me happy & proud that she's such a articulate child, it makes me sad that she feels that she's not normal in any sense of the word.

My thoughts are jumbled into a random mess this morning so forgive me for bouncing all over the place! One of our dear friends isn't feeling very well today and it is making his mom worried which in turn makes me worry. He's a lot like Alana, strong, brave, and prone to getting sick a lot. I hope that today ends up going smoother for them then yesterday, and that they know that all of us here are thinking of them!

P.S. Look how nicely her muscle biopsy incision is healing! She's so proud of it and she tells everyone that she didn't cry at all!

Chocolate milk anyone?

2009-07-01T17:03:00.004-08:00

Sorry to not update sooner but I was a walking zombie for most of the day.

On to the good news though--we are not in the hospital! She's still doing poor but she was drinking (more on the fact that she willingly drank the barium next) and ate some a today so we are still going to stay in a holding pattern. The fevers have remained lower, around 100-101, but she's also been getting very low as in hypothermia low (93-94). Last night was a crazy one, see above about the walking zombie, because she was up crying from about 3 a.m. until 6 a.m. for no reason i could find. On top of just crying she was so cold it was scary and even laying on my chest, wrapped in blankets in 70 degree weather she was unable to get warm. The final straw to make me really consider taking her to the ER was when she started tripping for a complete lack of a better word. She had her hands up in front of her face moving her fingers and clicking with her tongue. She wouldn't answer any questions I was asking her but would settle back down only to do it again 10-15 minutes later. This lasted right around an hour then we just went back to the crying and shivering until she finally fell back asleep. Go figure 'cause I have no bloody idea.

This morning she did have the upper GI study done with the barium and I have to tell you, Alana so rocks! I was thinking that this was going to be a long morning with her, even more so with her being so overtired, but alas, she drank it as she was told and when she was told. The tech made her smile by telling her some adults whine over the barium drinks and she should teach them how to do it. It did take a long time, around an hour and a half and they had to have her roll over from side to side on the table for awhile, then they decided to just wait a bit and she just laid there looking around (almost fell asleep) and then they resumed the scan again. They looked at her esophagus while she swallowed, they watched it enter the stomach, then work a bit into the intestinal tract. of course there is no results yet but by Thursday we should hear something. They did make us wait another 30 minutes while they talked with the doctor to make sure we could leave so not sure if that is standard or if they saw something of interest.

While we are on the GI issue today we made the appointment with the GI, August 24th, and there we will be going over the plans for her EGD and colonoscopy as well as the G tube talk. McDr will be keeping a close eye on her in the meantime and if things change; i.e. she goes inpatient for something else, drops more weight, we may address those sooner. Yesterday she ate 810 calories and today she's only up to 680 but we still haven't had dinner yet. The most that she's eaten since I've started keeping track a week ago is just over a 1000, 1010 to be exact. What are we going to do with you little girl!

Huh? Where'd that come from...

2009-06-29T15:50:00.004-08:00

So I spoke with McDr for a bit today about how Alana's been doing throughout the weekend and she also let me know of the *new* things/procedures that Alana needs to have done so here goes.

First with the procedures and those include on Wed having a barium upper GI study done. This outta be fun :/ They are at least going to try and flavor the barium to chocolate to try and encourage her to drink it. From there they will do the x-rays and we again, wait for results. On Wed as well I am to schedule Alana's appointment with the GI doctor and she will be having another EGD (Info) done as well as a colonoscopy both with biopsies. They are making sure she's not having reflux (for the 100th time) or having dramatic changes in her whole GI system due to the increase in overall GI problems she's been experencing. I am not 100% clear on what they hope to accomplish in those regards and will find out more when her appointment happens, sometime in August...yes having 1 traveling pedatric GI doc sucks almost all the time.

The other thing that came up was the dreaded NG/G tube issue. Well aparently the NG tube is no longer an issue as the GI doc feels that she doesn't need it. My bood started to boil when McDr mentioned that but then was put on ice as she told me he'd like to go straight to a G tube. After going over her issues he felt it best to just go straight to the G tube 'cause this isn't something that's just going to go away in her case and if it helps her gain and grow then just skip the month trial and get it done. This was both a relief and scary thought. I've been keeping a calorie log for her and she's consuming between 800-1000 calories a day and for a 5, almost 6 year old, that is not enough for even a couch potato kid. McDr and I will touch base again soon and talk about if/when we have to get to that point, is it now as in next month or is it now as in August. The other thing that he mentioned that took me completely by suprise was the thought that she may have to have what's called a Fundo (info). This I am 100% against. She's had no documented reflux and when she first got sick we tried every reflux med and had testing done b/c they did think her cough was caused by reflux and then that her pneumonias were from reflux asperation as well.

As for Alana overall she's doing crappy. She's been sleeping until 9-10 a.m. and still waking up tired and sore. She's wanting her back or her feet rubbed constantly and just very, very iritable and fussy. One minute she can be smiling and goofing off then the next it's like world war 3. She ran a good fever all weekend and then suddenly dropped down to 94 again. I am glad that the fever broke but just not glad that it went down to 94. She was shivering and cold to the touch under 2 blankets when it was 78 degrees outside! She's very pale and those damn cirlces under her eyes might as well be blackeyes from her getting into the ring with a heavy weight boxer! The patches that she gets on her tounge are back as well and that usually means that she's stressed and getting dehydrated. Neither is a good sign. McDr said if she's not doing at least a tiny bit better or drinking better by Wed to bring her in for an admit to get IV fluids and a workup. At this point I am offering her any soda she wants if she would just drink more than 4 ounces of it.

On to a great thing..today in the mail she got a package from one of her buddy's Deb and was she ever happy! It was a giraffe shirt and socks and she's refusing to take them off--ever. Nice to see a great big smile on her face!! Thanks Deb!

And we march on...

2009-06-25T18:22:00.003-08:00

Well things have been typical crazy around here. Aside from everyone adjusting to being home after a two week hiatus, there's always those things that come up out of the blue and shake you silly. It's nothing with Alana (besides the usual),it's something with someone else close as close can get and it is what it is.

Alana is staying typical untypical Alana, she's been running a fever for the last 2 days, between 100-101.8, she's not eating or drinking enough which it's at these times, and these times only, that I feel less resistant to the tube feeding issue. That is not a step am 100% sure of yet, it's just sitting there in the background of my mind. Other then the fever she's still battling her GI system. We had added the Phillips to the MiraLax and it worked great for 1 day then the next she had a major problem with it. We stopped both med's for a day and she was back to square 1, in pain and unable to move anything. :\ Right now I am trying a lower dose of the Phillips and keeping her MiraLax the same.

On Monday she starts back at PT and she does not want to go. I think once we get there and she gets to see Gail and Jess (her PTs) that she will get over that. Her inserts to help with her pronation (Picture) should be in and ready for her to try. Hopefully this will help with the foot pain she has.

Noelani & Shaylin are back in soccer full swing and it's taking sometime to adjust to these late night (8 and 8:30 pm) games x2 a wk. We have to divide to get them to their games and then the following game we rotate. Getting Shaylin to bed at 9:30-10 o'clock at night is rough. She needs her sleep and she awakes, no matter what, at 6 am every morning!

If Alana has a fever again tomorrow it's back to McDr for a checkup and check in. If she gets over 102 for more then 24 hours it's admit time and with the fevers coming closer and closer the last few weeks it's something that is weighing on me greatly.

I think this holds true:

“Be faithful in small things because it is in them that your strength lies.”

Back again...

2009-06-23T19:41:00.003-08:00

(The picture is on the way home from my parents house, I'd like to say that it's some backcountry road but it's not, it's Alaska all the way around all the time) Alright we are back again. We ended up staying at my parents house longer then anticipated and it was nice. Alana got to hang out with grandma and grandpa and we got to spend some one on one time with Noelani and Shaylin. I would have updated sooner but my moms connection did not like blogger or Caring bridge.

Things have been the same with Alana though, she's running hot right now (over 101) and has been for 2 days now but her leg looks good with no signs of infection. She's been sleeping a lot more then usual (and that's saying a lot because she sleeps so much) and still is tired.

So anyway, some of the things that I didn't touch on about Seattle were in regards to another idea that Dr. Haun had about a possible diagnosis for her. It's called familial dysautonomia and you check for it via labs which they did when they placed her IV line. No word on when those will come back but here's a quick background on it:

Familial dysautonomia is a genetic disorder that affects the development and survival of certain nerve cells. The disorder disturbs cells in the autonomic nervous system, which controls involuntary actions such as digestion, breathing, production of tears, and the regulation of blood pressure and body temperature. It also affects the sensory nervous system, which controls activities related to the senses, such as taste and the perception of pain, heat, and cold. Familial dysautonomia is also called hereditary sensory and autonomic neuropathy, type III.

Problems related to this disorder first appear during infancy. Early signs and symptoms include poor muscle tone (hypotonia), feeding difficulties, poor growth, lack of tears, frequent lung infections, and difficulty maintaining body temperature. Older infants and young children with familial dysautonomia may hold their breath for prolonged periods of time, which may cause a bluish appearance of the skin or lips (cyanosis) or fainting. This breath-holding behavior usually stops by age 6. Developmental milestones, such as walking and speech, are usually delayed, although some affected individuals show no signs of developmental delay.

Additional signs and symptoms in school-age children include bed wetting, episodes of vomiting, reduced sensitivity to temperature changes and pain, poor balance, abnormal curvature of the spine (scoliosis), poor bone quality and increased risk of bone fractures, and kidney and heart problems. Affected individuals also have poor regulation of blood pressure. They may experience a sharp drop in blood pressure upon standing (orthostatic hypotension), which can cause dizziness, blurred vision, or fainting. They can also have episodes of high blood pressure when nervous or excited, or during vomiting incidents. About one-third of children with familial dysautonomia have learning disabilities, such as a short attention span, that require special education classes. By adulthood, affected individuals often have increasing difficulties with balance and walking unaided. Other problems that may appear in adolescence or early adulthood include lung damage due to repeated infections, impaired kidney function, and worsening vision due to the shrinking size (atrophy) of optic nerves, which carry information from the eyes to the brain.

So that's a quickie version of FD and I am not sure where I sit with it. There are things of course that make sense and account for a lot of symptoms that she has and has had but then of course, there's things that don't fit. Go figure, the story of Alana right. There's so many things that seem promising when it comes to diganoses for her but then nothing pans out. I do know that if mitochondrial and now FD do not account for anything with her that we are going to apply for the NIH rare disease program (http://rarediseases.info.nih.gov/Resources.aspx?PageID=31) and see if we can get accepted and maybe some ideas on other possible avenues to follow.

I really struggle with when to stop. When do I stop taking her to other states, other doctors, other everythings to try and get a diagnosis for her. I know that even with some of these diagnoses that there is no treatment, only a name, but if we had a name then maybe someday there'd would be a treatment. It's bad enough knowing that she's battling something so intense that it's slowly robbing her of enjoying a healthy life but to add to it that it's either so rare or just completly unheard of that we can't even make those first steps towards treatment. Right now we are resigned to treating things as they come up, not heading anything off that's on the horizon. I know that I shouldn't take those things to heart like I do, but yet, I do. I feel like I should be trying harder to stop whatever this is and yes, I do know that some things are out of my control (gasp! that news was just broken to me recently lol), but with your children and their health you do what that to be something you want to be in control of.

All we can do now is wait on those results from the new testing and also we are still waiting on the results from the Muckle-Wells gene test. That's like a little skeleton waiting in the closet that one. It's making me nervous just waiting for it but clearly Dr. Haun thought it wasn't a great or even good possibility given the fact that he wanted the muscle biopsy and FD testing.

I ended up cancelling her eye appointment so we could hang at grandma's house longer so we will take care of that next month. It will keep and to be honest, she was done with appointments for a bit. We did go to her appointment with McDr and Dr. Demain and McDr again talked about the NG tube issue and what we are going to do with her increased GI motility issues. She had us add Phillips to the MiraLax to get things "moving" along and hopefully give her some relief from the pain of being backed up. She did also mention that she recived an email from Dr. Haun and was impressed by it. She said it was short but just letting her know that he would contact her as soon as the results came in and go over them. She is also going to contact the GI doc that rotates through here every couple of months (Alana has seen him in the past...) and talk with him about the increase in overall symptoms, of course in particular the GI issues, and also the NG/G tube talk. That was pretty much that appointment and we will check back in next week to see if she's gotten any ideas and make another appointment to see what the plan is in regards to the NG tube that I don't want to talk about...

Dr. Demain said that if the motility issues were being caused by her new allergies to wheat and soy they'd be better by now. She's been off those foods for more then enough time to see some resolution of symptoms in her GI system. He also noted that he didn't her any bowel sounds on examination and that wasn't good either. Think of it like a heart beat, just cause you don't hear it with the naked ear so to speak it's always making noise.

I know this was a long updated, kudos if you have stayed this far! Here's something I like to think about at times like this, it's from "Through the looking glass" (aka Alice in wonderland):

Alice laughed, "There's no use trying," she said, "one can't believe impossible things."

"I daresay you haven't had much practice," said the Queen. "When I was your age, I always did it for half-an-hour a day. Why, sometimes I've believed as many as six impossible things before breakfast."

Home sweet home...

2009-06-16T18:51:00.004-08:00

Just a quick update! We are home and boy does it feel good. Tomorrow we've got an appointment and then I am going to go fetch the girls from Grandmas so it's going to be a looong day all the way around.

Tomorrow night I will do an update once we are home about the appointments and a couple of things I didn't touch on while we were there. In the meantime THANK YOU, THANK YOU, & THANK YOU for all the prayers, love, and light for Alana this past week and every other week.

"A team" try outs...

2009-06-15T19:39:00.001-08:00

So tonight is our last night here in Seattle and I am happy to be heading home. I really miss Noelani and Shaylin and want to get back to our regular pace, crazy.

All last night she was alarming in the mid 80's with her saturation and heart rate (low) but once we got her O2 turned up we all slept soundly and comfortably. It was nice to not wake up until 8 am! When she did wake up though she had another fever and said that her leg felt stiff. Gave her some Tylenol and that seemed to help after awhile. I've given up trying to figure out what her highs and lows are from or even when they are coming. Well, at least for today I've stopped trying to figure it out. =)

She's having some major GI problems today & tonight and I don't know if it's being increased by the stress on her body right now or what but she's been very uncomfortable all day today.

Even though we are going on home the fun never ends 'round here. On Wed. she meets with the immunologist and allergist again to go over this appointment, the fact that her new diet hasn't helped her GI system, her growth, etc. On Thursday she meets with her ped. to go over this appointment (round 2) and talk about her growth and the calorie log we are going to start doing in order to try and figure out what is going on with her growth, or lack of it. Friday she has an appointment with the eye doc to make sure she's not having any inflammation in her eyes and just a good eye check. Lots and lots going on of course.

Well as we get ready to leave Seattle behind again I can say that it's been a good trip. We met a wonderful new doctor to try out for the "A team", we saw Linda and Jacob (yea!), even though her surgery had problems it was nothing we couldn't manage, and the best part, we get to go home with the promises of help in the future in regards to trying to find a diagnosis. I know we've been there, done that (about getting a diagnosis from a new doc) but if I lose faith that someday someone will come through with that, then I think I will have failed Alana on so many levels.

Freedom!

2009-06-14T14:01:00.002-08:00

So we are officially discharged! We are back at the hotel now and Larry made her a tent between the beds and she's laying under them chillin. We had another wonderful nurse today and that is something I can say holds true here just like every other hospital, 99% are just amazing and caring and all that, while 1% are total hags and rude. We didn't get a rude one, unfortunately for the new family next to us they did though. Can't win them all right? It was nice to not have to worry about the nursing staff though, it's one of those things that can make a visit or make it terrible.

So she's still terribly tired, more so then I'd like, but she's finally asking to color and wants to read a book. It's confusing how much something that's suppose to be a outpatient thing can be so hard for her. I think a lot of it still revolves around 2 key points;

1. Like a cold for her lasting 3 months, any invasive attack on her body, i.e. cold, flu, biopsy, triggers her already hyperactive immune system into massive overdrive therefor accounting for the completely unstable temperature shifts, the "erratic" heart rhythm, remaining lethargic for days after.
2. All of the above with the added complication of general anesthesia staying in her system longer then expected.

I think soon we will have to figure this out in order for her to have anymore procedures or just be sedated in general. I have a lot more thoughts and things to think about, including that we are testing for something that is hard to think about but for now we are back at the hotel, she's resting, I've spoken with Noelani and Shaylin and let them know we are coming home on Tuesday and that I cannot wait to see them. Shaylin was so cute, she told me she made me something and it's a big suprise and I will love it so much. Not more then I love you guys cupcake ;-)

Well I am ready...are they?

2009-06-14T09:47:00.004-08:00

So it seems that today we will go ahead and leave. Now I don't have the walking papers in my hand at the moment but we are already working on packing up and all that.

Last night still sucked and I am glad we stayed. By 8 p.m. vitals she had a slight fever of 100, considering that she wasn't above 96 since the surgery it was a good jump. From there she feel asleep and started to have heart rate fluxes that lasted 6 hours into the night, early morning. What would happen is she'd drop to the 50's & 60's, the alarms would go off enough to startle her system and she'd jump to 120's. Start to drift off again, heart rate would go down, repeat. By midnight vitals she was back down to 95 degrees and shivering under 3 blankets so it was just a very long and frustrating night all the way around.

She's still very sleepy and not wanting to get up and moving and she's only voided once today and it's 10:30 a.m. here. I will just keep pushing her to get up and drink and hopefully when we are out in the sunshine and in our own element she will perk up some. I am not even caring about her not eating right now as long as I can keep her drinking.

Just a bit ago they came in and pulled off the tegaderm over her incision and she was one p*ssed off camper! She's still favoring it but not complaining about it at all. If you go to touch it she just shy's away but still doesn't complain or even say anything. Wish she would say that it hurt or let us know how it feels. Sometimes it's hard having such a brave and strong little kid, they don't express fears and pains as you'd like them to.

Well I am going to try and keep packing and hope that it will hint to the nurses!

PS: Here's her "1 inch incision"

Okay..so that didn't work...

2009-06-13T18:27:00.001-08:00

Okay so yep, we are staying again tonight. When we asked the resident they told us, "well why don't we see how she's diong after her nap..." then it was "well why don't we see how she's doing after dinner..." because after her 2+ hour nap she was still tired, didn't want to get up to play or color and said her stomach was starting to hurt. By now it's about dinner time so then comes the after dinner part. Well after dinner her stomach hurt even more and she only ate a small amount of fish. blah.

So what we asked them to do was to lock her IV so she's having to fully drink on her own and not count on the IV to keep her hydrated, get us a cart and an O2 tank to make her get up and out and then walk part of the way back. So all those things happened and now she's even more pooped then she was before but I'd rather her be pooped and complaining here then at a hotel 30 minutes away (thanks Linda for that reminder ;-) and have our only means of getting back here be 911.

There it is in a nutshell, we are here for another night, she's got to drink and start working all on her own in order to get out of here. Hence the reason that "day surgery" does not apply to Alana at all.

Waiting...

2009-06-13T13:10:00.001-08:00

So it's 2 pm Seattle time and we are still inpatient. I *think* Dr. Haun said we could go if she was doing alright but then the senior res. came in and said they wanted to watch her some more and see how she does sleeping again. I think there's still a chance of blowing this Popsicle stand tonight but at the moment her SATs are dropping for no visible reason. They are not terribly low by any standard but they are a good 2-5% lower then they have been. So hmmm.

Her cheeks are a bit flushed right now but the swelling doesn't seem to be getting any worse so again, that's all great news. I think unless she does something totally kooky I am going to ask for her to be discharged by the resident when she wakes up.

On another note...our childrens hospital ROCKS! Now don't get me wrong, Seattle this time around has been wonderful and we've had fantastic luck with some amazing nurses & Dr. Haun. but--shared rooms SUCK! First we had a little boy who had no idea that he needed to stay on his side of the curtain and was so bloody loud telling his mother " Im gonna hit you!" over and over again. Luckily they only were here for the first day of our stay and went home. Great we were thinking, there was 5 other rooms on this floor that only had 1 kid in them so there was no reason to think that we'd get the short stick if someone else came up. Well luckily we didn't instead we got a roomie from another room who had complianed about her current roomie! They are not bad at all though, it's a mother and her 10 day oldish baby but she likes it dark and i like a lot of natural light :( I'll stop complaining though, much better then mean boy!

Well, I'll let you know when I know!

Hotel

2009-06-13T09:03:00.001-08:00

So we re getting out of the hospital sometime today as long as she keeps staying Alana simple. Her temperature still has not come up above 96 but in turn she does not have a fever either. We will go to the hotel for tonight and if things go smooth fly home tomorrow night. I think she will be able to make it if we make it an early evening flight instead of the red eye.

They should be coming in soon to remove the bandages and remove her IV so we shall see. Dr. Haun came in again this morning and talked with us and let us know that he hopes to be in touch within the next 2 months or sooner if something comes in. If after 2 months we don't hear from him we can give him a call. I will post more later on!

Surgery

2009-06-12T20:06:00.006-08:00

We are out of surgery and she’s in her room now (4018 bed 1 @ Seattle Children). The surgery went good and they used some new precautions so hopefully those will keep her on the path to going “home”, i.e. hotel, tomorrow afternoon.

Surgery was at 11 am, she was done around noon, and then she was finally brought upstairs around 1:30 once she was stable. She’s still pretty out of it and now that local on her thigh is wearing off so she’s getting uncomfortable. She was able to drink some apple juice so we are just making sure she can keep that down and she might be able to eat some dinner tonight J Tomorrow morning when surgery does rounds they will come in and remove the bandage so we will be able to see what it actually looks like under there. The surgeon said it was a good sized piece of her left, upper side muscle. He did reiterate that there’s a 9 out of 10 chance that even if she does have mitochondrial disorder that this will not pick it up because it’s such a rare and hard thing to find. We will cross that bridge when we get to it.

As of right now they are watching her closely and they’ve already had to reset her parameters on the machines as she keeps setting them off with a resting heart rate of 110 and a temperature of 95.2 degrees. Hopefully this is all we will see and she will do A-Okay and go from there.

Later tonight or tomorrow I will do another update but just wanted to do a quickie!

*UPDATE*

So that was how things were going earlier and things have stayed the same and changed all at the same time. Nothing to worrisome but she fell asleep and her heart went pretty low and then of course the rest soon followed, her face swelled up and she had perioral pallor while the rest of her face was beet red and hot and her temperature was 94.1. The nurse was paying attention at the desk and came in to see what was going on and got to see the swelling and redness so she made note of it to show Dr. Haun tomorrow. Now speaking of Dr. Haun I have to tell you, I really, really like him. I always get so nervous meeting new doctors with Alana 'cause there's no telling how it's going to go and particularly when we travel out of town there's no going back, know what I mean? I wasn't expecting to see him again while we were here but he came in tonight to see her and talk with her and he's so sweet and kind. I am really impressed. Even if he cannot figure her out for us I really feel like he's a special guy.

So here we are getting ready to go to bed and I don't know how much I am gong to sleep. She's restless but not in a lot of pain at this point (as long as we don't touch her leg). Tomorrow morning they will remove the bandage and we can get a better look at it. The surgeon did explain to us that it's not a pretty cut and it's all about where it's at and how it heals. It may "grow" with her for awhile because it's being stretched vertically along her leg. Such is life I suppose.

If she doesn't do this swollen face crap again tonight, continues to drink and eat enough to sustain, and her pain is undercontrol we should be able to go home tomorrow afternoon, and by home I mean the hotel. From there, again assuming that everything goes well, we will go ahead and fly home on Monday morning. I miss Noelani and Shaylin so very much and it makes me sad that they missed out on the giraffe's and the whole zoo trip. I don't know about anyone else but I remember when my brothers & sister would get something broken bone wise and I had never broken anything. I was also so jealous in that odd sense of the word because they were getting treats and extra attention and just being pampered. Now Alana's issues are no where near a broken bone, in fact they are much worse. I cannot imagine how Noelani and Shaylin feel about things some days. Even though she's in pain and in the hospital now, she did get to go to the zoo and go out to eat. Apples to oranges I suppose but when you are little those things are seen in the moment, not the quid pro quo of zoo=surgery.

Giraffes

2009-06-11T21:31:00.003-08:00

Okay, this will be a short update but to get the major stuff out of the way, surgery is tomorrow at 10 a.m. Seattle time, 9 a.m. Alaska time. The appointments were long today but productive. We met the surgeon who will be doing the surgery and he did reiterate what Dr. Haun told us; even if there is mito, 9 out of 10 they are unable to be diagnosed due to the unkown-ness of the disease even after a muscle biopsy. The muscle biospy is just the best way to look for it.

We did make it to the zoo today and she lasted through the giraffe with honors and managed to do well throughout the rest of the time. It was a sorta rushed trip and we didn't get to see all the animals but she really didn't care about them anyway. After that we went for her clinic appointments with the surgeon and GA clinic for the green lights for surgery and they were suppose to be 15-20 minutes tops but of course, once her history was asked it turned into about an hour each.

On top of going to the zoo though she got to meet Linda and Jacob! She was soooo happy to meet them, as were Larry and I, and had a blast. She thinks Jacob is the greatest thing since sliced bread and he is a pretty rocking kid. It was so nice to spend time with another family that faces some of the same issues we do and to see them thriving and to see Jacob, who has to use O2, be such a strong, smart, and witty kid. He was telling us about getting dirt in his canula after slidding into bases at baseball. Wow! They may have some limitations but they can still be such supa stars it's incredible!

Tomorrow I won't be updating until she's in her room and settled unless I really get a chance to so it may be mid-evening until that happens.

Forgot to mention this yesterday: Dr. Haun asked and recived our permission to include Alana into a research program that he is doing. He needed 20 kids and he said he had about 7. He is looking at other areas of mito. and gave me the information on it so I will post more tomorrow about it. To me it seemed him asking for her to be included was a sign, not good nor bad, that he thinks this a good possibility for a diagnosis. :/

Seattle V.2

2009-06-10T19:54:00.005-08:00

Well after a very long night we made it in one piece, at least physically! We arrived at 7:45 this morning and had just enough time to eat breakfast, take showers, and head over to Childrens for her first meeting at noon. Needless to say it was a very, very long morning/afternoon/evening. She's been so good but man, so tired. She has not wanted to walk at all and ate very little breakfast and lunch to make things worse.

So here's the good news, we met Dr. Haun here and he was so kind, patient, and attentive to our concerns! The appointment lasted 2 hours and he was very i depth in his explanations to us and it went rather smoothly 'cause Alana lasted through vitals and then she was out like a light! He tried to get her to wake up but she wouldn't so he let her be while we went over her history and progression. Here's the sum up of our appointment and what the plan is:

He did say that she has a multiorgan disease and this is evident by the lung, GI, muscle, neurological changes.
She is having the muscle biopsy for sure and we will find out the solid facts on the time tomorrow afternoon.
When those results come in, he estimates 2-3 months, she needs to have a brain MRI to see if something is going wrong that would account for the coordination, emotional delay.
She needs to have an EEG to look for seizures.
He said she has things that are mitochondrial in nature but lab work that is immune in nature so there's still no clearly defined etiology for her.
We need to get in touch with GI as soon as we get home to discuss her GI system, the tube issue, and really look at her caloric intake.
He is going to help us! He told Alana that he'd help "make her better" and that he "loved to hear her voice" and all of this made her smile, smile, smile!

She did end up waking up the last 15 minutes of the appointment and he did the physical portion of it. While she was sleeping she turned beet red on her hands and feet, eyes were terribly blood shot, she was shivering even though it was HOT in there, and she was saying her legs hurt her a lot. All of these things are not unusual in anyway but it was something I think is important for DOCTORS to see with their own eyes.

So it was a long day and tomorrow is going to be just as long. At 9 we are going to the zoo to meet with some friends before her appointment with the surgeon and GA clinic. She wants to go look at the space needle and seeing as we are 2 blocks from it I told her we could go over there tomorrow night after we get back and see how she's doing. She's so whipped that I she's been falling asleep every 10 minutes today and it got so back that she dozed while I was getting ready in the bathroom, fell backwards and hit her head on the tub. She's now got a goose egg to show for it! Agh!

I will do another update after tomorrows appointment 'cause that's when we get our surgery information. Much love everyone.

One more thing...

2009-06-10T02:24:00.000-08:00

Had to share this poem about having a child with disabilities...

c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

The wait begins...

2009-06-10T01:28:00.002-08:00

Well our flight is an hour behind, curses for not checking that before my parents left us at the airport at 12:30 a.m. It's now 1:29 in the morning and our flight isn't boarding until 3...1. more. hour.

On another completely sad note; Alana told my dad tonight that she's scared. If you know Alana then you know how doubly heart breaking this is. She is so brave and this is the first time she's ever admitted that she's scared. I had to turn away from where I was sitting so she didn't see me cry. There's nothing that can make her feel better right now except holding on tight and keeping her close. Alana doesn't talk to much about her health aside from saying she looks "like a freaky-goober" some days because of her canula and the asses who stare at her without smiling. Anyway--grandpa just hugged her harder and told her that that was alright. Everybody gets scared but she's the biggest and bravest.

I am whipped and unless I can muster some sleep on the plane, which I suffer from terribly insomnia even in my own bed, I will be up until at least 9 a.m. tomorrow (well technically this..) morning.

I will update when we get back from her first appointment unless something else comes up.

PS: I just wanted to thank everyone for all their thoughts and prayers for Alana. They mean the world to us in so many ways.

Today's the day...

2009-06-09T05:52:00.002-08:00

What a busy day yesterday was! I picked up the Sequel (a portable O2 concentrator) for our trip and I am going to have her use it today while we are around town to make sure we are all comfortable with it before we leave tonight. It's bulky and to be honest, I like her ladybug better!

She was so bloody cute yesterday when her friends James and Dylon came over. She hasn't seen them in a awhile and they brought her over a wonderful stuffed giraffe and the whole family prayed over it before they gave it to her so it was meaningful on so many levels. They played upstairs then went outside on the swing set and she was giggling and hugging them. She had taken off her O2 right as they got here...she thinks it makes her look funny, and for the 10-15 minutes that they were playing she was short of breath but refusing to settle down (I wasn't trying very hard to make her I admit) and as soon as they were gone and she put it on she was breathing a bit better but PT ended up being rough.

So today is finally here. I've got a few last minute things to take care of and hope to have a really good day with all of the girls. We leave late tonight so I will update again after her first appointment tomorrow afternoon.

“Each time we face our fear, we gain strength, courage, and confidence in the doing.”

Seattle--again...

2009-06-02T20:34:00.003-08:00

After all the ups and downs there is still things that have to be done and that includes the next big test she has to have. Every 6 months to a year Alana has to have an ECHO and EKG done to look at her heart. This is for a couple of reasons but the most important one is to look for pulmonary hypertension. When children, or anyone for that matter, is on O2 it is something that you need to keep an eye on and she in no different. I really like her heart doctor. He’s told me on more than one occasion I am Alana’s advocate, that if we are not making headway with someone to move on or make more noise until we do make some headway.

It’s nice when I can report great news here and so after having her ECHO and EKG the doctor told me the results right there, her heart looks great! Whooo HOOO! There’s no evidence of PH and everything else looks good. She’s still has her murmur and we are told to keep a close eye on the episodes of tachy and bradycardia that she’s having and if things get any worse or we notice fainting spells or other to bring her back right away. Otherwise we are good for 6-12 months. It’s so nice to have an appointment go smoothly and to leave with no thoughts of bad news to share. This was just last month so guess what?! We are here now, the present; crazy huh?

I’ve now been kicked back in my chair for awhile trying to think of where to start…she’s still having bad days, a lot of them in fact. To summarize this month in regards to her and her illness it’s been slow going. She’s been having fevers closer together and had a good set this weekend. As well as that she’s been really struggling with PT to the point that she was over 2 ltrs of O2 and still struggling to breath. I sit at poolside and watch her try with all her might to swim with help from a float or other and she cannot do it. Her feet and her hands no longer work in coordination with her mind. It makes me cry at night.

She went into McDr (yes, we are now calling her McDr, Dr. McArthur, according to Alana) today to see if she has developed pneumonia again because she’s coughing way more then she was even 5 days ago, she’s running fevers, she’s pale, and she’s needing O2 at higher flow rates. The good news is, that just from looking at the x-ray and not having the radiologist report yet, it looks OK. There are still infiltrates in her left lobe but they are always shifting and there. Tomorrow we should have the full report and will make the decision to start antibiotics.

We did talk about her increased lack of coordination, the fact that it’s been months now since the cough started again and then the biggie that she’s not gaining weight again. And in again I mean in the past 2 years. She still has not gained back the weight that she lost in October and December and in the words of the dietitian, her growth is “stagnant” all the way around. We’ve heard mention, from some of her other doctors that a NG or G-tube might be in her future but I’ve been resistant to think about it. I don’t know how long that can keep happening though. I was looking through photos of her way back in Craig when she was 3 and she has on this jacket that my mom made, well this weekend she was wearing that same jacket and it was still a bit big on her. *sigh* McDr said today that we might have to talk about this soon, like in a few weeks. If we do an NG tube and she gains weight then she will have to have a permanent g-tube placed to help her in the long run. Not something I want to think about right now.

McDr also mentions “progressive” again. I did it, I couldn’t help it; I started to cry. Please someone explain to me why I can’t seem to hear that word and not get upset?! I get it though, her increased loss of coordination, her increased O2, her increase in autonomic issues, her increased motility issues, the fatigue, the fevers being closer together, they all scream progression. Don’t cry just breath, right?

Another thing that I haven’t mentioned is that next week we leave for Seattle Childrens again. If you recall the last update I mentioned this and it’s now that time. Her appointments start on the 10th and we have no idea how long they will last. We are meeting with this guru on mitochondrial and other metabolic diseases and she will be having another surgery, this time a thigh muscle biopsy. No word on what day that is going to happen as we are meeting with the surgeon and the GA clinic on Thursday to see what they think about her going through another surgery. It will be while we are down there (don’t get me started on Seattle Childrens again!) is all we’ve been told.

So as usual we have a lot going on around here. Its summer break and the other 2 chicky-poos are home and I love having them around to myself during the days and taking them places with me. They are all giggling upstairs right now and its music to my ears! They are supposed to be folding their laundry but I think I will let it slide that they are not and in its place they are playing with each other.

Now that we are in the now, the present, the moment I will be doing the updates as they happen. The laptop is coming with me to Seattle so that will make things easy to keep current, also, here’s another link that I will be using while we are having “issues” or in-patient stays:

http://www.caringbridge.org/visit/alanastephens

On that page you can sign up for automatic updates whenever I post something. I will still always be on here, but it’s nice to have automatic updates when we are going through something big with her.

Seattle Genetics

2009-05-28T12:46:00.007-08:00

After confirmation of Alana’s new issues (low muscle tone, RR & HR issues, de-SATs, etc.) the referral to Genetics was pretty easy so on March 25th we met with Dr. Wallace from Seattle Childrens Genetics department here at the Alaska Clinic. Dr. Wallace was very nice, and seemed genuinely interested in Alana’s health and background. She did come in with a short list of things she felt Alana might have, Hyper IGD, Muckle-Wells, TRAPS, Mitochondrial disorder, and a few others. I was able to knock off a couple right away due to gene testing but a couple still remained, Muckle-Wells & Mitochondrial disease.

During the physical examination portion of the visit she pointed out things with Alana that we never really noticed, for instance, her eyes are a different shape then all of ours, her hair is sparser and coarser as well. She has a single palmar line on her hand (simian crease) were as no one else, all the way up to grandparents & aunties and uncles, do not, and a few other physical features. Then there were things confirmed that we did know, her hands and feet can be burning hot and red while her core is 96 degrees. She has rash “spots” on her body, almost eczema like in nature but do not respond to meds, she has mild over lapping toes and the list continues.

One thing that we were grateful for was that she noticed Alana’s teeth. They’ve been a concern of ours for awhile now as they are chipping away from the bottom up and pitted. The dentist keeps a close eye on her in regards to this, the technical term is Hypoplasia enamel and it’s just what it sounds like, low to no enamel on her teeth. The doctor thinks that this may be a clue as to what’s been going on with Alana all along but until more tests are done there’s no way to know for sure.

What ends up happening are 2 things, 1: Alana does a couple of special urinalysis’s to look at Muckle-Wells and Mitochondrial disorders. 2: If the testing for Muckle-Wells comes back negative then we will have a referral to see a mitochondrial specialist in Seattle and look down that alley. Here is some brief information on both diseases so you can see what we were looking at:

Muckle-Wells syndrome is a disorder characterized by periodic episodes of skin rash, fever, and joint pain. Progressive hearing loss and kidney damage also occur in this disorder. People with Muckle-Wells syndrome have recurrent "flare-ups" that begin during infancy or early childhood. These episodes may appear to arise spontaneously or be triggered by cold, heat, fatigue, or other stresses. Affected individuals typically develop a non-itchy rash, mild to moderate fever, painful and swollen joints, and in some cases redness in the whites of the eyes (conjunctivitis). Hearing loss caused by progressive nerve damage (sensorineural deafness) typically becomes apparent during the teenage years. Abnormal deposits of a protein called amyloid (amyloidosis) cause progressive kidney damage in about one-third of people with Muckle-Wells syndrome; these deposits may also damage other organs. In addition, pigmented skin lesions may occur in affected individuals.

Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common. Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.
Depending on which cells are affected, symptoms may include loss of motor control, muscle weakness and pain, gastro-intestinal disorders and swallowing difficulties, poor growth, cardiac disease, liver disease, diabetes, respiratory complications, seizures, visual/hearing problems, lactic acidosis, developmental delays and susceptibility to infection.

Either way you shake it they were scary things to look at. After waiting a few weeks the urinalysis came back and they were normal. Go figure. That did mean that we needed to start the next step, meeting with the doctor in Seattle about the mitochondrial disease.

While that is in the works things just keep on keeping on. Alana is working on another flare up and we are trying to keep her comfortable and most of all, out of the hospital. Her cough is increasing again, and the fevers are coming much faster and closer than they have in the past. She’s sleeping for 4-5 hours after PT and OT isn’t much better. She’s on 1.5 liters of O2 to function enough to do PT and OT but she’s still having incredible amounts of fatigue, not eating well nor drinking, mumbling and not making sense when she’s talking, de-SATing for no reason, chest rattles, rashes, red, angry spots on her tongue, and motility issues that are causing her a lot of pain.

Things stay this way and weeks go by with no change. Finally I bring her into the ped’s office and it’s just that, it’s just Alana unfortunately. The great news is that she’s able to stay out of the hospital and we just keep an eye on her. This works out well because she has an ECHO and EKG coming up as well as her patch testing for her allergies. Oh yippee right!

First is her allergy testing and this always makes me nervous. At the appointment I mention the bowel problems and pain she is having so Dr. Demain believes that we need to repeat the whole 21 food panel on her to make sure she’s not responding to wheat, milk, or soy which are known conspirators. This makes me even more nervous…what if she tests positive to those as well as the foods she’s already off; beef, chicken, pork, turkey, corn, carrots, rice, oats, eggs.

The patches go on Monday, off on Wednesday, and then final read on Thursday. No PT this week because they cannot get wet so yes, that also means no shower or bath for this water baby. By Monday night it was apparent that her back was itching and hurting. Argh.

Long story short the final read was typical Alana. Untypical. Dr. Demain could only think of 1 other child that he’d ever tested that was somewhat similar to what was going on. Great news, we tested negative to some foods. Bad news, she tested positive to some new foods. Here was the new list: beef, pork, wheat, soy, barley, rye, oat, & peas. Starting in 1 month we could pick one of these foods to try and reintroduce; rice, corn, eggs, chicken, turkey, & carrots. We could do one food, teaspoon a week over 3 weeks, and watch to see how she responds. *sigh*

You literally win some, you lose some. Up next; ECHO & EKG.

We are almost there...

2009-05-13T21:53:00.006-08:00

Shaylins birthday was wonderful! Agh, I cannot believe that she’s 9 now!! I don’t know about other families with medically fragile children, but I know for us it has truly affected every member of this house. While Shaylins birthday was a success it also showed us something we suspected was going on with Shaylin; she won’t rock the boat, she won’t speak up about things that she should. What I mean is she is all about trying to please us, trying to please Alana by giving into whatever Alana might be complaining about. Shaylins birthday was a surprise party a day later then her actual birthday was. We noticed that she didn’t even mention anything about not getting a gift or having a party. She was sad, trying to not cry, but just would not tell us what was wrong (even though we knew). Just trying to please everyone and not make a fuss, my little Shaylin.

Things do continue to change around here with everyone. We have been on the search for a counselor to work with us and the girls in regards to Alanas fascination with death, Shaylins inability to speak up and trust her voice, Noelanis unwillingness to acknowledge that she has a sick sister and all those other issues that have come with having not only a chronically ill child, but a child with no diagnosis and more testing then you can imagine. Things due work out finally though and we find not only 1, but 2 counselors who will work with us separately, and then as a whole unit.

In the meantime Alana needs to have another surgery. This one is to remove an enlarged lymph node from her neck that has been enlarged for years. I remember the first time we noticed it, it was actually my mom who did while she was rubbing Alanas neck trying to calm her down from a fit. We took her into the doctors right away but in the end they said it was just from an illness. After a few years and it getting larger with illnesses, larger than a grape, but no smaller then a blueberry it is decided that it needs to come out and be sent for pathology.

After meeting with the ENT, same one who did her tonsils, adenoids, and PE tubes, he asks us about the ear infections she still is having and the chronic fluid behind her drums. He tells me that when she comes back for pre-op in 3 weeks, if she still has fluid behind the ears she will need to have a second set of PE tubes put in. Okay, well wasn’t expecting that but not a big deal. I am more worried about her neck and the fact that she has such a hard time with general anesthesia. Dr. P goes over how this should be an outpatient procedure, she will spend about 2-3 hours in recovery and then should be well enough to go home right after that.

The day of surgery arrives and at least things are going along smoothly. We’ve been there done that so to speak and as long as the anesthesiologist will read her chart and band and see that she’s allergic to A LOT of tapes and stop trying to stick them to her for her IV we should be good to go!

Once the surgery is over they take me back to recovery to see her and she looks good. She’s still sleeping and they’ve got her O2 going. Right before the surgery Dr. P decided that he wanted her to go upstairs to sleep of the anesthesia and make sure she isn’t having any unexpected breathing problems and due to the fact that it's been hours and she is still sleeping.

Once we are upstairs, it again is only suppose to be for a few more hours, things do change. Sigh. She was sleeping and facing away from me so first I notice her SATs drop a bit, not drastic, but enough that I took notice. After a bit of this I go to move her and as soon as I see her face I see a red, swollen, patchy tomato lying where Alana once was. I don’t even move her but open the door and get the nurse in there within 30 seconds. They give her a push of Benadryl and call the doctor. Now we are in for the night for sure. Long story short, this happens all through the night, her SATs drop, her face swells and she has a pale ring around her nose and her mouth, and they shoot her up with Benadryl. By morning when it’s still happening they are starting to wonder if this is a G.A. or tape reaction or is this something else.

We spend 2 days in the hospital and have no idea what happened. She has done this before, the red face and pale ring around the mouth and nose, and it was after surgery. Hmmm another fish in the sea, symptom to wonder about. Once she is home it still happens over the next day and a half. We stop giving her Benadryl because it’s not helping it, what is helping is getting her up and moving around. It really seems to make at least the red and white patchiness go away. Again…hmmm.

Alana continues with PT and OT and as is now par for the course, things keep changing. After about 2 months of PT once a week the decision comes up to move her PT to twice a week in the hopes that we will see a better improvement? Stability in where she’s at? Something? Well the first session of the new sessions (say that 3 times fast!) ends up telling us a lot. Backing up a bit here, after each PT & OT session she’s falling asleep within 10 minutes of leaving each respective session and sleeping for 2-3 hours. Well after 30 minutes in her second PT of the week, Tuesday and Thursday, she was falling asleep in the pool. Well gee, that’s not good. After that PT she slept for almost 4 hours straight, was asleep even before we left the parking lot.

After that it was thought that we should move the PT down to 30 minutes for each session and see how she does; does she sleep less or is she able to stay awake longer? How is she through the session; is she able to function better both physically and cognitively? Well what we see is that she’s just back to where she was with the original 1 hour session in regards to sleep but…she is able to function through the session itself a bit better. What ends up happening is we stick with the 2, 30 minute sessions. It takes 1 1/2 L of O2 for her to be able to keep her SATs up (95%) enough while she’s in the pool but that’s alright, small steps.

Going back to the lymph node biopsy; the results finally come in from pathology and the cultures grew nothing. Go figure. On top of that the path results are the same, benign lymphatic tissue mass. It depends on the day on how I look at this, some days; we put her through another surgery, that didn’t go great, for no good reason. Other days, we ruled out 3 more things that were slight possibilities.

Can you believe we are almost to the present? As I’ve typed this over this past, well almost a year, there seems like so much but yet so little. It might seem like that’s an awfully long time to recount something but think about this--we’ve been on this journey for over 5 years now. There have been so many labs, 100’s and 100’s, CTs, surgeries, months of long illnesses. So, so, many tears, smiles, sleepless nights, and days of "are we going to make it through this"? There is more to come, we are almost there, to the present, but we are not there yet. Next we meet with Seattle Genetics to go over the “new” Alana. The Alana that we are at today.

PT & OT here we come...

2009-05-11T17:40:00.004-08:00

Things do continue in the same fashion as they have been, unfortunately. Her baseline is officially changed now from mediocre to crappy. On top of that she is having a harder time with shortness of breath, fatigue, and eating. Even at this point she has not gained back the weight that she lost over this last illness and she’s coughing more. Something new that does come up is a physical therapy evaluation. This is done for a few reasons, her fatigue, the evidence of muscle weakness and/or wasting, and her overall wellness level. We meet with a wonderful PT, Mrs. J, and after a few sessions with Alana she requests that another gal, an OT who works with children who have lung/breathing/heart issues sit in and give her input as well in regards to some issues Mrs. J is seeing in regards to Alana and her breathing issues. I am fine with this because heck, maybe she will notice something that can help.
Here is what they had to say in their report to the doctors:

“Alana is a bright and engaging 5 year old little girl” (okay, had to put that in here that that was the first thing they said =)

“Alana had difficulties coordinating co-contraction of her trunk for stability when attempts were made by therapist to elicit. Endurance is quite poor as a 5 year old child should be able to participate in an hour of sub-aerobic physical activity without immediately falling asleep upon cessation. However this lack of endurance doesn’t appear to be simply due to de-conditioning as Alana tries to be an active child. Alana appears to be a very busy active child with endless energy. However, it seems to this therapist that it is easier for Alana to remain in motion then to do stationary tasks that require core stability and subtle grading of muscle control.”

“Alana does demonstrate difficulties with coordination of her body following several minutes of sustained activity. At today’s evaluation Alana was propelling herself around the pool wearing floatation with nice coordinated movements of her upper and lower extremities initially. However, following 2.5 minutes of sustained activity Alana was no longer able to coordinate her movements and resorted to flailing type movements of her arms and legs to attempt to continue the activity because she wanted to”

“Alana demonstrates 85% open mouthed breathing but with increased demands she closes her mouth and creates a valsalva to increase intrathoracic pressure thereby increase her strength and stability. Her cough is non-productive with limited intra-abdominal pressure. Her mother reports that she will cough so long that she turns cyanotic. When asked to take deep breathes or after breathing increased for activity demands Alana demonstrated paradoxical breathing, this means that she activates her diaphragm on expiration not inspiration, limiting the volume of air she can inspire.”

Summary

Alana demonstrates:
1. Significant low tone especially abdominal muscles, also indicated by mild rib flares.
2. Mild to moderate dysregulated autonomic nervous system: indicated by inconsistent pulse, oxygen saturation levels not tied into activity needs, erratic temperature regulation and breathing pattern (several quick/shallow breathes and then large breath).
3. Inefficient breathing patterns. The diaphragm, intercostals, external/internal obliques, rectus abdominis are the predominant muscle in efficient breathing patterns doing approximately 60% of the work, while accessory muscles such as the paraspinals, pectoralis muscles, serratus anterior, scalenes, SCM, and trapezius are recruited for increased oxygen needs. Alana also demonstrates paradoxical breathing.
4. Possible undiagnosed venous return disorder. Red flags include constant movement without sensory seeking quality, desaturation without movement, and sitting down/flailing limbs with fatigue.

They did her SATs before and after PT and before (without O2) she was at 94%, after she was 86% and stayed that way for awhile. This was a very clear indication to us that she needed to be on O2 more than we had been putting her on it. This report went to her doctors not only here, but in Cincinnati as well. Within a day we heard from the pulmonologist in Cincy to discuss this “significant change in her status” in regards to her O2, muscles, and fatigue. Well HELLO! We’ve only been telling you she’s been having these issues for almost a year now!

Things do come of this though; Alana is put on O2 with activity and during PT and OT (OT was recommended as well due to her sensory issues—screaming, hands over ears, fight or flight reactions to car washes, some restaurants, music class, etc. and tactile issues to clothing and other), she is now going to PT once a week and OT once a week for an hour. The plan is that once her strength is increased that we will start working with the other OT in regards to her breathing issues to try and get her to utilize her lungs better, maybe in about 3-4 months is the goal.

I’d like to say I was surprised with what the OT & PT tell us, that she has the emotional maturity of a 2-3 year old, that she is hypotonic (low-tone), her O2 needs were greater than we appreciated, and that she needs long term, 12 months or more, of PT services. I would like to say that I didn’t see any of those things but I did. I just couldn’t seem to make them better for her—again.

There are still new things going on with her every month. It’s all day by day and minute by minute. She is such a strong and smart little girl, totally like her sisters in those regards. Rock stars the whole lot of em! We are finally to the end of February and we have Shaylins birthday in March to look forward to and boy, are we looking forward to some normal fun times! We all need a break and if all goes well Shaylins party will just be the ticket for that.

Our Christmas angel...

2009-04-29T18:04:00.002-08:00

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...

2009-04-20T12:09:00.003-08:00

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...

2009-04-05T15:21:00.003-08:00

“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

2009-04-03T08:59:00.005-08:00

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...

2009-04-02T20:15:00.006-08:00

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

2009-03-19T16:30:00.005-08:00

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...

2009-02-21T12:00:00.003-09:00

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...

2009-02-18T19:49:00.003-09:00

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...

2008-11-30T16:44:00.002-09:00

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.

Breaking stride...

2008-10-08T22:27:00.002-08:00

So I haven't been posting much these last few months as Alana has been having more and more episodes. They have finally come to a head and she's in the hospital again. She's down 2.5 pounds, seriously dehydrated, and her inflammation markers have tripled since Monday. As the days go on I'll update what we learn or don't learn. I am contacting the doctors that she's seen in Cincinnati (yep, did not get to tell you that part yet) to see what they want done as far as labs and/or any bright ideas they may have.

Will post more about the previous weeks tomorrow.

Random post

2008-09-07T08:39:00.003-08:00

Here is Alanas wordle. It's a collection of words from this blog arranged randomly. Pretty nifty.

Seattle Childrens

2008-09-04T19:44:00.002-08:00

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...

2008-08-13T12:23:00.003-08:00

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...

2008-07-25T12:22:00.007-08:00

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...

2008-07-16T19:25:00.006-08:00

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.

Here comes round 2...

2008-06-26T11:36:00.006-08:00

So where’d we leave off? Alana just did her first hospital stay and had her first surgery. We are at almost at 2 years of life for her. She’s such a trooper though. Through all this she’s still smiling, playing, and bringing joy to our family. She’s keeping not only Larry and I busy but her sister’s as well. They think she’s the doll-child and always want to hold her and play with her. Noelani and Shaylin are sometimes left out in this equations that is Alana. They are so good and understanding that it’s easy to forget that they are gong through this too. That they are seeing what she’s having done. Seeing the missed trips to the park because Alana isn’t well enough or the dinner out because she’s coughing so badly. The girls have so much compassion and strength in them that they make Larry and I look weak.

After Alana has healed from her surgery we hope and pray that this is going to be the thing that makes it alright. Having the tubes in will help with the ear infections and having her adenoids out will help with the coughing and swelling she’s having around her nose. It’s been almost 2 years now and it’s about time that she had a break from feeling cruddy you’d think. We did dream, and we did hope, and we did pray. The ear infections were cut in half! She was still having them and she still does but they are nothing like how they were. It was so nice to not have her on antibiotics for ear infections finally. And yes, on that note, her cough did not go away. We were still so very happy that at least one thing seemed to be moved outta the picture that maybe with time her cough would go away. It just needed more time to adjust to the new structure of her face right? So yea, the cough goes on and we go on and the world keeps moving around. She’s still in the doctors a ridiculous amount of time and we are still debating the use of antibiotics to try and clear what is a sinus infection? Cold? Flu ? Bad luck?

By July of 2006 she’s been sick again for months. Coughing, fevers, dehydration. Her CRP level keeps going up and they are not sure what to do about her. She’s now about 30 months old and has spent so much time sick. It isn’t long and she’s admitted yet again. Same diagnosis. Pneumonia. I remember being so upset that I could barley talk one night. Larry and I are fighting because things are not going well. Who do you take the most out of when things are not going well? Yea, your spouse or other significant person in your life. I was so very angry with the doctor at this point that I couldn’t see straight. This would be the last time this pediatrician was involved in Alana’s care. We were told that okay, now we can see that maybe this might not be normal even for a sicker kid who might just be unlucky and “catch” everything. We now say no, we want to see Dr. Demain now. This is it there is something wrong here and it’s not at all normal.

Dr. Demain comes in and sits beside her bed and tells us that now we are moving into a new area. It’s not just little tests anymore and the things they are looking at are dig deal immune problems. Okay we tell him. Better to know and deal with it than to not know what to expect at all. He then gets the ball rolling by giving her an adult dose of the Pneumococcal vaccination in her thigh. He tells us that the reasoning for this is that if her body does what it’s supposed to do then we will see redness and inflammation around the injection site. That means that her body is reacting to the shot/dose and there’s a sign that her body is indeed fighting foreign things.

So what has proven to be a consistent in Alanas life happened. It came back as a normal reaction. She did have a boat load of labs done at the same time and those came back normal for the most part with only a few things slightly eyebrow raising but nothing worrisome. T&B cell panel all within normal range, neutrophil oxidative burst: normal, 23 serotype S. Pneumoniae IgG: detectable but low, etc. etc.

This time they feel it would be beneficial for her to see the Infectious Disease doctor as well. He was a very nice man and I’ll give it to him that he tried. He ran a few tests and asked us a lot of questions about her and her past. He was very kind and told us that he would be grasping at straws to fit her into anything that he’d be able to help with. So again, we do our time and she improves. We try to remember that it’s not over this time and we are not taking any more of this “She’s just sicker than other kids” crap. We are now on the search for a new pediatrician on top of all the other things that we are trying to sort through.

Time goes on and she’s having good days and bad days. We are still seeing Dr. P. the ENT, and he’s trying to help us with her and come up with things that may help. She’s still not sleeping through the night and she snores on top of that. Given the size that her adenoids were we start talking about maybe taking her tonsils out. There is a relation to the size of one’s adenoids and their tonsils it seems so hey, maybe this route will at least work to help with the sleeping issue. If she could sleep through the night then maybe she will feel better and be able to stay a bit healthier in the long run.

October of 2006 she has just turned 3 years old! She’s a bright little bugger & is so used to seeing different doctors that at this point she’s calling them her “friends”. There were so many little things in the in-between that I’ve failed to mention. At 9 months of age she stopped growing. Just stopped growing and we were not sure why. She's not added failure to thrive to her list of "things" going on with her. Her bowel problems had remained consistent so there wasn’t any flair up in that area. She was just being how she’d been in the past. This went on until she was 18 months old (right before she went into the hospital). During that time we saw an Endocrinologist to see if there was anything in that realm that would account for what was going on with her. She, the Endo., ran tests for a few things like Turners syndrome & other genetic disorders, bone scan to check just that, her bone growth, and then a few other random things. Through all this Alana is just hanging tough. She’s sick for months at a time then better for a few weeks or even a month. I think she went 3 months without a cough or fever one time!! Through all this I think there was two different trains of though not only with the doctors but with our family.
They included:

1. She will outgrow this. You hear about kids all the time who have strange things going on with them and then one day *poof* they are better. No reason for them to be sick is ever found but keep the faith and in time she will show you that it’s going to go away.

2. That this isn’t a fluke thing. That this is the way that she is and wishing and hoping it away isn’t going to make it go away. Sitting back and just waiting for her to get sick again and then dealing with it isn’t an option.

3. And my personal favorite that we still hear off and on to this day "Well, she doesn't look sick to me."

As you can guess there were a lot of emotions running around by now. Every one of them was spoken with love and caring with no harm intended. It’s hard though when you are the one running to and fro for appointments. You are the one who’s always hearing that not only can they not fix it but they can’t even tell you what the bloody hell “it” is. No matter how strong your spirit and resolve are those things get hard and you can feel so lonely. Not lonely like alone in the wilderness but alone like you are screaming and people are just walking by, they can see you but it’s as if you are mute. That what you are saying isn’t important enough for them to take notice until it’s too late.

Taking a break from the past...

2008-06-23T20:25:00.004-08:00

So it's still tonight but I want to take the time to just talk. Reading Alanas story and the way that things were going and have been going may make you think that I've given up hope. That there was nothing good about her infancy/toddler-hood. This is not the case. Alana has been such a bright spot in our lives as well as the lives of so many. She's an extension of her sisters. The strength that they show. The love that they share. The hope that they bring.

We are so very, very blessed to have such amazing children. Nothing could ever happen that would make me want to change them. I know that reading through these early years seems depressing. That our lives were filled with nothing but doctors visits and hospital stays. And don't get me wrong, it was but there was also happiness. There was love and smiles and dreams coming true.

Nothing can ever take away the fact that we are so happy to have such amazing children. Things can get so bad but you know what's always there? Larry and I. We can make it and we do. We have such an amazing family. Not just the girls but our extended family. My parents, my siblings, our friends.

Please remember this, I know that I sound cynical and maybe even a little hopeless in my retelling of our journey but do know this and know it without a doubt. I love my children. I love my husband. I love my life and the things that I've been honored to receive.

Somewhere along the way...

2008-06-23T19:35:00.006-08:00

Dr. Demain felt that Alana was having sinus issues. Yes, she was a baby and sinus cavities are not formed and yes it’s unlikely that a sinus infection started in her when she was 3 months old but she had signs that lead him to think that. She had developed saddle bags across the bridge of her nose. She had dark circles under her eyes. Labs were done and she was having elevated white counts and CRP levels (C-reactive protein is a measure of inflammation on the body). His theory was that the cough was being caused by drainage dripping down the back of her throat and causing irritation. If we could clear the infection then she should be fine. This was going to be done by starting her on antibiotics above the normal amoxicillin (which we learn she’s allergic to) or z-packs. She had a series of x-rays done to see if the bitty sinuses she did have were compacted. I need to back up though; one of the things that I’ve failed to mention is that by 6 months of age Alana has had so many ear infections that I’ve truly lost count. That tied into Dr. Demains thoughts as well. If she was so blocked up then the ear infections could be a direct relation to her whole head issue. So onward and upward again. He starts her on stronger antibiotics, a 6 week course of Clindamycin. After that we will redo her sinus x-ray and hope that the coughing will stop.

Okay, alright, no worries…the coughing hasn’t stopped. I think she’s even managed to get another ear infection on top of all this. She’s continuing to have multiple things going on here. She’s falling further and further off the growth charts and she’s having rashes on her truck and legs. Her feet have started to peel so bad that she cannot walk; the skin is cracked and bloody. Her teeth have started to come in and they don’t look right. They are tan looking and pitted. We take her to the dentist and they ask if I used a lot of antibiotics while pregnant (no) or if she was a preemie (no, remember the “overcooked” comment). Again, now what the duck chuck is going on. We were seeing either Dr. Demain or her primary pediatrician every week. From her primary we were getting “Kids are sick all the time” or “We see children like this all the time in the office and they outgrow it” finally what became one of my favorites, “If you hear hoofs in the distance you think horse’s not zebra’s”.

All the while we are trying different antibiotics and different approaches. We’ve moved onto drugs like Ceftin, Biaxin, Sulfatrim, Predinsolone, Prevacid, Nasonex, and Albuterol. In a time period from January 2005-January 2006 she was on 17 different or repeat courses. Now tell me, where in the bloody hell was I?? Why didn’t I take more notice that my baby, my 16 monthish daughter, was on more drugs than most geriatric patients?? Yes I understand that again, nothing I can do, can’t go back, shouldn’t beat myself up over things that I didn’t know. The fact still remains that I trusted the doctors and they didn’t come through like they should have. My bad.

Now after 6 months of straight antibiotics things actually get worse. Alana was running a fever for a few days, nothing that unusual, but this time she was different. May of 2005 now and she seemed to be having a hard time breathing. We took her back to the primary and for the first time there is something very clear going on. She has gotten her first pneumonia. We were admitted straight from the primary’s office and she was given more antibiotics, more steroids, and albuterol. She tested negative for RSV, and other bacterial infections. She did however test positive that she had already HAD Epstein-Barr virus. Not that she had it then, but that she’d already had it and gotten rid of it. Now at the time the doctors chalked it up to a mistake with the lab. There was no way that an 18 month old had already contracted and gotten over EBV. Another thing that they take note of with Alana is that she’s again got an elevated CRP level. Normal range is below 12 (normally) and her’s is 30.

We do our time in the hospital and now both Larry and I are asking more questions. More detailed ones. How in the world could she have gotten pneumonia when she’s been on antibiotics for 6 months? How and why is this going on? We leave the hospital and are home for 2 days. She’s running a really high fever again and we rush her back to the doctors. She’s now got “otitis media with perforation”. I don’t know who cried more that day, me or her.

We spend the next months battling infections. Her skin, her ears, her body. We try more drugs. More time. More everything that we are told. The infection in her lungs doesn’t really go away now. She’s always coughing and she’s breathing faster with each little ear infection. She’s constantly miserable and we can seem to do nothing that will help her.

By January of 2006 we go and see an ENT on our own. He looks at her history, she’s at 30 or so ear infections and there’s been some mention that maybe her adenoids are large and causing blockage. That if they are removed, then possibly her sinus cavities can drain and stop the backing up of her face. The ENT we see is great. He feels that PE tubes would have benefited her awhile ago. He also believes that he can look at her adenoids while he’s in doing her ears and if they seem enlarged then he will remove them. We agree and in the middle of January she undergoes her first surgery.

I cried. I admit it. I was a wreck when they took her. She didn’t care. She was in la-la land with the help of some versed. I think when you are waiting for them to come and let you know that everything is A-OK time stands still. It’s so strange. That surgery was the first procedure that she truly had and it was the one I cried the most at. It had been such a long road and when they even hinted that she might be better after this. No more cough, no more ear infections. No more antibiotics. There was hope that this would end.

She pulled through like a champ. They adenoids did need to come out and they were large. Dr. P put the tubes in place and came out and told us how wonderful she had done. We waited to see her. And we waited. We saw kids who went in after her come out and go home and yet we waited. Finally after an hour or more they came and got us. It seemed that Alana didn’t want to wake up. She was quite fine in la-la land and was not to be disturbed. Well this wasn’t good enough for a nurse there. She got the bright idea to wake her up. Mentioning something like, “Oh sometimes they just need a little help waking up…”. Famous last words! Needless to say after Alana screaming her little head off for 45 minutes they decided that she was well enough to go home. Yea thanks lady. We had to listen to that screaming for another 4 hours. Again, looking back this showed us something with Alana that would come in handy in the future. If only I hadn’t been such a slow learner on that one though.

Home again

2008-06-20T09:49:00.008-08:00

And we return home…
We stayed a few days in Glenn-Allen for Christmas break then decided to head home a bit early. Alana was still having issues coughing but again, kids get colds. She had already proven herself to be different then her sisters and you hear that all the time. No matter how many children you have they may come from the same stock but they are each unique. We arrive home and make her a doctor’s appointment for the coughing and leave it at that. I take her in and yep, the pediatrician tells me that she’s got a cold. Probably caught it from her sisters. Take her home and steam her in the shower, make sure her head stays elevated and that will help her from getting the congestion that is sure to follow.

Looking back you can see through copies of the pediatricians phone logs how many times I call about this cold. This never ending cold. Hell, even looking back on it myself I think I was insane. We are just annoyed at this point. Aside from coughing all the time she’s nursing fairly well. In fact, she’s trying to nurse all the time. If there’s ever been a true point in my life that I felt like a cow it was then! She nursed every 2-3 hours and seemed almost never satisfied. She put on weight well and was in the 80% for a child her age. She did have reflux but Noelani and Shaylin did as well. She was having loose stools but nothing that was very worrisome at this point.

Time goes on and we’ve almost become complacent about her coughing. She’s now developing bags under her eyes. She’s not gaining weight all that well anymore and we have resorted to trying antibiotics. At 6 months old we start trying solid foods. We notice almost right away she’s having major bouts of diarrhea and becoming worse in general. She’s crying a lot and not sleeping well. She’s coughing even more than before and we are getting more and more concerned. The phone calls and visits to the pediatrician are ramped up. We finally get a referral to one of the kindest, most compassionate, brilliant doctors we have come in contact with, Dr. Jeffrey Demain.

Okay, so there are some things that we already know about allergies. Shaylin had a turbulent few first years as well. She was having diarrhea so bad that it was burning, literally burning, the skin off her bottom. Nothing virus related was coming back and she was failure to thrive when she was a year old. She had to have IV’s placed in the doctor’s office just to keep her hydrated. This went on for 2 years. She only ended up in the hospital twice, once for dehydration and the second for Rotavirus. We took her to an allergist who did the standard scratch testing but that didn’t reveal anything other than grapefruit, strawberries, and pork. This surely wasn’t what was causing all her problems. This were not things that were in her diet on any sort of regular basis so why/how could this be causing her so much distress. We continued to have problems. Dr. Demain is the allergist who we have been working with through all this and he’s just come back from The Childrens hospital of Philadelphia. I’ll never forget our follow up appointment with him. He was all smiles and very excited. He told us that in C.H.O.P. that he had learned about a new way to test for a different type of allergy. Not the standard type reactions that you get from scratch testing. You place this little food filled disks on people backs. They stay on for 48 hours. After that they are removed and then initially looked at. Depending on how many little bumps they see or feel it’s recorded as trace up to +3. Then another 24 hours go by and the actual reading is done. Again, following the same scale. Shaylin was one of the first handful of children that he had the chance to put these disks on. What it showed made a difference for Shaylin on such a level that she went from so far below growth to now at 8, petite but healthy. Shaylin has a disorder called Eosinophilic Gastroenteritis. What this means is that she has a type IV reaction to foods. Here is a clip from the American Partnership for Eosinophilic Disorders:

"A growing number of children and adults suffer from a chronic and complex group of disorders described as Eosinophilic (ee-oh-sin-oh-fill-ick) Gastrointestinal Disorders (EGID). These disorders are characterized by having above normal amounts of eosinophils, a type of white blood cell, in one or more specific places anywhere in the digestive system. EGID is further subdivided into organ-specific diagnosis. For example, Eosinophilic Gastritis means eosinophils infiltrating the stomach. "itis" means inflammation. While visual inflammation is not always present, inflammation may be apparent under the microscope."

Shaylin was +2 to +3 for Beef, Chicken, Pork, and Citrus. All things that were in her diet at this point. Orange juice, little Gerber beef sticks, diced chicken for protein. Once we eliminated those foods she was a completely different child within 8 weeks. Thank you Dr. Demain!

So how does this tie into Alana. Well aside from the coughing we thought Oh’ let’s get her allergy tested and she’ll be okay too. This really is just a super bad cold.

Looking back I personally feel so sad and ashamed that I didn’t do more to recognize that something was wrong. That I listened to the pediatrician that she just had a cold and she’d be better the next day, the next week, the next visit. Maybe will never change things but yes, maybe if I had been more proactive then she wouldn’t have to endure as much as she has now.

The allergy testing showed an Eosinophilic reaction like we’d expected. Beef, chicken, and pork. Dr. Demain was not content with her ongoing illness though. He now starts to point us in the direction that this isn’t just a bad cold, that 3 months of illness in a baby isn’t ever just a bad cold. And so begins the first set of testing and months of strong antibiotics.

And away we go!

2008-06-19T08:44:00.003-08:00

So where did we leave off. It’s almost Christmas time. My mom and dad are living in Glenn-Allen making it possible for us to visit them and vice versa. Christmas is a time for family to be together and we have no reason why this one should be any different.

If you know Larry at all you know for him being on time is being late. This means that when we were supposed to be on the road by 5 pm we were not on the road until 9 or 10 at night. Now for those of you that don’t know what it’s like to travel in Alaska in the middle of winter here is some background. Unlike the lower 48 where there are major highways lit up in the night with multiple options to exit onto small towns here, headed in that direction, there isn’t even street lights. If you happen upon a house it’s questionable to even approach as the thought is, if they live in the middle of nowhere do they really want to be bothered in the middle of the night? On top of all that you are along a mountain side with a chance of slides. Travelling snow covered roads in the dead of winter in the dark and in Alaska is not for the faint of heart. But I digress- we finally are on the road and away we go.

I remember Alana being fine because why, in my bloody mind, would we travel 3-4 hours with an ill 3 month old. We were half way to Glenn-Allen when both Larry and I were taken aback by this sudden cough coming from the back of the van. It was a very distinct cough and not one we had heard from our other 2 babes. That made us both take notice seeing as it was coming from Alana. We both assumed it was a onetime thing. Nothing to worry about right? A few minutes go by and again, here’s this terrible cough. By now I am looking at her car seat trying to see if she’s got her head in a position that is making it hard for her to swallow or breath. Larry and I are looking at each other wondering the heck is going on. This isn’t something in all our infinite wisdom as parents that we can remember hearing with the other 2 girls. Again, coughing, by this point I am scared and have Larry pull over. We are now on the side of the road, in B.F.E., -20 something degrees with a sick baby.

We do make the choice to go on. We have no real reason to fret. She’s not running a fever. She’s sleeping through this whole thing. She’s sticky sweaty from exerting herself during these spells of coughing and a bit red in the face but there really isn’t a huge red flag going up. I remember making it to my parent’s house and just being so tired. We manage to get everyone to bed and try to sleep through what’s left of the night. Tomorrow we will call the doctor. Tomorrow whatever fluke has taken place will be gone. There really was a time that I did think that everything would be okay. That there really was no reason to be worried about what had happened in those past few hours and that everything would be a-okay.

Okay now, in the beginning...

2008-06-17T12:52:00.007-08:00

Well in the beginning we created 3 amazing daughters. Noelani, Shaylin & Alana. All pictures of perfection. Blue eyed American girls. The quintessential dream come true. No space children here. You know the ones. Admit it, you smile and say how beautiful they are yet you wonder what type of alien they are.

I hope that with the creation of this blog I can keep you informed about Alana and how she's doing. How Shaylin & Noelani, the 2 greatest sisters that any little girl could ask for, are doing. And then tossed in somewhere along the way how Larry & I are.

Here's the background that you so need to understand how we got to this point of crude humor and twisted sense of entertainment. I am going to break this up into sections as it will be to much at once.

Alana was born in October 2003. She was a complicated pregnancy due to her inability to show us that she could move all her limbs and not be a stick in the mud. I think that she just liked being the center of attention. That she somehow knew in that little forming mind of hers that if she stayed really still, didn't move so much as a finger, we'd look at her. We'd spend hours gazing at her through a monitor, banging spoons on my stomach just waiting for her to honor us with a glimpse of movement. All and all she ended up being fine. We looked in on her twice a week for 4 & a half months until the day that she was delivered.

She was born at 38 weeks via C-Section. Upon entry the doctor commented on how "overdone" she appeared. Funny thing is is that she wasn't. We had a rather accurate date for her conception so we thought absolutely nothing of it. Looking back this was the start of a lot of things that Alana did that we just chalked up to Alana being different then her sisters.

She was a big girl, weighing in at 8 lbs plus a few ounces. She was red and rashy but nothing that gave us any big indication of what was coming in her little life. We stayed in the hospital for 4 days due to a few minor hiccups that I had going on. We arrived home on Halloween night just in time for the onslaught of trick or treater's. My mom and dad where here (thank god) to help with the girls. Larry & dad took the girls trick or treating and mom and I stayed at home with Alana.

Life was good. We had our 3 amazingly beautiful and smart daughters. Things settled into the routines that are the normal for a family with 3 kids. School, functions, sports, and chaos. Things stayed that way for a few months. Then came Christmas. Alana is just shy of 3 months old now. Here we begin a very long journey.

Why I am trying to do this??

2008-06-16T18:36:00.001-08:00

Well I thought that I'd be all witty and write my heart out about the girls and mainly what's been going on with Alana but given the fact that I can't get 2 minutes to think then I shall just try again tomorrow. (In between work, dinner, soccer, & bedtimes)