Why expect more...

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...

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...

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...

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...

(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...

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...

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!!