Moving forward...

Things have been rather busy here. As anyone with children knows things change all the time. Birthdays come and go and then they come again before your ready for them. Noelani is now 18, how the time does fly. You should listen to your mother about that, amongst other things. Shaylin is turning 14 next week and has blossomed into such a kind and gentle young woman. Alana has remained Alana. This next batch of photos will show some of her latest journey with what happened her last surgery, placement of a central line.

Long time no see...

It's been longer than it should have. What can I say, a lot has changed, a lot hasn't. This is going to be a story through pictures. There will be jumps and skips through this and it'll take me a few days to get them all uploaded.

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)