This is going to be a long one...

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...

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...

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...

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!

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?

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!