I think I can, I think I can, I think I can

I posted a status update of Facebook that we got the results back from Anabelle's latest sleep study. I didn't go to the appointment, but Kyle told me some numbers over the phone and they were awesome. 

But then I just sat down and really compared the numbers. And had to update everyone about how wonderful she is doing! 

Her previous sleep study was June 2012. Her last sleep study was December 2012. 
Here is the differences

                           JUNE          DECEMBER
Central apnea:        69               12
Mixed apnea:          24                0 (Yes, ZERO!)
Obstructive apnea:  216              2
Hypopnea:             580             38

Desaturations:        484            20

In June her oxygen saturation was 96.1% with a min of 52%. The time below 90% was 17.9%.
In December her oxygen saturation was 92% with a min of 65%. The time below 90% was 7%. 


There are more numbers listed. But these are the main (and most impressive) (oh and less confusing) numbers. 

They did say she needs to be on oxygen at night. Which she absolutely hates to the maximum. I tried to connect and get her all set up for tonight, but it's been so long it just wasn't working. There is a part for water (to have it not dry her nose out and keep it moist), but when I was connecting it and turned it on, water just shot up like a fountain. Alli was there to help me clean up though. :) 


But we are having a tech come by tomorrow to show us how to do it all again. Gotta count our lucky stars that it's been so long since we've used it that we've forgotten how to use it. 


While I'm at it...

We meet with her geneticist last week too. Nothing new. I guess he actually cancelled our appointment (forgot to tell us though) because the new tests he ran on her muscle biopsy didn't reveal anything new. And he has no answers or insight as to why what happened happened, or anything to suggests but to continue what were doing. Makes me feel so great to have put her through a muscle biopsy for that. But then again, if the news was different I would be singing a different song. 

We go to see Dr. Hall at the end of this month. She is a geneticist who has done lots and lots of AMC research and is supposedly the best around town. 


Ok. I'm not ranting and raving about how wonderful Anabelle is doing. 

AH! And Kyle took Allison to get her first haircut EVER. I'm still a little sad over it, but she looooooves it <3 font="font">

 This is her being a good. She's learned she can make funny noises with her mouth and tongue 

This is her 'Are we done yet mom?' look. I was just trying to get a picture with all of her gear, but she disagreed with the idea

 Gotta throw one in of Amelia too, just cause she's a cutie pie. 

With love, 
The Polks 
  

Pink!

I had a nice long (almost done) update all typed up, couldn't finish it because of life, so I saved instead... And when I get time to come back.. it's vanished. 

I apologize if I don't include everything now.. because time goes on and I just forget things :/ 

Man o man. Have we been a busy family lately. 

The most important update, one I am sure everyone is dying to know about. 

PHILADELPHIA! 

It was perfect! I'll start from the beginning. 

 <---- anabelle="anabelle" happy="happy" s="s" so="so">

We were flying out of SFO (San Francisco), which is about a 2 or so hours drive for us. We decided to drive to Richmond area and take the BART the rest of the way to the airport. All went well! We got there, check in was great. We had a layover in Denver. Oh and we sat next to a lady on the way to Denver that was SUPER sweet. And Ana smiled tons at her! Which is a huge improvement because she went (and sometimes is going) through a phase where she scream/cries at strangers. A little traumatized from doctors and nurses I think. We get to Philly, that went fine (minus the cab driver not knowing the city...) Go to our hotel. All is great. Ana did great sleeping somewhere foreign. 

We decided the next day to go to the hospital early to grab something to eat in the cafeteria before since we were in a hotel. We got to Shriner's about 2 hours before. We filled out all the registration papers and were told to go to clinic. I didn't think that the appointment would be as long as it was, so I think we can just grab something to eat after. Boy was I wrong. 

We got called back to a room early. We answered the same ol' medical history stuff. Waited. Saw Dr. Z (the hand expert). He was very nice! He knows and is willing to coordinate with the hand expert at our local Shriner's, whom we have no qualms with. She is actually helping us with our Miracle Flights paper work. He said that a muscle transfer will probably be unlikely for Ana. For two main reasons. 1) Taking good strong muscles from her legs to move them would only make her legs weaker, which doesn't makes sense, and 2) He needs a good nerve(s) to hook the muscle up to, which doesn't seem like it would happen or be easy with her arms. He did say to keep up the range of motion, and that he thinks some elbow splints would help with bending her elbows more. The more they bend, the bigger the possibility for independence. She might not be able to bring her hands to her face, but if she can get her forearm to a tables edge and use her weight to bend to her hand that's up on the table, then she could eat, brush teeth etc. I really hope that is an ok explanation. 

Then we meet with the PA (physician's assistant). Talked some more. 

Then waited some more. 

Then we meet with Dr. vB (lowers expert). He took measurements. How much she can bend (flex) and straighten (extend) everything; ankles, knees, hips, wrists, elbows, shoulders, head... We talked about different options. Explained what we have done so far to him. Never once did he say 'Let's try to get her knees to bend to at least a sitting position.' We decided serial casting would probably be a flop since she's kind of plateaued. So he thinks that AFO's and KAFO's will be better for her. So he put her in plaster casts until the braces are ready for pick up (1 months time). The AFO's will be for her to wear during the day, the KAFO's will be night time. The KAFO's will have a lock on them. This will allow us to flex her knees at night and lock them, and gradually over time keep flexing. But this also gives us the freedom to remove the braces and give her baths, continue therapy, and paint her toenails! For further down the road.. he likes to do hip surgeries around 1-1 1/2 years old. We didn't talk in too much detail only because we were already approaching the 4+ hour mark. But in short, he recommends doing a surgery that just nicks the tendon on her hip that is keeping her from laying flat. I have no idea what tendon, or if that has a name. He also talked about doing a hip osteotomy. This one is big and scary. And we will need some time to talk about it with him more. 

Oh and he took x-rays! I'm not sure why x-rays weren't taken locally before treatment started. I think we were just so excited to finally be getting ortho treatment that we were in a daze. But after a while we asked and were told they are not needed. Ok, not going to focus on the negative. He took films of Ana's ankles in a few different positions, her knees fully bent (her fully bent), fully extended, and of her hips. We learned that she has oblique talus. To my understanding, her ankle bones are in socket, but not perfectly. And he found it interesting. He thought she had vertical talus. So interesting to me that this was found, and treatment might differ, because of a simple x-ray. Sorry, I got sidetracked again. I mustn't dwell on the faults of previous doctors. And they point a little downwards. Her right foot arches too. Her knees look fine (need to ask about the patella, totally forgot), and her hips still look great and in socket. YAY!

I just have to say. The ENTIRE staff is amazing! I have tons of little stories. One family has a bunch of kids, two AMCer's, were there, and they also have a brand new baby. BRAND new. So while the kids were getting films (I believe..) the check in gal held the baby for the family! The PA helped us get subway info for our way back to the hotel (but we ended up cabbing it due to time). When people entered the room they didn't just sit and stare, they introduced themselves! Explained their role and why they were there! The entire staff offers their help. If I have any questions they make sure I know how to get a hold of them. And when I call/email, they RESPOND! In a timely fashion to top it off! Oh, another big plus... the doctor does the casting himself! And it's a single bed cast room vs. a 6 bed open curtain cast room where you hear the kid next to you screaming! Oh, and they offer you their left over pizza from their pizza party! Which was a life saver since it was after 5 and we hadn't eaten. 

It was so nice meeting other AMC families as well. Seeing their kiddos! We got to chat with a few different moms while waiting to get fitted for braces. 

Ana on the other had was not a happy camper. She was wanting sleep and was done with people touching her. Little did we know she was also coming down with a cold. 

I've heard Dr. vB's casts are painful, so I packed and used Ana's Tylenol with Codeine. 

So our flight home. It was ok.. I felt bad for Ana because she hated her legs being touched, and felt bad for those around us because she was screaming. During our layover in Denver a wonderfully nice lady came up to us. She asked if Ana just had surgery. Oh man, here we go. Explained no, AMC, and what were doing. Luckily Ana was sleeping. Surprise! The lady has a friend with AMC! YAY!! She asked where we were going/coming. Explained that as well. She was floored! Her friend has raved to her about Shriner's Philly, and how great their doctors are. So any adult AMC'er who had a friend in Denver on 11/6, I'd love to get in touch with her again! Very nice lady!!

We get home and Ana is feeling too great. We also had a nurse the day after we get back who was shadowing our regular nurse. But this nurse wanted DETAILS. We don't have everything in writing as most things are a "you try it out and figure out what works at home" kind of thing. Plus we didn't know she was coming, plus we had a doctor appointment. At the doctor appointment Ana spits some brown up. Weird. She has nothing in her stomach, especially nothing brown. The doc says to start an oral med again, but lowers the dose. It has to be oral because it coats the stomach lining, and if it goes through her tube then it won't get to her stomach. So at home I try to give her some. The new dose is .25 ML. 5 ML's equals roughly a teaspoon. 1 ML is about .2 teaspoons. So 0.25 ML is nothing! To us. To Ana it's tons. She chokes, gags, doesn't like it, doesn't know what to do with it. Then she throws up. Big time. And all brown. Off to the ER. I'm not waiting this out. They suspected a bowel obstruction. So they admitted her, to the ICU. Which means constant Pulse ox and monitoring (more wire when holding her, blah). Come to find out I was just giving her too much Tylenol with codeine and she was sick. I was trying to treat the symptoms of the Tylenol with more Tylenol. Mom of the year award goes to me... 

Finally four days later Ana was finally smiling again! And back to herself, thank goodness! 

OH. And we got the MRI results, but haven't had the follow up. So I'll update when we do that in the beginning of December. But nothing huge was found (at least from what I can read...)

We have a follow up sleep study next month too. 

Fun time!! Pictures! 

 Halloween- Tinkerbells!

 

Pooped after getting casts! 

Happy to be home!!

My three babes <3>

Amelia hugging her booked and 'sleeping' in Ana's bouncer :D

Until next time - 

With love, 

The Polks

The small stuff

What a week. Poor Anabelle has had eight, yes, eight, appointments this week. I'll give a brief summary of the major ones.

Sleep study/back to pulmonology: We did another sleep study. It was horrible. They were trying to jam two cannulas into her little nose. One to measure the co2 output and one to give her oxygen. After several failed attempts, they just did the co2 cannula with some blow by. She was uncomfortable the entire night, was screaming every 5 minutes. Finally we both got about an hour of solid sleep after they started the blow by. We went back to the Pulmonologist. I guess they finally took it serious because we got to meet the actual doctor this time. He said her second study was better, only about 110 apneas. (Not sure if that is total or just obstuctive apneas). However, it's still dangerously high. We started her on 24/7 oxygen today, 2 liters. She hates it. I think she's warming up to it a little now though, she's sleeping! But he said we will repeat the sleep study, and if that doesn't work then try a CPAP, repeat, then if that still doesn't work we will discuss a trach. This doctor really understands what a big decision it is, and told me how we will make the decision together. Very comforting. Hopefully the oxygen works though! We will repeat the sleep study next week to see where we are. Oh, and he said if this continues long term it could lead to brain damage because she only breaths about half the time. Ah.

Physical therapy/Shriners: We had our first visit with outpatient therapy (not with Shriners). They showed us some stuff, mostly what we already know and do. We also meet met with Shriners hand specialists. She gave us some stretching excersises, and said we'll regroup in 3 months to see where we are at. But good news is Anabelles range of motion is great! She can flex her elbow's a little more then 90 degress, and extend about 30-45. She explained how Anabelle is a little backwards from typical AMC kids. I guess they usually see then with extended arms and flexed legs. But that's Anabelle! :D Nothing is 'normal' when it comes to her medical care. *sigh* But she said she is too small for splints right now, but maybe in 3 months we will try them. Also, she said she doesn't want to be too agressive because Anabelle will show us what she can do and needs help with, from there we will see what we can do to help her. She doesn't want to do something now that will damage her later.

GI: Since her reflux is still horrible, we have switched her to some RX formula. We have three to try, one a week. In three weeks we will follow up with GI and determine if anything helped and if not we will switch to a J-tube. It will help *hopefully* with the reflux, which in turn will help with her breathing. I love her GI. She doesn't fool around. She gets things done, and doesn't make us feel dumb when we have silly questions. And she has lots of energy. Maybe a little to much at times for this sleepy mom. I just pray this works!


Other than that, little Anabelle is just too cute! She smiles all the time now! And her big sister love love love her!!
Kyle and Amelia had a conversation the other day. I wasn't there so I could be misquoting.
Kyle - "You know Anabelle's special right?"
Amelia - "Yeah, like a present!"
Kyle - "Do you know what that means?"
Amelia -"Yeah, it's good!"

Melts my heart. They don't see her any different. Honestly I tear up when I just think about them together. Amelia wanted to cuddle with Ana, and actually sleep with her! Well.. I couldn't let her do that, but she just cuddled as long as she could. Ah! Got to love my kiddos. They remind me of what is important in life. <3

With love,
The Polks

Trach?!

I have known for a while now that Anabelle doesn't breath as well as she should. I guess I never knew how bad it really was until they gave me some numbers behind it.
I just got back from our Pulmonology follow up. They had the results from her sleep study. And the results made me cry.  She started off easy... Anabelle has 5 central sleep apneas, 1 mixed (I forgot what this means), and here are the real kickers, 236 obstructive sleep apneas, and 224 hypopneas. Her miminum oxygen saturation was 40%... It should be 100%! Her total number of desaturations was 480 for the 9 hour study.

Then the really bad part came. The doc asked if I have ever heard of a Tracheostomy, or knew what it was. Going into the appointment today I had two fears. One being they would put her on oxygen. The second, larger fear was they would want to do a Tracheostomy. I've learned when the doctors ask if you have ever heard of a procedure or surgery, they are leaning towards recommending it. Highly recommending it.

Not only is this another surgery for Anabelle. But how the heck are we suppose to handle a trach at home!? I don't know the first things about trachs! Obviously we want what is best for Anabelle... I just don't know how this is going to work.

Tomorrow night we are repeating the sleep study with 1-2 liters of oxygen, they are going to review, and then we have an appointment on Thursday with the actual Pulmonologist. These past two times we've meet with the NP, who has confered with the doctor. I am praying that all she will need is some o2 while she sleeps and wal-la!  She's fine. I have a gut feeling that won't happen. I had a gut feeling about the g-tube, and I have that same feeling again. Just reading other kids stories, and the similarities.. It's just tough.

Well, we will keep you all updated on what happens this week. In all we have 8 appointments now! Wish us luck!! :D

With love,
The Polks

Sleep study

Last week we were scheduled to do a sleep study. They want to do it to check her breathing. Last week the day of Anabelle's appointment, we were at the ER because of her G-tube. I called to reschedule it and they said the next available was on the 5th, of July... Then yesterday morning they called and said they had an opening that night. Yay! I regret not taking a picture of Miss Belle during the study. She looked like she was half robot. She had two wires stuck to her head, three on her body (leads), a strap around her, a pulse ox, and a nasal cannula. They didn't give her oxygen through her nasal cannula, they just monitored her breathing through it. 
I tend to obsess over numbers on the machines. The one that monitored her in/out breaths should have been between 8-40, depending if she is breathing in, or out. Well... At times it would be 0. And stay at 0, for a while. Now, I'm not a doctor, but I know that's not good. And a few times it was high, above 40. The tech said (before we started) that she'd be surprised if she got above 25. Great. It takes about two weeks to get the full results. The pulmonologist has to read everything from her study, which was about 10-12 hours straight. I was impressed it will only take 2 weeks. I read an article warning to be prepared to wait for 6 weeks. 


On the plus side, she seems to be a happier baby! She will smile more, coo more, etc etc. It's wonderful!!! She is so visual and loves looking around. If we hold her, she will move from side to side looking at stuff. The older girls get too excited sometimes, and will talk to her and want her to look at them. But, they will do all of this about one inch away from her face. So naturally she dislikes it and looks the other way. They tend to get offended and ask why she won't look at them. But it's been really nice. They love her so much! Jealously will pop it's ugly head occasionally, but mostly it's them coming over to give her a hug and kiss. Amelia will whisper secrets into her ear. Honestly. Secrets. I can't hear what she says, and she won't tell me when I ask. It's adorable. They sing their made up songs about how they love her. Couldn't ask for more precious moments :)  

Here is a video of Anabelle smiling and talking

                                           

I feel like we *might* be getting into our groove. We still have no routine, or even a glimpse of a routine. But things are getting a little better. I didn't believe all the other moms when they told me things will get better with time, but thankfully they were right! Thanks AMC family! I couldn't have got this far without their help!!! 

Speaking of AMC... Just in case you missed it, AMC awareness day is June 30th. So please wear blue and help spread awareness. There are a few really cool sites where you can purchase AMC awareness gear. No only do you get cool stuff from it, but it helps raise money for awareness. Can I say awareness one more time? I don't think I've said it enough. 


Honestly though. Before Anabelle I has NO idea what AMC was. I don't think I could have pronounced it or even tried to spell it before hearing and seeing it hundreds of times before hand. I have learned so much since Anabelle has been born. I have even been lucky enough to educate some doctors! The doctors who followed my pregnancy (four perinatologist) told me that the combo of how her legs were and Gastroschisis is something none of them have seen, most being in the field for over 30 years. Then I find out that yes, it's common for AMC kids to have intestinal things go on. Oh, and just a little more education on AMC.... I think this helped me understand how to explain it better as well. 

(Click here if it's to hard to read below)

Off to bed, finally :D

With love, 

The Polks