Technical Difficulties

Sorry about the delay, again. Our oldest (and my favorite) laptop finally succumbed to the virus', and our newest (and the one I almost loathe) had been stepped on, so it's almost impossible to use. Luckily, I can pull some MacGyver tricks and use it. It just drives my slightly batty.

There's been a lot in the past few weeks. We took another trip to Philadelphia. It was just a follow up, to be sure all our plans for surgery are still on track. This appointment, although short, was important. We had a year break, so the doc wanted to be sure the surgery was still needed. A little refresher on the surgery. It will be to nick the tendons on top of the hip to help release those contractions, putting her in a better position to stand. The end result of the appointment was mediocre. The doc is pleased with her progress, and we will hopefully return July/August for surgery. We'll stay for a week; do some therapy and get her fitted for new braces before the surgery, have the surgery, then come home. Then return in a month to get the new braces and her casts off. All in all, it was a nice, smooth trip.

She also had a few other appointments with doctors here at our local Shriner's. Anabelle has a follow up with an ENT for her torticollis. He also is pleased with her progress, and said she should be able to avoid surgery. Which is great because I didn't even know that was in the cards! It's still very noticeable, especially when she is tired. The other appointment was a follow up with her upper extremities ortho. We talked about possibly doing dorsal carpal wedge osteotomy on one of her wrists. It's been decided after talking to wait and see how she is mobile after her legs are taken care of. It may be better for her to leave her wrists as is if she does use a wheelchair later. It'd be easier for her to transfer with flexed (bent) wrists rather than wrists stuck in extension (out straight). We plan to follow up in another 6 months. She also had a photo shoot so we could collaborate with the upper doc in Philly and see what he thinks. Luckily Ana has an amazing occupational therapist who accompanied us to this appointment. It helped to have someone there who spoke the same language as the doctor, and convey what we really wanted to. And just like the two doctors before, this one was just as pleased with Miss Bear's progress. We also got to show off Ana's WREX, although her doc didn't seemed to impressed. She didn't even ask for a demo. It's ok, Ana's still getting use to it.

Feeding therapy has also been in the picture lately. Not sure if I mentioned it before. Her therapist is happy with how in love with food this little one is. We are still under the directive to not even attempt to replace nutrition in any way with oral feeding, but with how much this girl loves her food, it's hard not to think she's getting tons of nutrition out of it. Looking back, she really has come so far with her breathing and eating. It's amazing to see.

Until the next time I feel like pretending to be McGyver...

With love,
The Polks

Helllo 2014!

Hey there! It's me, Kayla. I know, it's been a while. If I thought we were a busy family before... Let me take a moment and catch you up!

Miss Anabelle just turned TWO! I still can't believe it. She's such a big kid now. She talks and understands more than I am ready for. This year she was able to eat her cake! Although, she wasn't interested in the cake as much as she devoured the icing. 

                  



I will (mostly out of need) to keep it brief. I find myself starting an update, getting distracted, forgetting what I have already wrote, then just start over. I've done this about 8 times now...

In brief... Anabelle is eating orally now! She's still working on certain textures, but the desire is there. She loves eating. As apparent in the photo above. She does still eat through her feeding tube, which is a G-tube now. We have switched from a formula diet to a blended foods diet. At first, we were really crazy and counted calories and milliliters and charted and went crazy. Then meet with a nutritionist. She advised us to add protein and just put whatever we feed the older girls into a blend, and wa la. We have a 2 week follow up in 6 days. She's already gained 2 pounds. In just over a week. After 8 months of nothing... It's pretty spectacular. 

She's a scooting pro now. At times she does get frustrated because her sisters can outrun her. We are practicing with a power wheelchair in physical therapy, and she's doing alright. She's also practicing with a gait trainer. It's a tad too big for her still, so the focus is to get her comfortable in it.

Also.. Ana is now the proud owner of an exoskeleton. Her's is for her arms. You may have see an article circulating around.. and it's similar to that. She is also a work in progress with using this. Tonight she actually scooted over to it and asked for her arms. Then got distracted and changed her mind to shoes. 

Speaking of arms.. I recently spoke with Anabelle's uppers doctor. We talked about surgery. The surgery is called a dorsal carpal wedge osteotomy. She would also a procedure to help take Ana's thumb out of her palm a little. We have a follow up next month, so I'll post more then. 

Oh, and boy does this kid have attitude. She is very vocal. Sometimes she may not being saying words necessarily, but she will make sure you know how she is feeling. I feel she is getting frustrated with not being able to always communicate her needs, or do it herself, so she becomes Monster Ana. She actually had her first time out last night because of it. I mean... you think she's so small and cute and nice... just wait until you are in the path of something she wants. Let's put it like this, she has no in-between. It's high or low. Happy or Freak Out. The terrible twos have hit the Polk house yet again. So, it's something we're working on. 

Amelia and Allison are really showing more interest in her. Before their interest primarily laid in helping with the baby, not so much in the baby itself. Don't get me wrong, they still cuddled and loved her. But now they play with her. They are including her and realizing knows what's going on now. All of this is just age stuff. And she's finally caught up to where she's realizing (to a certain point) what they are playing, and plays along. It's wonderful to see the bonds they are creating. 


And that's a 'brief' update. I will post again on Sunday. If I don't, please hold me accountable! I am so easily distracted, it's not even funny. 

With love, 
The Polks

Tooth fairy, surgery, friends and more!

Let's see.. where to start.

Last post I mentioned we were moving. Well, we've moved! We love being closer to family, and not to mention back to a slower paced town. It helps reminds us to stop and smell the roses while we are running around like chickens with our heads cut off.

Also, Ana finally had her procedures/surgery that we were waiting over 6 months for. About a month ago she went in and they put Botox into a couple muscles in her neck, removed her adenoids and did a bronchoscopy. There was a different doctor doing each part. The Botox doctor was first and quickest. Then the bronch doctor. He came and talked to us after he was done. He said that everything looks great. That even in deep sleep she protects her airway. And there are no missing pieces, and it's all in working order. So the issue is she just needs to learn to coordinate closing her airway while swallowing. Then the adenoids doc came in a few minutes later. Kyle had stepped out of the waiting room for a minute. The doc came in holding a specimen jar. Wait. Let me digress.

For this big day, we had to get to the hospital to sign in and wait around very early, about 6:30. For the Polks, that's awfully early. So while we were waiting, we were playing around with Ana. Kyle was holding her upside down and we noticed how her top tooth is finally coming in!

 

 

Who knew that fuzzy, upside down, can't even really see it picture would be the only one we'd get with that tooth inside her mouth.

 

Let's continue. The adenoid doc came in, holding a specimen jar. I thought maybe he was going to show me how massive her adenoids were and that's why she would get stuffy at night and sick so easily. But he looked as if was wearing sandbags around his neck. Heavy. Then I got a little panicked. Just as we were walking back, Kyle walked in. We followed the doctor from the waiting room back to the holding room. He paused. I feel as if I blurted out "Is she ok!?". He calmed my fears, letting me know she did great, but is down a tooth. He accidently knocked out that previously mentioned tooth. Then handed over the specimen jar. It held her tooth. Not adenoids. He explained how he had the gag(not sure what that is) in position to hold her mouth open, and it tipped and pulled her tooth out. It wasn't a clean pull either. A little flab of gum got ripped and pulled too.

 

 

It's a small thing. She's fine. She recovered wonderfully. Although those first few hours were horrible because she wasn't feel good, and her only soother AKA pacifier couldn't be used because of the tooth incident. But she woke up the next day with a big toothless smile on her face.

Even though it's small and she's fine, Kyle and I feel slightly robbed. It's just another unnecessary baby thing taken away. Her first haircut was in the NICU. And it was the whole side of her head, which wasn't even used. And now her first tooth was taken before it ever really came in. Ok, just had to throw myself a small pity party:)

 

But in closing, the doctor feels horrible! Right after I said it was ok, things happen. And he said "No. It's unacceptable." What do I do with that!?  Things do happen, we understand. As long as she is doing ok, we're ok. He checked on her in recovery (which is rare), called me later that night, and continued to apologize at the follow up.

 

It's been pretty quite around here. Just trying to get things in order, line up appointments and changing things to our new address. Which is proving more difficult than thought with some of her services. We also decided to nix the in home nurse for now. So Kyle is being super dad and juggling the home and appointments. We're also still trying to get her to tolerate things through her G tube. Remember, she now has a GJ tube, so there is a part for each. She's still getting a majority of formula through the J. We're also going to be introducing some blended foods through her G too. I'm just still nervous, but I need to just go for it! I've heard wonderful things about blended diets and how people flourish on them. I'll keep you updated on how it goes.

 

We also just went to our first AMC annual conference! It was amazing. Some kids didn't really want to play with Ana since she's not mobile, and I think it motivated her. She was able to see how these other kids scooted around, roll around, wheel around, etc. And since they didn't just stay by her and play with her like her sisters do, I think it pushed her to get mobile. She's a pro and pivoting, and she's starting to slowly scoot! I need to edit the video and post it.  I'll have another blog about how wonderful it was, in detail.

 

She also is very interested in food. We're scheduling another swallow study (and sleep study), then moving forward from there towards orally eating.

 

It's been a crazy few months. I'm promising myself to keep the blog more updated. I really do apologize!

 

With love,

The Polks

Quick update

I realized that I haven't updated as much as I should! In over a month actually... I apologize..

What's new with Miss Anabear?

Well, she's learning to use her arms to the best way she can. She will swing her arms and knock things over, or use her mouth.
She's still working on scooting. It's kind of hit a wall, so we're talking with her therapists about getting something to help.
She still refuses to say 'mama'. Even though she tries. She looks intensely at our mouths as we say it and tries.

When we got home from our last Philly trip we had to change her tube. I think I updated about that. NIGHTMARE! Then about a week ago the tube broke again. Now, Anabelle has/had a straight J tube. And the size she had only came in a tube that had an external portion, 'high-profile'. Like the picture below. Except (obviously) that's not Ana, it's a photo off of the internet. Hopefully I don't get in trouble because of it. :)

So when it broke for the second time in roughly a month, I was done with it! We took her to the ER, because it was after hours. I had the ER call her GI doctor, because I wasn't able to get a hold of her. Finally we talk, and come up with a plan. We switched her to a low-profile GJ tube. The picture below isn't wonderful. I'm not sure why there is an apple in it.. But I hope it makes sense. The very top (where the apple is) is what lays against her belly. Then the balloon (the bottom of the apple) is what is inside her belly, along with the rest of the tubing. That way the only part sticking out is flat. It's wonderful. And since the main issues with the straight J-tube are that they broke on the outside, I am hoping this is much better. Plus, we get to see if she will tolerate food through the G-tube again. We haven't tried it yet, because she's been sick, but it's an option!



We meet with her GI last week. She isn't gaining enough weight. She has a registered dietitian (RD) that comes in before the doc, and we discuss options. It's nice to have that available! She said she'd like Ana to be gaining around 15 grams a day, and she's around 2.5. So she's plateaued. Which is difficult because she doesn't tolerate a high volume of food. And in a larger picture it's frusterating because I'd love her to be on a blended diet (BD). Which is literally food, blended. Then we push it through a syringe to feed her, rather then being on a pump. But we can't BD feed her through her J tube, per her RD. And I'm nervous about trying to feed her again through her G. So we are waiting until she is better to try it out and see if we can't get her on a BD through her G. That way we can add more calories, she won't be hooked up all the time, she's getting REAL food.. just good all around.

Let's see, what else. We are STILL trying to coordinate a procedure. Well. Procedure/surgery now. We're still waiting for them to get their stuff together and do a bronchoscopy and the Botox injection in her neck. We had a meeting with her ENT doc, and he said he agrees that removing her adenoids would be beneficial. So now we're adding that on to it. It's a lot, but it's not the easiest for her to be put under multiple times.. I feel bad for doing so much to her at once, but the risks are less if it's once rather then three separate times. Plus, she gets put under so much already, why not try and combine? But we've been waiting for just the bronch since around December.. so we'll see. I've expressed my frustrations plenty, I just hope they understand.

After we do the all of that, next step depends on the results. If her Pulmonologist thinks that we can move forward with another swallow study, we will. If not, then we discuss. I talked with her ENT, and if the reason she aspirates is other than her just being underdeveloped, then it'd be surgical. And if it's surgical, most likely it's be very invasive surgery. We're hoping not go have to even discuss it further than that. We're hoping her muscles are just immature and need to develop more.

But boy oh boy does that girl love to taste food! A few nights back we had a lazy pizza dinner night. And Alli was sitting on the couch next to Ana, and Ana leaned over and started to suck on her pizza! I was also on the couch, so I let her taste the sauce, and she was in love! She was very upset with me when I had to take it away. She's starting to bite food now. It's amazing, yes, but terrifying for me. I am scared she is going to take a bit and aspirate a whole bit of actual food. AH! I don't think her doctor would be pleased with me. I don't even know really what would happen, I hope she wouldn't choke. But ugh. It's wonderful she understands that's what you do, but terrifying.

Other then those big things, the only other thing is that we are moving! We're moving closer to family, so it will be nice. It's where Kyle and I grew up, so we're happy we get to raise our girls there too. Only downside is we have to switch therapists. Which is a huge downside. Once that I didn't realize until after we had everything set up to move. Not only do we have to start over, but I'm nervous it will take Ana a long time (again) to get use to new people. People that move her and make her work.. She's one determined little girl. If she doesn't want to do something, rarely are you going to get her to do it.

Thanks again for keeping in the loop with Anabear! And the support and love! It's amazing to look back on the past year plus and see how far our little fighter has come! Like I always say, we wouldn't be here if it wasn't for you :)

With love,

The Polks



Philly Part 2

WE ARE HOME! 

Well, we've been home since Saturday technically...


Let me pick up from where I left off last.

On Thursday we had more therapy. It was actually very beneficial because we were able to move the time so Ana wasn't so exhausted from no naps. She was a bit tired still, but actually worked! We focused on rolling and scooting. Anabelle really liked to roll. We tried to get her into a prone position when she would roll on to her belly, she cried. Prone means she is up putting weight onto her forearms, like in an army crawl position. I think it might be to much of a stretch on the muscles in her shoulder/arm pit area since those muscles are really tight. There was also a wedge we used to help her learn to scoot. They has a smaller wedge that basically looked like a little slide, Ana sized. It has an inch lip on each side, and it was about 18 inches from top to bottom of the slide. We put Ana at the top, and at first helped her scoot down to show her how to do it. She was fine if we were doing it for her. But she got the idea, and did it solo! However... it scared her, and she cried. She's not use to moving on her own, so it was scary not to have our hands on her while she moved,  she didn't feel secure. But she is getting the idea, and I think she will progress quickly!

Friday: We had PT first. It was another good day! More of the same, but she is getting the idea, and I think it will only take a few months until she is all over the place and we will have to really baby proof again!

Saturday was our flight home. It was an early flight, especially since we had to return a car and was worried about it being spring break and the cut backs to TSA. We had a flight from Philly to Chicago, then Chicago home. With a 3 hour layover in Chicago. Unfortunately plans changed. On the flight to Chicago Anabelle's feeding tube broke. Now, she has a straight J tube. J is for Jejunum. Jejunum is the first part of your small intestine. The more popular tube is a G tube, and that goes directly into your stomach. Ana's tube bypass the stomach and goes directly to the jejunum. Her tube has to be replaced in the hospital under anesthesia. We aren't even give a replacement just in case we run into this issue. Also, because of the size she has, the part that comes out of her stomach hangs out about 6 inches or so. The tube broke in that part. I couldn't clamp it, so it just kept leaking. I had no choice but to stop her food. I dried it all up, and used some tape to try to stop it. So we finally get to Chicago. I learn that our plane is delayed by two hours. So 3 hour layover is now a 5 hour layover. This means Ana won't be eating this whole time, and it means getting her to the ER will take even longer. I tried calling doctors to get them to help expedite our process. Nope. Ana couldn't sleep well. She doesn't feel hunger, but her sugars and whatnot drop. You know that feeling when you haven't eaten for awhile, and you feel kind of sick? Yep, that's it. Then the plane was delayed another hour. At this point had been up traveling for 11 hours. Then our plane got in, and we had about a 4 1/2 hr plane ride home. Make that 11 hours 15 1/2. We land, go to get our bags, and learn because of the delays, they misplaced one bag. So I file a baggage claim. Then finally off the hospital!

We have to go to the ER, see if the department who replaces tubes will do it that night, and of course they won't. They have to admit Ana since by this point it has been over 24 hours since she's had anything, and she's dehydrated and her numbers have dropped. I usually have a really good experience with the nurses and doctors.. However this nurse rubbed me the wrong way. Maybe it was the lack of sleep and food on my end. Long story short, we finally get admitted, get her in bed, get her some sugar and electrolytes, sulfate, etc.. Which I learned the next morning they did incorrectly... And she starts to do better, but still exhausted. Get her a new tube, finally, and get out of dodge.

We got home Sunday evening. I am not sure how mom's travel solo for this trip repeatedly. I don't think I can do it again. I am still trying to recoup from this trip. Luckily Ana is able to nap:) However today (Tuesday) she is acting like her tube is in the wrong place. We can wait till morning to check, but hopefully it's fine.

Oh, but on top of this, Anabelle has been battling pink eye and a cold. She is one little trooper. Makes me feel like a big wimp sometimes :)


Hopefully that is the end to our excitement for awhile.

With love,
The Polks

The more you know

With Anabelle's first birthday just days away, I have been looking back on our lives. 

I looked back at a couple blog entries from December 2011. I knew absolutely nothing. I am still learning. However, I know mountains more now then 15 months ago. I didn't know how to correctly spell Gastroschisis, I didn't even know what exactly Arthrogryposis was. I was terrified of what was to come. 

It seemed like eternity for Anabelle to join us. I remember having such mixed emotions. I couldn't wait to hold my baby girl. But no one could tell me how long I might have with her, or what to expect at all. I knew that she was ok while still in my belly, but how would she be outside?! It was terrifying. And then the day came. March 1, 2012. Such a roller coaster day. I was so out of it because of all the lovely pain killers they gave me. I remember when I finally got to see her, and tried to ask, 'How is she doing?', I'm sure it came out as 'Keolnlsdu aslkdnfo uoasdnl'. Or at least that's how it felt by the stares I got. 

Now were just shy of a year later. If you told me we'd be here be a year ago, I'd probably laugh in your face. I didn't even want to think this far into the future. It was because the doctors didn't think that Anabelle was as amazing as she is. I couldn't imagine life without her. 

I won't lie though. It hasn't been all rainbows and unicorns. The beginning was rough. Really rough. Thankfully we have had amazing support. Not only from our family and friends, but from total strangers. We have been blessed to meet tons of other families online who have years of experience. I'm not sure we'd be as strong of a family now if we didn't have this support. It's a group of people who have already been there, done that. They have encouraged us to do what we think is best, as well as offer up some well learned tips. We have only meet a few of the families in person, but I don't feel like they are strangers. With them this road isn't so rough. It's so wonderful to have questions and have tons of people to turn to for advice. 

When I think about how far Anabelle has come, I am overcome with joy. From doctors telling us that she'd never do anything, to today, where she sat for hours and played with her sisters and laughed her head off. But that's a mix of emotions. Doctors don't know everything. And some people think what they say is stone. And to me that is terrifying. Because sometimes they are wrong. Like they were with my wonderful baby. They told me to consider my options. The genetic counselor we were working with during that time push our 'options' on us. During our most fragile time. Just hurts to think that these people can influence families to make these life changing choices and take their wonderful babies away from them. I am so thankful we didn't listen to those doctors. Don't get me wrong, they were nice during our care, but... only after we made them understand our feelings. That this is our baby, and a diagnosis, or lack thereof, doesn't change that. 

Today I tried to do a photo shot of the girls for their birthdays. Spring time in the Polk household is Birthday season! Four out of five members celebrate. Today was primarily for Anabelle's birthday. It is just wonderful to have all of them home, together, healthy and happy (mostly). 

(And yes, we are that family who does matching outfits for pictures)

She is a natural! 

Hanging out upside down! Loves it because it allows her to move her arms :)

Time for all three!

She's not sure about this pose...

And...she's done!

Not even a wardrobe change and her animals could salvage this shot

She was so tired from sitting for so long, she took a nap from about 3 to 6, and then woke up for about an hour, and slept since. I guess a photo shot is a great way to knock her out :) Good thing, because this one didn't turn out as I planned or wanted exactly. Which just means another great night of sleeping for the parents! 

Thanks to everyone for EVERYTHING. Everything has gotten us to this this point, and I am so thankful. 

More to come. 

With love, 

The Polks

Needing a traperkeeper

I always think of great topics to post here. Then I finally make myself sit here and write, and I can't think of any of them! 

Well.. we can start with what's going on, maybe they will come to me. 

Ana meet with her Rehabilitation doc last week. He says our treatment plan sounds great. And he threw out the idea of possibly doing Botox injections in Ana's neck to help relive the Torticollis. I'm still not on board 100%. I wasn't there, so I have to call and talk to him a little more. 

We're still trying to get her Bronchoscope scheduled. They are running into issues because I want to add on getting a new Jtube while she's under. You would think that I asked them to move the Earth by the way the are acting. If they go about it the way they are thinking, then Anabelle will be put under, have the scope done on the fifth floor, be transferred while under to the first floor and have the tube switched. You might ask why, if it's such a hassle, am I wanting the tubes switched. Well. Because her tube cap broke, and this is very inconvenient. Also, because if she is under, we might as well do it. She's had this tube for about 5 months or so now, and they say this tube last about 6. So why put her out in a month again if we can just do it now? Oh, and now the Rehab doctor said if we want to do the Botox, he can combine this too. That will be fun to call and try to explain and coordinate. 

Good GI news! I talked with the new dietitian  and we are starting to increase Anabelle's hourly intake while decreasing her time on. For a long time Ana has been at 25 MLs for 18 hours, plus 4 hours of Pedialyte for more fluids. There are 30 MLs in an ounce, so she was getting 15 oz of formula a day (24 hr period), plus another 3.33 oz of Pedialyte just to keep her hydrated, but not pack on too many calories. So now, each week we are increasing her by 5 ML's. This week she is at 30 ML's an hour for 15 hours. Next week she'll be at 35 MLs for 13 hours, and so on and on. She'll still be getting 15 oz of formula, just not have to be hooked up to the pump all the time. Then when we get up to 50 MLs an hour for 9 hours, we're going to call her back or meet with her to discuss bolus feeds. Then we will start on a more strict blended foods diet. My hope is to get off formula all together and have her just eat whatever we do, just through her tube. That way, when she's ready to eat orally, it won't be a shock to her intestines. 

We've also had two playdates with other AMC families. It was nice to finally meet some of the people who have been such huge helps to us since we first heard the word Arthrogryposis. And it was great to meet there wonderful kids and see how they figure stuff out on their own. It was a tad frustrating though. Amelia and Allison don't treat Anabelle any different then anyone else. However, when around kids their age with AMC, they acted as if they've never seen a brace in their life. I understand that they've never seen a kid their age with the same stuff as Ana, but I didn't expect them to be so shy about it. We deal with kids who stare at us daily. And I don't mind usually, but I never thought my kids would be those kids. I know eventually with time, and more time being inclusive, they will get it an not even think about it. I just it was just a shock to my system since they don't even see anything with their sister. But then again, it's their sister... But besides my kids shocking me, both meet ups were awesome! 


My new goal is to post a new post weekly, every Sunday. I know I've said this before. If I haven't, I've at least thought of it. I need to keep my word on this so I can hopefully post about the things I think about during the day long enough to post them here. Or at least write them down! :)

Goal for next update: To update about AMC. I defiantly don't know everything about it, but many people outside the AMC world have asked lots of questions. So AMC, maybe combined with feeding tube/Gastroschisis information. 

And please, if you have ANY questions about Miss Bear, feel free to ask. I look at is as spreading awareness, not offensive. You can do very little to offend me. 


Until Sunday!

With love, 

The Polks

Big long words

Our trip to Seattle was well worth it. Dr. Judith Hall is one of the kindest doctors I have meet. Not only does she have a wealth of knowledge to offer up, she sincerely showed that she cared. Not only for the time she was sitting with us, but for Anabelle's future. For the future of our family. She wanted to focus on Anabelle as a person, not just a diagnosis. 

I know that seems like an oxymoron because I went searching for a more definitive diagnosis. But I didn't go searching because I needed to validate anything. But I just wanted to have more information.

But to the juicy parts. 

Dr. Hall believes Anabelle is in the Amyoplasia spectrum. And on the severe side of Amyoplasia. She cannot say definitively, but believes this is correct due to the Gastroschisis along with the AMC. I'm getting ahead of myself. 

The reasons she cannot say it's Amyoplasia 100%:

 - Kids are typically presented with their arms stuck straight and legs bent,  kind of like a pretzel.  

 - Anabelle is opposite of this. Even though no two people are effected exactly the same way, Anabelle is pretty far from the typical Amyoplasia kid. 

 - Anabelle has these long dimples on her forearms and legs. Dimples in AMC are areas that didn't move much at all in utero, causing little to no muscle (and other things) to develop, so the skin basically stays to the bone, making a dimple. Ana has them on her shoulders, elbows, wrists.. All typical places. But Dr. Hall has never seen the ones on the forearms and legs quite as large as Anabelle's. Also, she was puzzled because she has seen kids with dimples on the legs, but shorter ones, and when they do have them, it was caused by the placement of the foot/feet. Meaning the toes were facing up towards the shin. Ana also does not fit that category as her feet were turned out. 

Back to why she thinks Amyoplasia in relations to Gastroschisis. She explained that in every big study anyone's done in Gastroschisis that 3-4% of the participants have Arthrogryposis, and of those with AMC, all are Amyoplasia. 

She also thinks it was all caused in relations with the vascular system. I can't remember exactly what she was meaning (I know, totally dumb of me), so I will be emailing her shortly for a refresher. 

But she cannot say exactly what caused any of it. The AMC or Gastroschisis. But she did say she is running a study of AMC and Gastroschisis/bowel adhesions soon, and will be looking for similarities. 

She also said if she finds anyone else with long dimples like Ana that she will be in touch with us. There is another genetic test they can run, more fine tooth then the two we have already ran. 

She did say that she recommends us returning to our local geneticists when Anabelle is about 16-18 years. 

AND. She said that of course she cannot guarantee anything.. however, in her observations, kids with good hip movement can walk. Anabelle has GREAT hip movement. I know that she isn't so amazing she has a crystal ball or anything... But Ana's uppers (hands/arms) don't function too great, (though she is making great strides!!) so if she can have walking in her pocket, I won't say no to it. No matter what happens she'll be fine, it'd just be nice to have the benefits of lowers.  

We also got the chance to meet other AMC families. It was really fun. There was an adorable 3 year old little boy. At first, very shy, but then he started joking around with me. Just made me miss Amelia and Allison all that more. I'm not sure how we're going to make it in March for a week when we go to Philadelphia. 

Side note: She is interested in food! We have been giving her little taste of it, and now when she sees food she yells at us if we don't give her tastes. By little tastes, I mean we dap our fingers in what we are eating and put it on her tongue. I cannot wait for the bronchoscope to be done with so we can hopefully start feeding her orally. Although, I don't think that will happen anytime soon. When we give her tastes, she is still swallowing the way she was when she was orally eating. Imagine eating air. That's the closest I can come to to describe what it sounds like. As if she is eating and breathing at once. 

Side note #2: So far this month, besides therapy, she has a total of 2 out of the home appointments. TWO! Last summer it was 20. Just shows how much she has improved in the health department. 

Promise, pictures in the next post. :D

With love, 

The Polk's

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 
  

Quick recap

So I know it's been far too long since I have updated. It's mostly due to lack of time. So I am forcing myself to sit here and do it!

The last update was just before we went on our second trip to Philadelphia. 

So now that was almost a month ago now. Realizing that I feel pretty bad for not updating. 

It went really good! It was just me and Anabelle for this trip. We also used Hosts for Hospitals (H4H) for the first time too. All in all, it was a good trip. The family we stayed with was sweet beyond words. They opened their entire house to us. The casts were removed. And legs and ankles look amazing. 

The top picture is before, then after is on bottom! Oh, and see how chunky her lil thighs are now too! 

So casts came off, then we went to make sure her braces still fit. We tried out the AFO's and KAFO's. The AFO's just needed to be made a little wider for her new chunk. The KAFO's needed a lot of reworking. She grew about an inch on one leg, and almost an inch on the other leg. And they also needed to be made a bit wider. They had to mail them to us since their metal worker already left and it was the weekend. Below is a picture of what a KAFO looks like. Ana's looks pretty similar, and even has a butterfly pattern!

So our appointment was December 7th. It's the 27th now. And still no KAFO's. Thanks to lovely UPS. They said we refused the package. Right... So they are on their way back to Philly, then back to us. As long as we get them eventually...

We are going to go back in March for a week. A week of intensive therapy. That means daily therapy geared to help her focus on ... wait for it ... WALKING! So our goal until then is to get her to sit unassisted. Over the past few weeks she is getting a lot better at holding her head by herself. I think it is do-able! 

The flight back Anabelle must have caught another bug. She has been sick on and off since then. We started her on Albuterol for the congestion, coughing, and ickyness. We also did another sleep study. We will get the results on the 7th. Really hoping for good results. We also saw her pulmonologist. He recommended doing a bronchoscopy when she is fully over all this ickyness. 


She is getting use to her AFO's. They are keeping her ankles straight. 

Another awesome note. Her GI said that she is getting too big! Well, to quickly at least. So we get to cut down from continuous 24 hours feedings to 18 hours a day. Last weigh in she weighed 15 pounds 10 oz!! CHUNKY! That's almost 11 pounds since she was born. Amazing. 


And she is talking up a storm. She says 'Da da da da da', and 'Ya ya ya ya'. She's become a lot more social too. As long as she has sleep in her. And she is sleeping better most nights, as long as she is feeling good. 

It seems like the appointments are slowly dwindling. I remember when we had 20+ appointments a month, and AMC families told me that it gets easier after a year, and appointments will slow down. I didn't believe it when they said it, but it's slowly starting to! 


Oh, and we had a wonderful Christmas! Ana didn't cry when she meet Santa! 

I can't believe we are creeping up on a year already. Just a few more months. I know time flies with kids, but it seems like this last year has gone by much quicker than normal. Luckily even though it is going fast, it is getting better and better. 

Just so thankful for my beautiful family. And everyone for the support. We couldn't have made it this far without it. 

Thank you!!

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