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

Philly!

So it's been a while, and I have lots to catch up on. I am very unorganized and the 'every Sunday post' thing isn't working as well as planned. I apologize. 


Last weekend was Anabelle's birthday! YAY! It was a blast. In summary, we spend quality time with quality people. Anabelle was a tad fussy since she decided to skip nap time. But she did enjoy walking around the arcade with the lights and noises! 


This week we are in Philadelphia. Dad and two big sisters stayed back home so the girls could stay in school, and because having all 5 of us here would be complete chaos. 

To recap thus far:

Monday: She went to clinic. Followed up with uppers and lowers. Uppers said he likes the range she has gained. Still just wants to watch to see how she grows and develops before thinking about any kind of surgery. I showed him the night splints we had made at home, and he is having the O/T here makes some others. The ones we had were very large and bulky. And honestly we never used them because of this. Lowers said he is also pleased. He is happy her ankles have stayed in place, and how much range she has gained in her knees and legs. One knee bends about 60 degrees while he other is about 70. Which is really good considering a year ago they were about hyper-extended about 10 degrees. We had to lengthen her KAFO's because she's grown a few inches! Very exciting! Also, he doesn't think she will need an osteotomy. An osteotomy is an invasive surgery where they go in, cut the bone (depending on what type of osteotomy, in Ana's case, it'd be her femur), re-position it in a better place, then pin it up, sew them up, and cast them for weeks. Instead of doing that, he thinks he can correct her legs by a simple soft tissue release. For Ana, since she was born with her toes by her ears, the tissues/joints in the front hip area, is very tight. This makes it impossible for her legs to come down and lay flat. It seems like a fairly simple surgery, in and out. She'd be casted for 4 weeks, with a bar in between her legs to keep them out. Now sure why that is, forgot to ask. But once that is done, that means she will be able to stand up straight. Which means we can really focus on walking!

She also had PT this day. In PT she learned quickly how to manipulate the therapist to get her sympathy. We discussed our goals for the week. Mine is mobility. She sees her sisters running around, and I can see she wants to be involved. I just don't know how to get her to be.. So we came up with the solution of scooting, or rocking.. Just moving her legs and bottom around so she can get around. Anabelle isn't totally on board yet with this idea. 

Tuesday: In the morning we meet with OT. They made her some wrist splits. These splits are night splits. Made of a lighter/thinner material. They stretch her wrist into extension (out straight), her thumbs out into extension, and her fingers into extension while flexing her knuckles. Lots of stretches from one split. Then we had a few hours before PT. And kind of slept on and off. Not very well though. Then PT. She didn't like it. But she worked. A little. She showed off her kicking skills at least :)

Wednesday: This morning was OT again. They made some adjustments to the splits, and put them on her for about 20 minutes. They didn't have time for a full session... for whatever reason... so elbow splits were not made. Then we had a few hours before PT. I thought Ana would sleep since she was tired......NOPE. She decided to go into zombie mode. Cry. Whine. Anything but sleep. So PT time rolled around, and she was already done. She didn't even want to sit up half the time. I had to take a 5 minute break and walk around with her to calm her down. There were times that she was ok with being there, but still wouldn't work. It was really frustrating. Only because we didn't have her OT scheduled originally, and then they decided they didn't have time. So that means we got very little accomplished today...

The joys of it all. Oh, then we got back to the Ronald McDonald house, and I realized I left my phone charger somewhere. I thought in the cafeteria, but they didn't find it.. So now I get to go on a hunt for a Target to find one. But of course I threw in some laundry before I noticed the missing charger, so I have to wait for that to be done. 

Blah. I wish Target stocked some magical pills that took away sleep deprivation  I'm sure the line would be out the door for those! This whole day light savings/time zone change has really messed my sleep up. I'm a total wimp! 

While the laundry was going, I thought I could catch up on some work.. But it seems like the password I had before I left no longer works for my work email.. and I don't want to drain my phone to call the help desk to fix it before I get a new charger since I use it for GPS... So I'm sorry boss ladies! I am trying to work! Promise! 

But I have meet some great AMC families while here. We had dinner last night together. Unfortunately I am staying at a different Ronald McDonald house, but the drive isn't bad at all. Just over a huge scary terrifying bridge. No big. :)


I will update more later with pictures to boot! 


Thanks again to EVERYONE! We wouldn't be here without everyone doing what they do to help us :) 

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

Picturetastic

As promised, here are pictures! 

This is Anabelle's first helmet. They obviously sent the wrong design. 

You can see how sweaty it makes her head.             

   I mistakenly left the camera lens and she had fun kicking it while I tried to take pictures.

This is the toy Kyle made for Ana. Made out of PVC Pipe. Then wrapped in pink polka dot duct tape for a girly touch. She loves this thing. 

We call it her kick stand.

                                        

She can really kick her toys with this thing and enjoy playing independently!

                                      

We finally got the right helmet. It was a little to plain for Amelia and Allison's taste, so they decorated it with their stickers. 

Ana sitting! 

She can do it all alone now. We have to get her into the seating position, but once there, she does great! 

Occasionally she will fall forward, like so. But she can sometimes bring herself back up. Sometimes she falls to the sides. 

                                    

The girls playing with play dough. When Ana gets to touch new things, she usually makes a face like she is disgusted. It took her a while to warm up to play dough...

Ana and a silly face. 

 

My crazies. 

Alli Paige 

(wearing my first crocheted hat)

 Until next time. 

With love, 
The Polk's

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