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

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

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

Travelin the states

So just a brief update. Without pictures. Booo. I know.


We finally have Anabelle's helmet. The right one. I guess the manufacturer sent the wrong one or wrong material or something... and she ended up with a BLUE SCOOPY DOO helmet for a week.. Very opposite of a little girls helmet.

We ordered a plain white helmet, and are going to order sticker decals for it to dress it up! She is getting more use to it, although her head is still sweating like a pig in it. It's really gross, but we're told that calms down after a few weeks.

Tomorrow we fly out to Seattle for our appointment with the infamous Dr. Judith Hall. Very excited. And nervous. I know it seems silly and pointless for me to seach for the answers we may never get. Like they why, or the specific diagnosis, etc. I am not sure it will help any. But at the same time it might. Even if not, I would like to be prepared just in case Anabelle asks me those questions when she gets older. Not to mention try to be as educated as I can be. I learned last week that I know very little while talking to other AMC families.



They have a luncheon during the clinic for all the AMC families there, which I am really looking forward to.

Oh! And we had a cardiology appointment a while back, and he said she only has a small PFO. And that it's very common, 1 in 5 adults have it and don't even know it. He did notice that her port line is in her heart, which he doesn't like. But he talked with her surgeon and he said it's fine. So we can check cardiology off the list!
And big news!!! She is starting to sit unassisted!!

We sit her on the couch, propped up but laying back, and she will sit herself up off the couch. Sometimes she falls face forward, or falls to the sides. But she is doing so wonderful! It's like she decided that she was done with always using something for support!

This is extra special news. Besides being a huge developmental milestone that is. We are going back to Philadelphia in March for a week of intensive therapy. If she can sit unassisted, it opens the doors for possibilities of assistive devices such as standers, walkers, etc. Also, it opens the door for the possibility of the WREX (a device to help with arm use).

She is getting stronger everyday. We play a game where we hold her hand up and she has to use her own strength to pull it down. She love its and thinks it's hilarious. It's mostly shoulder use, but I'll take it!
AND!! More big news! She was playing on the floor the other day, and I look over, and she's on her tummy. All by herself! She doesn't like that too much because she is face down since she can't get her arms up, but she did it! TWICE. All by herself! She's going to miss her regular therapy this week because of our trip, but I can't wait to tell them.
She is thriving. And learning. And growing. And doing wonderful all around. I am such a proud mama. She is one in about a month, and to see her go through everything she has this past year, and come out on top. I am in awe.
She's a pretty amazing lil gal if I do say so myself.
Thanks everyone for the continued love and support. It really has made a world of difference.
I promise (well.. I hope to) have pictures next time.

With love,

The Polk's

Holland

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo

David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandt's.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

I learned that this is an old poem. But, I think it will outlast time. I am very fond of this poem. It speaks volumes to our new life that we've accepted. 

I typed out a few paragraphs of how sad and upset I get at times because I realize the difference in development between our older two girls and Anabelle. Then quickly deleted it. I realize that ever so often I get caught up in feeling sad because of the missed milestones.

I need to refocus my attention and energy. Focus on how far she has come.

So much farther than I ever thought was imaginable. Yes, the road so far looks like it needs a little fixing, but we're getting there. 

Unfortunately Anabelle has pneumonia again. They said it was viral again, but gave her antibiotics. Last time they said it was pneumonia and antibiotics would do nothing. Slightly confused. And this time she is much worse. Luckily they discharged her from the ER and didn't admit her though. We have everything she would need at the hospital at home, minus a doctor and nurses obviously. But we have a pulse ox, oxygen if needed, apnea monitor, etc. She is non stop coughing, congested, raspy from crying and coughing, and plain old beat. She can't sleep very well, but she wants to oh so much. It's pathetic. 
Our older two have had this bronchial cough for a month or so now. They did 10 days of antibiotics themselves. But it hasn't done anything. And their noses won't stop running either. I'm curious now if they have some sort of pneumonia too, but their doctor isn't open until Monday. 

Even with all the coughing and not feeling good going around, the girls are still smiling. I just love them. They remind me that it's all worth it. 

Last night before we knew Anabelle has pneumonia, she was just non stop crying, wouldn't sleep, etc. I had no idea what was going on. It was my turn to watch her that night. Kyle and I switch some weekends so one of us can get a good nights sleep and stay sane to help balance out the others lack there of. But this night was horrible. I was up with her until 1:45, then she slept for 3 hours. That's it. Around 1:00 I woke Kyle because I was loosing it. She kept choking on her saliva. No matter what I did she wouldn't sleep. So we decided to take her to the ER. I didn't feel comfortable with just one going with her alone in the back seat due to her choking, so we woke Amelia and Alli, and all loaded up. About 3/4 of the way there we realized she was OUT. Totally asleep. For those that are not familiar with the ER... If you take a baby in who doesn't immediately look like they are sick, they will think you are a crazy overreacting parent. They even told me I overdosed her once.. Yeah, that nurse got a piece of my mind.

Anyway. Since she was out, we decided she must have just been tired and now she is asleep and we'll go home. So we get home, she sleeps until 4:45. I try to deal with it. I don't. I lost my cool. I fought with Kyle (before and after our attempt to the ER), I couldn't figure out why she was acting this way and got mad with her. I realize it was very illogical to get mad with a 8 month old baby. But I did. My tone with her was not a nice one. And today I feel like I deserve the worlds worst mother award. Kyle took her into the ER this time, around 6 AM. They got home around 1:30 pm, and I felt like she was mad at me, like she didn't want to look me in the eyes, let alone in my direction. It could have been that she was just tired, but it didn't feel like it. It felt as if she was holding a well deserved grudge at me for loosing my cool with her last night. It is defiantly my lowest moment as a mother so far. I mean, I get upset with the Milly and Alli, but they are older, they can talk and tell me what's going on usually.. But to get angry with a baby, and come to find out she was acting like she was due to pneumonia! Yep. I earned that award..




Hopefully this ugly bout of pneumonia will only last a few days. We go back to Philadelphia soon, and I'm a little nervous that her doc will say she can't fly if she is still sick. Obviously I don't want her to travel if she is sick either... I just want things to run smoothly. I'm always worried about when that monkey is going to throw his wrench in our plans. Dang monkey is always running our plans...

And thank you everyone for the continued love, support, good vibes, etc. It means the world to us. Honestly. I get a little teary when I think about how many people have helped us get to where we are today. 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

The new normal

nor·mal

 [ náwrm'l ]   

1. usual: conforming to the usual standard, type, or custom
2. healthy: physically, mentally, and emotionally healthy

Firstly, I don't want anyone to be offended. My intend is solely to express my feelings. If you disagree, that is perfectly ok and I accept that. As I hope that you accept that mine differ from yours and we can still respect each other. 

I have three daughters, Anabelle is our youngest. I have done the baby things that majority of people deal with, what you typically think comes along with having a new addition to the family. 

With our older girls there were the middle of the night feedings, burping, spit up, introducing foods, tummy time (with ease), learning to crawl, holding their heads up (basically from birth..), doctor appointments for check ups and only very rarely between, playing with toys, etc etc. 

With Anabelle there are continuous feeds, no need for burping because she has a bag that does it for her, and nothing is in her stomach anyways, spit up that is bile only and due to severe reflux, nothing orally because she can aspirate, a variety of medications and hospital visits, and very physically delayed. Oh, and about 20 doctor appointments a month. 

I do not say any of this in a negative light. I mention these things because they are not normal. People continue to tell me that Anabelle is normal. Yes. There are things about her that are normal. She is a beautiful little girl, a wonderful addition to our family, I wouldn't trade her for any other baby, and her basic needs are normal as any other baby out there. 

However, her feeding bags and list of medications are not normal. 

Her formula being prescription formula is not normal. 

Her going to therapy twice weekly is not normal. 

Yada yada yada....

I hope everyone understand what my point is. I am a little tired so it makes sense in my head, and I just hope it makes sense as I type it. 

So medical update! yay.

We have the first set of results back from the muscle biopsy. From the conversation with the Geneticist all positive. The basic: It's non progressive. Which is the answer we were looking for. In the consult, he said that sometimes conditions start out looking like just Arthrogryposis, then become degenerative. So finding out that she can only get better was a huge relief. He also said she has Fiber Type 1 & 2 disproportion. Also, he said something about active necrosis. I am confused by all of this. It seems a little oxymoron to me...  And the nurse was trying to explain the blood results over the phone, and I was totally confused. I asked her to send me the report because it was all just too much to understand over the phone. Oh, and they are still doing tests on the muscle to see what else they can find since the first tests were somewhat inconclusive. 

These are just a few paragraphs from the records, just to explain how easily I can get confused:

'The muscle biopsy shows necrosis in two adjacent fascicles with near complete necrosis of their fibers that are being replaced by an infiltrate of histiocytes at the periphery of the necrotic fascicles. That change, which essentially represents infarctions of the two muscle fascicles, is not present in the remaining fascicles of this biopsy. The necrotic fascicles are not associated with significant inflammatory infiltrates other than the histiocytes at the periphery of the fascicles, and the remaining fascicles of the biopsy remain free of inflammation  There is no evidence of vascular thrombosis.....

Trichrome stain does not show the presence of "ragged red" fibers that could indicate a mitochondrial myopathy. Likewise, stains with SDH and COX (both mitochondrial specific enzymes) show no evidence of an abnormal pattern of staining. The fibers retain a normal amount of glycogen with PSA stains with and without diastase and normal amounts of lipids are detected with Oil-red-O stains. NADH stain does not show the presence of target or core fibers.'

So... with that said... if you understand all of this please let me know what the heck is means. I understand bits and pieces. But.. 

So with that and her appointment with the neurologist, we are moving forward with another MRI. I don't really know what the right path is, or if we are choosing the right steps, and all I can do is hope that we are. It will be about 3 hours and it will be on her brain and spine. So that means anesthesia for the 6th time since she was born. Not my favorite, but it's what we have to do. 

I wouldn't trade any of this crazy chaos for the world. Of course there are times I want to just throw the white flag up. Then one of the girls or Kyle will do something to remind me it's all worth it. I am remind to appreciate every day given to us. 

When we first found out there was something different with Anabelle, we were told she probably wasn't going to make it to birth. So here, 7 months and 14 days post birth, I can only be thankful for our journey, no matter where it leads. It might not be the normal path, or the path we expected, but it is ours and I wouldn't change it for anything. 

With love, 

The Polks

crazy. beautiful

Brace yourself, it's a long one. 

I've been thinking about doing an update daily. What to write, include, etc. I've been thinking about how personal to make it, what parts of our lives to include. I know personally I read other blogs and feel like I belong. Belong to a club of sorts. A club that doesn't judge, a club where I am not alone, a club where everyone understands. 

First I will update about Ana's medical stuff... 


We have had more therapy visits. One 'feeding evaluation', and some occupational therapy (OT). 

Of course Anabelle didn't have a normal feeding evaluation. Because she doesn't eat. But it was just following up with her speech therapist and see what we can do to help her be ready to eat when she can. Setting up the appointment was frustrating. The receptionist said the have her hungry, but not starving. This way she will show them how she eats. Well, she doesn't eat. She proceeded to disagree with me and was totally confused and baffled with my responses. I told her I know what needs to happen, and don't worry about it. 

She is currently receiving occupation therapy twice a week through CCS. They have to submit a plan to her doctor for approval. The plan includes where she is at, goals, expectations, etc. It was nice reading the parts about how she is a very visual and happy baby. It was devastating reading the parts about her being at a 12 week development mark, how drastic her AMC really is, and just how much therapy they think she needs and how much she needs help with. 

I think it hits so hard because I have procrastinated thinking about life to come. Since we found out that Anabelle had something different going on (at 20 weeks prenatally), it's been a wait and see process. It still is, but some things are becoming evident. She is 7 months old (today, YAY!) but physically where a 3 months old is. It's hard to see it written down on paper. It's hard to see other kids her age at the store, in waiting rooms, at the park. Anywhere life normally takes you.  

Like I said, it only gets to me sometimes. Mostly I am just so thrilled that she is gaining weight and is happy! But I think these are reasons I have avoided updating because it makes me sit down and think about her progress. Her very very slow progress. 

But good news! She was plateauing for a few weeks on weight. We slowly upped her feedings, and now she is doing just fine! Her GI is micromanaging her for a few weeks to ensure things are under control and she is on the right track. This means weekly appointments. And since both us and her are so busy, we get to see her Monday mornings at 6:45 AM. Anabelle isn't loving these appointments, and gets mad when I wake her up. She has done good though! She has joined the 12 pound club! Next Monday if things keep going good we are going to switch from formula to a blended diet. This means blending foods! Actual food! I don't know all about it yet, but she mentioned spinach, kael, oil... and that kids usually do much better on blended diets, especially kids with reflux. She is an awesome doctor. Always listens, very open to ideas, not all medical. Realized that we are people that have to live with this. We talked about this on Monday, and she was explaining how she advocates for her families. That right there was awesome. Her families, not patients. She realizes this effects the whole family. LOVE her! 
*update to the update* 
I wrote this a week ago, and GI has been.. iffy lately. Not the doc. Wednesday I was giving night meds and her tube clogged. It happens. Every time I stress, but we can always get it unclogged. I guess we put too much pressure on it and the tube popped. Totally broke. Done. Off to the ER. Since IR didn't want to come in that night, she had to be admitted until they came in the next morning to get her a new tube. They said it'd be an easy experience, smooth. Put a guide wire in the tube, take the old tube out, put the new tube over the guide wire, place it, then take the guide wire out. Easy! WRONG. The tube was so clogged they couldn't put the guide wire through, so they had to do the entire procedure. And since she doesn't do great with anesthesia, and they said it'd be simple and anesthesia wouldn't be necessary, we didn't do it. WRONG, again. I got to be with her, and the entire time was horrid. I hate myself for letting it happen and her go through that pain. Then Saturday night I was doing meds again, and the damn tube clogged, again. I just balled. I called the GI to see if there was anything else I could do that I hadn't tried, there wasn't. Per her instructions we went in Sunday morning. We went in at 7:00 am and IR couldn't change her tube until 4:00 pm. ALL day without food. And this time we used Propofol and she didn't have to be awake. But then all last night and today she just isn't her. So I took her into the ER. They say she has gas and it's just the side effects from Propofol. We see her GI in the morning so I was done with them. There were a lot of people with coughs and contagious stuff today. So done with the hospital. I wish I could run it and make things run smoothly. 


Last Friday Anabelle meet with Neurology. He wants to wait until the muscle biopsy comes back, but depending on what the results are wants to do another MRI. I'm not exactly sure why (I didn't go), but wanting to check out the nervous system is my guess. 

OH!!! BIG NEWS!!! We have an appointment for Shriner's in Philadelphia! November 5th :) I am terrified things will all fall apart and we won't be able to make it. I also haven't really ever traveled, so I am terrified. Scared of traveling with a baby, a baby with feeding tube, etc etc. But I am hoping things work out, and am trying to prevent them from falling apart. I really want to go out there and see those doctors. The lower extremity doctors here are upsetting. They are unwilling to coordinate. Last week she was suppose to get casted again. Kyle took her in, and they make it seem like we are burdening them. In the end, he walked out without casts. The last casts we got, a few weeks ago, were too tight. Since they use soft casts I am able to take them off at home, which I did. I felt like her care was being compromised. So I am excited and can't wait to see what Philadelphia has to offer. 

She is doing ok overall. Hopefully tomorrow her GI can give us more answers. 


Amelia and Allison are doing good. Amelia is wanting more attention from me, and I can't give it to her all the time. It's hard to balance attention. 

Lately I have been conflicted. Conflicted over having 'typical' kids and a special needs kid. I am so happy and grateful that my older girls are healthy, are able to do things on their own, for the fact that I have never worried if they will be able to live independently, and on and on and on. In the other hand, I am frustrated because I know these things will be hard for Anabelle. I don't know exactly how to explain it. I try to not let AMC effect things in our lives so much. Just take it for what it is and conquer it. Again, the moments of frustration know how to creep its way in. 

But I wouldn't change our life for anything. I am thrilled with each and every day that we have these beautiful girls, and that they are ours. I love them all so much and couldn't imagine life without them. I am blessed to have them, blessed to have such a wonderful partner to go through this crazy beautiful life with. 

With love, 
The Polks