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

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

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

Just keep swimming

I started out writing this post a few days ago. Then put it off, then  got busy. I think I delay updating because it makes it a little to real sometimes. Sorry. It's easy to go through the motions and not think due to being to distracted by what is currently happening. And our hospital and doctors are very into having us make big decisions. So we focus on that instead of, "Hey, this is all really happening to our infant daughter."


So here it goes.. as always, very busy little girl.

Yesterday she had her surgery. Her surgeon found a port small enough for Anabelle. Which was so exciting, that way she didn't have to have a broviac, meaning there are no extra cords or tubing sticking out of her. So she had the port place, had a muscle biopsy and they switch her J-tube to the same type of tube, just a new tube. 


So in preop, we meet the anesthesiologist. After looking over her veins, he asked us why we were getting a port, and that she has great veins. ARE YOU SERIOUS?! This has been something Kyle and I have been deliberating over. Because it is optional. But then we keep reminding ourselves that winter is coming, and that we don't want her to go through what she's been through for blood or an IV one more time. Plus, if she gets sick again, and dehydrated, and they can't get an IV started, she could go into shock and things could be 100% worse. Thanks Dr. Anesthesiologist for making us doubt our decision in the 12th hour. Thankfully her surgeon (who has known her since birth) agreed with us and went forward with the port.   

The surgery took longer then expected. The surgeon said it should all take about two hours. When the 1 hour 45 min mark came, I called back just to see where they were, how she was doing, etc. The nurse said that they just finished the port placement and J-tube, and were starting the muscle biopsy. AH. Why? What happened? Is she ok? Well.. she is/was fine. 
The issue was the doctor had a tough time placing the port due to her anatomy. I understood what that meant when I saw her. Typically a port goes right around or below the collar bone. She had about 4 nicks where he attempted to place it there, then one on her neck covered with a steri-strip. He ended up placing it on her side, below her chest, but kind of on her ribs. There is a little cut about an inch or so above it where he cut to place it.  Before we got to see Anabelle, the surgeon talked to us. He explained all of this to us. We were scared, having no idea what to expect and all and asked how she was. He said she was fine, she did great. But that he was frustrated and not thrilled with what he had to do. He didn't like where he had to put it, and that it is going to her jugular, rather then normally coming out by the heart area (from my understanding). But that it is fine, will all work the same and she won't know the difference. This was huge coming from him. He is a very reserved fellow, who only talks when he needs to, and doesn't speak very loud. Him telling us how he was frustrated made me realize how different Anabelle's body is then other kids. 
But all in all it went pretty smoothly. The anesthesiologist let us know, again, that she has a small airway, and it was difficult to intubate her. But it was nice because he gave me pointers to let the next anesthesiologist know ahead of time what to expect and how to make it easier. 

We finally got to see our baby girl about 4 hours after handing her over. She was not a happy baby. We discussed which pain meds to give to her with the nurse. She said Morphine. I was TERRIFIED. The last time she had Morphine was after her Gastroschisis repair. And she was on a ventilator for 5 days. And they had to bag her time and time again. Like I said, the hospital is very parent guided. Great. Make me make this choice while my daughter is waking up from surgery, in pain... Ok, let's do it. Her normal dose would be 1.5 MLs, and we started out at .2 MLs. About 15 minutes later we realized that didn't do much. So she got another .2 MLs. Then she was feeling much much better. No more crying. Her crying wasn't full on crying, it was more like whimpering. And no side effects!! She did wonderful with it! Still hate giving it to her... but it worked. Then we were still suppose to go home that same day. Neither Kyle or I felt comfortable with that since she had so much done. We just didn't feel  comfortable we could control her pain, and what happens if her breathing acts up? Nooo thank you. Finally her surgeon said we could stay overnight. Oh, and I got my honorary nursing degree yesterday too. I wish it could work in real life and I could skip the schooling.. Ana's nurse in recover just mentioned how I know everything about her and how I could do everything she was doing. So maybe I could just be Ana's nurse :)

Later that night the nurse on the floor was giving Ana her meds, and she clogged her brand new tube. Now... I've done this countless times, so I knew it could be unclogged and didn't panic. But I guess her nurse is new, because she mentioned, 'Great, the rookie nurse clogged your tube.' Next time ya might want to keep that thought in your head, just sayin'. So I walked her through it. Told her what to grab to unclog it. But man, she clogged that thing up good... It was tough to get undone, but after about 20 minutes it was done. 

Anabelle slept all night, with only waking up her and there to whimper and complain. But overall her pain was controlled. She did have some weird breathing until about 11 PM. She was breathing like you would after a good hard cry. Kind of a hyperventilating breathing. But she got Tylenol with codeine at 10, and by 11 it stopped. 

I do have to mention that after she got her Morphine and opened her eyes in the recovery room, we got a few half heart-ed smiles out of her :)

In other news..earlier this week we meet with her GI. She has plateaued with her weight. She is hovering around 11 lbs now. She wants us to slowly increase her feeds again, until she can no longer tolerate it. We have to find her threshold. We think that with the extra therapy, and how she is almost always sweating, that she is burning to many calories. And since we can't stop either of those things... So she was at 16 ML/hour, and the GI wants us to go up 1 ML every day to every other day. As we see fit. Another thing where it's great because we went to medical school and all. 

I really can't complain. I would be upset if they were telling us what to do and not asking our opinion. It just makes me nervous because I'm not a nurse, a doctor, or anything close to having any say over anything medical. We will see how far up we can get Anabelle. The goal is at least 20, but who knows what she needs, it might be higher. Her GI is trying to avoid the use of TPN. It can cause liver failure, and we don't need to go down that road. She is a big advocate of NOT using it. 


In family news!

Allison started preschool (the same day as surgery...)!!! She will be in school 5 hours a day, for 5 days a week! She said that she liked it and had a good time. Of course I feel horrible though. Since it was planned the same day as surgery, we didn't pick her up and Kyle's mom did. Thankfully we have someone to do it (Thanks Cindy!), but it's something I would want to have been able to do. She did get cut on the chin by a little boy though... :( 
And Amelia had her first day in a 5 hour class, with a new teacher. She said that she liked it too!! And that she had fun, which is such a huge relief. OH. I called during the day to check on them. The teacher said that Amelia didn't take her nap, as usual. But she kept saying that she had to go potty. This is her way of avoiding naps and bed time. But every time she got up she did her business, so I guess you can't get too mad. 

But they are such awesome little girls. The other day Amelia asked if the doctors were going to cut Anabelle. We have decided it is best to keep the girls in the loop, and try to prepare them and explain things about Anabelle as best as we can. We don't keep anything a secret, we are an open book with them. I know they are only toddlers, but.. we think its best. We'll see how it plays out in a few years, but, for now we are doing what we think is best.

I digress. She asked about the doctors cutting Ana. I said yes, they are going to do it tomorrow. She got up and come over to us (I was holding her), and said how she doesn't want the doctors to cut her. We explained what they were doing, and why. What to expect the after, and how she will need to be careful with her. She said that she was sad, and that she didn't want them to cut her. And how she wanted to cry. Oh. My. Goodness. I love her!!!! She didn't cry. But just to know that she is so mature at four to realize this, and what's going one. Meanwhile, Allison is in the background jumping up and down, saying, 'They're going to cut her', kind of in a singing voice... 

Now, some might think this is horrible, because it's too much on a four year old. You might be right. To me though, I interpret it as her understanding what her baby sister is going through, showing proper emotions, and showing that she loves and cares about her sister. 

With each passing day Anabelle is getting better and stronger. Tomorrow we get to schedule an appointment to use her port for a blood draw. I'm terrified. I'm scared that it will still hurt, but mostly I'm scared it won't work. We will see... 

But we appreciate everyone's support, prayers, thoughts and good vibes through everything. It really makes a difference knowing people are there for us, and more importantly for Anabelle. Thank you!

With love, 
The Polks 

Busy lady

It's never a dull moment with Anabelle! 

Last week her GI called, and said that if I am not comfortable with her pain level to bring her in, again. To the hospital because it was Friday after 5:00 PM, and that's how our life works. So she had been screaming all day, and then I get home from work and no change. Even with meds. 

I took her in. We got her comfortable and admitted. I meet with the attending doc. I should mention we are VERY familiar with all of the doctors, and most nurses. Probably my least favorite thing. So he comes in and we discuss. He noted she was boarderline for an ear infection, but just not sure. So he wanted a complete work up. yay.... I explain how tough it is to get an IV and blood, and how we're getting a broviac cause of this very reason. I explain how last time they stuck her 14+ times, and still nada. He puts a cap on the times they can stick her, 3. Love it!! 

So two nurses come in to do the IV. These are new nurses I have never seen, which is weird... I explain how hard it is. I explain how I'd like IV therapy or a NICU nurse.. They reassure me that they are pros and can get it. I tell them about the 3 sticks max.... 

Two sticks in, they tell me I'm right, and mama knows best. DUH!
NICU nurses come down. Another 4 sticks. They got an IV twice, but both turned. Meaning they no longer went into the vein, but rather just into her arms.. filling it with meds and fluids. Painful.. 

So no IV. Then lab comes. They did another few sticks and got enough only for 1 of the 4 tests. Great. 

We saw her GI the next morning. Of course Anabelle was all smiles. Making me look insane. Then her GI remembered that in the hospital they blend the formula. Literally, just put the formula and water into a blender and wa-lah! She said that it takes out a lot of the gas.. still seems like she's crazy, but I guess it worked, for a few days. Anabelle came home on Sunday. 
She did pretty ok during the week, only needing Tylenol w/ codeine once a day.. however, Thursday she started getting back to herself. And now, Friday, I called her GI again. 

She also meet with surgery for a follow up. We told him what's been going on. He said that she is too small for a port, and she will get a broviac. But I guess there is such thing as a low profile broviac... (I can't remember if I have already said this..sorry!)

We meet with pulmonology this week. He said that he's happy with how she's progressed. Cancelled a med, added another one. Important news. He said that he doesn't want to repeat her swallow study for at least six months. And he said that'd be early. With that said, she will have a feeding tube, be it J or G, or GJ, for at least another year. In order to get her tube out she has to 
1. Do a repeat swallow study and PASS, showing no aspirating or iffy like behavior. 
2. Eat on her own without touching her tube AND thrive for another solid 6 months. 

That was a little upsetting to hear, only because her GI ok'd a repeat swallow study for as soon as it could be approved.. but it's ok. What ever is best for Anabanana. 

OHH and she is 11 lbs now :D Such a chunky monkey! Her hair has gotten so out of control too.. It reaches her eyes now! I put together some pictures and sent them to a doc for his opinion, so they have some funny words on the, but look how far she's come! 

See what I mean about the hair! 

(yawning because she is bored with me...)

 Her knees BENDING

 Ana laying out :)

As far as family news::: I just got a promotion/new position at work! It will be awesome. I think I will really enjoy the work, and am super super super excited to start!! 
Amelia and Allison are doing awesome. Amelia is really loving school. We have our first parent teacher conference next week! But there is this little girl who I can't stand. She tells Amelia that her shoes are not cute, or her hair or whatever.. then Amelia comes back and says she needs to wear different shoes, or she can't wear braids because this little girl said they are ugly.. UGH. I never thought I could be angry with a 4 year old!! Don't worry, I alerted her teacher to it and she has gotten better, I think.. 
I really don't think Ana and all of her needed attention has affected them to much. People always ask how they are doing with everything. I honestly haven't seen a huge drastic change in them. I feared jealous, outbursts, and just plain hell when we took Ana home from the NICU. But nada. I gotta say, our kids are pretty dang awesome. 

With love, 

The Polks

Testing and guessing

Anabelle has been a busy busy little lady. 


Let's see. Medical stuff... 


She's finally back in serial casting.. It's been a while since her on and off again hospital stays and trips. She has seen Endrocrinology, a Pulmonology team (a nurse, dietitian, social worker, etc.), her Rehabilitation coordinator, and GI. 

Endo wants blood. Pulmonology wants follow ups. Rehab wants therapy. GI wants the most. 

GI wanted an X-ray to check what, I don't know. But we did it this morning. I swear she might grow up and glow from all of this radiation... From my understanding; depending on what her GI sees in the x-ray...maybe not.. on Monday we will switch her back to a G-tube. Then observe. This will be done in the office. We will wait and watch her reflux. Unfortunately I have a feeling that it will be the same. She's still refluxing with a J-Tube, and that goes straight the the small intestine and was suppose to fix all of our worries. So if she still has severe reflux that affects her breathing horribly, again, then we will do a Nissen fundoplication. (gotta love Wikipedia!) So if we do that then we will combine the muscle biopsy and broviac, or a long PICC, like she had when she was in the NICU, and the Nissen. 

Man oh man. My hope is that by 7... 7 1/2 months... things will slow down and we will be settled and things will be smooooth. I feel like we're getting there.. Just a little hiccup in the road.

Like I've said, I'm back at work. And I have been asked more than once how Anabelle is. Honestly it doesn't bother me. It's just though because do you really want to know, because we're just passing by each other in the hall, and I can't fit it all in in these short 5 seconds. And also, I don't want some people to think I'm complaining, or being sad. It's just her story. I don't want them to feel bad or pity her or our family. We are blessed. Honestly. I look at her and always just imagine how much worse it could be. And how far she's come, and what a little trooper and fighter she is. 

Like today! She had such an awesome day! She was laughing (which it's been a while since she's laughed), and was all smiles. And she watches what we do, interacts with us. And most of all doesn't let all of this medical junk get in her way of being her, a happy, loving life baby girl. I have to say though, I think she loves her sisters most. Every time they talk and play with her she is ALL smiles! They love to help so so much. I am so thankful that our family has come together so well and we can all just see our precious Ana :) 


Hopefully we will have more answers as to what the next few weeks look like ahead by tomorrow. 

Oh, and the prayers, good thoughts, vibes, mojo, can we please keep it coming? I really feel it's been working wonders. Thanks <3 font="font">


With love, 
The Polks

Hospital stay #3!

Anabelle's GI decided that it was time to switch her from a G-tube to a J-tube.  Her reflux was so bad that sometimes she would turn blue. No good. 

July 12th we went in and had them switch it out. With a G-tube the tube just goes into the stomach, very easy to switch out for another. With a J-tube it's a little more tricky. 

First, to place it she had to be put under. An Interventional Radiologist placed it. Basically they used a X-ray machine that took current and live pictures. I think... I'm still fuzzy on how they did it. It was all very short notice. It was done as an outpatient procedure. Well. It was meant to be. 

We went in to the hospital. After much confusion regarding size, procedure, etc etc.. We finally went to the room. I was able to sit by Anabelle and kiss and talk to her until she fell asleep.. Until they put her to sleep. 

July 13th. The entire night she had been extremely fussy. Couldn't sleep. Her tube site (on the outside) leaked a few times. I just figured it was because they had just messed with it. But by morning I knew it just wasn't right. She had been trying to sleep. But there was just something preventing her. I thought I was overreacting, like I tend to do. So I called the on call pediatrician. He suggested I take her in to see them. So I did. Her doctor wasn't sure what it was. She said to take her to the ER and have them Xray to check the J-tube placement. So me and little miss Ana were off to the ER. I really hate going to the ER. Not only due to the long waits, germ filled people, people who don't need to be there, etc etc. But also because most of the nurses and doctors have no idea what to do for her. They usually have to call in a doctor who has already seen her before. And also because I have to explain Arthrogryposis to almost everyone, patients, nurses, doctors... I don't mind spreading awareness, however, when we are in the ER it is usually because she has some issue and I don't want to explain it 100 times. Or explain that no, she just has leg casts, not a spica cast. No, they are not to bring her legs down or relocate her hips, they are to bend her knees. The tubes you see are her feeding tubes. Nope, the only thing she takes orally is her binkie. *sigh* Sorry, it's just exhausting. More so when she is crying the entire time. 

What was I trying to get at again? Oh yeah. So we get back to the ER 'room'. They actually just gave us a gurney in the hallway. The nurse was nice and after triage-ing us took us back rather then sending us out to the masses again. 

The doctor took his time seeing us. Then said that he will have to confer with the pediatric intensivist, then get back to us. Meanwhile Ana's tubes are filling up with yellow... stuff. The ER doctor has no idea what it is.. Her bag had about 40 ML's. (just over an ounce).. What is going on!?! 

So finally after a few hours and and xray showing things are in place, he comes over and says that we have to go upstairs and they will figure it out up there. And as I should know, this won't be able to be fixed as an outpatient. Um, excuse me? I did not think this is the path we'd be going down today. I hadn't eaten yet because I thought we'd be home after her appointment with her pediatrician. I didn't have my purse, extra diapers, food for Ana, nothing! 

After a good chunk of time, the PICU (Pediatric ICU) doc sees her. Says she needs an IV because we haven't been able to feed her and she's dehydrated. FUN! It is beyond hard to get an IV started on this little lady. I honestly lost number of how many attempts were made. The doc said if they couldn't get one soon they'd have to do something where they stick the needle in her bone... I don't remember what it was called, but it sounded horrible. He was just trying to avoid putting her under anesthesia. I think an hour after starting, and about 5 people trying later, he finally got one in her foot. We got the IV started. But still no food.

In the end they think the balloon was just inflated to much for her little body. They believe it kept slipping and blocking things it isn't suppose to, causing food to come back out, and great pain for Anabelle. Also, her GI told us that they used the wrong J-tube. They were suppose to use a low profile J-tube, which would enable us to be able to hold her chest to chest with ease, have a medicine port closer to her so meds got to her faster, and not have a tube sticking out that can get pulled, wallow (again), etc. The first few days she was miserable. She was throwing up, non-stop crying, not sleeping. Then they figured her feeds where to much at once for her. So for about a week we played with her volume. The PICU doctor was threatening we'd have to use TPN. Luckily her GI is amazing, and has a few tricks up her sleeve. Typically babies eat 20 calories per ounce, however, she's bumped up to 45 calories per ounce. Unfortunately that isn't enough. So we've added a few medications to help. A few that are not cheap and not covered. Even with all of this she is still irritable. The GI has told us she thinks it's now because she is anticipating reflux, discomfort and/or pain, therefore she cries. The PICU doc thinks it's something GI related. So since no one can agree and figure it out, she is temporarily on Ativan. Awesome. My four month old is on an anti anxiety med. I have mixed feelings about it. And question if it is the right thing to do. 

After 13 days, they finally let us out. She still needs to gain to be on the low side of normal. Another reason they started Ativan. If she is fussy and crying all the time, she is burning calories. 

Oh. And today we meet with genetics. I thought that since we had an amniocentesis and microarray done that they wouldn't have much to tell us. Boy was I wrong. In short, he wants to do a muscle biopsy. And he was upset that they didn't do it while they had her asleep for this last ordeal. So in a few months they will put her to sleep for the fourth time and take a piece of her leg muscle and do a battery of tests on it to search for answers. The genetics doc said he wants to give her a break since she's been through so much so far. Thankfully!! 

And! I go back to work in one week. YIKES! Luckily I think we have everything for her in check. And hopefully we can get some nursing set up soon to help while I'm away. It's been a long (almost) 5 months. And I know it's just the beginning. I'm just happy that she will get a break for a little while and hope that she can just relax and grow and be happy :) 

It's tough to think that my little baby isn't healthy. It's hard to say that she is special needs. I always say that all I want for my kids are to be happy and healthy. And it's tough when I don't know if or when Anabelle is going to be healthy. Where we don't have a page full of specialist phone numbers, and have a list of meds she is or has taken, or tons of medical equipment laying around. I know things will be ok eventually. It's just hard at times to admit that right now they are not great. Thanks for letting me vent :):);)

With love, 

The Polks