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

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

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

122-79 = 43

I just counted all of Anabelle's doctor appointments, ER visits, and hospital stays thus far, and it drives me crazy how much this little girl has to be bothered by doctors. I could have missed some doctor appointments because sometimes I don't get them written down, but this is what I counted as of today:


 41 doctor appointments 
 36 days in the hospital
+2 ER visits
79  doctor things in her short 122 days of life


This means we've only had 43 days of relaxing. Ugh.


I have to share this story to get it off my chest. This past weekend we all went out as a family to a fundraiser. I was sitting their holding Anabelle as she was sleeping. People were walking back and forth, not really paying to much attention to us. But this one group of people... It appeared to be a family; mom, dad, older kids. The dad made it obvious to stare. I think he was staring at the bag that had medical tubing running from it to my daughter. But still.. just ask! As he walked toward us, he STARED. As he passed us, he STARED. As he walked past us, he STARED. I looked up and when he noticed me, he smiled. I said hi, he said hi back. And continued to stare. Seriously!? I wish I was more experienced with handling these situations. I know I will get there over the years. But c'mon man! 
I just find it unfair. And right now I'm having one of those moments where I question why. Don't worry, it'll pass in a few minutes :)


Last week was suppose to be easy. On Monday we only had casting, P/T and a follow up with her surgeon. (Whom I love by the way!) But in about 30 minutes we added a sleep study and follow up with her pulmonologist. 


First, her casting. It went awesome! Except she has a small pressure sore, so this set of cast only lasted a few days. They use soft casts so we can take them off at home if she needs, which is nice because she needed them off yesterday! But her doctor is so happy with her progress. Even though it's slow. But her knees will barely go hyper extended anymore! We're going forward still until he feels she hits a wall.

Just look how far she's come! From not even being able to take a picture of her legs separate to... 

This!! 

Progress! I asked the doc if he thinks she'll need braces (AFO's or KAFO's) to walk, and he said probably. Just because the muscle there isn't what she really needs to walk. But I'll take it! Now, he's not saying he's 100% sure he thinks she'll walk, but he said if she does. Now if he says no, we're getting a second opinion, of course. 

Then we had P/T. She laughed for her p/t lady! She's only laughed a little for me, but this was a hardy full on laugh. Stinker butt. Overall though, she hates p/t.

Then the sleep study. Mind you this was all on Tuesday. Casts at 8, P/T at 3:30, then the sleep study from 7:30PM-6 AM. FUN day. 

Wednesday we met with Dr. Chipps, her pulmonologist. He said that her sleep study was improving. Not good, not great, but better then the first two. Good news was she doesn't have to be on oxygen anymore, only when she needs it, and we don't have to see him again for 2 months! YAY!!!! I wanted to hug him! But he's not really a people person and I think he would have told us to find a new pulmonologist... 

These past few days have been hard though. She's been really crabby. Little princess doesn't like to be put down. She likes to sleep chest to chest. Which makes sleeping for us muy difficult. I usually sleep on the couch with her so that she isn't crying all night. With our first two we'd let them try to sooth themselves. But Ana can't really do that. She has trouble keeping her binki in by herself, and we (I) always think it's something bad when she cries. 'Are her casts bugging her?' 'Is her reflux bad right now?' 'Does her tube hurt her?' 'Does she have gas?' And on and on and on. It's a guessing game. 

But then she makes up for it. She'll relax and calm down, and give us the biggest smile ever! Underneath it all, she is a happy baby. At least she wants to be. She love talking to her sisters! She'll talk their ears off! And I think they love her too! 

OH! And Amelia started preschool last week!

I know, my kids are cute :) 

This weekend Kyle and I get a night away for my birthday :):):):)

Thanks Leah!!!!

Yes! A family member is crazy enough to take on all three of our kids overnight, plus their own! And we get to escape to San Francisco for the night, and go to Alcatraz. I am nervous, terrified, thrilled, scared, excited, panicked, and more all at the same time. I know we have enough equipment to make sure Ana stays safe and sound. It's just scary. And I know we're leaving her in capable hands. I don't doubt that. I just know it can be rough when Kyle and I are home, and we have just our own kids. Anabelle requires a lot of attention...

But we love her. So so much <3

With love, 

The Polks

Trach?!

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

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

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

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

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

With love,
The Polks

Home at last! - again

I'm not very good at keeping up with whole posting twice a week. Opps!


Well we got home with Miss Anabelle on Saturday, so we've been home for 5 days now. On our own. With this stinking G-tube stuff. It honestly hasn't been horrible. It's just difficult to move around the house while holding her. I feel like our home has transformed into some sort of hospital clinic now. We have an IV pole and all. Oh, and our dumb apnea monitor doesn't work correctly, or we're using incorrectly. 


She is still having pretty bad reflux. She is just so darn sensitive. We lay her flat to change a diaper and it starts. And it's horrible. It usually doesn't come up, it just hang out in the back of her throat. Which isn't fun. I'm sure it is just sitting there burning her, and then she cries in pain, and I fear she will some day aspirate it. And she favors being held chest to chest, and dislikes being put down. We can sometimes get away with it, but usually not for long. We have to pad her swing so that she is basically sitting straight up. And she favors this one particular head position that makes it a little tough to lay her down on her back. Luckily our home therapy people let us borrow this pillow thing. It's called a Versa pillow. It's full of little beads, and you shape it how you want, then you can suck out all of the air and it holds its shape! We finally got to use it today. She is actually liking it, kinda. 

I am also excited because they do craniosacral therapy! I just learned about it earlier this week, and was sure it would be hard to find, and expensive. Luckily I asked, and they do it! And it's free! I'm just hoping we can get enough visits for us to become comfortable enough to do it if we don't get visits for it at least weekly. 


I don't know how I am going to get use to this life of doctors. I know we have just begun, but shoot! How do other families do this?! This week we have 6, yes 6! doctor appointments. In 5 days! This week we had pulmonology, two home visits, Shriner's for castings, a post op check, and sleep study. 


Pulmonology didn't do too much. They said that they want her to see cardiology, neurology, and be followed by the pulmonology team. As far as positioning, just don't put her head to her chest. Duh. You see, Anabelle likes to arch her back and keep her airway open. It's hard for her to breath if she is in any other position, and sometimes even that position is hard. I mentioned   Stridor, and they agreed, but didn't say yes or no. Just 'yeah, that's a possibility.' UGH. Like I tell Amelia, it's either yes or no. 


On a good note, we weighed Anabelle at Shriner's on Tuesday. We use their scale for her weighted since she is there weekly. And guess what!? Little miss Belle is 7 POUNDS! So in twelve weeks, she's gained two pounds. Hopefully it's all due to the G-tube and it will just keep going up and up. Also, she is super visual. As always. In the NICU the nurses said she was very 'social'. She loves looking around and seeing what's going on. But now, she is starting to really look at you. She is smiling more and more, cooing, and just being such a cutie pie! It's tough because just laying her back a little to look at her and talk with her gives her reflux. It's just a balancing act. Right now her voice is pretty raspy from that, combined with crying a bunch. It's sad. We've been giving her Tylenol for any pain she might be having, as well as Zantac and Prevacid. 


It's just nice having her home though. I'll take having us all together over a few extra hours of sleep and a more organized home. <3


With love, 
The Polks

Oh, and here are a few pics of the latest hospital trek

'Uhh... mom, What is this thing?!'

She loved this thing! Don't tell, but it made it home with us :D 

Comfy at the hospital

After surgery, hooked up to some machines; Her feeding pump, leads, pulse ox, as well as oxygen. She liked the bouncer/swing they had though!