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