Hoping for the Best and Preparing for the Worst

I thought it was time to finally update everyone on how baby Everly is doing and what we learned at the appointment with the specialist. Thank you for being patient with me. Although this blog is therapeutic, the topic is extremely heavy and I really think it took me a while to prepare (emotionally) to sit down and write this. Also, her defects are crazy! I don't understand the anatomy of a healthy heart, so to be thrown into this world and have to learn about all of her defects has been very confusing and overwhelming.

Okay, so it was Friday, July 28th. Drew, Urban, Roger, and I had flown into Salt Lake THAT MORNING from Columbus, Ohio to get to our appointment up at the University of Utah. We had to leave for the airport around 4:30 AM, so we knew it was going to be an exhausting day. Want to hear something awesome? I literally didn't sleep the night before... I never closed my eyes. I just hung out and watched the time go by. Call it nerves, I guess. I was a complete zombie, and I guess I was trying to emotionally prepare myself for a life changing doctors appointment! Also, just picture Drew and I trying to entertain a crazy toddler and a crazy dog on our flight home. "I got two words.. NEVER AGAIN!"- Lelan Pack

Don't let this picture fool you. These naps only lasted 15 minutes of the whole 8 hours of travel time.

When we got into SLC, we dropped Urban and Roger off at the in-laws and we headed up to the U. We got there a little early and as we sat in the waiting room I couldn't help but look at all the people around me and wonder what their journey looked like. To my right I kept staring at a cute young couple and their brand new baby. Drew always says how creepy I am because I love staring at babies so much. This baby couldn't have been more than a week old. She had an oxygen tube connected to her but you could barely even notice that through all her cuteness! I thought about Everly and how I hope one day we will be sitting in this room for her check ups.

When they called my name, we walked into a room and began the hour long ultrasound of Everly's heart. What a boring hour that was. The equipment is so fine tuned that the entire monitor was of her heart (that was no bigger than a quarter at the time). We didn't get to see her cute face, or her wiggly little body. Just an hour in a dark room looking at something that was so foreign to us. I kept asking questions like, "should that little black dot be there?" and "Oh, is that a view of the 4 chambers?" The ultrasound technician was so nice to not make fun of me when I asked those questions. Drew couldn't help but giggle. I was just so impatient! I wanted to know what she saw!

After the ultrasound, I went to use the bathroom while they took Drew into a different room. When I walked into the room there were some new faces. A few moments later I found out that they were social workers and they were there for support. The room was small, but big enough to hold 7 or 8 chairs and a coffee table in the center of the room. In front of us, there was a big wall of file organizers with papers in each slot. Everly's cardiologist went through that wall and pulled several papers that had pictures and definitions of heart defects. Now here is where it gets confusing..

Everly has 4 separate and rare heart defects. He would set down the appropriate picture and show us a defect but then he would scribble on it to show how hers differed from the picture. THEY DID NOT HAVE PAPERWORK TO SHOW ALL OF HER DEFECTS. We left the appointment with a lot of drawn pictures to help us understand. It was very intimidating! I will go into each heart defect so you have some idea of whats going on with her.

Defect #1: "Right Dominant" Hypoplastic Left Heart Syndrome.
HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis.

Defect #2: Unbalanced AV Canal

Complete atrial ventricular septal defects (AV Canal) generally have right and left valve components equally divided. However, in unbalanced AV canal either right or left ventricle dominance may occur. The spectrum may vary between those readily able to undergo biventricular repair at no increased risk to those requiring a single ventricle approach in cases with severe hypoplasia of the ipsilateral ventricle.

Defect #3: Double Outlet Right Ventricle

DORV is when the pulmonary after and the aorta (the hearts two major arteries) both connect to the right ventricle. IN a normal heart, the pulmonary artery connects to the right ventricle, and the aorta connects to the left ventricle. DORV creates a problem because the right ventricle carries oxygen-poor blood, which then gets circulated in the body.

Defect #4: Pulmonary Stenosis

Pulmonary Stinosis is a condition characterized by the obstruction of blood flow from the right ventricle to the pulmonary artery, This obstruction is caused by the narrowing (stenosis) at one or more points from the right ventricle to the pulmonary artery.

Are you confused yet? I sure am. There is still so much more I have to learn. I make jokes that her heart is just a big ball of mush; Poor thing. Who knows how all this happened?! This picture below is the picture that her doctor drew for us to show her defects.

So... how am I doing? Great question. It is different everyday. Somedays I feel really positive and hopeful. Other days I cry a lot. I am so sick of crying to be completely honest. Before my 20 week ultrasound, I never cried this much. Just the other day, I cried in target when I ran my fingers through some baby girl jammies. Yes, I am that person who cries randomly in a store and I make everyone around me uncomfortable. I try to keep busy but it still gets to me. Thank goodness I have Urban and thank goodness I have amazing family and friends that keep me busy. Since being back in Utah I have had company stay over almost every weekend. I have friends that bring me dinner on weeknights. I have lunch dates with my old coworkers that I love and miss so much. Ikea has been my best friend lately. Mini house projects keep me sane while Drew is still away. I am so blessed to have these amazing people (and projects) in my life. People keep telling me how "strong" I am but the truth is, I don't have a choice. I wake up, feed Urban breakfast, change his diaper, run errands, pay bills, clean my house, do laundry, etc.. I wake up and I keep living because that is all I can do.

This picture of Urban was when I was crying and he walked over and put his face against mine and hugged me. You can see a perfect mascara mark on his forehead. What would I do without this kid?!? 

Obviously, we are going to do everything we can to help our little girl fight for her life, but at the same time, we don't want her to be in pain. The truth is, she has FOUR major defects. Not just 1 or 2. Even if every single surgery goes perfectly and she is a complete champ, which I know she will be, there is no telling how her quality of life will be. It kills me to think that she will be in so much pain when she joins us. When she is born and my body isn't supporting her anymore, she will really struggle for her life. I guess what I am trying to say is we are hoping for the best but preparing for the worst. I would really love to watch Everly grow up, but if I am not able to in this life, at least I know she is free from pain and she will be with our Savior. What better caretaker to watch over her while we are waiting to be together again?! I get tears in my eyes when I picture mine and Drew's grandparents playing games like peek-a-boo and hide-and-seek with our baby girl.

Thank you to everyone who has reached out, and thank you for all the prayers. We feel them. It is truly humbling. We have the most amazing support system and we wouldn't be able to live this life without you. Also, the heart community is AMAZING!! Intermountain Healing Hearts is a group that I recently joined. I don't want to go too much into them quite yet, but lets just say that I have made some life long friends in the short time we have known about Everly's heart. 

We are truly blessed.

Love,

Markie