Frazier's Full House Farm and the Adventure of Addy's Heart: July 2011

Our date for Addy's cardiac MRI & bronchoscopy has been set for August 12th. We will have to be there at the ungodly hour of 6am. They will do the bronchoscopy 1st about 8am, then anesthesia will take her for the MRI from 10-12 o'clock. We will expect to be in radiology recovery for most of the day but they said if she does well she should be able to go home that afternoon. It makes me want to vomit to think about the possibilities that can happen in the 4 hours that she is away from us. We know she needs this though. As she gets more active, (i.e. closer to talking all by herself!) she becomes more out of breath and labored with her breathing. She often has a dusky color to her lips that improves as soon as I pick her up and make her settle down. How much of this is normal? Who knows.... we know she will have some degree of activity intolerance, it doesn't seem to hold her back so that's a good thing.

On the other hand, this will take place exactly one week before Addy's 1st birthday! We are planning a little party to celebrate this occasion with friends and family. One year ago, we didn't know if we would get to celebrate a first birthday with Addy. We had no clue what the future would hold. While that remains unchanged, the last year has been one wonderful worldwind as we have gotten to know our baby and rejoice in every step she takes! I'm so proud of my baby girl, she is amazing. I have realized that the fear of what will happen in the future will never go away. I just have to love her every day that she is here and know she is meant for big things.

I love my baby so much...I can't tell you how many times a day I think that to myself...I don't know what I'd do if I lost her. Unfortunately, these are things that a heart parent faces every day. However, I don't think I could possibly take more joy than when my baby girl squeals at the sight of me, breaks out in a big cheesy grin, or makes a new noise/word. I wish that I could take a picture or video of every second of every day, so that I never forget a single moment...we never know when the last might be. That's why when we have those hiccups that are expected- having half a heart and all, it is so scary.

Our last cards checkup looked good except that they were unable to get very good pictures due to Addy's unwillingness to cooperate...but it did look like she might have some left Pulmonary Artery narrowing (that goes to her lungs). Hence, we have been waiting for 2 the last 2 months for Cardiology to decide whether we were going to have a sedated Echo (by sedation team) or a cardiac MRI (intubated by cardiac anesthesiology). After a few weeks of watching sats we decided that yes, in fact, her sats DO run in the 80's and we should be ok with the Echo. However, then Heath and I started noticing something....

Over the last few months, we have noticed that Addy makes a very peculiar sound when she cries. Now we are also hearing it at night while she sleeps sometimes as well as if she gets overly excited. Granted, my overly joyous baby doesn't truly CRY very often...although whining is a totally different story. When she cries we hear this rather loud and disturbing "eeeeee!" when she breaths in. This mommy (& daddy's) trained ears immediately cue in that this is not a normal sound (no matter what other people say!). Stridor. Not something we want to hear. When I think the word stridor this is what runs through this ICU nurses head.....stridor...laryg/tracheo/broncheomalacia....difficult intubation...airway trauma....trach...no beautiful words when talking.... OK OK, yea I know it's kind of twisted. But you can't turn off the ICU nurse in me and Heath...and we have seen that progression TOOOO many times. While this would not change how I love my sweet baby, no parent wants to go through that or have to make their child. So, we insisted on an ENT evaluation before we would allow them to decide on the Echo or MRI. Once again we are thinking in overtime of...stridor of unknown cause...sedated...unstable airway...bad situation.... Are all other heart parents brains thinking like this too or is it just our over education in the subject of possibilities that could happen?

So...we saw the ENT specialist today to ease Mommy & Daddy's fears. Although we have been told by several well meaning people that this can be a normal sound, you aren't going to convince us. Right off, Addy was not a fan of the fellow or the attending. It really PISSED her off that they dare to look in her mouth and ears. Those of you who have never actually met Addy might not know that she doesn't fit the profile of a "hypoplast" heart kid. She is bulldozer- hard headed, never tires (or doesn't stop if she does), not afraid of anything, and opinionated...just like her mommy & daddy! Needless to say, she let them know that their behavior was not going to be tolerated with loud screams- while at the same time demonstrating what we were talking about! So then they decided that they wanted to take a quick peek at her vocal cords by shoving this little camera scope up her nose....thank God we swaddled her because otherwise I'm fairly sure she would have decked them! See, when she still had that NG tube she was fairly used to having things put down her nose, then she didn't even cry. But now, I'm sure she thought that we were putting it back in or something because she was terrified! Luckily I had enough forethought to remember to make sure we had a pacifier, but more importantly "Beary Manilow". As soon as they finished and stepped back away from her she quickly calmed and shot them a dirty look while hugging Beary and clinging to me. I felt so bad for her! However, it did give us some answers....

So it appears that Addy has some extra skin that is kind of sitting up above her vocal cords that sometimes gets in the way when she cries, hence making the stridorous sound. Most likely this is an anatomical thing, not something that was a result of her previous intubations. Sometimes as kids grow, these pieces of extra tissue may grow as well, thus creating more problems. Most likely, nothing will ever have to be done, but it is good to know as it could potentially create more difficulty with accessing her vocal cords for future intubations. However, the doc would like to take a more thorough look at everything further down below the cords to make sure their is no other stenosis or narrowing that could also be causing it. So...that pretty much secures our future of a cardiac MRI. Sedation team is not going to be thrilled about sedating her with these questions and then they can bronch her at the same time as the MRI (which anesthesia will be present at). On one hand we are glad that we aren't crazy and neurotic overprotective parents that are making up imaginary things; on the other hand, I hope and pray that this is nothing that will affect her in the future.

On a lighter note, Addy is thriving in every other way! She is THE MOST active baby I have ever seen. As my mother tells me almost every day, "She's just like you!" She crawls amazingly fast, pulls up in a heartbeat, and is starting to play around with letting go of the furniture! She says "no,no, no" a lot, she has learned "uh-oh" and drops things a lot so she can say it, and seems to be repeating an assortment of other words. She is the only baby I have ever seen that actually whistles! I think it is because sometimes she purse-lip breaths to pace herself and figured out that she can make a noise when she does it. She loves playing in the swimming pool and is a true water baby. She now has 3 teeth and eats everything! At our last checkup she was almost 18 pounds, no puny heart baby by far! We recently went on vacation and she had her first trip to the beach! She didn't think much of the ocean, instead she spent more time in her baby pool on the shore! As I feel the need to photograph every event of every day, I have lots of recent pictures to post but here are just a few...please enjoy our sweet girl, we think she is awfully cute!