Thursday, February 12, 2015

Faces of CHD: Meet Ava

February 7-14 is Congenital Heart Defect Awareness Week. To help raise awareness, spread knowledge and share the effects of this disease, Jayme of The Paper Deer Photography Blog, Christie of Lemon Squeezy Home and Kierra of The Irvine Home are each sharing one story of a child with CHD every day to showcase the trials, triumphs, journey and heartache of this devastating disease. We invite you to read and share this project to further our voices. Thank you. #Facesof CHD



Meet Ava Monroe!  Chandler and I (Kaila) have the privilege of raising this extraordinary little, now 6 month old, life together.  We live in Georgia, about 45 miles west of Atlanta, and have been married for just shy of 3 years.  After being diagnosed with PCOS several years ago, we did not see a baby in our near future at all.  Especially after being told we would need to seek fertility to conceive.  But just before Christmas 2013, we were shocked to find out that we would be welcoming our little miracle in the summer.

Ava’s journey in the CICU (Cardiac Intensive Care Unit) began at just 12 hours old.  Our brand new baby girl was transferred to Egleston very quickly.  And thanks to a very easy delivery, I was discharged at the same time.  This particular CICU was open-concept.  There were 26 bed-spaces, and every last one was full.  This was seemingly nice for the first… several hours.  We met a few parents, listened to their experiences and swapped pictures.  But then, roughly 6 hours into our stay, the baby in the bed space next to us lost his battle.  My heart still drops as I replay the mother’s screams in my head.  They echoed through the halls and into my soul.  I remember telling God, “do not let that be me”.
Other than surrounding chaos, our first five days were full of optimism.  The week consisted of meeting with doctors & surgeons.  They came up with her plan of care and informed us that we were looking at 10-14 days of recovery before we would be home-bound.  Ava was not allowed food that first week due to her specific CHD (Congenital Heart Defect) & required Prostaglandins to keep a special duct open until her surgery.  She had no oxygen requirements, just IV’s & central lines (which prevented us from holding her).  On day 6, we walked alongside our baby girl as they wheeled her back to the OR.  There was really no time to process any of it as we were immediately dumped into a waiting room FULL of people, including much of our large family.  After hours of waiting, the surgeon came and found us.  He greeted us with sweat dripping from his surgical cap.  Waiting for his words was the most nervous I had been up to that point.  He told us that he had repaired Ava’s aortic arch and tried to get to her VSD but couldn’t cover it all.  He went on to say that her pressures in her right ventricle were “a little funky” and that he would watch that closely but he hoped it would work itself out as her heart got accustomed to it’s new “flow”.  Thankfully, it did.

Nothing could have prepared for what we walked in to.  Just hours prior, we had handed off our seemingly perfect, 5 lb, 14 oz. child.  The sight that greeted us when we walked back in to the CICU was simply terrifying.  They had to leave Ava’s chest open to allow room for swelling and extra fluid that had been pumped into her during surgery.  All that was covering her tiny beating heart was a clear patch stitched between her chest bones.  The nurses were whispering to us, assuring us that she looked very good and letting us know we could touch her hands and feet.  They didn’t want us talking to her too much or stimulating her, being that her little body had just experienced such major trauma.  It was all we could do to text our families goodbyes.  Truly, we could not have faced anyone in those 4 days that she remained open.  They were excruciatingly long.  Once Ava was closed, they started lifting her sedations relatively quickly.  There was such hope in seeing her big round eyes open after so long.  I felt so disconnected from her, but so admirable of her at the same time.  I just remember feeling like, we finally made it!  Our new life is about to start.

Two days later, they were ready to extubate her (remove her breathing tube)!  We were anxiously awaiting this moment because this was a huge step toward going home.  As soon as they allowed us back in the unit, there were nearly 10 people scrambling around Ava’s bed-side.  They were already switching her from the oxygen she was on to a RAM Cannula (which gives additional pressure to help expand the lungs) and they assured us that it was very common for post-op babies to need a little extra respiratory help immediately following a surgery such as hers.  However, within the hour, it was clear that Ava’s respiratory status was declining and they emergently re-intubated her.  Little did we know, this was the beginning of a new journey for us. 

Ava’s first three extubations, were chalked up to her just needing to regain strength.  After all, time was not much to ask for after what our girl had endured.  So time we gave her.  After nearly a month since surgery, it was time to extubate for the fourth time, things were going surprisingly well.  So well in fact, that my mom came to sit with Ava while my husband and I made the hour+ drive home for the first time.  The second, and I literally mean the second, that we opened our front door.. I received a call from the CICU.  The doctor was rambling and rambling and I just remember screaming, “What Happened?!”.  Ava had been re-intubated.. but that was not all.  She had a line that had been in her right femoral artery for over 3 weeks.  They speculated that it must have formed a clot because after the nurse flushed her line.. Ava lost all profusion to the lower half of her body.  There was not adequate blood flow being delivered to her lower extremities.  We immediately got in the car and headed back to Atlanta.  My mom had been rushed out in the haste of things.  Ava was started on blood thinners and they were hoping that things would resolve quickly.  By the time we arrived, her left side looked seemingly normal, but her right leg was very alarming.  It was modeled, and there were spots of black and purple from her hip to her toes.  The lower you looked, the worse/darker it got.  The vascular surgeons sent their best ultrasound tech over to see what they could conclude.  Our doctors seemed to be shocked by what had happened, they said if this happened once a year that would be a lot.  The vascular team could not locate the clot, but informed us that it was not likely that our daughter would keep her right leg.  Devastated is too light a word.  When you are battling such a devil as CHD, and trying to make your mental list of things to prepare for, losing limbs is not on that list. 
The following days, despite what the vascular doctor had expected, her leg continued to improve.  And after a week, losing her leg had turned into, as the doctors put it, “JUST losing her toe”.  I remember dwelling on that word, “just”.  Many may have counted their blessings at that point, but I was just so exhausted and so bitter.  And after roughly a month and a half in the hospital, nothing had gone right, nothing.  Once we got over that hump, we were back to the old grind of trying to successfully extubate.  But again, the days were turning to weeks and every failed extubation turned into another.  Each time more devastating than the last.  Then, we had a terrible scare.  One morning, about a dozen doctors were gathered around Ava’s bedside as we walked in.  I had seen this before, around other babies, and it was never good.  They were all looking at her x-ray.  Some on their phones with other doctors, some with pharmacy, others checking labs and speculating amongst themselves.  We had seen her chest x-ray every single morning since we had been there.  That being said, we noticed it immediately:  Her heart was doubled in size.  To any heart parent, this is a nightmare, as it typically indicates heart failure.  She had several close calls with cardiac arrest and her heart began taking on the stress from all of her respiratory troubles.  Thankfully, with the help of cardiac medication and another intubation, the stress on her heart slowly retreated.

But Ava was just pitiful.  Her respiratory decline came quicker with every extubation.  I will never forget hearing the whistle of air as she fought for every unassisted breath.  Her violent retractions bore visibility to every centimeter of her ribs.  My heart was breaking, and my faith, I would have laughed at the very word.  The doctors did not think we would be bringing our daughter home, and although we never spoke it to each other, my husband and I felt that dream slipping away.

During these horrible months, Ava had gone to the OR several times for bronchoscopies to look at her airway.  It was the same each time.  She had suffered damage from the breathing tube.  But from what they could see, nothing significant enough to be causing her respiratory failure.  They then did an exploratory cath procedure.  But came back with nothing, her lungs and pulmonary arteries were working well. 
Every time we would check something off the list, the more time had been wasted.  All the while, this tiny person was fighting for every breath and we were no closer to offering her relief.  The days were so long, and the nights were longer.  Something as simple as gas pain was enough to send her over the edge, and cause her to need to be emergently re-intubated.  She had no energy left.  My baby was aging, but not growing.  I reached my limit of emotional, spiritual, physical exhaustion and pain.  I could not watch her go on like this any longer.  I felt defeated.  As I laid my head on my precious daughter that night, I whispered to God, with a lump in my throat, and a shattered heart, that he could have her back.  Nothing in this life will ever be so painful.  My husband and I sought nothing but each others arms, we cried until we were shells of human beings.  My husband told me that, as long as Ava fought, we would fight with her.  All it took was one look to see that there was not much left in her tiny, beautiful body.

That Wednesday, after a rough start to her final attempt at kicking the breathing tube, Chandler demanded her to be re-intubated.  We knew where it was headed and did not desire to see her struggle, that intubation was simply a relief.  From there, things moved quickly in the direction of Ava getting a Tracheostomy and G-tube.  It was the only way we would have a chance at taking her home, and to relieve her of her burden.  Within 48 hours, she had already had the surgeries.  And for the first time, in over 3 months, things were looking up.  Ava began to smile and stay awake for longer periods of time.  She had been on so many sedations for so long, and every wean just brought out more of her incredible personality.  Hope for her future flooded our hearts!  We were moved to the TICU (Technology Dependent ICU) and her recovery only expedited.  Within two weeks, she had weaned off the ventilator for 12 hours at a time!  The TICU team was blown away!  We had thought that we would be going home with nothing more than an empty car-seat, but our Ava fought for us, and I am so proud to say that she proved us wrong.  After 102 of the longest days of our lives, we were discharged with a positive prognosis in every way. The trach is temporary while she outgrows her airway trauma, for her lungs get to healthy and strong. We had fought through all of her drug withdrawal, we managed to keep all 10 fingers AND toes, & lastly, her heart was stable!  They told us that other than the need for the mandatory medications and monitoring, Ava would grow to have no limitations. 
From conception to survival, she is our miracle.

I have to say a thank you to the family and community that held us together when we had reached the end of ourselves.  When we had no faith, you were our intercessors.  When our emergency funds became depleted, we not once needed for anything.  We could not have made it without the support of these beautiful people.

It is hard to sum up what CHD means to me.. I suppose I have to say, it is a reckless love.  I have never known a more reckless, selfless, scary, or beautiful love since coming to know so many families in the CHD world.  The parents, the siblings, the families of donors have shown us/displayed such a variance of this.  Not one of them/us can control our fighter’s outcome.. and even so, the love is immeasurable.


- Kaila (Ava's mom)

You are welcome to follow the Biagini family on Instagram: @urbnmama @kailabiagini and @chandlerbiagini

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