All 4 of my daughters are all special and unique, however Annabelle has a very special heart. At about 26 weeks pregnant, we found out she had a rare heart condition.
A variation of HLHS (Hypoplastic Left Heart Syndrome,) her official diagnosis was an unbalanced AVSD (Atrioventricular Septal Defect) with a hypoplastic left heart and aorta.
Basically, instead of one valve to the right ventricle and one to the left, she had a hole in between them which make them one big valve. On top of that, it was "unbalanced" meaning the one big valve was more over the right ventricle than the left, which caused limited blood flow to the left ventricle, thus the hypoplastic left heart and aorta.
There is no "fix" for a hypoplastic left heart. Annabelle's left heart was too small to be viable, so she had to undergo a single ventricle repair. The goal was to reroute the blood flow to use only half of her heart. This is not ideal, obviously, and many kids that undergo this procedure must eventually have a heart transplant, however the hope was to get her through for many years.
The repair itself involves three surgeries, the Norwood within the first week or two of birth, the Glenn between 3-6 months, and the Fontan at age 15 months to 2 years (although the time frame much depends on the baby so this could be earlier or later.)
This was a temporary fix at best. The rate of survival through all stages is about 60%, a little less or more depending on who you talk to. As a mother, those odds scared the living daylights out of me. As a Christian and follower of Jesus, I remained confident that God's hand was on my daughter and the odds meant nothing, knowing that God was in control.
The Beginning of Annabelle's Journey -
Annabelle had her Norwood surgery when she was 6 days old. She had several complications, including one very scary night 2 days after surgery where we thought she might not make it and another day in September where she coded twice and had to be "cooled" to prevent brain damage. (Picture walking in to your daughter wrapped with ice packs and almost freezing to the touch.)
I lost count at home many times she was extubated only to fail and have to be put back on a ventilator.
In October 2010, since she'd been unable to sustain herself off the ventilator, they felt the issue was due to a leaky valve and decided to do her Glenn surgery early, which would hopefully take much pressure off the heart and help her leaky valve. Unfortunately, when they took her into the OR to do surgery, she turned blue and her O2 Sats were bad, so instead, they tried to repair the valve and changed her to a different type of shunt (a BT shunt to a RA-PA condoit.) This new shunt was bigger and, theoretically, should have helped her to go a few more months until she was ready for her Glenn.
After a few more attempts, she was finally able to come off the ventilator with her new shunt. We were unable to leave the hospital though, as her respiratory status wasn't stable.
Because of severe reflux and an inability to eat by mouth due to fear of aspiration, she had a Nissen/GTube surgery as well in early December.
We were set to go home on Christmas Eve for the first time... but Annabelle ran a fever of 102 that morning.
A week later, we had a heart-cath, then were set to go home again. This time, she was rushed to the ICU due to respiratory issues on the morning of our intended home-going.
Annabelle's New Direction
The next week, we sat down with doctors and were told that due to Annabelle's decreased heart function and moderate to severe AV Valve regurge (the attempt to fix in October had failed) that they no longer thought she would be able to survive her planned Glenn reconstruction. Her safest, and really only option, was to be listed for a heart transplant.
On Thursday, January 13th, 2011, Annabelle was officially added to the Heart Transplant list with a 1b status. The next day, she suffered another episode of acute respiratory distress, was rushed to the ICU, and was placed on special IV heart medication to preserve her heart function, and was raised in status to 1a, the highest and most urgent status for transplant.
We prayed and waited for a new heart, very saddened to know this could only come about if another parent faced the unimaginable loss of a child.
But God is a big God. And we determined to trust Him during those times.
She was taken to the OR at 4:30 that afternoon, and received the donor heart late that evening and was out of the OR by midnight.
We are deeply indebted to the donor family, for their gift amidst the very horrible tragedy they were facing. Words cannot express our gratitude.
The Journey Home
Annabelle was finally released from the hospital for the first time on May 24th, 2011, 308 days after birth. She was readmitted six days later for possible rejection.
Four days later... home...
Four days later... back in the hospital, fearing greater rejection. They did a heart cath on June 8th, 2011, but no rejection was found. They did find a few issues with her new heart (a compressed coronary artery and a narrow pulmonary artery.) Heart surgery was scheduled for the following day, which is a good thing as her condition deteriorated over night and she was emergently put on the ventilator in the early morning hours.
After 10 days in the hospital, we went home yet again, another heart surgery complete.
In July, 2011, a week before Annabelle's 1st birthday, she was admitted to the hospital again, for one day, due to fluid issues.
She was able to spend her birthday at home, but on Saturday morning, July 23rd, we woke to Annabelle screaming. I rocked her, but noticed her breathing funny. Checking her oxygen level, we found it to be 40, when it should be 90+
We called 911, and by the time they got to our house, her eyes were glassed over and she was non-responsive. She was taken to the nearest hospital, intubated in the ambulance, and life-flighted to Vanderbilt Children's Hospital.
But she's come so far. She's walking and running and even starting to jump a little! She's using her big girl words, even though her speech is delayed and communication isn't simple. Yet she is so smart, so intelligent. She loves to play with her big sisters and is just such a joy. We've recently weaned her from her feeding tube and she is for the FIRST time eating all her nutrition by mouth!
We've learned a lot with Annabelle. To treasure every single day as a gift from God. To love fiercely, knowing we are never promised tomorrow. Heaven is our goal, no matter how many days on Earth God allows us.
You can read through the posts about our Annabelle's journey here, and earlier (pre-named) here.
The most up-to-date information on Annabelle is found on her Facebook prayer page at www.facebook.com/prayersforprincessannabelle.
There are a few specific posts I'd like to point out though, as they are ones I refer back to often during the journey, and pray that they may be helpful to you as well.
Do Dreams really come true?
I'm happy to talk about congenital heart defects and organ donation awareness, and answer any questions you may have. Please feel free to contact me (contact info is at the bottom!)