On July 20th, 2010, we welcomed our 4th daughter into the world, Annabelle Lillian Faith Phillips.
All 4 of my daughters are all special and unique, however Annabelle was born with a very special heart.
The Diagnosis:
At 26 weeks, an ultrasound confirmed that Annabelle had 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 repairs they recommended were temporary, meant to give her the best quality life possible. The rate of survival through all stages is about 60%, a little less or more depending on who you talk to. We were told that most kids end up having to have a heart transplant at some point, if they live long enough.
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 first 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 into 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 next surgery early. Unfortunately, when they took her into the OR to do surgery, she turned blue and her O2 Sats were bad, so they changed out her shunt to a different kind, hoping it would relieve the pressure on the heart and give her a few more months to grow.
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, they no longer felt she would survive her planned Glenn reconstruction. Her safest, and really the 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.
On Saturday, April 9th, at 3:30 in the morning, we received THE call we’d been waiting to get for almost 3 months. A donor heart was available, and it was a good match for Annabelle!
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 overnight 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. Her oxygen levels were dangerously low, and by the time the ambulance arrived, she was non-responsive. She was life-flighted to Vanderbilt Children’s hospital in critical condition.
The cause of her distress was never officially diagnosed. She was treated for rejection because signs pointed toward heart failure, and she got better. After an 11 day hospital stay, she came home again.
Annabelle is now an enegentic 11-year-old little girl. Life isn’t without its struggles. Her risks for infection and other illnesses are great. Her long journey has resulted in significant medical trauma that presents as various mental health struggles.
But she’s come so far. She graduated from gtube feeding at age 5. She LOVES to music, LOVES to play outside (on the swing especially!), and is the bravest young lady I know!
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.
The most up-to-date information on Annabelle is found on her Facebook prayer page at www.facebook.com/prayersforprincessannabelle. (Although thankfully it is updated fairly infrequently the last few years!)
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.