We all know the power of sharing your personal story through illness. As a child life specialist, I work closely with kids to help them find their voice, document it in words, photos or illustrations. It’s therapeutic for the child, but also inspiring to others. I’m excited to share a new children’s book, created by two young siblings, Jack and Everly.
Guest Blogger, Lauren Backe; Mother and Advocate
Various media outlets caught wind of our Jack winning the national Starlight Children’s Foundation’s ‘Design a Gown’ contest— where people across the country were encouraged to design a creative hospital gown for children facing challenges in the hospital. He entered the contest for his sister, Everly, who has a Congenital Heart Defect (CHD). Upon his win, 30,000 comfortable, imaginative hospital gowns will be made and distributed in the likeness of his heartfelt creation: hearts and lots of printed glitter! Since winning, numerous people have approached my husband and I wanting to learn more about CHDs and Everly’s journey with it thus far.
I didn’t know much about CHD until July 2017. I didn’t think I knew many babies who had it (turns out, I do know a few: an old neighbor’s son, a classmate of Jack’s brother, a friend of a friend’s daughter….) CHD is the most common birth defect. I asked LOTS of specialists (everyone we saw in July and August) what could’ve caused Everly’s CHD. They all assured me that I didn’t do anything to have caused it. Everly’s genetic testing came back clean. The heart starts as a tube and grows and branches off from there. From all I’ve learned about fetal heart development- it seems to me- it’s just a complete miracle that so many hearts form “normally.”
Everly was born in August. We found out a few months before birth that she had a CHD that would need to be repaired before she left the hospital. I remember the day vividly. After an ultrasound at around 30 weeks, my local OB said the heart looked a little off and we should get an ECHO to rule anything out. Not expecting to hear anything “bad” at the ECHO, I went by myself. I knew the news wasn’t great when the cardiologist said to me “So you came by yourself, huh?” I left the appointment with pictures drawn of our unborn baby’s heart compared to a “normal” heart and the cardiologist offered to call my husband that evening.
After learning her diagnosis, we set up to find the best surgeon for our girl. We are lucky that there are several “3 Star” rated heart hospitals near by. One particular surgeon at one of these hospitals came very highly recommended by just about anyone you asked. After meeting with him, we knew he was the one!
We met with an Maternal Fetal Medicine Specialist (high risk OB) and set up an induction day so that all of the right people for Everly would be ready for her when she was born. A few minutes after birth, the NICU swept her away to get her lines set up to receive medication. They also performed an ECHO on her to get more pictures of her heart outside of my belly.
CHD is 1 in 100 births.
IAA is 2 in 100,000. IAA is about 1% of CHDs.
Everly had the Norwood surgery to repair her IAA at 3 days old. The Norwood surgery for the IAA defect is not commonly needed. We had hoped that Everly would have ONLY ONE open heart/bypass surgery right after birth that would fix the IAA and the VSD. Unfortunately, her heart did not grow as expected between the fetal ECHO and birth, and she was not able to have that surgery (which is a less risky open heart/bypass surgery- STAT 4). STAT levels range from 1 to 5 with 5 being the most complex/dangerous.
Instead, she needed to have a very complex surgery (STAT 5 level). The Norwood corrected the IAA, but not the VSD (some parts of her heart needed time to grow before repairing the VSD). She will need to have multiple open heart/bypass surgeries in her life.
In October, Everly was briefly hospitalized for a virus. Thankfully, we were able to take care of her the majority of the time at home with home oxygen tanks/machines.
She had a “cath” (cardiac catheter) in November 2017- this looked at the functioning of her heart to determine next steps- it’s a surgery with intubation and anesthesia but we were only there one night- it is not “open heart”. In the cath, they were able to do some “ballooning” to help buy a little bit of time.
In December 2017, Everly had an additional shunt put in (STAT 4) because she was not yet big enough for the “full repair”- the Rastelli procedure. She now has two shunts- one from the Norwood and then the additional one put in, in December.
In April 2018, Everly was briefly hospitalized for a virus- she needed some extra IV fluids to make sure the 2 shunts in her heart didn’t risk clotting.
The Rastelli surgery will be next. This will put a conduit in- tube and valve and fix the VSD. She needs to be big enough for the Rastelli (STAT 3). The conduit is bigger than a shunt so there needs to be enough space in her chest/heart for this conduit to fit. After the Rastelli, we will hopefully have about 3-5 years until the next open heart surgery.
Approximately every 3-5 years, she will need to have the conduit replaced. When she is big enough for an adult size conduit, the valve in the tube can be replaced through her leg (vs. an open heart surgery). However, the adult size conduit will not last forever and she will need to have the conduit replaced (more open heart surgery). It is too hard to predict how many valve and/or conduit replacements she will need in her lifetime.
Everly is very medically fragile currently since her heart functions like it only has one chamber (not two like a normal heart), but will hopefully be considerably stronger after the Rastelli surgery. The time between the Norwood and the Rastelli is called the “interstage”- Everly is part of a “Norwood Clinic” where we have a lot of specialists for just a few patients because of the extreme fragile-ness. (If Everly had had the one time repair we initially had hoped for, we wouldn’t be part of this “group”). We hadn’t planned for this (the Norwood surgery) so we didn’t know the specialists. BUT we were so lucky to be handed this amazing team. They are truly amazing and have helped us so much in these very critical months. We will continue to be part of this “Norwood Clinic” until the Rastelli.
So in short…. What have we learned over the past 10ish months?
Choose joy each day, find joy in small things, celebrate all victories- even small ones, take things one day at a time, try not to take time with family and friends for granted, try to live in the moment, try not to sweat the small stuff, breathe…. This little girl and her strength and Jack’s ability to adapt to all the changes has taught us so much in 9 short months. Everyday, we are grateful for both of them. Perspective is everything.
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