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.
Play is powerful and therapeutic. When children have the opportunity to play out familiar experiences, they gain control and build resiliency. The media has drawn some attention to commercial dolls and toys that also represent children with differences.
As a child life specialist, I love hearing how others are making a positive impact on a child’s life. I’m excited to share Everly’s story with you.
Guest Blogger, Lauren Backe
Our daughter, Everly, was born with a congenital heart defect. That means that her heart didn’t form in a way that would be able to sustain her out of utero. She had her first open-heart surgery, a category 5 mortality- the worst ranking there is, at just 3 days old. Her heart was the size of a walnut. With a total of 3 open-heart surgeries, 2 cardiac catheterization procedures, and 1 infection washout surgery that reopened her chest all before her first birthday. That means that although we’ve gotten almost a year “break” from surgeries, we know there are many, many, many more open-heart surgeries in her future because there is no cure for CHD, just a fix for an unknown time.
But today’s story doesn’t focus on her heart directly. During her first hospitalization, she had an IV that leaked out into her foot. This caused a HUGE, yellow, open wound on her foot for almost the entire first year of her life. There were lots of extra appointments, painful dressing changes, new specialists, new medications. This meant visiting the high-risk cardiology team located (on a good day) 1.75 hours from our house, then stopping at the pediatric plastic surgeon’s campus then driving home, every single week for months with a tiny newborn who was functioning with a half a heart and about 75% of the oxygen you and I do.
Although we’re grateful this was the biggest complication she had after this first (HUGE) open heart surgery, it was one more thing on a long list of things we had to keep an eye on when we brought our newborn home from the hospital. When she came home from the hospital, she wasn’t allowed to cry. We checked her oxygen levels and heart rates multiple times throughout the day. She was on a feeding pump with a feeding tube in her nose that ran every 3 hours, for a total of 8 hours of the day. She had a strict medication schedule with too many medications and doses daily to count.
While the most important thing to fix is her heart, as a mom I do wonder if the infiltration injury will impact her in other ways. What if her feet are never the same size? And what if she can’t splurge on a nice pair of shoes because she has to buy 2 pairs? What if she has a large scar and is embarrassed about getting a pedicure with friends? We’ll cross that bridge when we come to it—but for now, we’re happy that she’s alive because of the care she’s received. A scar is a small thing, comparatively, when her heart condition is literally life or death. We’ll work to find beauty and strength in the scars because they are a symbol of her will to fight.
Fast forward from the infiltrate almost 2 years… and Everly’s right foot is still larger than her left. There also appears to be some nerve and circulation issues as she doesn’t like to keep a shoe on that foot and bangs that foot repeatedly when walking or crawling. Everly’s team of specialists decided that we should try a compression sock. Let me tell you finding someone who could make a custom compression sock for a toddler was not easy! But we found someone, after lots of phone calls and emails and research.
A few weeks ago, we met with a CPed/LPed (Sheck and Siress) who measured Everly to have a custom sock made for her. She shared with someone at the company who was making the sock (Jobst) all about our little fashionista Everly and how I was worried she wasn’t going to keep the sock on or tolerate it well. At our first appointment, I asked if we had color options, etc. and she said she would ask but she thought it would just be a plain nude sock. And then I didn’t think much of it, except maybe I could get some patches to sew on it or something if she totally hated it.
I got a call two weeks ago that the sock was in. To be honest, I kinda dragged my feet about scheduling the appointment to go in and get them, It’s 50 minutes each way. And we had fun summer plans and nice weather in the days ahead. (Remember this is our first summer in 2 years without an open heart surgery scheduled… and Everly has finally been cleared to get out of the house and do stuff- she wasn’t allowed out of the house or able to be exposed to people- even close family and friends until recently to help keep her health). So I scheduled the appointment for Friday and purposefully planned a trip to a friend’s house after to take the sting off the stock I figured she’d be angry about.
Well, we walked into the appointment and the CPed/LPed surprised us with what the lady at Jobst created. Not only did she use PINK stitching…. She also attached butterfly patches and she went out and bought a baby doll with butterflies on her clothes and made Everly’s doll a sock that matches Everly’s.
I mean, can you even believe this?! Some stranger who doesn’t even know us thought of all of this and planned it all out. I was just truly blown away with the thoughtfulness and kindness.
We’ve been lucky enough to have and create the very best, most compassionate medical team for Everly. They love her like she is their own. I could write something about every single one of them. But what really blows me away about this, is that this lady at Jobst was a stranger. She doesn’t know us. She doesn’t know Everly. She hasn’t had the opportunity to spend time with Evie and fall in love with our little sweet and spicy firecracker.
Sometimes days feel hard and it’s hard to watch/hear what’s going on in the world in the news, but then something like this happens and you remember that there are a lot of really good people in the world. I wish we could hear more stories like this. About the good people in the world.
Please note: We are so incredibly grateful there is something that can be done to/for Everly’s heart. We are so grateful for all the doctors, nurses, clinicians, etc. who have dedicated their lives to working to find a cure for CHD and developing surgeries to help our children with CHD live longer lives. It is not lost on us that had Everly been born a few decades ago, we would not have even had these two beautiful years with her so far. Because of lots of hard work and research, her prognosis is to live into adulthood. And while taking care of Everly’s foot is/was hard work and she is not thrilled with wearing the compression sock, we remind ourselves of our family motto “We can do anything for a short time”. We remind ourselves how lucky we are for every single day and moment with her. Everly will be 2 in about a month and is thriving thanks to advances made in CHD care.
Lauren is a stay at home mom to her two children, Jack (7) and Everly (soon to be 2). Lauren is a former teacher and literacy coach. Lauren and her husband Matt feel strongly about sharing their CHD journey and life with a medically fragile child so that other families will hopefully not feel so alone. “Sometimes strength comes in knowing you are not alone.”
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 nearby. 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 the next steps- it’s 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 valves 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 have taught us so much in 9 short months. Every day, we are grateful for both of them. Perspective is everything.