Amy was 32 weeks pregnant when she was sent for a maternal-fetal consultation.
Something concerning had shown up on her ultrasound, but Amy and her husband, Jason, weren’t worried at that point. They had already had two healthy pregnancies, and this one seemed no different.
“We were really hoping things would be fine, and we could grab a quick date night snack before picking up our kids at daycare and school,” Amy said.
“Within an hour, we were at OSF HealthCare Children’s Hospital of Illinois, we were sitting down with a pediatric cardiologist and he explained to us a diagnosis we had never heard of before.”
Their daughter, whom they would name Hannah, had heterotaxy – a rare condition that affects how the child’s internal organs, including her heart, were forming.
“We were in shock. It just seemed so surreal because we had had two seemingly normal, healthy pregnancies. And then they are telling me she might need multiple heart surgeries in her first year of life,” Amy said.
Heterotaxy: ‘A unique arrangement of organs’
Nearly five years later, Amy and Jason are well versed in the list of things that makes Hannah’s body one of a kind, a “unique arrangement of organs,” Amy calls it.
Due to Hannah’s heterotaxy, her liver is unusually placed, and she has two right-shaped lungs. She also has intestinal malrotation – which means her intestines are abnormally twisted. And she was born without a spleen, which plays an important role in the immune system.
But what doctors were most concerned about was Hannah’s heart. She had complex congenital heart disease, including holes in both the upper and lower chambers of her heart.
“They were thinking within 6 to 9 months – maybe a year – she would be needing major surgery,” Jason said.
Hannah had her first heart surgery when she was 3 weeks old when her oxygen levels unexpectedly dropped. Surgeons installed a Blalock-Taussig (BT) shunt, a small tube to create a pathway for blood to go from the heart to the lungs.
“That’s when we got to know all the doctors and all the pediatric ICU nurses because that hospital stay was 4 weeks,” Amy said.
But Hannah’s heart journey was just beginning. Because of the complexity of her heart defects, Hannah would sooner or later need a major repair.
A life-saving surgery
Knowing that their child was facing a difficult journey, Jason and Amy decided to seek second opinions with other congenital heart programs to see what the best part forward for Hannah would be.
“We wanted to see the bigger picture,” Jason said.
Hannah had two congenital defects that would potentially be addressed: atrial septal defect, or ASD, which is a hole between the top two chambers of the heart, and ventricular septal defect, or VSD, a hole between the lower chambers.
As they were searching for a surgical solution, Hannah’s parents and doctors would need to determine whether only one of the holes could be closed or if surgeons could close both, giving Hannah a four-chambered heart.
Hannah’s doctors at OSF Children’s Hospital encouraged the family to look at all the options available, sharing their imaging and consulting with teams from other health systems. They even built a 3D model of Hannah’s heart to help visualize her unique anatomy.
OSF Children’s Hospital in partnership with the Advanced Imaging and Modeling (AIM) Lab developed a way to turn 2D images seen on a traditional MRI into 3D images that can be printed into an exact physical model of a patient’s heart. The couple still has their plaster copy of Hannah’s heart at their home in Washington, Illinois.
Taking this imaging technology to another level, Hannah’s team at OSF Children’s Hospital was able to view that 3D model in virtual reality, so her surgeon, Mark Plunkett, MD, could not just see, but interact with Hannah’s heart so he could confidently lay out a surgical plan that would give Hannah the outcome her parents hoped for.
“We knew we wanted that four-chambered heart because it would help Hannah in the long run and would have a better overall life expectancy. They had that scan and were able to look at the heart as if it was real to be able to tell if there was enough room to give us that four-chambered heart. We were much more confident with OSF and their ability to do the surgery,” Amy said.
Hannah had her bi-ventricle repair in November 2018, when she was 2 ½ years old. After four weeks in the hospital, she recovered and has an active life.
Hannah’s parents are grateful for the life she’s had so far. And while she will continue to face challenges, she will face them with parents who love her and a team of health care professionals committed to giving her a full and healthy life.
“We really trust our team. We feel like they are a great team of nurses and doctors. We have handed our baby over to them many times for her surgeries, and they have been able to give her an amazing life. She goes to pre-school and the babysitters, and she’s living a normal 4-year-old’s life right now,” Amy said.
For Amy and Jason, connecting with other parents has been an important part of their support system, whether local organizations like TOUCH or through Facebook and online groups.
“I just want other families to have hope that their child can live, can survive, and live a healthy life with heart defects. I want them to know that there are places they can reach out to for support,” Amy said. “I just want other families to know they are not alone on this journey.”