Cody and Hayley Smith knew their lives would change with the birth of their first child. But they never imagined just how heartbreaking, joyful and profound those changes would be.
They credit the lung transplant team and staff at St. Louis Children’s Hospital with helping them navigate a harrowing medical journey that has given two of their children a second chance at life.
The Smiths are former high-school sweethearts from Alabama. Seven years ago, they were living in Washington state where Haley was stationed with the U.S. Navy. There, she gave birth to Kimberlyn, their first child.
At first, the baby appeared healthy. But staff at the hospital noticed she had difficulty breathing and thought she may have amniotic fluid in her lungs. When she didn’t get better, they suspected a lung infection and transferred her to an Army hospital in Tacoma.
Doctors there struggled to find the cause of her breathing problem. One Army physician, who had trained at St. Louis Children’s Hospital, suspected that Kimberlyn, called “Kimmy,” was suffering from a rare genetic lung disease, ABCA3 deficiency. A genetic testing and a biopsy confirmed the grim diagnosis.
With ABCA3 deficiency, the lungs don’t produce enough surfactant — the slippery substance that coats the lining of the lungs. Without surfactant, the lungs can’t expand to take in enough oxygen. The condition, caused by mutations in the ABCA3 gene, is very rare and usually fatal.
With the help of the Army doctor, the Smiths contacted the St. Louis Children’s Hospital lung transplant team and made the decision to transport their baby to St. Louis to be evaluated.
Only a few babies each year receive lung transplants. Children’s experience and expertise with this procedure is unmatched in the U.S., says Joshua Blatter, MD, MPH, Washington University transplant pulmonologist at St. Louis Children’s Hospital.
“It's a unique multidisciplinary team that takes care of patients and families who are considered for lung transplant,” he says. “Not just nurses and surgeons and medical doctors, but also social workers, psychologists, dietitians, physical therapists, respiratory therapists, and Child Life specialists, among others. We receive questions about lung transplant from physicians across the country — and around the world.”
Arriving in St. Louis on Halloween week, 2015, the Smiths learned their newborn daughter’s best chance at survival was a lung transplant.
The lung transplant team gave them a thorough, realistic picture of the transplant process and what Kimmy’s future might look like. So, the Smiths knew that even with a new set of lungs, Kimmy would probably have some long-term issues, says Cody.
“Education is essential to this process,” Dr. Blatter says. “Transplant is not ultimately the ‘right choice’ for some families. A family can be told by doctors that transplant is an option — but they might still independently decide that they don’t want to proceed. They might have fears about the procedure. They might not want to relocate to our transplant center to await organ offers. They need to hear what the entire experience looks like so they can make a decision that represents them and their own values.”
This comprehensive information helped the Smiths decide to keep letting Kimmy fight, Cody says. She was added to the transplant waiting list.
The wait that followed included “very dark days” when it looked like Kimmy wouldn’t make it to transplant. Finally, on Jan. 24, 2016, the call came that donor lungs had become available. Kimmy received her lifesaving transplant the next day.
She remained at Children’s for several months after the surgery. Although Kimmy’s recovery was slow and often bumpy, meeting other patients and their families dealing with similar or worse problems taught the Smiths to savor every moment, says Cody.
Kimmy was discharged after spending 334 days at Children’s.
But more problems lay ahead as the Smiths had their second child. The Smith’s son Harrison was born in 2017. He also had ABCA3 deficiency and died at 22 weeks old.
Daughter Riley was born in 2018, without ABCA3 deficiency and healthy.
When Hayley became pregnant with their fourth child, prenatal testing revealed that the baby also carried the rare mutations and would be born with ABCA3 deficiency.
After much thought, discussion and consultation, the Smiths, who had been transferred to Virginia, decided that after this child was born, they’d keep him as comfortable as possible and let nature take its course.
Their son Houston was born Jan. 31, 2020. From the start, he fought the dire diagnosis that hung over him. His parents and doctors noted that “this little boy has got some spunk,” says Cody.
After conferring with the team at St. Louis Children’s Hospital and learning that Houston was also a lung transplant candidate, the Smiths returned to St. Louis.
But shortly thereafter, life threw the Smiths and the rest of the world another curveball – COVID-19.
Because of COVID-19 restrictions, Haley spent most of her time at Children’s with Houston in the neonatal ICU, while Cody stayed hunkered down at a nearby apartment with their daughters. The Smiths say it was just another hurdle to overcome.
On June 24, Houston received a healthy pair of donor lungs. The Smiths say his recovery has been more straightforward with fewer complications than his sister’s.
Now stationed at Scott Air Force Base, with all their children at home, the Smiths have been able to focus on the future.
Kimmy still faces several health and developmental challenges. But she’s a happy child who loves meeting people and is “best friends” with her sister Riley. Helping her to survive and meet her challenges has helped the family to grow and their bond to strengthen, they say.
“We know we had Kimmy for a reason,” says Cody.
Meanwhile, Houston continues to make steady progress. He’s no longer on oxygen and he’s scheduled to have his tracheostomy canula removed this summer.
The Smiths also anticipate moving this summer, after Hayley Smith receives new orders. Ideally, the family would like to be sent to the Naval Air Station in Jacksonville, Fla., which is near the University of Florida pediatric lung transplant program in Gainesville.
Wherever they end up, Hayley plans to begin nursing school and Cody would like to start classes to become a teacher. Both say they the experience with their children has inspired them to pursue careers that allow them give back. The Smiths have also participated in ongoing research studies at Washington University to help other infants and children with this rare disease.
Both consider St. Louis a second home — and the staff at St. Louis Children’s Hospital a second family.
Says Cody, “They’ve changed our lives forever.”