Hope Leads the Way for Girl With Rare Lung Disorder

Stanford Medicine Children’s Health performs first-in-world simultaneous lung transplant and chest wall expansion surgery on a young child

Meet 6-year-old Violet from Shasta Lake, California. She’s feisty and brave. She rolls with the punches. She has her own pace to life. And her middle name is Hope.

“We chose Hope because life can be so hard without hope,” says Tawna, Violet’s mom. “We didn’t know how much we would need hope in the years ahead.”

When Violet was born, everything seemed fine. She was full-term and healthy. Then, at 1 month old, she developed a cough that wouldn’t go away. Her doctor thought it was a lung infection, but further testing revealed that Violet had a very rare lung disease called surfactant protein C deficiency, a genetic disorder. Surfactant is a crucial substance within the lungs that helps keep the alveoli (air sacs) open. Without enough of it, the alveoli collapse, making it hard to breathe.

“For the next four years, Violet was on oxygen, but her lungs kept getting worse. She developed secondary pulmonary hypertension,” Tawna says. “Our doctors said her only hope for survival was a lung transplant.”

At age 4, Violet was referred to Stanford Medicine Children’s Health’s Lung and Heart-Lung Transplant program. It’s where she earned her honorary title of Lung Warrior Princess. And it’s where the family’s hope was truly tested. It’s also where they came up with their family motto: Hope will lead the way.

Awaiting donor lungs for nearly a year

Tawna likes to refer to Violet’s long wait for donor lungs as an adventure rather than a journey. It’s this positivity that helped fuel the family’s enduring hope. It carried them through two dry runs for possible lung transplantation before finding the right donor for Violet.

The family—Tawna, dad Andrew, and older brother Jameson—basically lived at the hospital with Violet. She needed to stay to receive continual advanced care for her very sick lungs. One of them was always there at her bedside.

“Andrew was there the most. He would stay all night and day,” Tawna says.

The family appreciated how the care team supported them and how they understood Violet’s need to go at her own pace, due to autism. The family wore masks continually and asked everyone who came in contact with Violet to do the same to protect her vulnerable lungs from infection.

“Everybody was really thoughtful, and they respected our boundaries,” Andrew says.

“I remember a doctor saying early on they were going to go at Violet’s pace,” Tawna says. “That sparked our motto that hope will lead the way. Throughout Violet Hope’s care adventure, we followed her lead, and that is how we did so well.”

Taking a creative approach to make space for new lungs

A challenge for getting new lungs for Violet was that her chest was too small to fit lungs large enough to sustain her as she grew. The lung transplant team came up with the innovative idea of partnering with the Chest Wall Program at Stanford Children’s to consider a chest expansion surgery, a modified Nuss procedure, at the same time as performing the lung transplant surgery. The dual surgery has been done a handful of times worldwide, but never before on a child as young as Violet.

“Violet developed pectus excavatum, or sunken chest, because she was trying so hard to breathe over the years,” says Stephanie Chao, MD, pediatric surgeon and director of the Chest Wall Program. “As a young child, the chest wall is flexible cartilage rather than bony rib, so when Violet breathed hard, she would suck in her chest from the force of inhaling. Doing so essentially remodeled her chest wall, making it concaved.”

Pectus excavatum is considered a risk factor for lung transplant failure, so resolving it was vital. Planning to expand Violet’s chest and make room for donor lungs gave her doctors a better chance of finding donor lungs, where otherwise it might not have been possible. Also, with Violet’s concave chest repaired, her new lungs would function better after transplantation. Importantly, combining the procedures meant only one surgery for Violet instead of two.

“Thinking outside the box to bring different services together for a better outcome for a child is something we do often at Stanford Children’s,” Dr. Chao says. “We don’t shy away from challenges.”

Deciding on this innovative solution involved bringing together several specialty teams, including the Lung and Heart-Lung Transplant, Chest Wall Program, Pulmonary Vascular Disease Program, Radiology, and intensive care teams.

“Other national centers refer some of their most challenging lung and heart-lung transplant cases to us because we don’t hesitate to act innovatively to give children a chance at a longer or better quality of life, even for those children who are deemed inoperable or too high-risk,” says Michael Ma, MD, chief of Pediatric Heart Surgery and surgical director of Lung and Heart-Lung Transplant at Stanford Children’s.

The multidisciplinary approach included Frandics Chan, MD, pediatric radiologist, and David Cornfield, MD, chief of Pulmonary, Asthma, and Sleep Medicine. The two worked together to provide innovative 3-D virtual imaging of Violet’s chest, developed partly by Dr. Chan, to determine which potential donors were a good match.

“Prior to transplant, Violet spent most of her life in the hospital, including 330 days in the pediatric intensive care at Stanford Children’s,” says Dr. Cornfield. “Dozens and dozens of people cared deeply and carefully for Violet so that she was healthy enough to undergo transplant. Violet’s course, from birth until today, is a story of love, hope, resilience, and perseverance—a true inspiration!”

Undergoing a simultaneous lung transplant and modified Nuss procedure

Finally, the family got the good news they had been waiting on for months. The lung transplant care team had donor lungs for Violet.

“When Dr. Cornfield came in and said, ‘We have lungs for you,’ I didn’t think transplant was possible because Violet had a cold. He said they were comfortable moving forward, and it went really fast and smoothly from there,” Tawna says. “You wait so long, so when it finally happens, it feels really sudden.”

During the surgery, the lung transplant team and the chest wall team worked side by side. First, Violet’s damaged lungs were removed. Next, the surgical team performed cryoablation to freeze the nerves in Violet’s chest cavity to provide pain relief for several weeks after.

“At that point, the transplant surgeon placed the new lungs, and right after, I performed the modified Nuss procedure by fitting two curved titanium bars to elevate Violet’s sternum and create more space in her thoracic cavity,” Dr. Chao says.

Since the modified Nuss procedure is rarely done on 5-year-olds, Dr. Chao worked with different manufacturers to ensure that she had small enough bars ready for Violet. The manufacturer representatives were present during her procedure to help as needed. The surgery went exactly as planned. The team’s strategy on how much space the modified Nuss procedure would give them, and which size lungs they could accept, was spot-on.

“Violet did amazing, and the surgeons did amazing. We felt so comfortable and confident with them,” Tawna says.

Violet’s recovery went smoothly. The breathing tube came out sooner than the family expected, and she only needed a little oxygen at first. Just over two weeks later, Violet was discharged from the hospital to live nearby at the Ronald McDonald House at Stanford with her family for her first three months after her surgery.

Paving the way for better care for other kids

Violet is the youngest reported patient in the world to undergo these two surgeries at once. She is paving the way for other children at Stanford Children’s, and beyond, to receive the same chance at a better outcome.

“Violet and her experience have helped inspire new treatments. It’s her legacy,” Tawna says.

Dr. Chao is proud of what Stanford Children’s was able to do for Violet, but she gives a lot of credit to the family for her good outcome: “Living in an ICU room for almost a year—not sleeping well, not having your home—takes a toll. It’s hard to hold on to hope through all the ups and downs, but Violet’s family was always gracious and never gave up hope. I respect the lengths they went to for their daughter.”

Home and enjoying life today

Violet is happy to finally be home. She loves watching her favorite PBS shows—Daniel Tiger’s Neighborhood, Rosie’s Rules, and others. She enjoys playing with technology and learning toys. “She loves to dress up like a princess. We change her dress every hour,” Andrew says. He has noticed a change in Violet since her lung transplant. “She’s happier and she laughs more.”

Violet’s transplanted lungs are doing well. She no longer needs oxygen, a great relief for her family. She receives regular checkups from her Stanford Children’s team.

The family’s hope prevailed. “She’s doing better than we could have ever imagined,” Tawna says.

Learn more about the Lung and Heart-Lung Transplant Program at Stanford Children’s >