Siblings breathe easier following double-lung transplants

Before being wheeled into surgery for a double-lung transplant in March, David Diaz, 9, benefited from the knowledge of a close adviser: His older sister had gone through the same rare surgery just three years earlier.

“She told him, ‘David, don’t be scared. I’ve been through this, and you don’t feel pain or anything. They give you medicine so you can go to sleep,’” recalled their mother, Corina. “She was a very big help to him and answered all his questions.”

David’s big sister, Doris, now 12, was diagnosed with cystic fibrosis in 2006, when she was 6 months old. In 2007, 1-month-old David was also diagnosed with CF. Their mother and father, David Diaz Sr., became experts in the daily care their children required, from administering medications to performing airway clearance to making regular doctor’s visits with the pulmonology team at Lucile Packard Children’s Hospital Stanford.

“Both of these kids had very severe cystic fibrosis,” said Carol Conrad, MD, medical director of the Pediatric Lung and Heart-Lung Transplantation Program and Pediatric Pulmonary Function Lab at the hospital. “Despite thorough and adequate treatment at home, and despite everybody’s best efforts, they both developed end-stage lung disease quite early in life.”

Rare surgery in young patients

In June 2014, Doris underwent a double-lung transplant at Packard Children’s, which has the only pediatric lung and heart-lung transplant program on the West Coast. Yet at the same time the Diaz family was celebrating Doris’ successful surgery and quick recovery, David’s health was declining rapidly. His pulmonologist, Carlos Milla, MD, associate professor of pediatric pulmonary medicine at the School of Medicine, kept a close eye on David’s case as his parents continued providing home care as long as they could.

“When David would cough, or his lips would turn purple, or when he started to cry, Doris was scared,” Corina said. “She would say to me, ‘Every day he seems worse than me.’ But to David, she said, ‘David please be faithful. Remember how I looked before? Look at me now.’”

In late 2016, David was admitted to Packard Children’s, where he was hospitalized for two months. He needed around-the-clock oxygen and the support of a bi-level positive airway pressure machine. Katsuhide Maeda, MD, clinical associate professor of cardiothoracic surgery at the School of Medicine, sat with Milla in the pediatric intensive care unit as they examined David’s chest X-ray.

Although David clearly needed an intervention, the decision to do a lung transplant was not an easy one. “His right lung was completely collapsed and not working at all,” Maeda said. “He was surviving only with his left lung, which was also very sick and had expanded, pushing his heart to the other side of his chest.”

At ‘much higher risk’ than his sister

When it comes to transplants, doctors must carefully consider whether a patient is too sick to survive the procedure, in which case the rare, much-needed donor lungs — for which most pediatric patients wait a year or more — should be allocated to another patient. Maeda had done Doris’ high-risk, double-lung transplant, as well as several others. But David “was much higher risk than Doris was, or anybody we’ve ever known,” said Conrad, who is also an associate professor of pediatric pulmonary medicine at the School of Medicine.

Milla and Maeda weighed the risks and benefits. “Then, finally,” Maeda said, “we decided to accept him as a transplant candidate.”

I see my kids healthy and playing like normal kids.

In mid-January, David’s doctors put him on the national organ transplant waiting list. Then, in March, the family learned that donor lungs were available for him. “He was so happy. When the doctor told him the news, he started crying,” Corina said.

Going into surgery, “David looked more comfortable than other patients,” Maeda said, “because he knew his sister had done the same surgery, and she was doing just fine.”

David’s surgery began at 7 p.m. in the evening and lasted 12 hours. “It was exactly as I expected when I opened up his chest,” Maeda said. “But every time we started dissecting the right lung, we lost blood pressure, and David became very hemodynamically unstable,” meaning he had unstable blood pressure.

“So we had to use the cardiopulmonary bypass machine, even for the lung dissection,” Maeda said. “After that was completed, we pushed his heart back into normal position and put in the two new, healthy lungs. And, luckily, those two lungs functioned very well.”

‘An amazing surgeon’

Conrad had advised David’s parents not to expect David’s recovery to be as rapid as Doris’ had been. “I just didn’t expect that to happen with David,” Corina said. “But Dr. Maeda is such an amazing surgeon, he has such excellent technique, and he’s so fast. And David was amazing, just the perfect little patient. He had been listening very hard to what he needed to do prior to surgery, and afterwards he just set out to get better. And he did great.”

David’s recovery was remarkable given how fragile he had been just days before the surgery. His breathing tube was removed within 24 hours of the surgery, and he was discharged from the hospital within two weeks.

A week after discharge, he was playing soccer with other kids at the Ronald McDonald House at Stanford, where his family stayed through most of June to be close by during his follow-up care.

Corina said that with his two new lungs, David is like a new kid. “I see my kids healthy and playing like normal kids, which is something amazing,” she said. “It’s funny; sometimes it’s 8 or 9 o’clock at night, and David still wants to kick the ball around.”