Teenager to graduate, thanks to 500 caregivers, whole lot of hope

Robert Dicks/Packard Children's Hospital

At the May 14 “An Evening in Wonderland” prom at Packard Children’s, Ori Shadmon celebrated his upcoming Gunn High School graduation with Palo Alto Unified School District teachers Thayer Gershon and Kathy Ho.

Ori Shadmon was in a coma. He was unable to breathe without a medical ventilator, and most of his organs were failing.

The culprit, an adenovirus, typically afflicts its victims with flu-like symptoms for a week or so. But for Ori, 16, the virus had landed him in the intensive care unit at Lucile Packard Children’s Hospital, where he was diagnosed with acute respiratory distress syndrome — a deadly condition in which fluid accumulates in the lungs, inhibiting oxygen from entering the bloodstream.

“Most people didn’t believe he could survive,” said David Cornfield, MD, the hospital’s chief of pulmonary, asthma and critical care. But Cornfield and his team were not among them. Ori, who is now 18, will graduate in two weeks from Gunn High School in Palo Alto. He plans to attend college in the fall.

What happened between his arrival at Packard Children’s on a cold winter night in 2008 and his discharge — a staggering seven-and-a-half months later — is a testament to the vigorous care he received from more than 500 physicians, surgeons, nurses and other hospital staff. But it’s also the story of the patient and his family’s steadfast sense of hope, and a team of doctors with a nuanced understanding of a respiratory illness that had a particularly nasty grip on this immunodeficient patient.

David Cornfield

A native of Tel Aviv, Ori underwent heart surgery when he was 3 weeks old to repair his aorta. At age 9, he got a kidney transplant at Packard Children’s. His mother donated the kidney. As a result of the drugs Ori had to take to keep his body from rejecting the organ, his immune system was compromised and especially vulnerable to ARDS.

So when the condition struck him at the start of 2008, he was left severely weakened. He could barely stand and threw up anything he tried to eat or drink. By the time he was admitted on Jan. 10, the situation was desperate. “I was exhausted. I thought it was the end,” he recalled. “Unless they do something drastic — it’s over.”

Once Ori’s lungs failed, the doctors placed him in a medically-induced coma, began mechanical ventilation and hooked him up to a dialysis machine to support his failing kidney. In addition, Ori developed pancreatitis, bloodstream infections, vocal cord paralysis and disseminated clots.

Cornfield, also the Anne T. and Robert M. Bass Professor in Pediatric Pulmonary Medicine, said that Ori lived “on the edge of a knife” for two months, but the doctor never wavered in his belief that the teenager could pull through.

Based on his experience in treating ARDS and on an earlier study of the condition, Cornfield knew that Ori’s chances of survival would increase by almost 40 percent if he could be kept alive for two weeks. “ARDS is one of the few disease states in which if you’re not getting worse, you’re actually getting better,” Cornfield said. “Most deaths from ARDS are in the first two weeks; nearly none are after five weeks. A number of trials have reproduced this.”

In its efforts to keep Ori alive and minimize damage to his lungs, the critical-care team adopted less-than-conventional strategies.

First, the team used an innovative and unusual treatment: nitric oxide. Not to be confused with nitrous oxide (an anesthetic), nitric oxide is a molecule that signals the pulmonary blood vessels in newborns to relax and start absorbing oxygen.

The therapy has risks: The molecule can cause a dangerous build-up of methemoglobin, a kind of hemoglobin that does not carry oxygen, as well as other cells that can hamper oxygenation. Still, Cornfield was familiar with a 1999 study showing that while nitric oxide did not help typical pediatric cases of ARDS, it had major benefits for children with severe cases of ARDS and compromised immune systems — that is, patients like Ori. In those cases, it was shown to actually increase oxygenation to the bloodstream.

To complement the nitric oxide strategy, the doctors put Ori on a ventilator that delivered small puffs of oxygen at very high rates — for Ori, as high as 700 puffs per minute. The aim was to keep the lungs from stretching too much, which can produce inflammation, Cornfield said.

This two-pronged treatment — small sips of oxygen at high frequency, plus inhaled nitric oxide — is setting a new standard for how certain ARDS patients should be cared for, Cornfield said, adding that he believes it’s what helped Ori turn the corner.

Yet Ori’s eventual recovery was not just about the excellent medical care he received at Packard Children’s.

Cornfield gave a recent presentation to colleagues and residents about the case and was joined by Ori, who spoke about the importance of a more abstract kind of treatment. “Doctors need to understand that hope can start with the smallest interaction with the child or his parents,” Ori said.

“Think about my mom: She was sleeping outside the pediatric intensive care unit 24/7, terrified of receiving bad news,” Ori said, seeking to explain how critical it was that she receive some sort of encouragement. “Dr. Cornfield told my dad when I was in the PICU that it will take time, but ‘Ori will be able to ride his bike.’ That message provided strength and hope.”

And his parents’ hope gave him strength to fight, he said. “They made a promise that as long as I don’t give up, they wouldn’t give up.”

Letting Ori’s family know he had a chance of surviving, despite the severity of his illness, was essential, Cornfield said. “It’s OK, in my estimation, to warn people that the odds are long and the journey will be hard, but that there still remains hope,” he said.

Many doctors and nurses also played an important role in keeping the family’s spirits up, said Rachel Shadmon, Ori’s mother.

One spring day, as Ori was still recovering but could neither move nor speak, two pediatricians managed to maneuver him and the medical machines that were keeping him alive to the hospital’s rooftop garden.

“There was fresh air, and Ori could smell flowers and see birds,” Rachel said. “It helped him to understand there is a light at the end of the tunnel.”

On May 14, Ori came to the Packard Children’s prom, which is held annually for patients who are attending or have attended the hospital school. On that evening, he talked happily about playing the clarinet and going to the gym again. In June, he’s taking a vacation to Paris with his family. In the fall, he plans to attend Foothill College.

“When I came to this prom the first time, I was fully intubated, I had tons and tons of IVs and I was paralyzed from the waist down,” Ori said. “This time, the prom feels like closure. I’m really excited about what lies ahead.”