Benefield: Heart-lung transplant a breath of fresh air for Montgomery grad

For 23 years, Florence Sheffer had lived in fear of her daughter running out of breath. So when Kathleen Sheffer stopped cold on a walk one day two years ago, Florence girded herself like she had done so many times before.

But this time it was different. This time Kathleen Sheffer was smiling.

“She said, ‘No, it's OK,'” Florence recalled. “She's kind of laughing. She said, ‘I caught my breath, Mom. I caught my breath.'”

Getting gassed and recovering, giving full effort and knowing your body can regulate itself and bring your heart rate and breathing back down to normal - these are things that athletes, and probably most people, take for granted. Not Kathleen Sheffer.

Now a three-time gold medalist at the Transplant Games of America who cycles and hikes and does so many things no one ever thought she'd do, Kathleen Sheffer knows every breath she takes is something of a miracle.

When Kathleen Sheffer, a Montgomery grad, was born 25 years ago, her dad Craig was there happily snapping pictures of his first-born child. But doctors knew immediately something was wrong. She wasn't turning a healthy pinkish color.

“It was transposition of the great arteries. When I heard that, when I studied it, it was a death sentence,” said Craig, who is a nurse and the clinical services manager at St. Joseph Health. “I passed out. I just dropped.”

When she was 7 days old, doctors opened Kathleen's chest in an attempt to correct the problem. They could not, so they created a shunt from one side of her heart to the other, hoping that would correct the problem.

But even with the shunt, she always struggled to breathe.

“As soon as she could talk, she would say ‘I can't breathe,'” Florence Sheffer said.

Regular visits with a cardiologist were generally positive. But one day when Kathleen was probably six years old, she was at a swim lesson at La Cantera Raquet and Swim Club in Santa Rosa. The young guy giving her the lesson knew viscerally that something was wrong.

“This kid says, ‘I don't care what any doctor says, something is up with this kid. It's like her heart is going to jump out of her chest,'” Florence Sheffer remembered.

The Sheffers took their daughter to a different doctor for a second opinion.

That was in February of 2000. When the doctor came to greet the Sheffers, they knew the news was not good.

“He said, ‘I got a private room because the news I have is really bad,'” Florence Sheffer said.

The news was that as a result of the procedure when she was 7 days old, Kathleen had developed pulmonary hypertension.

“I said, ‘What do we do to fix that?'” Florence Sheffer said. “And he said, ‘Well, there is no fix.'”

“When she was diagnosed, 80 percent of people died within five years,” she said.

In the face of doctors telling the Sheffers to prepare for a life expectancy of perhaps one to three years and to consider palliative care for their 6-year-old, friends and family went to work, researching doctors and therapies that might help. But it was dire.

“My good friend said, ‘Do not go on the internet. Do not,'” Florence Sheffer said.

Florence Sheffer's rule for incoming information on her daughter's disease? “If a doctor tells you she's screwed, don't pass that along to me.”

The Sheffers found Dr. Robyn Barst at the College of Physicians and Surgeons at Columbia University in New York. Under Barst's direction, a central line was inserted into Kathleen Sheffer's chest and she began receiving constant - and life sustaining - medicine that helped Kathleen's blood vessels open so blood could flow through them.

The tube connected to a pump she carried in a backpack 24 hours a day. When she showered, she had to manage the entry point with wound care. She could not swim. Contact sports were out of the question. She slept with the line in.

And if for any reason the Flolan stopped flowing, its efficacy dropped to 50 percent in six minutes. At that point, Kathleen Sheffer would be in a life-or -death situation. And it happened.

“We were hooking her up to this lifeline that had a very short window of air,” Craig Sheffer said. “The other option was to keep her comfortable and allow her to die, or we can try this very aggressive medicine.”

Kathleen Sheffer went for it. And despite being on intense blood thinners, she rode a bike, she scootered, she ice skated - with a helmet. But catching her breath, getting winded and deeply fatigued, was a constant struggle.

Craig Sheffer said there were too many tales of close calls from pinched lines or broken ports to recall them all. When the phone would ring, he spent years scanning his wife's face for any sign of distress. It was a precarious life of worry over a kid described as stubborn and feisty - someone intent on living her life.

Trips to the ER were frequent. Her younger sister Monica could play soccer and do karate and swim, but she could not. Her body may have been built unable to sustain those pursuits, but her spirit had other ideas.

“I think I definitely recognize that I have a competitive drive,” she said.

After graduating from Montgomery, Sheffer went to Cal. She rode an electric bike. She studied architecture. She explained to roommates and friends her frequent naps.

“I was relatively stable through high school and college,” she said. “The last year of college I was declining a bit. There would be months where I would use supplemental oxygen. I would sometimes carry a concentrator with me.”

After graduating from Cal, she began working as a professional photographer. On a work trip to Seattle in 2016, Kathleen Sheffer awoke in the middle of the night coughing up large amounts of blood.

“Her lungs were hemorrhaging,” Florence Sheffer said. “You don't usually survive it.”

After years of pulmonary hypertension, her lungs were worn out. And the situation was so acute, the rest of her organs began to shut down, too.

Florence Sheffer recalls a doctor telling her that if something like the incident in Seattle happened again - and it likely would - Kathleen would surely not survive.

Transplant had always been an option, but it had always brought a tremendous amount of worry. The Sheffers said that for Kathleen, it would not be a surefire panacea. And with her need for both heart and lungs, it would be extraordinarily complicated. And Kathleen, who has always been active in a community built around dealing with pulmonary hypertension, had seen more friends die than most elderly people.

But in the ICU in Seattle, the Sheffers all knew it was the only reasonable option that remained.

“I thought, ‘How am I going to tell her this?'” Florence Sheffer recalled. “I went in and I talked to her. I had just braided her hair. I looked at her and with her big, wide eyes, she said, ‘OK.'”

Twenty-six days later, the Sheffers got a call that a match was made and organs were available. It was terrifying but it was a lifeline.

On July 1, 2016 at Stanford Medical Center, Kathleen Sheffer got a new heart and new lungs. And it was during her post-op stay that she was walking with her mom around the Stanford campus that it happened. They were walking, she got winded and stopped. She took a deep breath.

“The feeling of catching my breath was different from anything I have ever experienced,” Kathleen Sheffer said. “I cried when I first felt it.”

Her recovery from transplant surgery has been phenomenal.

She goes rock climbing, she rides her bike, she hikes. And in August she flew to Salt Lake City to participate in the Transplant Games of America.

She competed in the 5K and 20K bike races, table tennis and badminton. She won three gold medals. And the person who for 17 years was connected to a tube and pump and life-giving medicine was now feeling connected to something else entirely.

“I just felt really connected to my heart and lungs and could feel every time I was breathing deeply, which I have never done before, feel that connection,” she said. “It made me feel grateful and really connected to the person who gave me this chance.”

An organ donor gave Kathleen Sheffer a second chance and she has done everything in her power to honor that gift.

She climbed Half Dome last month. Craig Sheffer was traveling when a photo of his older daughter, seemingly in the skies above Yosemite Valley, popped up on his phone.

“That is who this kid is,” Craig Sheffer said. “She is an incredible force and an extraordinary young woman.”

Parents worry for their kids long after they stop being kids. The Sheffers are no different. When one has spent every waking moment in various degrees of worry over a child, that is hard to shake.

Rejection can happen at any time. Simple illness for a transplant recipient can blossom into something much more ominous.

“I'm still waiting for the other shoe to drop,” Florence Sheffer said. “She has had issues her whole life. We are just pinching ourselves.”

As she balances pride with worry, Florence Sheffer watches her daughter flourish.

“It's just crazy,” she said. “We go on a trip and she carries her own suitcase. Now I'm lagging behind. I just want to say, ‘Wait.'”

But Florence Sheffer would never tell her daughter to wait. Or to slow down. Or not do something on her wish list.

“No matter what happens, she is leading a life that is entirely different than the one she did before,” she said. “I'm so happy.”

Craig Sheffer is indescribably grateful to the donor who gave his daughter another shot at life, but he knows Kathleen deserves credit too.

“Kathleen is alive today because Kathleen fought to be alive,” he said.

The surgery, the seemingly endless medications, the dietary restrictions, the masks she must sometimes wear - Kathleen Sheffer has embraced it all as pieces of the puzzle that keep her healthy.

“It's not an easy course and not everyone comes through it like Kathleen has,” he said. “It's really, really, really hard even when things go right.

“What we got from transplant is like winning the lottery,” he said. “We got the best we could have ever expected.”

Kathleen Sheffer continues to write letters to the family of the anonymous person who donated their organs. She hasn't heard back, but she continues to write.

She knows that their gift was likely borne of acute sadness. So she writes about her new life and her happiness. She writes about winning three gold medals at the Transplant Games, of hiking with her family, of day-to-day joys like catching her breath.

“I say thank you.”

You can reach staff columnist Kerry Benefield at 707-526-8671 or kerry.benefield@pressdemocrat.com, on Twitter @benefield and on Instagram at kerry.benefield. Podcasting on iTunes, “Overtime with Kerry Benefield.”