By Andra Bryan Stefanoni
Globe Staff Writer
JOPLIN, Mo. —
It was a no-brainer when Sherri Petry was asked to give away her husband’s heart.
For 22 years, Paul Petry’s heart hadn’t worked well, and since her husband now has a new one, they’re hoping others will one day benefit from the clues the defective one could provide.
“Paul’s particular disease is very rare, and they know very little about it,” Sherri said.
Paul, 45, a pediatrician for Freeman Health System, received a heart transplant on March 3 — more than two decades after first learning he had a rare genetic defect.
“There’s a guy that’s doing research on this particular genotype, and I say he can have it,” Paul said of the old heart. “Do some research on it — find out what you can. I didn’t have any use for it.”
It’s not beyond the realm of possibility that that research might someday benefit two of the Petrys’ children, who also have been diagnosed with the same problem.
“In my mind, anything they can learn that would help my kids or anyone else in the future is definitely a benefit,” Sherri said.
Paul Petry grew up in Lake of the Ozarks, the son of a family physician. He was 23 and in medical school at Kirksville in 1991 when he had what he described as “a sudden cardiac death episode.”
“I was walking to class, and happened to be with all my classmates — some were paramedics, some were nurses — and they resuscitated me. They got me to the hospital, which was just right there, and I was told I have hypertrophic cardiomyopathy.”
That’s medical-speak for an enlarged heart muscle, Petry noted.
“He has a very rare condition,” said Dr. John Cox, a Freeman cardiologist familiar with Petry’s case. “Cardiomyopathy is very common, but usually from a heart attack, or uncontrolled high blood pressure, or sometimes during pregnancy the body attacks its own heart protein. In some, that just happens spontaneously. But it’s rare for them to present with the syndrome that he has.
“He had a congenital condition that created a variable response as far as how it affects people. In his case, it created a cardiomyopathy. It had thickened his heart muscle, which created the potential for electrical short-circuits.”
Doctors implanted a defibrillator — a small, battery-powered electrical impulse generator for patients who are at risk of sudden cardiac death.
“So if I had an arrhythmia, it would shock me back into the right rhythm,” Petry said. “I lived for 22 years with just this defibrillator, and it went off once in a while.”
He graduated from medical school in 1996, did his residency in Kirksville and came to Freeman six years ago.
“It’s always been in the back of my mind — Dr. (John) Swartz, my doctor, warned me that this was in my future,” Petry said. “I really wanted to deny it.”
But denying it didn’t work.
“It was just all of a sudden. I had been working, I felt fine. Then my heart started having a funny rhythm. An irregular rhythm,” he said of the episode that led to his heart transplant.
He had gone to bed on Feb. 17. His wife, a night owl, had stayed awake, and she remembers him getting up with the irregular rhythms. She drove him to the hospital that night. They shocked his heart back to its normal rhythm, but his problems continued.
Over the coming days, doctors tried other procedures, but nothing was working, and they decided it was time to get help from Barnes-Jewish Hospital in St. Louis.
“We have a close affiliation with the heart transplant department at Barnes,” Cox said. “We have one of their doctors come to our office once a month. When we find that people have deteriorated to the point that something like a transplant or an artificial heart ... is necessary, we can make an appointment with them to see a specialist right on campus. Then he can determine ‘now what.’”
Petry’s condition had deteriorated so quickly that he was admitted to the St. Louis hospital, where doctors even installed an artificial heart. But it did not work and he was put on a heart transplant list.
“It is all kind of a blur. I really don’t remember well what happened between going to the hospital and waking up in the ICU with a new heart,” Petry said.
“My husband’s disease we’ve been dealing with the entirety of our married life — four months after we were married,” Sherri Petry said.
Their oldest son, Luke, 20, now a junior in college at Pittsburg (Kan.) State University, was diagnosed with cardiomyopathy at age 10.
“We found it because he randomly had chest pain one day,” Sherri said. “Because of Paul’s history we watched them very closely.”
Father and son went to the Mayo Clinic in Minnesota in 2003 and learned the diagnosis: Luke tested positive for the gene for cardiomyopathy. It was a stressful time, Sherri recalls.
“I was pregnant with our last child. At that time, given Paul’s history, they decided to implant a defibrillator in Luke.”
Paul wonders about that decision.
“I wish we hadn’t, because the dark side of defibrillators is that you can get shocked when you don’t need it, and it hurts — really badly — and you’re not expecting it. So he’s had several unnecessary shocks.”
But Luke is doing well as a student in the construction program, his proud parents said.
“He’s a tough kid,” Sherri added.
A couple of years ago, the Petrys learned another child, Jonathan, now 13 and a seventh-grader in Carl Junction, also has the gene. The other four children tested negative for it.
A new heart
On March 2, Sherri received the call that a heart had been found for Paul. They were in luck as it was a man’s heart, which Paul needed because of his large frame. But it was unfortunate timing — Sherri was more than seven hours away in Oklahoma City.
“Our daughter had started her competitive dance season. Paul’s sister was with him in St. Louis,” Sherri recalled. “It was a very stressful time, trying to juggle everything.”
Sherri pointed her car northeast.
“It was a 6 1/2-hour procedure, and they let me know each hour where he was at and how he was doing,” she said.
In the weeks that followed, Sherri, who is an avid reader and likes to know details, would consume everything in print she could find to describe the situation.
“We had a lot of things thrown at us very quickly. I like to know what’s happening, and I asked a lot of questions,” she said. “They’ve all been very good about explaining everything.”
Luke, their 20-year-old, and Jacob, their 18-year-old, managed the routine with their four younger siblings at home, and a grandmother and aunt took turns helping with the children.
Paul had a few complications, including an infection after surgery. Sherri, who works at Carl Junction schools as a paraprofessional in the K-1 building, said the support the district gave her “was wonderful.”
“I was initially gone a lot. They covered for me, got subs, sent gift cards and meals. I just felt like we had a huge support system,” she said.
Support also came from the congregation at Carl Junction United Methodist Church, which brought meals and sent cards.
Dr. Gregory Ewald, transplant cardiologist and medical director of the heart transplant program at Barnes-Jewish and Washington University Medical Center, specializes in clinical research into various heart conditions, including the one that affects the Petry family.
“What we’ve done here, for a few years, is developed a tissue bank for heart specimens,” he said. “A few other places are trying to do similar work, but there are some unique aspects about ours. We work with Mid-America Transplant Services, so when there is a heart donated that is not suitable to be transplanted, we can sometimes get tissue, so we have a pretty robust system.”
Ewald works with others at the university to test electrical properties of heart tissue.
“We look at ways different genes get turned off and on when there is heart failure,” he said.
The bank has tissue samples from 100 to 200 patients, he estimated.
“The power of this is to have many patients who have been through the same thing to look at some of the characteristics of the tissue,” Ewald said.
“There’s always potential applications. There are a lot of things that cause cardiomyopathy. A lot of the pathways that lead to damage and dysfunction look a lot alike if you did a heart biopsy or took tissue. You can’t always look at it and clearly say, ‘This is X.’
“Upstream from what we’re doing is figuring out the genetics — figuring out how to turn certain genes on and off to sort of prevent the thing from happening in the first place.”
The family hopes Paul will be home in time to see his son, Jacob, graduate from high school on May 12.
“He was critically ill when he got here, and he struggled a bit to get through the transplant, but he’s working on it,” Ewald said.
Sherri said a milestone was reached last week when Paul was moved to a rehabilitation unit, where he likely will be “about a week or two.”
“The goal now is for him to get home,” Sherri said. “He’s talking about going back to work, and they’ve said that is most definitely a possibility maybe even this summer. He misses his patients, loves what he does, loves medicine.”