A heart impossible to fix? Here’s what knowledge can do

Whether or not there was divine intervention in the life of Rebekah Hughes, as she believes, depends on your faith. What can be known for certain is that the intervention and care of Cardiovascular Surgeon Dr. Tirone David and a multidisciplinary team at the PMCC – nurses, pulmonologists, respiratory therapists, pharmacists – made it possible for her to come home to her husband and their children after spending 80 days in the Cardiovascular Intensive Care Unit (CVICU).

Those days included her 38th birthday and her 15th wedding anniversary, a Thanksgiving dinner and her son’s ninth birthday.

“She was going to die otherwise, so I had no choice,” says Dr. David. “She’s so young, a wonderful human being, with three kids at home.” The bond between patient and surgeon goes beyond what happened in that operating room. “Every day he was in the hospital, he came to see me,” she says.

An unexpected diagnosis
The story of Rebekah Hughes really begins with a three-year-old girl who swallowed a penny. She travelled to Britain with her parents in 1979 to visit relatives there and somehow managed to swallow an English penny. An X-ray found more than the penny, which was easily removed. The scan revealed a sarcoma, a rare soft-tissue malignant tumour with a terrible prognosis, growing large in the child’s chest, but as yet causing no symptoms. The family flew home, and within four days she was undergoing surgery at SickKids, followed by intense radiation and two years of chemotherapy.

“They did kill the cancer,” she says, “and it never returned. But I was left with significant scars from the radiation, one functioning lung and ongoing health issues.” What was not known 35 years ago was the damage that cancer treatment could cause to the heart. In recent years, because of the prolonged survival of cancer patients, an important new medical field of onco-cardiology has evolved to screen cancer patients at risk of heart damage from treatment, to prevent cardiac complications and to treat damage that might occur. Decades ago, no one knew that irradiating the chest of a young person can cause severe injury to the heart and lungs that becomes evident only many years later.

“The consequence of the radiation was that everything turned and twisted in her heart, and arteries closed with scarring from being burned and one lung was destroyed,” explains Dr. David. “The main artery that feeds the heart muscle was 99 per cent narrowed. Radiation damaged both nerves that make the lungs move.” Rebekah was left with a 30% lung capacity. Only after her youngest child was born in 2010 did her body begin to fail. Simple household tasks made her short of breath. So did reading out loud to her children.

How the family coped
The night before the operation, on the Labour Day 2014 weekend, her husband, Jon, and the three children came to her bedside. She knew she might be saying goodbye to them, “bearing this weight of not knowing what would happen,” she recalls.

“My daughter was 13, my son was eight and the youngest was four. They understood that my heart was sick, and that Dr. David was going to do his best to help make it better. The 13-year old knew there was a risk.” The children went to stay with her sister’s family. Her husband packed a suitcase to stay in downtown Toronto to be with her. He packed enough for the seven to 10 days she was expected to be in hospital. He ended up staying until she was discharged, 80 days later. It would be almost three months before the Hughes family returned home. The surgery itself was supposed to be an aortic valve replacement that normally takes about two hours. Instead, it was an unexpected and complex operation that lasted seven hours.

Parts of her heart were so scarred and calcified that they shattered like eggshells. Weeks later, Dr. David told her that had he known what he would find when he opened me up, he would not have attempted the surgery. It was even riskier than what he’d anticipated. “At one point he looked up around the operating table and said he wasn’t sure he should continue. Then he said, ‘This woman has three children to go home to.’ So he carried on and did his job.”

How knowledge saved Rebekah’s life
This was the job Dr. David did: “I cut her leg, took the vein, cut the main artery, replaced the aortic valve, the main pipe, as well. It was not conventional surgery. It was something creative that I do sporadically and only out of desperation.” Around nine in the evening, he came out of the operating room to speak to Rebekah’s family. He told them that she had come through the surgery. But he was sombre. When her sister cheered, he admonished her, “Don’t cheer yet. I’m not sure she’ll make it through the night or the week.”

Cardiac Surgeon Dr. Tirone David

Cardiac Surgeon Dr. Tirone David

Rebekah recalls those first days and weeks: “I struggled to breathe and I struggled with hallucinations and delusions because the carbon dioxide was elevating in my blood. It was days of up and down.” After a week, Rebekah was discharged from CVICU, but was brought back 24 hours later when she went into a coma. Shortly after, her only functioning lung collapsed with pneumonia.

“I was fighting to live,” she says, “and it was such a hard fight physically and emotionally. I had no fear of death. I am fully confident that this world is not the end. I had the hope of being with Jesus. And I wasn’t sure that I could keep that fight going. But I realized, No, it’s not just about me. I was determined to keep fighting. I did it for my family, to experience with them the joys we have in this world, as well as the hardships.”

Rebekah’s physical condition began to improve after she underwent a tracheotomy. A tube inserted in her windpipe allowed a ventilator to push oxygen into her one working lung. As long as she needed the ventilator, Rebekah remained in the CVICU. Although the breathing tube was removed after a month – she still required breathing support. She was discharged from the CVICU and sent to a rehab hospital to learn to use a BiPAP machine, similar to the CPAP machine used by people with sleep apnea.

And then at last, Rebekah went home.  In early fall 2015 —  a year after her surgery — an echocardiogram showed that her heart was healing. She was given the go-ahead to begin exercising.

Rebekah attributes her survival to the providential hand of God, the expertise of Dr. David and the PMCC, and the steadfastness and love of her husband and family. She is, against all reason, in defiance of every prognosis but one: alive.