When Zofia Fenyrch was pregnant with her second child — a girl with a rare and complex heart defect named Dorothea — she and her husband, Rob Velez, hoped to increase their baby’s chances of survival through fetal intervention. The couple told Insider their story in April while they tried to raise $3. 25 million to cover the cost of the in-utero surgery that only a leading cardiologist in Houston, Texas, could perform.
They fell short of their financial goal. It meant that Dorothea, whom they said doctors had suggested they terminate, would have a less-than-10% survival rate at birth. She had hypoplastic left-heart syndrome — a condition that occurs when the left side of the organ is chronically under-developed.
In her case, the prognosis was worse because her condition came with an “intact atrial septum,” meaning there was no hole in the upper chambers of her heart. It meant that blood couldn’t return from her lungs to her already compromised heart, causing backup and subsequent damage. A paper published by the Journal of the American Heart Association described the combination of the two conditions as “highly lethal.
” Fenyrch and Velez said that it felt like a miracle when Dorothea beat the odds and was safely delivered by C-section on July 7, 2022. “We always hung onto hope. There was fear, but faith and hope dominated because we’d loved her and fought for her for so long,” Fenyrch said.
However, she said that they needed to cling to that hope “even harder” because Dorothea faced a second challenge — a surgery with a similarly low rate of survival to that of her birth. Fenyrch, who is from Brighton, England, said that medical staff told her even if Dorothea survived the surgery, which doctors needed to perform during her first week of life, there would be “neurological complications. ” Velez said they were “strongly pushed to decide on palliative care.
” But, he said, they refused to give up on Dorothea. Velez said that they managed to find possibly the only surgeon in the UK who was willing to carry out the procedure. The consultant who agreed to take on their case was Guido Michielon , a lead congenital surgeon at Royal Brompton Hospital.
Michielon told Insider that the “complex anatomy” of Dorothea’s heart meant the operation was “highly likely to be unsuccessful. ” Fenyrch described the upcoming surgery as “altering the plumbing” of Dorothea’s heart so that her blood could circulate more efficiently. She said that Michielon told her that he was motivated by the knowledge that she and Velez “had already come so far with Dorothea and were determined that she would live.
” “I told him that I wanted her to know that we did everything we could in our power to help save her,” she said. But the parents hit a roadblock. They said that two days before the planned surgery, the hospital canceled the procedure.
The couple said that the administration told them the chances of Dorothea surviving the surgery were too low. “It felt like they cut our wings off,” Fenyrch said. The couple, who have another daughter, 2-year-old Bathsheba, hired a human-rights lawyer.
“We were devastated, but we were determined to continue our fight. We started bombarding the hospital with personal and legal letters,” Velez said. He said that the strategy worked.
Michelion was able to perform the surgery on Dorothea when she was almost a week old. The surgeon told Insider that it was an all-day procedure. He said that, to the surprise of many of his colleagues, the baby pulled through and appeared to be stable.
However, Michelion said, Dorothea had a sudden drop in blood pressure after she was transferred to the pediatric intensive-care unit. She went into cardiac arrest and medical staff performed CPR. “They turned on the alarms, and it was scary sounding,” Fenyrch said.
The 41-year-old, who, like her husband, is Catholic, said that they had to wait in a separate room while the hospital staff worked on their newborn. “We thought, ‘God, if the six days we’ve had with Dorothea are all you can give us, then we are thankful for the time we had together,'” Fenyrch said. To their relief, she said, a nurse later told them that the CPR — which had lasted more than an hour — had been successful.
Dorothea was placed on an ECMO machine, which temporarily took over the function of her heart and lungs by oxygenating blood outside her body. “It was a scary sight because she was swollen with an open heart and dozens of tubes and drains in her body,” Velez said. “But we looked beyond all that because our miracle was still happening and she was alive.
” Dorothea experienced a series of complications. “We were told there were potential horrific outcomes including brain death,” Fenyrch said. She said that a nurse told her to have Dorothea’s sister and half-brother, Cyprian, visit.
“She said that it might be their only memory of her,” Fenrych added. But Dorothea battled on. She was moved from the PICU to the high-dependency ward at the end of August.
A recent MRI proved that, despite the risky surgery and her cardiac arrest, she’d suffered no brain damage. “She smiles all the time,” Fenyrch said. “She’s an amazing and smart little girl.
” Dorothea needs to have a second reconstruction of the heart, known as the Glenn procedure, in three months’ time to further improve the flow of blood to her lungs. But, as Michelion pointed out, Dorothea’s case remains more complex than most others. “It’s not going to be a straightforward procedure,” Michelion, one of a handful of surgeons in the world who are skilled enough to perform the intricate surgery on Dorothea, said.
Fenyrch and Velez must clear another hurdle on their daughter’s behalf. Michelion will shortly leave the Royal Brompton Hospital to work at a hospital in Genoa, Italy. The only way that Dorothea can achieve continuity of care — and the highest rate of survival — is to have her second surgery there.
Velez, who has set up a fund to raise money for the latest step, said that the medical and travel costs are expected to reach more than $100,000. “We’ve come so far and we will keep fighting for Dorothea,” the father said. He noted that the family sings “Happy Birthday” to the baby every morning to celebrate her new day of life.
Meanwhile, Michelion said that Dorothea’s “miracle” is “not just the work of science. ” “It’s got a lot to do with love,” the surgeon said. .
From: insider
URL: https://www.insider.com/surgeon-love-not-only-science-is-saving-a-baby-girl-2022-10