Two-organ transplant changes life of Dracut girlBy JOHN COLLINS , Associated Press
Mar. 24, 2013 3:04 AM ET
DRACUT, Mass. (AP) — Never have two parents been more amazed to see their toddler crawl up a flight of stairs.
Only 13 days after a then 2-year-old, 21-pound, Sofia Groff received a donated aorta and attached kidney in a first-of-its-kind, 12-hour transplant operation at Boston Children's Hospital, there she was, surgery scars already fading, clambering up the stairs of her Dracut home as her wide-eyed parents, Linda and Marcus Groff, watched in near disbelief.
"We were looking at each other, saying, should we really be letting her do that?" said Linda Groff, recalling the unforgettable day Sofia celebrated the family's return home by exercising her climbing instinct to reach her very own bed upstairs.
"It really was a testament to the unbelievable work they do at Children's. It's also a tribute to Sofia's spirit," said Marcus Groff of his daughter's being able to move about normally so soon after beating the longest of medical odds.
Marcus and Linda Groff fully credit their daughter's new lease on life to the surgeon, Dr. Heung Bae KIm, and the team of medical professionals at Children's Hospital who detected and ultimately "fixed" her extremely rare medical condition, known as "mid-aortic syndrome."
MAS is defined by the narrowing of the aortic valves, the main arteries that deliver oxygen-rich blood throughout the body. If the aorta is narrowed or damaged, it can cause serious spikes in blood pressure by restricting blood flow through the thoracic and abdominal region and to the kidneys. In Sofia's case, an especially aggressive form of MAS was wreaking havoc inside her tiny, 5-pound body.
Sofia's condition was likely to be fatal, the Groffs learned. It was only due to a combination of Dr. Kim's coming up with a novel solution to MAS, the availability of the donor organs, and Kim's and his surgical team's skill and stamina in performing the marathon operation that Sofia is alive today, her father said.
Born prematurely at 32 weeks on July 1, 2008, Sofia, the Groffs' only child, was quickly found by her neonatal caretakers at Tufts New England Medical Center in Boston to have dangerously high blood pressure.
In an effort to treat the high blood pressure that was threatening to permanently damage Sofia's brain and eyesight in the hours after her birth, doctors administered a drug known to have a possible side effect of kidney failure. In fact, Sofia soon suffered "full renal failure," with the Tufts medical team scrambling to save her partly by inserting a catheter through her navel to draw out toxins, her father said. Though the catheter solved one problem, it opened the door to Sofia soon contracting bacterial and fungal infections, also considered life-threatening.
With their baby daughter at risk of dying for the first four months of her life, Linda Groff said she and her husband "basically lived at the hospital," signing up daily to sleep in a room set aside for the parents of whichever child is believed to be in the most dire physical condition that day.
Daily for four months, the nurses and doctors at Tufts got Sofia past one life-threatening hurdle after another, Linda Groff said.
"We had gone through episodes where Sofia had gone into cardiac distress and they couldn't get a handle on the root cause for the hypertension and her heart and breathing problems," said Marcus Groff.
Acting on expert advice, the couple sought a consultation at Children's Hospital, where they later received a verified diagnosis of "a really aggressive form" of MAS.
Before she was transferred by ambulance from Tufts to Children's in November 2008, several nurses at Tufts all said tearful goodbyes to Sofia.
"A few of the nurses confided to us years later that as we were leaving the NICU at Tufts after our four-month stay, a few of them had thought that Sofia wouldn't survive," said Linda Groff. "Of course, they would never have said that at the time. They were our rocks, and I don't know how Marc and I could have handled it without them."
Over several months, Sofia underwent a series of angioplasty operations to keep blood flowing through her constantly constricting aortic valve and arteries. Angioplasty and the insertion of stents bought Sofia valuable time, allowing her to grow from a 5-pound baby to a 21-pound toddler. But continuing with angioplasty was considered a losing proposition for Sofia, her parents were told.
"This was around the time, when Sofia was about 1 1/2, that Dr. Kim decided Sofia would require a novel approach," said Marcus Groff. "It turned out to be something as unique as she is. Something fantastic. Dr. Kim came up with this out-of-the-box thinking of an aorta-and-kidney transplant, which had never been done on a child of Sofia's size."
Children's Hospital placed an organ-donor request for "the trunk of an aorta along with the kidney, which they could kind of plug into Sofia's chest as one unit," her father explained.
The Groffs would wait nine months to receive the life-altering phone call that a donor had been located. During those nine months, Sofia's parents and nurses painstakingly guided her to maintain the minimum 20 pounds of body weight needed to withstand the transplant surgery.
"Getting her to grow was a challenge unto itself at that point in time. We were injecting formula directly into her system," said Marcus Groff.
It was Oct. 27, 2010 when the Groffs received the call that there was a donor. The father of a 5-year-old girl, who had died in a highly publicized high-speed car crash on a Massachusetts highway a day earlier, had agreed to donate his daughter's organs so four other children, including Sofia, might live.
The Groffs remember feeling a jumble of emotions as they brought their 2-year-old to Children's for the transplant operation.
"Because of the nature of the organ request we knew it would be a child of similar size and would be a deceased donor," said Marcus Groff. "The strange this is, when you're in this situation you're very focused on getting that call, but as soon as you get it you're incredibly ambivalent about it. You find yourself thinking: Are we sure we want to do this? In the end, of course, it's an extraordinary blessing."
On Thursday, Oct. 28, Dr. Kim and a team of doctors that included Dr. Khashayar Vakili, performed the first-ever organ transplant where a length of aorta was transplanted along with a kidney, simultaneously treating both Sofia's renal failure and MAS.
"Normally there would be two different teams to do the extraction and transplantation, but Dr. Kim wanted to make sure everything was done just right, given this was not your typical kidney transplant," Marcus Groff said of the two consecutive surgical procedures that spanned 26 consecutive hours. "It really is amazing."
While anxiously waiting out the operation on their daughter, the Groffs took a memorable walk along the Charles River in Boston.
"My wife is Italian and Italians believe if a bird poops on you, it's good luck," Marcus Groff noted. "So we as we walked and a bird pooped on me at one point, it actually provided great mental benefit. I'm not superstitious myself, but I felt very grateful to have had that experience on that particular day. It made me feel like, OK, this is going to work out."
Though there have been other health challenges for Sofia since she came home from the hospital only 13 days after the transplant and climbed the stairs, "She has basically been a rocket ship out of the gate since then, and never looked back," said Marcus Groff.
"We don't know exactly what the future is going to bring because, as was explained to us, these organs have a shelf life, they don't last forever, and she'll probably need a donor again in 12 to 20 years," said Linda Groff. "But it's amazing what a difference it's made. She didn't walk until the surgery. She wasn't strong enough. By December she was fully walking. First day after she got home from the surgery she was crawling up the stairs.
"We were saying, 'I don't know if we should be letting her do that. But all right. She was 2 1/2 years old at that point. We think about it."
Aside from having a feeding tube permanently implanted in her belly and needing to take a set of medications like clockwork several times a day, Sofia is otherwise a healthy 4-year-old who has nearly caught up to her peers physically, her mother said.
"Sofia carries the hopes and dreams of two families now — and the promise of a better day for other children suffering from MAS," her father said. "She is ready, and finally able, to thrive."