Jack Eigel, an active, vivacious 53-year-old, couldn't be more kind-hearted. But for much of his life people would kid him about having his heart in the wrong place ... and it wasn't a figure of speech.
Eigel, who lives near Milwaukee and has worked at a tabletop and home decor store for the last 20 years, has a condition called Situs Inversus. It causes all of his organs, including his heart, to be reversed.
"You know, when I was little, I tried to pledge allegiance the wrong way and got a few teachers mad at me," he joked.
On the inside, Eigel is a mirror image of someone with normal anatomy -- every organ in his chest and abdomen is flipped. His intestines, liver, lungs and heart are all on the opposite side of where they should be.
"I've had Situs Inversus all of my life, so it's something I don't even really think about," he said.
Situs Inversus is very rare.
"The exact incidence is not really known, but it probably affects somewhere around one in 10,000 people," said Dr. Alfred Nicolosi, a cardiothoracic surgeon at Milwaukee's Froedtert & Medical College of Wisconsin. "The majority of patients …really don't have any real problem from it. It's just everything inside is backwards."
It is a condition Eigel could have lived with his entire life with no problems, but three years ago, his heart started to fail. He had experienced shortness of breath and fatigue but attributed it to getting older, until he suffered a heart attack two days after Christmas 2006.
"Jack has something drastically wrong with his heart muscle and it doesn't contract normally," Nicolosi said. "It's to the point where we would call it stage D heart failure, or end stage heart failure."
Eigel's only hope of survival was a heart transplant, but given his unique anatomy, surgeons at Froedtert weren't sure it could be done.
"My first reaction was, is this anatomy going to be prohibitive to doing a heart transplant and has it been done before?" Nicolosi recalled.
There are only a handful of other heart transplants like this mentioned in medical literature. With 2,500 heart transplants performed annually in the U.S., Nicolosi said, "you can assume that, at most, that there are maybe one of these cases done every four or five years."
Waiting for a Heart
The problem for surgeons is that the space in Eigel's chest is a different shape than for a normal heart, and any new heart will have arteries and veins that don't line up with his flip-flopped anatomy.
"We're going to have to change quite a bit the way we implant this heart," said Nicolosi, who estimates he's performed between 50 and 60 heart transplants. "It's going to be sitting in the chest different; we may need to use pieces of artificial graft material to make some of the connections."
But doctors had faith they could do it, if they found a heart in time.
"Obviously I'm not going to wait for a Situs Inversus heart or I'd be waiting for a couple of lifetimes ... a regular heart will go into me and it will be flipped," Eigel said. Until then, he had to wait and hope.
"As long as the medicine keeps working, great -- but it's not a medicine that works forever," Eigel said.
As the summer went on, Eigel's health declined, and still there was no heart available.
"One of the things I probably miss the most is walking. Just, you know, being able to walk wherever I wanted to go or hop on public transportation ... now I simply just can't do that," he said.
"I suppose, I hate to say it, I miss going to work," he added.
To increase his odds of getting a heart, Eigel was put on the waiting list at a second hospital, the University of Wisconsin Hospital and Clinics in Madison, where a whole new team of doctors had to learn about his rare condition.
In August, after waiting nine long months, they had a heart for him.
"It's going to be a neat puzzle trying to put this together in the right way," said Dr. Niloo Edwards, the chairman of cardiothoracic surgery and director of the heart transplant program at University of Wisconsin Hospital who performed the procedure.
Unique Transplant Surgery
Even as he was being prepped for surgery, Eigel couldn't quite believe it was happening.
"Until I wake up and it's over with, that's when we'll know it happened," he said, adding that he had "total faith" in his doctors.
"It's literally you are taking two mirror images and trying to make them match," Edwards said. "So it's sort of a three-dimensional Rubix cube and that's what makes him particularly unusual."
The operation lasted six hours as the surgical team worked to fit the new heart into Eigel's backward anatomy.
"It's certainly one of the most unique transplants I have done," Edwards said. "At the end of all this rewiring -- it sort of looked like the inside of a 1970s Cadillac -- there were sort of pipes and tubes running in different directions where they shouldn't be."
After the surgery was over, even before he was fully conscious, Eigel could feel the difference.
"When I actually woke up after the surgery, Dr. Edwards, the surgeon, was actually in my room," he recalled. "I noticed I felt perfectly fine and I thought, oh, it was a false alarm, I thought it hadn't happened."
Now, five weeks later, Eigel is doing better than he ever could have imagined, and he returned to Froedtert Hospital not for medical reasons, but for a ceremony honoring organ donors.
"Just being able to walk around, I don't have any -- I am not hooked up to anything," he said. "I don't tire, I can go for a 40-minute walk, just things I wondered if I would ever be able to do again."
Eigel and other organ recipients tied ribbons around tree branches -- tiny tokens of gratitude for a second chance at life.
"I am just so thankful," he said. "It's given me my life back."
Froedtert & Medical College: http://www.froedtert.com/home.aspx
University of Wisconsin Hospital: http://www.uwhealth.org/