Normal lungs successfully transplanted into bodies with reversed organs

By Sandee LaMotte, CNN

(CNN) — Fifty-year-old psychologist Dennis Deer’s lungs were failing, scarred by a rare inflammatory condition called polymyositis. Despite carrying supplemental oxygen, the Cook County Commissioner for the 2nd district in Illinois was finding it harder and harder to perform his job.

“It was terrible. I would walk 10 steps and I was gasping for air,” Deer said in a news conference Monday.

Deer was added to the lung transplant list, but he faced an additional hurdle. All of the organs in his chest and abdomen — including his lungs — were flip-flopped in his body, with organs that should be on the right existing on the left and vice versa.

The genetic condition, called situs inversus, affects about 1 in every 10,000 people, according to the Cleveland Clinic. Occurring during fetal development, the disorder has been linked to some 100 different genes.

“As the fetus is developing and the organs are swapped, unless it’s consistent with life, the fetus is often naturally aborted by the body,” said Dr. Ankit Bharat, chief of thoracic surgery and director of the Northwestern Medicine Canning Thoracic Institute.

“Once the fetus is born during a normal delivery, then it has already demonstrated the organ swapping that happened is consistent with life and the child grows normally,” he said.

Years ago, people may have lived without realizing their organs were backward. Today, however, most people find out about the condition during a routine medical exam, Bharat said.

“Someone has tried to listen to their heart, and then they don’t find the heartbeat on the left side, so they get an X-ray, and everything looks reversed,” he said.

A second rare patient

In Elgin, Illinois, a city along the Fox River about 35 miles northwest of Chicago, 27-year-old Yahaira Vega was also struggling to breathe. She, too, had been born with situs inversus, which can sometimes come hand in hand with another rare disorder called primary ciliary dyskinesia, or PCD.

“Situs inversus is a rare condition. But having yet another rare condition that ends up causing enough lung damage that someone would need a lung transplant is even rarer,” Bharat said.

The genetic condition PCD is caused by defects in tiny hairlike structures in the lungs called cilia that move germs and debris caught in mucus out of the airways. When cilia don’t function properly, the lungs cannot properly expel mucus and it can build up, at times to dangerous levels.

As her condition worsened, Vega said she could fill a 32-ounce cup or two with mucus she had coughed up each day.

“I felt like a prisoner — best way to describe it like a bird in a cage. You know, you can still hear the bird’s singing — it sings a beautiful song, but the song is still very sad,” she said at the news conference.

Complicated surgery

Like Deer, Vega was put on the lung transplant waiting list, and like Deer, needed individualized treatment. Performing an organ transplant on patients with situs inversus is an extraordinary surgical challenge, Bharat said.

“We have to replace the old lungs — which are reversed in the body — with new lungs from a donor who has a typical or normal placement,” he said. “Because the new lungs need to fit into a chest cavity that’s a mirror image, we have to make technical modifications in order to do the surgery.”

Just reattaching blood vessels created in utero for a left lung to openings on a “normal” right lung, for example, was especially difficult. In addition, Deer’s and Vega’s lungs were unique in how their backward organs had developed and grown new blood vessels to compensate for the reversal.

“All these alterations in the anatomy require very careful planning. We had to use specialized 3D scans, mold the new lung to fit into the chest cavity, and then figure out how to tailor vessels and structures to fit in the lung that’s on the normal side,” Bharat said.

“It’s like taking a left sleeve off a shirt and then figuring out how to reattach it to the right side of the shirt instead.”

Two successful transplants in a month

After nine days on the transplant waiting list, doctors found a matching double lung donor for Vega — she received her new lungs at Northwestern Memorial Hospital on April 28.  Asked what she wanted to do first with her new lungs, Vega said “laugh.”

“I strive to make other people laugh because seeing the joy on other people’s faces brings me a joy that feels almost healing,” she said. “And when I was sick, I could never really laugh like those ugly, snotty, kind of like piggy laughs because I would always be choking on my mucus or coughing. I could never really focus on the joy,”

While Vega is still not strong enough to laugh as fully as she hopes to one day, she does feel like a new person physically.

“My body feels great, and I feel amazing. Now I can actually strive to be something more than just some sick disabled girl that can barely get up off the couch,” she said, adding with a chuckle: “I just know I’m going to have the ugliest laugh ever.”

Deer was next. He received his new lungs on May 22. When he woke up from surgery, he was shocked to be breathing without assistance.

“I immediately said, ‘Where’s my oxygen?’ And then my wife said to me, ‘Well, you don’t need it anymore.’ So, I said to her, ‘Give me my oximeter.’ I put it on my finger and the reading was 99%,” he said.

“It was exhilarating. It is certainly a miracle,” Deer said. Gesturing to Vega, he added: “We are both walking miracles.”

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