Editor's note: This story is part of an ongoing investigative series about organ transplant. If you or someone you know would like to talk about your experience being an organ donor or recipient, contact investigative reporter Nora Eckert at neckert@postbulletin.com. Read previous stories in the series here.

Whenever Nithya Rao’s phone buzzes in the middle of the night, she gets a jolt. She's spent the last nine months on high alert, anticipating a call from Mayo Clinic that the heart-lung donation she desperately needs is finally available.

So far, the call hasn't come.

Every morning brings a temporary sigh of relief, followed swiftly by sadness. On one hand, Rao doesn't have to worry about the possible complications of such a complex surgery. On the other, she must continue battling the illness that's ravaged her pulmonary system.

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“Every day has an anxious moment,” Rao said. “It's been like that for the last nine months, where I'm like, every night hoping, maybe today is the day. Or maybe it is tomorrow. And then waking up in the morning and feeling a sigh of relief ... at times I feel that. At times, I’m sad I didn’t get the call.”

The 55-year-old is one of 38 candidates in the United States who await a heart-lung transplant. The procedure is extremely rare: just 58 were performed in the United States in 2020, 13 more than the year prior. Fewer than a handful are performed at Mayo Clinic each year.

While finding a donor who can provide one compatible organ is difficult, the chances of getting a donor with two fitting organs is even slimmer. Patients must sometimes withstand the stress of undergoing two separate operations when only one organ is available at a given time.

“It’s a really hard process being ill enough that you're going to be dying from a disease with your heart or your lungs. And then the option is transplantation, but it's not off the shelf,” said Dr. Richard Daly, a cardiothoracic surgeon and director of heart-lung transplant at Mayo Clinic. “You're waiting, you're just hoping that this will happen while you have a chance. So it’s a lot for people to go through.”

“We don’t know how long you’re going to live”

In July 2000, during a visit to see her parents in Washington, D.C., Rao nearly passed out while walking down the street.

She’d grown more fatigued and short of breath over the months since her second daughter was born, but she regarded these symptoms as complications from her pregnancy. Several doctor visits later, she received a dire diagnosis: primary pulmonary hypertension.

The chronic disease narrows pulmonary blood vessels, affecting vital arteries that carry blood from around the body back to the lungs. Unmanaged pulmonary hypertension can lead to heart failure.

Nithya Rao, second from left, stands with her two daughters and husband. The 55-year-old is one of the few people in the nation awaiting a heart-lung double transplant. Contributed / Nithya Rao
Nithya Rao, second from left, stands with her two daughters and husband. The 55-year-old is one of the few people in the nation awaiting a heart-lung double transplant. Contributed / Nithya Rao

“They didn’t have a timeline. They said ‘We don’t know how long you’re going to live. Maybe a few days, or weeks, or months.’ That’s all they gave me,” Rao said.

Rao went through a series of clinical trials and tests. She lived her life connected to an IV line and an oxygen tank.

Then, in 2011, a new doctor discovered a hole in her heart, which she thought might be the cause of Rao’s condition. Surgeons closed the hole and slowly weaned her off of medication. By 2014, Rao was told she was free of primary pulmonary hypertension.

“That was my happiest day. I think I was so happy,” Rao said. “Looking back, I didn't think I would live that long. That I would survive, that was like a victory for me.”

She engaged in all the activities she’d missed for years. She worked out at the gym and went for long walks. Her life returned to normal.

Three years later, the symptoms crept back in.

She started a new job as a data analyst for Allstate — reclaiming a part of her life she lost to the disease — and began experiencing dizzy and fainting spells at work. Walking from her car to the office building left her breathless. The PPH specialists were aghast. They’d rarely, if ever, seen a case resurge after treatment. Rao was the exception.

She went back on her medication, eventually reaching the maximum dose possible. As these treatments grew less effective, her options narrowed. Rao was referred to Mayo Clinic, where she found that a single avenue remained: a double lung and heart transplant.

Double transplant, triple listing

After going through rounds of physical and psychological tests to be evaluated as a potential organ recipient, in addition to receiving antibiotic therapy for a latent tuberculosis condition, Rao was listed as an eligible organ recipient at Mayo Clinic. She was also added to the national registry for the United Network for Organ Sharing.

It is doubly difficult to find a suitable donor for Rao. One reason is the already narrow list of eligible organ donors is even slimmer when one is in need of two compatible organs.

“The donor has to have suitable lungs and heart for transplant. It's not uncommon for donors just to have one or the other organ system working well enough to be suitable for the rigors of transplant,” Daly said. Often this is because organ donors die as the result of a trauma that can leave them brain dead, affecting lung function.

At Mayo Clinic, cardiac surgeons perform heart-lung operations, typically transplanting the two organs as a single block. There may be situations where only a lung or heart is available for the patient, but not both. In these cases, one organ may be transplanted before another is available.

This can be difficult for the patient as the new lungs struggle to work with the existing heart, or vice versa. Plus, it means the patient must endure two transplant procedures and recoveries. Such decisions weigh heavily on the transplant team and rely on the health of each candidate.

Rao attempted to be added to Mayo Clinic-Jacksonville's transplant list to improve her chances of finding a donor, but she was deferred because of her distance from the hospital. Heart and lung transplants are extremely time sensitive in order to maintain organ integrity, so recipients must be able to travel to the medical center as quickly as possible.

Additionally, Daly said the organ allocation system can cause difficulties for those waiting on more than one transplant. There is a complicated set of rules for those in this rare spot. A heart-lung transplant patient is listed three times for the transplant center and on the UNOS national registry: the heart, lung and heart-lung lists for each.

A UNOS representative said the patient is listed multiple times as a way to cover all potential transplant opportunities.

“By doing so, the center ensures that their heart-lung candidates will appear in all the appropriate heart, lung and heart-lung match runs. If they were to list their candidates on only one or two of the lists, they could potentially miss suitable offers, particularly in the case of hearts,” wrote Anne Paschke, UNOS media relation specialist.

'How does a normal person breathe?'

Twenty-one years ago, Rao wasn't sure if she would live more than a few days.

Her life has since swung between bouts of intense PPH and brief periods of reprieve.

"There are days where I'm constantly crying thinking about it. Am I going to be alive? Am I going to be able to see my grandkids and live to enjoy my life later? I do think about that," Rao said. "Being on the transplant list, there's a sense of hope."

She's had fleeting glimpses of what a healthy life feels like. Now, she craves that normalcy.

She wants to go to the gym, walk outside on a humid day, tend to her garden without growing exhausted. She wants to visit her family in India, a trip she's avoided for fear that she'll receive the call from Mayo while overseas.

“I don’t know if I might get transplanted next month, or a few months or years. I don’t know. But I have a hope that, yes, I might get something. So I can live longer to see my kids be successful. And be able to live longer to enjoy life,” Rao said. “How does a normal person feel or breathe? Because I never know what is normal to breathe.”