U of U Health Transplant Team Provides Hope After Rare Lung Disease Diagnosis

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In her big, blended family of nine, Maylee Lemon is an optimist who loves to keep everyone laughing. When she and her husband got married, he had four children, she had one, and they had two more together—today those kids range from 40 to 15 years old.

It was during her pregnancy with her youngest daughter in 2005 that she first noticed some respiratory issues, but at the time she thought it was pregnancy-related. After her daughter was born she got pretty sick and went to the doctor for a chest X-ray.

“I’m not a doctor, but when he brought that chest X-ray up, I knew something was wrong,” said Maylee. “I seriously asked him how long I had to live.”

Her doctor sent her to a local pulmonology specialist for treatment. Two years later (after a lot of tests) the pulmonologist diagnosed her with pulmonary fibrosis, but still didn’t think that was actually what was going on. He sent her to Mayo Clinic, where specialists eventually arrived at a very rare diagnosis of pulmonary pleuroparenchymal fibroelastosis.

The cause of this condition is unknown, but experts think it could be the result of recurrent lung infections. Almost everyone diagnosed with the condition is a nonsmoker. It may also have a genetic link. That part made sense to Maylee because her older sister was having similar respiratory issues and was seeing a doctor in Seattle already (although she didn’t have an official diagnosis). He asked Maylee to come to his clinic so he could learn more about the condition so she was traveling up to Seattle regularly for checkups.

It wasn’t long before Maylee’s dad also started having symptoms. He would need a lung transplant, but he wasn’t interested in traveling to Seattle for treatment, so he inquired at University of Utah Health. He found a pulmonology specialist, and eventually got a single lung transplant from the lung transplant team.

By that time Maylee’s older sister had passed away, but she was still traveling to Seattle to see the specialist regularly. The trips weren’t as fun when they didn’t include time with her sister, and she knew that she would eventually need a transplant like her dad, so she decided to move her care to U of U Health too.

“I really liked the transplant team at the University of Utah, they were great with my dad and treated him really well.”

She didn’t need a transplant at the time—the disease progresses slowly—so she started seeing a pulmonologist.

By 2014 her disease had progressed to the point that she needed to be on oxygen. She tried not to let it stop her from doing the things she loved, but would notice signs that it was still getting worse. Her morning walks got shorter, and it was difficult to walk up and down two flights of stairs. When she was doing housework she would have to stop to catch her breath, and had to keep turning up her oxygen levels to feel like she could breathe.  

“I was blessed with a positive attitude so I didn’t let it stop me, my mindset was that I was not going to let it slow me down,” said Maylee. Maybe it helped that she knew from the start that the disease would get progressively worse over time, so that none of it was a surprise.

Meanwhile her pulmonologist was monitoring her condition and considering a lung transplant. As the condition progresses it leads to scarring in the lungs and eventually the tissues harden, making it very difficult to breathe. Figuring out the exact right time to get on the list for a transplant is a delicate line to walk. Doctors don’t want to recommend it too soon because it’s a significant operation with its own risks. They also don’t want to wait too long, because nobody knows how much time it will take to find a suitable donor match—the average is four to six months, but for some it can take years.  

The U of U Health lung transplant team discussed it with her and decided to put her on the list to find a donor in November 2019. She got a call three weeks later that they found a match.

She didn’t know what to expect, only that she was excited to get her lung transplant, and not at all nervous about what was about to happen. She was taken into surgery around 11:00 pm on December 13, 2019.

The surgery took almost 13 hours, in part because of her history of lung disease. Back around the time she was first diagnosed, she visited the emergency room in severe pain and unable to breathe. ER doctors discovered that her lung had collapsed, and she had to get surgery that “glued” the outside of her lung to her chest wall to keep it from collapsing.

During her transplant procedure, surgeons had to carefully remove that glued lung without causing additional damage or potentially life-threatening bleeding. Ultimately they got the whole thing except one small section, but it took a long time.

Despite those initial challenges, her recovery went well. She spent about three weeks in the hospital, then went home to continue with her recovery. In the first year after her dad’s lung transplant she watched him struggle with a lot of complications, frequently in and out of the hospital. By contrast, her recovery went smoothly. Her husband took a couple months off work to care for her, but she had no major complications.  

Surgeons often tell patients that with a lung transplant it’s not a matter of if your body will reject the new lung, it’s really a matter of when. But so far that hasn’t happened for her. Perhaps the most difficult part of her recovery was simply letting go of her oxygen tank. A couple weeks after the surgery when she went to a doctor’s appointment they told her she could stop using her oxygen. Psychologically she had a hard time realizing that she didn’t need that extra help anymore, and would constantly check her oxygen levels when it was off.  

“It’s the most amazing feeling in the world to be able to breathe,” said Maylee. “People take it for granted, but it is a miracle.”

Her doctors have talked to her about the fact that lung transplant patients usually don’t live as long, but it’s not something she dwells on. For now, she can breathe and do all the things that she couldn’t do for years as her disease progressed. She is focused on raising her two youngest children and spending time with her grandchildren, and looking forward to many more years with all of them.

“I love the team of U of U Health, even more now than after my transplant,” she said. “They are like family, genuinely concerned about my health and about me personally.”

Learn More About the Lung Transplant Program

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