Catching her breath

Cathy Cuenin first lost her breath five years ago. At the time, she was 48 and running five miles daily to train for the Klondike Road Relay race. One morning she awoke with strange back pain. Later, on the way up a fight of stairs, she inhaled but couldn't pull in enough oxygen. Suddenly, she was gasping.

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"I went to the ER and I had a collapsed lung," Cuenin said.

She was hospitalized and doctors were able to stabilize the lung. But when she went home, she again found she couldn't breathe.

"I just sneezed and it collapsed again. That is when they did a CAT scan," she said.

The scan revealed something doctors almost never see: Cuenin's lung was full of blebs, or air-filled pockets.

"It looked like Swiss cheese," she said.

Cuenin's doctor, William Palmer, diagnosed her with lymphangioleiomyomatosis, known as LAM disease, an incredibly rare and potentially fatal lung disease that affects only women.

Some women with LAM have only one incident, while others deteriorate quickly, needing first oxygen, and then a risky lung transplant. So far, the disease has no proven cure and there are few clues to what triggers it.

Only 1,000 pre-menopausal women in the United States have been given the same diagnosis, according to the nonprofit Ohio-based LAM Foundation. Cuenin was the second woman to be diagnosed in Juneau because Palmer, unlike many doctors, happens to be familiar with the disease.

"It was horrible and it was amazing that (Dr. Palmer) diagnosed it," Cuenin said. "Some women have it for 10 years before they know."

Cuenin, once an avid hiker and outdoor sports enthusiast, says adventure brought her and her husband, Loren Gerhard, north to Juneau after she finished nursing school in 1974. She took her first nursing job at Bartlett Regional Hospital. The couple later started a business in Sitka running tugboats and hauling freight. Cuenin worked for 10 years as a hospice nurse and has been the school nurse at Riverbend Elementary since 1999. Cuenin and Gerhard have one son, named A.D., a 25-year-old computer programmer who lives in Minnesota.

Though she was scaling mountain peaks five years ago, Cuenin must now wear oxygen just to run up a flight of stairs. She has been placed on a lung-transplant recipient list, and is moving to the San Francisco Bay area on New Year's Eve to be close to the transplant hospital.

"I miss those mountaintops, desperately," she said.

Cuenin says Gerhard, who works for the Southeast Conference, looks at the LAM as if both of them are afflicted, not just her. When she talks about the disease, she often uses the pronoun "we."

"Obviously, this is an incredible disappointment to both of us," Cuenin said. "I think we think of it as a real opportunity to be challenged, to ask, 'What is the real meaning of life?' "

On the Internet Cuenin and Gerhard found the LAM Foundation, a nonprofit group started in 1998 that funds research. According to the site, LAM is similar to cancer, in which cells grow in a mutated formation. LAM also is linked to kidney tumors, and a more common disease called tuberous sclerosis that causes muscle tissue to grow in the brain, liver and lungs.

A few months after her diagnosis, Cuenin was surprised to find she was not the only woman in Juneau to have the disease. Sally Slater, 55, had been diagnosed by Palmer a few years earlier after a similar episode with a collapsed lung. A mutual friend gave Slater Cuenin's number and the two soon became friends. They now e-mail or talk weekly.

"She contacted me to say, 'By the way, there will be life after these five months," Cuenin said. "You just feel like life is over. She said, 'Look, I look normal, and you will too.' "

Comparing Slater's story to Cuenin's illustrates the cruel randomness of LAM disease. Slater was diagnosed in 1995, when she was 48 and living in Haines. She first noticed a little shortness of breath, which she attributed to smoking. Then her lung collapsed and couldn't be repaired for weeks. Slater had a procedure called a pleurodesis in which doctors surgically stabilize the lung. Since then, she hasn't had many problems.

"Since 1995 I have been fine other than I had one other collapse, but it just resolved itself without a chest tube. I have a mild case," she said.

The women have compared not only the progression of their disease but their lives before diagnosis, trying to find something they might have in common, such as exposure to similar chemicals. They found nothing.

"They have done a lot of surveys in that regard, trying to find a common thread of some sort and they just haven't been able to at this point," Slater said. "We are both similar in age and we both live here where there is clean air. She's progressing at a much more rapid pace. There is just no rhyme or reason to it."

Cuenin says life with her worsening disease is like the life of a climber at altitude where the oxygen is thin.

"The energy it takes me to do a day is what it used to take me to climb Mount Juneau," she said.

Despite her shortness of breath, Cuenin is spry and chronically high-energy. Her longtime friend Mary Tonsmeire says she is the "kind of woman who can't even get up out of a chair without going 60 miles hour." Cuenin continues to work at the school and walks regularly in an effort keep in good shape. Transplant recipients must try to be as healthy as possible to survive a transplant, but must be seriously ill to get on the "hot list," where they are most likely to receive new lungs.

Cuenin carries a petite oxygen tank in a small sporty backpack when she works at Riverbend or walks the treadmill at the gym.

"I might have to graduate to something more heavy-duty soon," Cuenin said, joking about carrying a larger oxygen tank in a jogging stroller.

Her energy and sense of humor have no doubt helped Cuenin as she struggled for the last five years with the looming possibility of her own death from LAM. She estimates that if the disease continues to progress at this rate, she could live only a few years. She also knows the statistics associated with surviving a lung transplant - only half of transplant recipients live for five years. The most successful transplants extend life 10 years.

There have been times, Cuenin said, when she was walking alone on the airport wetlands trail and felt a cool panic, thinking about what might happen if her lung were to collapse with no one around to get her to a hospital. She has felt tremendous sadness, too, she said.

"I try to be able to see the fear and move past it. That is how I deal with fear of death," Cuenin said. "This has been slow enough. When it started, it was more in theory. Now I own it."

Over the years Cuenin has tried alternative healing, exotic vitamin supplements and hormones. Now, she just exercises regularly, takes multivitamins and tries to enjoy every moment, she said. Prayer, meditation and the support of her family and friends sustain her. She also loves her job working with elementary students.

"Nursing is a calling, not a vocation, for me," she said. "You get to work each day to empower people to be well and take care of themselves."

Through the LAM Foundation Web site, Cuenin made contact with a woman who stopped the progression of her LAM disease with the help of an acupuncturist in the San Francisco Bay area. Cuenin plans to give the acupuncturist a try while she waits for her transplant. She also plans to start training for the Bay to Breakers race with others in the San Francisco transplant support group. She says she remains open to the possibility that the mysterious disease may inexplicably change course, that she will be healed.

"I would love to see my grandkids," she said. "And I suspect I will. It amazes me in some ways that I am quite happy."

Julia O'Malley can be reached at jomalley@juneauempire.com.