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WAITING to INHALE

In April 2003, Pat Hovis learned a new word.

Lymphangioleiomyomatosis.

LAM for short.

LAM is a rare lung disease, and her doctor told her she had it. A CT scan revealed cysts throughout her lungs, leaving them looking like Swiss cheese.

"I was 48 and had 50 percent of my lung use," Hovis said.

"The doctors looking at the scan kept asking me, 'How much did you smoke? How long did you live with smokers?' "I never smoked."

Looking back, Hovis realized that the disease probably started several years before her diagnosis. Following the birth of her second child, she struggled to keep up with the active toddler, though she always had been a high-energy person.

Her breathing problems were misdiagnosed as asthma - misdiagnosis is very common with LAM - but asthma treatments were ineffective. Her doctor began to suspect some more serious lung problem.

LAM has no cure, no effective treatment.

But if you think despair is the only option, meet Pat Hovis. She lives with the hope of a double lung transplant and enthusiasm for the work of the LAM Foundation.

After her diagnosis, Hovis threw herself into the foundation's work, attending international conferences and traveling to the National Institutes of Health (NIH) in Bethesda, Md., every six months to be tested for research.

She also took on fundraising - in a big way.

"I've brought in $150,000 since I was diagnosed," she said. Most of that came from personal donations and is being used for research and patient care.

Unfortunately, that is only a fraction of the cost of a transplant, which runs about $640,000. The post-transplant medications required for the rest of her life now cost $4,000 to $6,000 per month.

"Going to NIH really helped me take control of my disease. They're up front about what you can do," she said.

Although she cannot travel to the conferences anymore, she stays in touch with other LAM patients through a worldwide patient registry established by the LAM Foundation in 2007. And she has more plans to contribute to research.

"After my transplant," Hovis said, "my diseased lungs will go off to 15 researchers. I'm hoping we'll be able to grow our own lungs or hearts through stem cell research someday."

Her first roommate at NIH was a woman in her early 20s from Costa Rica. She had suffered five lung collapses and lost a kidney to the disease.

"These are strong people, I tell you," Hovis said. "The sad part is that there are so many young women with this."

Preparation for the lung transplant includes pulmonary rehabilitation at Olympic Medical Center's Sequim facility. She also is part of a "lung support group" started by another local woman who had a lung transplant. Although there are no other LAM patients nearby, the support of others who face lung disease is valuable to her.

Hovis has found an additional form of pulmonary therapy: the harmonica. She and a friend practice together and the breathing in and blowing out help her lung function.

"Right now my job is to keep my other body organs as healthy as possible," she said.

Hovis discontinued using an experimental medication in order to go "on-list" for a transplant.

"I tried it but didn't see a big difference," she said.

"I couldn't do the transplant on the drug. I had to make a choice."

With her typical enthusiasm, Hovis is planning her post-transplant life.

"I'm making my list for when this is over," she said. "My husband and I want to travel. I want to get back into photography."

A couple of factors improve her transplant odds:

_ She's never had a collapsed lung, which makes the transplant easier to do.

_ Ironically, because of the growing cysts, her chest cavity is now larger, giving her more chances for a tissue match.

"I can have a man's lungs because my chest has expanded," Hovis said.

She celebrated along with the University of Washington Medical Center when they announced in June the recent completion of their 500th lung transplant and 500th heart transplant.

Now that she's on the list, she said, "I can't go west of Port Angeles, and I have to have a phone on me all the time."

Although there are people higher on the transplant list than she is, factors such as tissue matching make it possible that she could get a transplant soon.

"I keep saying it's going to happen in 2009," Hovis said. Meanwhile, she is tethered to an oxygen tank by a long plastic tube.

Her attitude remains upbeat.

"If you have to have a rare disease, I guess this is a good one," she said, "because there is so much support."



Sandra Frykholm can be reached at sfrykholm@sequimgazette.com.



What is LAM?



Facts about lymphangioleiomyomatosis:

_ LAM is a progressive lung disease that usually occurs in women during their childbearing years.

_ LAM cells form clusters and cysts in the lungs, destroying healthy tissue. Eventually, breathing is impaired more and more severely.

_ The exact number of people living with LAM is not known. Although just 1,500 cases of LAM have been identified, scientists estimate that as many as 250,000 women may be undiagnosed or misdiagnosed because the symptoms are similar to those of asthma, bronchitis and emphysema.

_ Scientists have discovered a genetic link between LAM and another rare disease, tubular sclerosis, which causes benign kidney tumors in most cases. About 40 percent of LAM patients have these tumors.

_ Researchers believe the hormone estrogen may play a role in LAM, but at this time the connection is unclear.

_ Because pregnancy may accelerate the progression of LAM, patients are urged to consult a health care professional before getting pregnant.

_ Treatment options are very limited at this time, although a major study is under way testing a drug now used to prevent kidney transplant rejection. Supplementary oxygen use is common.

_ Because of their high risk, lung transplants are considered a last resort.

More information on LAM can be found online at www.thelamfoundation.org.



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