Breath of Hope | Page 2 of 2

Breath of Hope

What happens when a woman is diagnosed with a rare lung disease and given a short time to live? Sometimes, life happens

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Stojic and her husband, Steve, a Washington attorney, enjoy a quiet moment at their farm in  Fairfield, Pa. Photo by Erick GibsonProgesterone didn’t seem to help her lungs, but it definitely affected her behavior, Stojic says. “I did not handle things as diplomatically as I had prior to progesterone. I became the bluntest woman in the county. When I say blunt, I’m being nice,” she says with a laugh. “No holds were barred. My husband, of course, bore the brunt of that.”

From the time she was diagnosed, Stojic has participated in an ongoing LAM study launched by Moss in 1995. For 10 years, she checked into the National Institutes of Health’s Clinical Center, about a mile from her home, twice a year for three or four days of intensive testing. Now she usually goes just once a year. Her daughters used to refer to it as “Mom’s NIH sleepover.”

About 500 patients have enrolled in the study over the years, says Moss, who lives a block from the NIH campus where he has worked for 38 years. Many come from other countries. NHLBI covers the cost of their travel within the United States and their stay at the Clinical Center.

Moss didn’t earn his M.D. and Ph.D. in biochemistry with the goal of studying the disease. But in the 1990s, he says, “I was given the opportunity to start a clinical research program, and I was trying to figure out where I could do the most good. LAM was one where I had the expertise.” 

“He’s a role model for the majority of us who study patients with rare diseases,” says Dr. Richard Childs, clinical director at NHLBI. “Trust is absolutely the key element.”

Stojic recalls leaving Moss a voice mail message from her hospital bed one weekend. “I said my lung collapsed again, and we’re debating how to do surgery,” she recalls. It was her second operation on the same lung, and “each time you have surgery it becomes more and more complicated.”

She didn’t have to wait long for Moss to call back. “He was actually in the office working. He’s very, very responsive to patients and their physicians,” she says. “I think it’s very reassuring for patients who are participating in the protocol [study] at NIH to know that he’s willing to do that.”

Reassurance is in great demand by new enrollees in Moss’ study.

“Some of the patients who were referred here early on were told they have LAM and they’re going to die soon,” he says. But as patients like Stojic kept returning to the Clinical Center year after year, Moss says he learned that LAM  “tends to be much more of a chronic disease” than a terminal one. “We have patients with LAM who are in their late 70s,” he says. “It’s clear they had symptoms for 20 or 30 years or more. They just weren’t diagnosed.”

Patients want to know whether they’ll be among the lucky ones whose disease progresses slowly, but Moss can’t yet answer that. “We don’t know why some patients move more quickly than others,” he says.

Many patients wonder whether pregnancy would be risky because of the accompanying estrogen surge. “There’s disagreement in the field,” Moss acknowledges. “What I’m going to say is controversial to some extent, but if the patient has mild disease, they can go through pregnancy without progression” of LAM.

On Tuesday or Wednesday afternoons, Moss meets with LAM patients who are staying at the Clinical Center that week. Some bring a husband or friend. “What we give them is a follow-up on what we’ve learned over the past year,” Moss says.

 At a recent session, five patients, one husband and one friend sit around a table in a Clinical Center conference room. One woman was diagnosed only a month earlier, another in 1989. “The idea here is to be very interactive,” Moss tells them. “You’ll learn as much from other patients as you will learn from me. We’ve made some of our patients very good microbiologists. Somebody has to go back and educate the doctors locally.”

In response to the patients’ questions, Moss covers a large whiteboard with notes and diagrams about how LAM affects the lungs. He recounts what a longtime patient once told newcomers: “When we first started, that board was empty.”

Stojic, who has hosted a talk by Moss at her home, reaches out to newly diagnosed women as The LAM Foundation’s regional liaison. About 50 women in Maryland, Virginia and Delaware are living with a LAM diagnosis, including three or four in Montgomery County, Stojic says.

“Her attitude about LAM is just so positive,” says Sally Lamb, director of patient services at the foundation. “When she talks to new patients, they get a great sense of reassurance from her.”

It isn’t always easy, Stojic says. “My heart breaks for the young women in their 20s who don’t know if they should have children.” They’ve heard of women not much older than they are who’ve died of LAM, Stojic says, and, just as she once did, they worry that they won’t live to see their kids grow up.

There is still no cure for LAM, but a study co-authored by Moss and published in 2011 in The New England Journal of Medicine­ found that the drug sirolimus stabilized lung function and improved the quality of life in LAM patients. Marketed as Rapamune, the drug is approved to prevent rejection in kidney transplant patients. But because it suppresses the immune system, it increases a patient’s risk of infection and cancer.

As a result of the study, Stojic has been taking sirolimus for about a year and a half. Her lung function has still continued to decline, but she attributes that to her surgeries for collapsed lungs, rather than a failure of the drug. 

Stojic isn’t nearly sick enough to go on the waiting list for a lung transplant, but if her lung function continues to diminish, she might have to go on oxygen. She’s not thrilled at the prospect.

When she’s in her 50s, “I want to be able to fly to see my grandchildren without the hassle of oxygen,” she says.

At least now she believes she’ll live long enough to have them. 

LAM or LYMPHANGIOLEIOMYOMATOSIS

THE FACTS:

  • LAM is a rare lung disease that affects only women.
  • LAM usually strikes women between the onset of puberty and menopause.
  • Symptoms may include shortness of breath, cough, chest pain, fatigue and collapsed lung.
  • An angiomyolipoma, a benign kidney tumor, is found in 50 percent of women with LAM.
  • LAM is frequently misdiagnosed as asthma or emphysema.
  • Nearly 300,000 women worldwide are unaware that they have LAM.

For more information on LAM, go to www.thelamfoundation.org.

Rita Rubin, a former health writer for USA Today, lives in Bethesda. 

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