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No more waiting to exhale

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Special to The Times

Three years ago, Doris Rheaume, a retired addiction counselor, could barely breathe.

“I was on oxygen 24 hours a day,” she says. “If I turned my head without it, I huffed and puffed.”

There is no mystery why the Needham, Mass., woman was so sick. She had smoked “a few packs a day for many, many years.” And, like 15% to 20% of smokers, Rheaume, now 71, has paid a terrible price for her habit: chronic obstructive pulmonary disease. People with the disease, known as COPD, have chronic bronchitis (inflammation of bronchial tubes), emphysema (destruction of the tiny air sacs in the lungs) or both.

Better drugs, daring surgery -- the subject of a just-released report -- and better pulmonary rehabilitation are significantly improving the outlook and quality of life for many people with COPD.

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But it remains a miserable condition in which sufferers slowly suffocate, breath by breath, over many years. The lungs swell and lose their normal elasticity. Stale air gets trapped in over-inflated air sacs. By using accessory muscles, people still inhale, with effort, but exhaling is nearly impossible. Instead of looking healthy and sponge-like, the lungs begin to resemble cotton candy.

People with severe COPD “can’t get a whole sentence out without taking a breath,” says Dr. Gail Weinmann, director of the airways biology and disease program at the National Heart, Lung and Blood Institute. “They have to rest after putting on a sock.”

According to the federal Centers for Disease Control and Prevention in Atlanta, 10 million Americans have been diagnosed with COPD and an additional 14 million have somewhat impaired lung function. The death toll is 120,000 Americans a year -- and rising.

“COPD is the only cause of death that is rising in the U.S. and in the world,” says Dr. Bartolome Celli, chief of pulmonary and critical care medicine at Caritas St. Elizabeth’s Medical Center in Boston. It ranks as the fourth-leading cause of death in the U.S. and is expected to be No. 3 in the U.S. and worldwide by 2020.

Moreover, the death rate is rising fastest in women. Men in the U.S. have begun responding to anti-smoking campaigns, but in general women have not.

In the U.S., even teenage smokers show “unmistakable evidence” of early COPD, says Dr. Donald Tashkin, a professor of medicine and specialist in pulmonary critical care at UCLA, citing data from lung function tests and autopsies of teens who died in car crashes.

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In COPD, as in asthma, the big culprit is inflammation of the lungs and airways. White blood cells pump out chemicals called cytokines that cause mucus glands to produce excess mucus. Inflammation also leads to scar tissue that makes the windpipe more rigid, which makes breathing harder.

In the delicate air sacs of the lungs, inflammatory cells pump out digestive enzymes such as elastase, collagenase and some metalloproteinases that eat away lung tissue. “The air sacs get larger and larger and their number decreases,” Celli says. “The result is a net decrease in surface area, and air gets trapped inside.”

So, what to do? First, if you smoke, stop. Some loss of lung function is normal with aging, but COPD speeds up that decline, says Dr. Gregory Diette, assistant professor of medicine and epidemiology at Johns Hopkins University. “People who quit smoking return to a normal rate of lung-function decline within weeks.”

Second, get influenza and pneumococcus vaccinations to avoid bronchial infections. Third, exercise, but judiciously -- when air quality is good.

Beyond that, watch for signs of COPD and ask your doctor for a lung function test called spirometry if you’re worried. Worrisome signs include shortness of breath without much exertion, chronic cough or excess mucus production.

If you are diagnosed with COPD, don’t despair; treatments are getting somewhat better. Among medications, there are three main options, which can be used together.

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Inhaled bronchodilators (drugs that keep airways open) are key. Beta-agonists, anti-cholinergics and theophylline-based drugs are the three main types.

A totally different approach is to use anti-inflammatory drugs such as the inhaled corticosteroids Budesonide, Fluticasone and Beclomethasone. Yet another approach is to use antioxidants, which combat the dangerous free radicals triggered by inflammation. One antioxidant drug (N-acetylcysteine) has been shown to be helpful in European studies but is not approved for use in COPD in the U.S. yet. There’s also a five-center study underway to determine whether some forms of retinoid (vitamin A) can induce new lung tissue growth in people, as it does in rats.

Portable oxygen tanks also can make life more livable for people with COPD, as can pulmonary rehabilitation, which involves learning how to conserve energy while dressing, talking and climbing stairs and how to exercise without undue shortness of breath.

Lung transplants are a last resort, and donor lungs are in short supply. That has led surgeons to try a radical alternative: cutting away parts of the diseased lungs to reduce overall lung size so that there is more room in the chest cavity for the remaining lung tissue to deflate and inflate properly.

One new study on lung-reduction surgery combined data on 150 patients (from studies in Massachusetts and Canada). It showed that the surgery works better than medications at improving lung function, says Celli, who was part of the study.

Another study, the National Emphysema Therapy Trial, was released last week. It was set up in 1996 after Medicare stopped paying for lung reduction surgery after questions arose about its benefits.

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The new study found that “patients with severe emphysema who undergo lung volume reduction surgery along with medical management are more likely to function better and face no increased risk of death after two years compared to those treated with medical management alone,” according to a prepared statement from Johns Hopkins researchers, who led the study.

But some patients fared worse with the surgery: those whose disease was located primarily in the upper lobes of the lungs and who had relatively good exercise capacity before surgery. The study is expected to affect Medicare coverage.

Though the operation is clearly not for everyone, some patients who’ve had it swear by it, among them Rheaume. “I can’t do like I used to do,” she says. “But at least I can go around with oxygen and go places. That is most important.”

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