Forgotten disease

Frank Nichols no longer takes breathing for granted.

“You don’t think about this air business until you don’t have it,” Nichols, a 79-year-old Spokane man, says over the quiet thunk-thunk of his portable oxygen machine.

Nicholas suffers from COPD — chronic obstructive pulmonary disease. He’s one of nearly 11 million Americans who have been diagnosed with the incurable lung disease, according to the American Lung Association.

COPD is currently the fourth leading cause of death in this country, behind heart disease, stroke and cancer, and its numbers are on the rise. Death rates from COPD have doubled over the past 30 years, and the disease is expected to be the nation’s third largest killer by 2020, according to a recent report in the Journal of the American Medical Association. Some experts suspect there are millions more who are undiagnosed.

And yet, many people have never heard of it.

“This is a forgotten disease,” says Cindy Thompson, regional director for the American Lung Association. “And there are just a lot of people suffering from it.”

COPD is really a combination of two diseases, both of which make it difficult to expel air from the lungs. A mix of chronic bronchitis and emphysema causes COPD patients to suffer shortness of breath and cough. The disease also makes them more susceptible to respiratory infections.

COPD limits the lungs’ ability to take in oxygen and remove carbon dioxide. And it causes the small airways in the walls of the lungs to lose elasticity. The passages then become clogged with mucous.

“It’s a disease in which the symptoms can be managed,” says Heidi Jibby, a respiratory therapist and assistant manager of the Medically Directed Wellness program at St. Luke’s Rehabilitation Institute. “It’s not really a treatable disease. … The more they manage their symptoms, the better quality of life they will have.”

The vast majority of people with COPD – some 80 to 90 percent – are or have been smokers. (A small percentage of COPD cases are caused by genetic or environmental factors.) The link to smoking is likely the reason the disease doesn’t get the attention of other common ailments, many say.

“There’s a lot of shame involved,” Jibby says. “It’s difficult for people sometimes to cope with the disease, based on the fact that it might have been self-inflicted.”

Dr. Sam Joseph, a Spokane lung specialist who is the pulmonary rehabilitation medical director at St. Luke’s, knows of some people who won’t even call the disease COPD.

“A lot of patients, even some doctors, will call it something else,” Joseph says. “They call it asthma. There’s a stigma about calling it what it is. It does have the connotation that it’s self-induced. They’ll try to blame it on Mount St. Helens or that they worked in a mill.”

Nichols says he smoked for “oh, God, like 100 years,” before finally quitting in 1999, shortly after the death of his wife. He’s been diagnosed with COPD for the past five or six years but has felt the symptoms creeping up on him much longer than that. Last year, the doctor put him on oxygen, and he’s now hooked to the tank 24 hours a day.

He’s currently one of about 24 COPD patients enrolled in the pulmonary rehabilitation program at St. Luke’s. Participants learn more about the disease, including how to prevent infection, how to breathe more efficiently, and how to conserve energy and oxygen.

During a session last week, Nichols walked slowly on the treadmill and pedaled on a stationary bike.

Jibby monitors the patients’ oxygen levels and blood pressure during the exercise.

“It’s to help increase strength and endurance so they can do more with less shortness of breath,” she says. “That, in turn, helps to improve their quality of life.”

Nichols, for one, says he already has noticed feeling less shortness of breath when climbing stairs after his exercise class.

With so many people suffering from the disease, COPD is a fertile area for medical research. New treatments have come along just in the past several years, Joseph says.

The newest medication is called tiotropium, or Spiriva, he says. The inhaled drug needs to be taken only once a day, and there is evidence it slows progression of the disease, he says.

And simple measures, such as getting flu and pneumonia shots, can go a long way toward preventing complications from COPD, he says.

But the most important way to stem progression of the disease is an obvious one: Quit smoking.

“Of course, if you’re still smoking you should stop. That is the No. 1 thing,” Thompson says. “There will be people that will continue smoking even when they’re on oxygen, because of the nature of it being so addictive.”

There is some work, at a national level, to raise awareness of COPD. And much of that effort has been spearheaded by Sen. Mike Crapo (R-Idaho), who formed the Congressional COPD Caucus last year.

Crapo started the group after joining his brothers, who are both doctors specializing in lung research, at a COPD conference.

“This is so important and is such a big thing,” Crapo said in a recent phone interview. “And it has such a low level of understanding in the public as well as, I believe, in Congress, … There is no congressional district and there is no state in which there aren’t thousands and thousands of constituents who face problems.”

The group has pushed for greater funding for COPD research, as well as making sure members of Congress are informed about the disease. Most recently, the caucus has pressed the Federal Aviation Administration to change the rules to allow people on oxygen to travel by air.

A report out late last week by the Centers for Disease Control and Prevention shows that smoking rates continue to slowly decline in the United States, with just under 21 percent of adults having smoked in the past year.

But as that population of smokers gets older, doctors are expecting to see many more COPD cases.

“There’s always going to be a repository of patients,” Joseph says. “Even if everyone stops smoking today, we’re going to see fallout of this for two decades.”