NEAL CONAN, HOST:
This is TALK OF THE NATION. I'm Neal Conan in Washington. There are treatments but no cure for COPD. So the many millions who suffer from chronic obstructive pulmonary disease have to learn to live with the coughing, wheezing and shortness of breath and with the knowledge that COPD is now our third leading killer after cancer and heart disease.
Earlier this week we spoke with victims of rare diseases. Today we want to hear what we don't know about life with a disease that afflicts at least 12 million and maybe many more. According to a report released last week by the American Lung Association, the majority are female. Women now account for more than half of all deaths from the disease and are 37 percent more likely to have COPD than men.
So what don't we know about life with COPD? Tell us your story. 800-989-8255 is our phone number. Email us, firstname.lastname@example.org. You can also join the conversation on our website. That's at npr.org. Click on TALK OF THE NATION.
Later in the program, another in our series of conversations looking ahead. Today, Suleika Jaouad, diagnosed with acute leukemia at the age of 22, author of the Life Interrupted blog, now cancer-free. But we begin with Grace Anne Dorney Koppel, who got her COPD diagnosis in 2001. She's now a patient advocate and national spokesperson for the National Heart Lung and Blood Institute's campaign to raise awareness of COPD. She joins us now from her home in Potomac, Maryland. Welcome to TALK OF THE NATION.
GRACE ANNE DORNEY KOPPEL: Thank you very much. I would like to correct just one thing. Our numbers are estimated to be 25 million. Only half of us have been diagnosed and are being treated.
CONAN: I was just using the Lung Association's numbers, so that's why we had them. Take us back. When did you first hear those initials, COPD?
KOPPEL: I first heard those initials probably when I was diagnosed in 2001. I had not been successful with my family doctor in getting a diagnosis for this extreme shortness of breath, inability to go up stairs, couldn't do things I had easily done before. And it seemed to be sudden. But it's a progressive disease, and that was misleading.
The first large medical institution that I went to for a diagnosis immediately pegged it at chronic obstructive pulmonary disease. It's easily diagnosed with a simple breathing test, and I only had a quarter of the lung function that I should have had at that time, in 2001.
CONAN: And the prognosis, as I understand it, was pretty dire.
KOPPEL: Oh, it was very dire. If anyone in a white coat ever looks at you, and when you say how long do I have, you know, when will I get better, and someone says to you, well, Grace Anne, you know, you really should make end-of-life preparations, maybe three to five years, maybe you'll be on oxygen full time in just a couple years.
And I was then in a wheelchair going from appointment to appointment because I was simply too weak to walk.
CONAN: And what has happened in those intervening years? Obviously you're still with us.
KOPPEL: I am very much with us. What has happened is that I have realized that as a result of the proper treatment, the proper use of medications, and an awful lot of hard work on my part - exercise, diet, nutrition - that I have not only been able to regain some of my lost lung function, but maintain it.
So today I have 50 percent of what everyone else has and takes for granted, but it is enough for me. I'm able to walk two to three miles a day at an aerobic level. I can - you know, I'm very active. I travel by plane and train and car. I'm involved in life. And I would not have been had I not had pulmonary rehabilitation.
CONAN: We want to hear from others who suffer from COPD. What don't we know about life with this disease? Give us a call, 800-989-8255. Email us, email@example.com. Let's start with Judy(ph), and Judy's on the line with us from Juneau in Alaska.
JUDY: Hi, I'm glad to talk to you.
CONAN: Hi, go ahead, please.
JUDY: I'm a nurse, and I just didn't realize how difficult it is for people on oxygen, how difficult the tubing is and accident-prone they become. I got the disease two years ago, I believe from a medication that I took for asthma that suddenly turned into COPD, which is much more severe and life-threatening.
And I almost died, and as your earlier speaker said, I did go to pulmonary training and an exercise program and have regained a fairly normal life, although I can't go out very long.
CONAN: Can't go out because of fears of...
JUDY: Well, I'm so weak. I've become so weakened.
CONAN: And do you use oxygen all the time?
JUDY: Yes, I do.
CONAN: What about you, Grace Anne?
KOPPEL: I no longer require oxygen. I did - I was on oxygen after lung cancer surgery, and I've probably spent, of these 13 years, two to three years on oxygen. But I don't require oxygen except when I fly long distances.
JUDY: That's very encouraging. That's very encouraging, and I - we do need you speaking out because the doctors don't know much about taking care of this. I've been amazed how awful the steroids have been and have not been using steroids and have been using other medications and have gotten better.
CONAN: Well, Judy, we wish you the best of luck. Thank you very much for the phone call.
JUDY: Thank you for taking this subject.
CONAN: And we're going to talk with a doctor who's a lung specialist and research at Johns Hopkins University School of Medicine. Dr. Enid Neptune treat patients with COPD. She joins us now from member station WYPR in Baltimore. And good of you to be with us today.
ENID NEPTUNE: Well, I'm very glad to be here, and thanks for inviting me.
CONAN: What are the main risk factors for COPD?
NEPTUNE: To some extent the risk profile depends on which country you're talking about. In First World countries, such as, you know, the United States, we're largely talking about people who have exposure to chronic cigarette smoke. And the vast majority of patients I see have very significant histories of cigarette smoke exposure, and we're talking maybe 20, 30, 40 years.
However in the Third World we're talking about exposures to what we call biomass, and those are particulates that are generated when people use wood-burning stoves inside. And so the global spectrum of COPD is very much dependent on the country of origin and also on kind of the economics of that particular country.
CONAN: And while relatively few of us are familiar with that term, COPD - emphysema, bronchitis, chronic bronchitis, those are lumped together with - as part of COPD?
NEPTUNE: Yeah, sometimes it's a little confusing because the terms are used interchangeably. But I think the most convenient way of looking at it is to see COPD as encompassing aspects of both chronic bronchitis and emphysema. Now, what those terms really refer to are different compartments of the lungs.
So the goal and the ultimate strategy of the lung is to deliver oxygen to the bloodstream so that you can oxygenate your tissues. And the structures that actually do this are either tubes, or the conducting structures or the highways of the lung, and these little sacular structures which are the sites where oxygen kind of moves from the lung into the bloodstream.
And the unfortunate aspect of COPD is that both of these kind of compartments are compromised, and so you have two reasons to have compromised delivery of oxygen. And as a corollary to that, what happens is that you have a problem getting rid of air from which oxygen has already been extracted, and therefore you're breathing at very, very high lung volumes, and that causes your respiratory muscles to fatigue.
And you enter into what Grace was describing as the cycle of disability, where you become more short of breath, you become more deconditioned, you become more short of breath and more deconditioned. And that's why the aggressive efforts for pulmonary rehabilitation have been so effective.
CONAN: So the disease, I guess in this country if it's attributable to cigarette smoke largely, it's preventable. The treatment, though, as she describes it, is a treatment, not a cure.
NEPTUNE: Exactly, and those of us who do research on this disease are actively trying to find interventions that can restore normal lung function in patients who are very compromised by the disease. But a point that I want to make is that the treatments are effective. And so there should be no patients who have the diagnosis who aren't being placed on an armamentarium of interventions that have proven to be quite successful.
And obviously Grace has had a very, you know, auspicious response, but that's not unusual, actually, and so we would want to call patients to arms so that they can talk to their physicians and come up with a treatment plan that's likely to make them kind of much more comfortable and much less short of breath.
CONAN: Grace Anne Dorney Koppel, as you talk with others who have this disease, what is the most difficult thing to overcome? Is there a sense of hopelessness because of that lost of breath?
KOPPEL: Yes, many can feel that, and as the disease progresses, people tend to become more and more isolated and more tied to their own homes, to the oxygen tubing that your caller described. But we do live in a time when there are portable oxygen concentrators, perhaps not as available to all as they should be, and it is very possible to lead a more or less normal life if you have a portable oxygen concentrator.
But I do want to focus on one thing, Neal, and that is that we have only come to realize lately how very severely women have been affected by the disease. We apparently suffer more. Our symptoms are worse. There are many, many causes postulated for this. We have smaller bodies. Our lungs are smaller. Our airways are smaller. But women do suffer more. They have more flare-ups of the disease.
They are less likely to be diagnosed by their doctors because they're women. There is gender bias, and I do think that the American Lung Association white paper clearly underlines this.
CONAN: We're talking with Grace Anne Dorney Koppel and with Dr. Enid Neptune about what we don't know about life with COPD. Tell us your story if that's you. 800-989-8255 is the phone number. Email us, firstname.lastname@example.org. Stay with us. I'm Neal Conan. It's the TALK OF THE NATION from NPR News.
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CONAN: This is TALK OF THE NATION, from NPR News. I'm Neal Conan. We're talking about life with COPD, chronic obstructive pulmonary disease. The World Health Organization counts some 64 million with the disease worldwide. Many are, or were, smokers. Many lived with or worked with smokers. But a significant percentage of COPD patients never smoked.
What used to be a disease that largely affected men - or largely seen as affecting men - now more common among women. So what don't we know about life with COPD? Call, tell us your story: 800-989-8255. Email us: email@example.com. Our guests are Grace Anne Dorney Koppel, first diagnosed with COPD in 2001, now a patient advocate and national spokesperson for the National Heart Lung and Blood Institute. Also with us, Dr. Enid Neptune, a lung specialist and researcher at Johns Hopkins University. Let's get Bill on the program, Bill's calling us from Sacramento.
BILL: Hello. Thank you for taking my call. I think I'm not the typical COPD patient. I'm 64 years old. Approximately nine years ago I, was diagnosed with COPD, because as a long-distance runner, when I would start running, after about a mile, I would have severe chest pain, and it just stopped me. And, of course, I went to the doctor, and I had no heart disease.
I was told then after diagnostic x-rays and pulmonary function tests that I had COPD, and that's why I was having chest pains. That still has greatly affected my exercise life. I was also a mountain climber and have a great deal of difficulty above 10,000 feet, say, in breathing and need to use inhalers at this time. So I didn't know there were people like me that never smoked, never had asthma and have COPD.
CONAN: Dr. Neptune, is - how common is that?
NEPTUNE: It's not common, but it's also not rare. We find that a certain subset of patients with COPD have minimal, or even no kind of cigarette smoke exposure. And in those cases, we consider other possible risk factors, which include, you know, exposure to other environmental toxins. Some persons have worked in factories or worked in industries where they're exposed to a lot of toxic substances.
Alternatively, people can have genetic risk factors, and that's something we haven't talked about. And one of those that's the most kind of prominent and associated with cigarette smoke exposure is alpha-1 antitrypsin deficiency. But I also see patients, actually, who develop COPD and emphysema because they have underlying what we call matrix disorder, and therefore the structure of their lungs are altered.
And so there are other possible causes, and I would advise anyone who carries a diagnosis, who's a nonsmoker, to be evaluated at a tertiary care institution, where they're able to do this kind of very incisive analysis of what the possible risk factors are, and come up with a likely scenario of what's going on.
CONAN: Barbara in Keokuk, Iowa, emailed us: My husband has alpha-1 antitrypsin deficiency. That's the genetic form of COPD. She writes: It's an often misdiagnosed cause for COPD that occurs in younger patients, and can also involve the liver. It's diagnosed with a simple blood test. I give my husband a weekly IV to replace the protein in his liver, which does not release to counteract an enzyme that cleans the lung.
It's imperative that any person that has a diagnosis of COPD be checked for alpha-1. Bill, have you been checked for that?
BILL: I'm sorry. Can you repeat that?
CONAN: Have you been checked for alpha-1 antitrypsin deficiency?
BILL: It's the first time I've heard of it.
CONAN: Well, maybe that's something you might want to look into.
BILL: I will, and interestingly enough, she mentioned this, my mother had breathing difficulties towards the end of her life, and never was diagnosed with COPD. And I think also another comorbidity was a long-distance runner in the Sacramento environment for 25 years probably didn't help.
CONAN: Well, Bill, good luck. Thank you very much for the phone call.
BILL: Thank you.
CONAN: And Grace Anne, a lot of these conditions are easily detected once there's a test. Getting the test, that's the hard part.
KOPPEL: That was in my case, and I think it is in many cases. Too few general practitioners make use of spirometry, and it's a very simple breathing test. I think it should be administered anytime someone complains, and they're in the right age range - that's over the age of 45 - of shortness of breath, cough, wheeze and difficulty doing things that they easily did before.
And we're trying to find ways to make the test even more simple, so that general practitioners will just routinely do this in their offices. But it is the sad fact that most of us who are finally diagnosed have lost 50 percent, if not more, of our lung function.
CONAN: And you mentioned earlier that it is women who are most often misdiagnosed. Why do you think that is?
KOPPEL: Well, there is a gender bias, because it was for years thought of as an elderly man's disease. Women started smoking heavily in the, oh, '30s, '40s, '50s, and we're seeing the results of that now. But that's just one of the known risk factors. We also are more aware of things like secondhand smoke, third hand smoke, air pollution.
It's interesting that in the Centers for Disease Control's first analysis of behavioral risk factors - and it focused on questions on COPD - that almost 25 percent of those who responded that they had COPD had never smoked. So more and more, we need to explore and are exploring in the COPD gene study, for example, the genetic factors that make us more susceptible.
And we do know women are more susceptible, but we don't know the other factors. Once this massive study is completed, I hope we'll know more. Some of the researchers like Dr. Sonia Buist say that COPD starts in the womb. We're born with certain factors, and then we engage in certain risky behaviors ourselves, and some of us end up with the third-leading killer of Americans as our disease.
CONAN: Here's an email from Anthony Williams: I remember watching my father in a cool hospital room in Seattle with the window open wearing only underwear, with sweat beading on his brow and a look of intense concentration just trying to get the next breath. That is shortness of breath from COPD and emphysema.
Let's get James on the line, James with us from New London in Wisconsin.
CONAN: Go ahead, please.
JAMES: I have COPD, and I recently learned that there is or can be an inherited factor to the disease. I also just recently learned that there is a surgical procedure to help with COPD, and I just underwent that procedure.
CONAN: And how did it go for you?
JAMES: I'm doing great. I'm three months out, and I have improved drastically.
CONAN: Dr. Neptune, I'm sure this is something you're aware of?
NEPTUNE: Yes, there are surgical options. The requirements for undergoing those procedures are very specific. And so it's not most patients, actually, who have COPD that are considered candidates for these. However, if you are - and some of that depends on the distribution of your emphysema, your degree of disability as determined by pulmonary function tests, as well as by exercise studies, and by, you know, the evidence of other what we call co-morbidities or other conditions.
And in a certain fraction of those patients who fulfill those criteria, they're considered good candidates for what we call lung volume reduction surgery. And I imagine that that is the intervention that the caller had. And that's a major thoracic procedure, where you remove the parts of the lungs that are most - in which you see the greatest degree of emphysema.
And that allows the other portions of the lungs that are less involved to expand, and therefore they're able to kind of take over in terms of ventilation, in terms of gas exchange. It's performed in certain centers. It's not a procedure that's done everywhere. And so if a person is a candidate for it, he should definitely get a second opinion. And, you know, if it seems that he might respond to it, then that would be a good choice to proceed with.
CONAN: James, I'm glad it worked out in your case.
JAMES: Yes. There is only a few centers in the country that will do that procedure, but it has been really great for me, and I advise anyone to seek information on that possibility.
CONAN: James, thanks, and continued good luck to you.
JAMES: Thank you.
CONAN: And I wanted to ask you, Dr. Neptune, there is - we're talking about surgery. We're talking about various, well, diet and Grace Anne was talking earlier about diet and exercise and various other treatments. There are so many people who have this disease who don't know they have it. Is it difficult for people to understand this shortness of breath? Why don't the symptoms become more evident?
NEPTUNE: Well, the problem is the lag phase, and that's something that Grace mentioned. And the fact is that when you start smoking, it's going - you're going to have maybe a 20, 30, 40-year lag phase before you start developing symptoms. And so we're largely looking at people who are over the age of 50, 60, 70 years of age.
And unfortunately not just patients, but often physicians think that one's exercise capacity should fall with age. And so when patients complain or report that they're a little more short of breath, they're unable to do activities that they had done before, sometimes that's just attributed to getting older.
And so a problem is on the level of the patients not really telling the doctor, you know, I think I should be able to do this but I'm not, but also on the level of the physician who may not kind of pursue this particular symptom as aggressively as he should.
I should also say that the general recommendation is that any person with a significant smoking history, and that's usually quantified by what we call pack years, and that's the number of years you smoke, and the number of packs per day on the average that you smoke. And if you have a greater than 20 pack year exposure history and you have any symptoms, you should undergo spirometry.
And so I know that sounds a little convoluted for patients. And so for patients I say, if you're a smoker and you have any respiratory symptoms, you should ask your physician if he or she thinks that spirometry would be indicated to make this diagnosis.
CONAN: Dr. Enid Neptune, a lung specialist and a researcher at Johns Hopkins. Also with us, Grace Anne Dorney Koppel, who's got COPD and now national spokesperson for the National Heart, Lung and Blood Institute's campaign to raise awareness of that disease. You're listening to TALK OF THE NATION from NPR News.
And Kim is on the line, with us from Wichita.
KIM: Hi. I'm a respiratory therapist, and I've got 30 years working in the field. One of the things that I run across on a regular basis is patients who are newly diagnosed and they don't realize that the elastic lung tissue that they've lost is never going to grow back. So they've got to change the way they live, which includes quitting smoking.
I also encounter a lot of asthmatics who think, oh, it's just asthma. It's not a big deal. And they don't realize that in addition to the fact that asthma kills thousands of people every year, that over time if it's not treated it causes something called airway remodeling and eventually turns into chronic obstructive pulmonary disease.
CONAN: So that sense of denial that, oh, it'll get better.
KIM: They come in with what we call an exacerbation, and they want us to treat them with medications and IV fluids and steroids and bronchodilators and all of that. And then they get out and they think that they should be just as good as they were like a long time ago.
And it takes a lot of education to convince them that, you know, this is a disease that if it's untreated, it's progressive. And if it's treated, we could just maybe slow it down or stabilize it a little bit, but it's not going to go away.
CONAN: All right. Kim...
KIM: Once you ruin your lung tissue, it's gone. Education is a big key.
CONAN: And Grace Anne Dorney Koppel, that's where you come in.
KOPPEL: Well, I hope so. I'm a board member of the COPD Foundation. I care deeply about getting the message through to people A) that if they have symptoms, they've got to be tested; B) if they know their history is such that they're probably likely to have symptoms, they should ask for a breathing test; and thirdly, once you're diagnosed, don't lose hope...
KOPPEL: ...because I think we have it within ourselves to change our lifestyle, to begin exercising if we're not regular exercisers, and to spread this message. The message is essentially one of hope, because the earlier the diagnosis, the better the prognosis. And for example, I had stopped smoking almost 10 years before I was diagnosed. I didn't realize I was still at risk, but I was.
So I think that people have to realize that when you say the words heart disease or cancer, they fear the disease. I think they have to begin to fear COPD and say what risk factors do I have in my life, whether it's occupation, former smoking, present smoking, or whether there's some genetic possibility if many people in my family or even one other person has the disease.
So I think we have to empower ourselves and try to bring this disease, which is killing 125,000 Americans a year, under control.
CONAN: Kim, thanks very much for the call.
KIM: Thank you.
CONAN: And just a few seconds left, but I did want to ask you, Grace Anne Dorney Koppel, former smokers, most of them, are the people - the risk factors, the risky behavior you talked about. Are there people who blame themselves for their condition?
KOPPEL: Oh, that is so common. And I'll tell you, I am convinced that we don't know more about this disease than we do know, and smoking is clearly the biggest risk factor. But we're only now discovering the susceptibilities that I referred to earlier with the COPDGenes study. And I do think that everyone is at risk. The numbers that I've read from the World Health Organization - and you correct me, Enid, if I'm wrong - is something like 318 million worldwide have COPD. So it's not just a problem in this country, and it is a growing problem because of our poor air quality.
NEPTUNE: And one aspect that we didn't have time to get into is the fact that tobacco companies, many people feel that they're - have been marginalized, but they are very actively kind of encouraging people to smoke and use cigarettes, especially in Third World countries. So that's an expanding population of smokers.
CONAN: Going to be a big problem in China in the years to come. Thank you both very much for your time today. We do appreciate it. Dr. Enid Neptune joined us from WYPR in Baltimore, where she's a lung specialist and researcher at Johns Hopkins; Grace Anne Dorney Koppel joined us from her home in Maryland. She's national spokesperson for the National Heart Lung and Blood Institute's campaign to raise awareness of COPD. Transcript provided by NPR, Copyright NPR.