image2Peadar Noone, MD / Photo by Chris Polydoroff
Dr. Peadar Noone introduces us to the UNC Center for Bronchiectasis Care, which recently opened in Meadowmont in Chapel Hill, NC. Dr. Noone is a Professor of Medicine in the Division of Pulmonary & Critical Care Medicine, and he is Medical Director for this new center.

“Many times, patients ask me…What is this strange disease, bronchiectasis? What does it mean for me? Is it going to limit my life? Can you cure it? And if not cure, what kinds of treatments can you give me? Is there anything that will improve my quality of life?

-Dr. Peadar Noone

 

Falk: Hello, this is Ron Falk for the Department of Medicine at the University of North Carolina. Welcome to the Chair’s Corner.

Today, we welcome Dr. Peadar Noone who is a Professor of Medicine in our Pulmonary division, and is the Medical Director for the UNC Center for Bronchiectasis Care. This is a brand-new center that specifically focuses on the diagnosis and treatment of bronchiectasis and mycobacterial lung infections, which we will be discussing today. Welcome, Dr. Noone.

Noone: Thank you very much, Dr. Falk.

Falk: So what on earth is this word “bronchiectasis”? What does it mean?

Noone: That’s a question I get very often in the clinic. It’s a disease that is characterized by abnormalities in the airway of patients with chronic lung disease. Classically, it is associated with chronic cough, productive of fair amounts of sputum, and it’s often associated with quality of life issues. The cough interrupts people’s ability to go to church, to go to restaurants, be in company, even on public transport, because it attracts a lot of attention. Sputum production is bothersome, obviously. Then it’s often interspersed with periods of stability, punctuated by periods of instability—what we call exacerbations or flare-ups that require treatment.

Falk: The word “bronchiectasis”-let’s talk about that for a minute. In your lung you have lots of bronchi..what’s the “-ectasis” in “bronchiectasis?”

Noone: That’s a great question, because many people confuse it with bronchitis, which is kind of similar-but different. Bronchitis is inflammation of the airways, typically associated with infections.

Falk: So you’re saying “airways” instead of “bronchi” – help with that differentiation.

Noone: Airways are the tissues that help us get air into our lungs, and the interchange of blood gases, carbon dioxide occurs at the end of the airway. But the airway’s an important conducting organ to get the air in and out. So inflammation and problems in the airways, obviously can lead to lots of symptoms.

Falk: When you take a breath in, the air goes down your treachea, and then what goes after the trachea?

Noone: Larger bronchi, smaller bronchi–airways, and then tiny sort of microscopic airways which lead to the air sacs, also known as alveoli.

Falk: Bronchiectasis involves that portion of the airway, are any of those bronchi?

Noone: Generally not the trachea or the very large airways, but after that, yes, it can involve any portion and can cause inflammation. The airway loses its nice tapering quality, which is critical for the normal function of the airway to clear the sort of thing we breathe in and out every day. We’re exposed to air particles, bacteria, dust, pollen..It’s obviously critical that our airways have the ability to clear themselves. We have a very good automatic mechanism for clearing the airways in a healthy lung. If that gets disrupted in any way, it can lead to abnormalities and disease and then the airway loses its ability to clean itself. It gets sort of sludgey, bacteria take hold, and then bronchiectasis may ensue.

Falk: So the differentiation of the word bronchitis, inflammation of bronchi. In contrast to bronchiectasis what’s the different characteristic in the lung if you could zoom in, into a bronchus? How would you describe that?

Noone: The way I describe it to patients is if you imagine your airway is like the branches of a tree with large branches tapering to small branches, and now envisage that there are abnormalities in those branches that lead to pockets where pus and mucus can build up. If it was a river there would be eddies and currents and sometimes those enlarge and you get ponds and pools that become relatively stagnant. Bacteria love that environment. The airway doesn’t clean itself very well in those areas. So you get dilated or enlarged airways in those pockets which can vary from smaller pockets in smaller airways to quite larger pockets in larger airways, sometimes leading to what we call cavities—very large pockets of pus, mucus, infection.

Falk: Right. So those pockets, then, of infection, and the pooling of normal mucus is what is coughed up and causes the symptoms that you so beautifully described early on. Is that correct?

Noone: Yes. The bronchiectasis entity leads to stagnation, and of course we’re all exposed to environmental bacteria—germs, and if those germs take root in the lung, in that environment of stagnation, they can proliferate and cause further damage, so you get into this cycle of damage, inflammation, infection with germs causing more damage and inflammation, and it goes round and round. Ultimately it can damage the lung and the airways quite significantly.

Falk: What started it?

Noone:That’s an excellent question. If we see small children with these kinds of symptoms, we think genetics—you inherited something from your parents. Cystic fibrosis is a classic kind of disease that we think of in small children. Or younger adults with this kind of disease, they often have life-long symptoms since birth. There are other kinds of genetic diseases that predisposes one to bronchiectasis.

Typically in my Bronchiectasis Center clinic, though, I see older people, typically in their 50’s, 60’s, 70’s who have really had no symptoms for most of their childhood or early adulthood. Then they suddenly develop these symptoms of cough, sputum, production, flare-ups requiring antibiotics repeatedly, particularly over winter periods. Often we don’t find a cause in those kinds of people. Sometimes they’ve had a very nasty lung infection that somehow damages their airway and the airway’s ability to clean itself. Sometimes they have exposure to very noxious agents—gases, industrial solvents, that kind of thing, but they are a small proportion of people. Most often, it’s what we call idiopathic—we simply don’t ever find a cause.

Falk: In the naming of your new center, you include mycobacterial lung infections. Does that imply that some of these patients may have tuberculosis, or other non-tuberculous kinds of bronchiectasis, or are those two entities separable?

Noone: Mycobacteria are a family of organisms of which tuberculosis is one member, so there is some overlap in similarity of symptoms and the way patients present, but they are different bacteria completely in the sense that tuberculosis is very communicable between even healthy humans.

Mycobacteria are more environmental organisms that generally are not that easy to acquire in our lung to cause actual overt infection. People with chronic lung disease who have injured lungs, particularly those people with bronchiectasis, they’re more prone to acquire these organisms and then those organisms can further add insult to injury, further, worse than the bronchiectasis.

There are some people, who, for reasons that aren’t completely clear, can acquire mycobacteria even with apparently healthy lungs prior to acquiring the infection. Often their symptoms match those of bronchiectasis patients, with cough, weight loss, fevers, night sweats, that kind of thing. Generally a chest x-ray or ct-scan will pick up changes consistent with either bronchiectasis and/or mycobacteria. Generally we try to gather sputum—collect the mucus that’s expectorated from the lung to see what bacteria are there and we particularly look for mycobacteria.

The diagnosis is important, because the treatment is quite different for mycobacteria than for standards kinds of germs.

Falk: How does somebody know that they have bronchiectasis? It’s an uncommon presenting diagnosis. It must have been called or diagnosed more than likely as something else: “recurrent bronchitis” or “recurrent pneumonia” or some other condition. How does a patient, then, understand that this really is bronchiectasis and they may really need specialized care?

Noone: That’s a key question, actually. Many of these patients, because of the vagueness and the chronicity of the symptoms, sometimes building up over years, rather than weeks or months, are labeled as having COPD, smoker’s related lung disease, even if they haven’t smoked. They’re often mislabeled as having asthma because, of course, they wheeze and cough much like patients with asthma do.

But the key concept is, these patients have typically years-worth of symptoms and they produce typically more sputum, more mucus from their lungs, than your average patient with COPD or asthma. Generally they bring up a lot of green stuff from their lungs, and if you ask them to describe the volume, they’ll tell you it’s maybe a half cup full per day, sometimes interspersed with blood, which is another key symptom which typically people with asthma and COPD do not experience.

Falk: The diagnostic tests that you may do, kinds of treatments that you may prescribe–give us a glimmer of what kinds of things you do in your center.

Noone: Well, you know, we do quite old-fashioned things, which I teach my medical students. Good history is critical: How much do you cough? How much do you cough up in an average day? How long have you been coughing? When did symptoms start? A careful history, in other words. That can often really open the door for the key diagnostic tests that we might want to do.

Physical exam can be relatively non-specific. There are certainly some things that we look for on a physical exam. If I was to pick out one or two tests that are really important for the diagnosis, I would say gather sputum, send it to our laboratory for bacteria and germ growth, various stains, and so on. And a CAT scan. A CAT scan really has superceded, actually, all other tests that were done prior to this, because we have such an exquisitely good picture of the inside of one’s lungs, particularly the airways and those pockets that I described earlier.

Falk: When a patient first comes to this bronchiectasis center now at Meadowmont, what kinds of questions do they ask you?

Noone: You know, many times, patients ask me many of the same things you’ve asked me—What is this strange disease, bronchiectasis? What does it mean for me? Is it going to limit my life? Can you cure it? And if not cure, what kinds of treatments can you give me? Is there anything that will improve my quality of life? I would like to be able to sing in the choir. I would like to be able to go on the subway without people saying to me, “Gee, you need to go see a doctor. You may have TB.” That kind of thing, that really bothers people. Sometimes they’ve lost weight and they don’t feel very well, which leads to depression and that kind of thing. So, often vague sorts of symptoms really punish these patients to the point where they’re desperate for, if not a cure, at least something that will help them tolerate their life better.

Falk: Can you cure bronchiectasis?

Noone: For most patients there is no cure. Very rarely we can ask our surgeons to take out a particularly bad area of bronchiectasis lung, but for the most part, the lungs are affected in a general kind of way and it’s not possible to operate, but we certainly have very good management programs to control symptoms, improve quality of life, decrease what we call those exacerbations, or flare-ups, which really are the things that destroy people’s quality of life. Needing antibiotics several times a year is no picnic, and often people experience side effects from medications and so on. So if we can reduce the number of flare-ups, and more importantly reduce the number of hospitalizations, because many times these people end up in emergency rooms, they’re admitted to the hospital, they’re patched up, so to speak, for a few days, sent home without a rigorous future plan of care. So trying to prevent that and reduce those occasions when people feel really terrible—that’s the key concept.

Falk: Are there FDA-approved drugs specific for bronchiectasis?

Noone: That’s one of the things that we’re really working hard on now. I have to say. Bronchiectasis is a disease that has flown under the radar screen for many years. It’s sort of come back to the fore, so to speak, and has garnered the attention of clinicians like myself around the country and the world, to look at this disease that we once thought was cured. After antibiotics were developed in the 1950’s, I think people lost interest in many chronic infections and lung diseases. But it’s back, we know it’s out there, we know it’s common, commoner than appreciated.

So it’s very important that we harness people into centers like the UNC center here and around the country. We’ve established a registry, to capture patients into the registry with their consent, so then we can do good research studies, particularly looking at new medicines to try to combat this disease and obviously FDA approval is important, because prescribing medicines is one thing, but getting the patient the medicine, through various hurdles such as insurance is very important. FDA approval is one of those things that improves access to good care and good medicines.

Falk: You collaborate with the Bronchiectasis and NTM Initiative – NTM meaning non-tuberculous mycobacteria, that organization sponsors wonderful research and a registry for patients with these conditions. Can you tell us a little bit about this initiative, who should join, and what sort of studies are coming out from there?

Noone: That’s a wonderful national initiative that was started several years ago mainly by patient advocates who had the disease themselves, particularly the mycobacterial lung infection spectrum. If we think bronchiectasis is a strange term, you can imagine your average lay person hearing about nontuberculous mycobacteria – MAC which is a particular germ in that family, they virtually know nothing about that disease and those infections. So having access to informative web sites and patient advocacy groups where they can become informed, and they can feel that they are not alone, which is very important. People feel quite alone with these kinds of diseases, because it’s not the sort of thing that their next-door neighbor is likely to have.

Obviously that then leads to collaboration with industry, with professionals like myself and colleagues which is how the registry got started. We’re trying to collect all of the patients around the country who have either the diagnosis of mycobacteria or bronchiectasis. That leads to good access to research studies.

From the patient perspective, I think the most important thing is being able to feel that they’re part of the group where there’s activity. There’s actually interest from professionals, from societies, leading to good research that will ultimately improve their quality of life.

Falk: There’s a social support network called Bronch and NTM 360 Social. That’s a social network that’s really aimed at doing just exactly what you described.

Noone: Exactly, yes, the COPD Foundation actually started much of this several years ago. UNC was a founding member in the registry which sprang from that and received early funding from that initiative. A more recent development is the Bronch 360 which is a sort of a blog web site where patients can participate, questions and answers, gain resources locally—because not everybody lives in Chapel Hill and has access to a care center—so they can see where the resources are locally or maybe regionally and liaise with their own care provider to establish best practice of care for those patients.

Falk: This new center, the UNC Center for Bronchiectasis Care is a recent new phenomenon. It’s not only comprised of lung doctors – pulmonary doctors. There are a number of other kinds of disciplines that are available for a patient. Could you describe some of those other services that a patient could expect to have?

Noone: One of the reasons that we created the site is that we have been doing this for many years – I started doing this kind of thing as a fellow with Dr. Knowles, and really you could argue that we’ve been doing this, why create a center? Increased visibility, and better access to care, prompted me to create this center but also I thought it would be a good idea to harness the expertise of other people at UNC besides just lung doctors. Many people who have very great expertise at UNC have agreed to help us manage this center. For example, Ear, Nose and Throat doctors have agreed to help us and expedite care as best as possible, because many of these patients have sinus disease, nose disease, ear disease. I’ve enlisted the help of an infectious disease doctor, because managing these germs, managing the antibiotics, the complex regime of treatments, is not trivial. And many other experts. Radiologists, for example. Sometimes we really have to pore over a CAT scan to find very specific areas of disease, for example, those that might be amenable to surgery—access to a good chest radiologist is so important.

Falk: Let me ask you one last patient-focused question. This is a chronic condition. This is a condition that, as you describe as socially debilitating. How do patients cope with this disease? What strategies have you learned from listening to patients?

Noone: It depends whether their disease is managed appropriately or not. There are some patients, who if they really don’t get a grip on their symptoms, they actually can become housebound. Those of them that are a bit more savvy, they’re on good treatments, often do the best they can to clear their lungs, clear their airways in the morning, do all their treatments, so that they can have a relatively symptom-free day and then refocus on all their treatments again in the evening when they’ve gone home. Many patients do strategize like that and have very complete, fulfilling lives, so I certainly don’t want to end on the note of a house-bound patient. There are many patients who work, get married, live lives, travel, enjoy vacations, so while there’s no cure, appropriately-managed people can have a very fulfilled, excellent quality of life.

Falk: That’s wonderful. Thank you so much for spending time with me today.

Noone: Thank you so much for the opportunity, Dr. Falk.

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Visit these sites for more information on bronchiectasis:

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