黑料网

Falk: What started it?

Noone:That鈥檚 an excellent question. If we see small children with these kinds of symptoms, we think genetics鈥攜ou 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鈥檚, 60鈥檚, 70鈥檚 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鈥檛 find a cause in those kinds of people. Sometimes they鈥檝e had a very nasty lung infection that somehow damages their airway and the airway鈥檚 ability to clean itself. Sometimes they have exposure to very noxious agents鈥攇ases, industrial solvents, that kind of thing, but they are a small proportion of people. Most often, it鈥檚 what we call idiopathic鈥攚e simply don鈥檛 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鈥檙e 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鈥檛 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鈥攃ollect the mucus that鈥檚 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鈥檚 an uncommon presenting diagnosis. It must have been called or diagnosed more than likely as something else: 鈥渞ecurrent bronchitis鈥� or 鈥渞ecurrent pneumonia鈥� or some other condition. How does a patient, then, understand that this really is bronchiectasis and they may really need specialized care?

Noone: That鈥檚 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鈥檚 related lung disease, even if they haven鈥檛 smoked. They鈥檙e 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鈥檒l tell you it鈥檚 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鈥檚 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鈥檝e asked me鈥�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, 鈥淕ee, you need to go see a doctor. You may have TB.鈥� That kind of thing, that really bothers people. Sometimes they鈥檝e lost weight and they don鈥檛 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鈥檙e 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鈥檚 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鈥檚 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鈥檙e admitted to the hospital, they鈥檙e 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鈥攖hat鈥檚 the key concept.

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

Noone: That鈥檚 one of the things that we鈥檙e really working hard on now. I have to say. Bronchiectasis is a disease that has flown under the radar screen for many years. It鈥檚 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鈥檚, I think people lost interest in many chronic infections and lung diseases. But it鈥檚 back, we know it鈥檚 out there, we know it鈥檚 common, commoner than appreciated.

So it鈥檚 very important that we harness people into centers like the 黑料网 center here and around the country. We鈥檝e 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 鈥� 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鈥檚 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 鈥� 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鈥檚 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鈥檙e 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鈥檙e part of the group where there鈥檚 activity. There鈥檚 actually interest from professionals, from societies, leading to good research that will ultimately improve their quality of life.

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

Noone: Exactly, yes, the COPD Foundation actually started much of this several years ago. 黑料网 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鈥攂ecause not everybody lives in Chapel Hill and has access to a care center鈥攕o 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 黑料网 Center for Bronchiectasis Care is a recent new phenomenon. It鈥檚 not only comprised of lung doctors 鈥� 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 鈥� I started doing this kind of thing as a fellow with Dr. Knowles, and really you could argue that we鈥檝e 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 黑料网 besides just lung doctors. Many people who have very great expertise at 黑料网 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鈥檝e 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鈥攁ccess 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鈥檛 get a grip on their symptoms, they actually can become housebound. Those of them that are a bit more savvy, they鈥檙e 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鈥檝e gone home. Many patients do strategize like that and have very complete, fulfilling lives, so I certainly don鈥檛 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鈥檚 no cure, appropriately-managed people can have a very fulfilled, excellent quality of life.

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

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