Real NTM Stories

Experts Explain NTM Lung Disease

Chapter 1: Important Facts About NTM Lung Disease

What is NTM?

NTM stands for nontuberculous mycobacteria.

These are not contagious and commonly found in the environment in places like water and soil.

Essentially, we all live around nontuberculous mycobacteria, or NTM. Fortunately, these don't cause disease in the majority of humans, but for patients that are at risk for NTM, these can cause substantial progressive lung disease. MAC stands for Mycobacterium avium complex, and this is the most common NTM lung disease in the United States. It actually accounts for about 80% of what we see in NTM.

Who is at risk of getting NTM lung disease?

Individuals with certain underlying lung disease, diseases like bronchiectasis or dilated floppy bronchial tubes, or other diseases, like asthma or COPD, are often associated with NTM. Other patients that are on drugs which suppress the immune system are also at risk to develop NTM.

Who else might be at risk?

We often see NTM in older populations. It is more common in females rather than in males. We also see other characteristics, like being tall or thin. It is more common in the white population, and common in people with diseases like scoliosis, reflux, or other lung diseases.

Where in the United States is NTM most common?

Location matters with NTM. We know that NTM is living in many water sources—so patients that live around states near the ocean, near large bodies of water, are at higher risk. So we see lots of NTM in states like Hawaii, Florida, certain parts of California, and all along the Gulf Coast.

Why is getting diagnosed so important?

Timely diagnosis is so critical in this patient population because the disease can be progressive over time if left unidentified and untreated. I think it's also really important, too, in patients who have had such chronic symptoms. I think that there's a huge feeling of relief, number 1, and confidence, number 2, that we finally have a diagnosis and something that we can treat and help them feel better.

Chapter 2: Diagnosing NTM Lung Disease

Why does it take time to be diagnosed?

I think that there's a number of reasons why it may take some time before patients get diagnosed. A lot of these patients already have preexisting lung disease and, so, a lot of times, these patients simply attribute their symptoms, or even progression of their symptoms, to their known lung disease, such as COPD or even asthma. I think that's really what can prompt patients to advocate for themselves, or even physicians to look a little more deeply when patients are not improving on standard treatments.

Another factor might include recurrent respiratory infections, in addition to their chronic cough in airway disease. And that is often a red flag for providers, and also patients, to look a little more deeply into what may be going on with them.

What are the symptoms of NTM?

As a consequence of getting this infection, patients can develop a chronic cough, with or without sputum production, fatigue, loss of stamina, shortness of breath, and, in more advanced cases, some patients may have night sweats and fevers.

What symptoms make you think of NTM rather than another lung condition?

In my practice, when I hear a patient say, "Cough, cough, cough. I have a cough that won't go away and I'm extremely, extremely tired." Those are 2 trigger points for me, and I often think, "Could this person be at risk? Could they have NTM lung disease?"

What else do you look for?

Well, I think about the symptoms in terms of cough, the frequency of cough, how much it impacts someone's life, how miserable they are with the cough. I also think about symptoms that persist despite treatment. So, if a patient has been treated for things like reflux and asthma and multiple bouts of bronchitis, I start to think about other things that may be at play and, often, one of those is NTM lung disease.

We do often see patients that have been treated for things like asthma, or COPD, or reflux disease and, oftentimes, what we find that those patients actually have cultures in their sputum positive for NTM.

Chapter 3: Treating NTM Lung Disease

How do you confirm that a person has NTM lung disease?

In addition to symptoms, we need microbiologic confirmation. In other words, we need to see NTM in the sputum itself. And so we usually get at least 2 positive sputum cultures to make the diagnosis of NTM—or sometimes we can do a bronchoscopy, and a sample from a bronchoscopy also suffices.

The third thing that we need in addition to symptoms and positive microbiologic confirmation is radiographs that are consistent with the disease, and this usually comes in the form of a CT scan of the chest or a chest x-ray.

Why do you prescribe more than 1 antibiotic?

The reason that multiple antibiotics are required for the treatment of MAC lung disease is because there really is no single antibiotic that's effective in killing the bacteria. We've learned over time that it really does require multiple antibiotics, taken at the same time, to effectively kill the bacteria.

And not only that, it helps limit the development of resistance while patients are on treatment.

What are the goals of treatment?

The goals of successful treatment are to convert the patient's sputum to negative, and that means that they no longer grow NTM in their sputum—and successful treatment implies that they take 12 months of therapy with negative sputum cultures. Other goals include improvement in radiographs, things like CAT scans or chest x-rays, although that can be complicated in patients with underlying structural lung disease, but also the patient's symptoms, improvement in symptoms is also incredibly important.

What would you tell someone starting antibiotic treatment?

When I'm starting a patient on treatment for NTM lung disease, I really take the time to have a long discussion with them to try to review expectations of treatment. We do take an extensive amount of time reviewing each of the antibiotics individually to review potential side effects, but, more importantly, to try to provide strategies to limit the side effects in the first place and try to give them some control over how they're taking the medications.

I also discuss routine monitoring while they're on therapy, and that includes laboratory monitoring, referral to an ophthalmologist, and also the potential for audiometry.

I think it's really important on starting treatment to be very honest about duration of therapy. You know, unlike other infections that we may treat where they're 5 to 7 days of treatment, this is a much longer treatment duration. And as trite as it may sound, it's more of a marathon than a sprint. So, I'm very up front about treatment duration, explaining to them that you're going to be on treatment at least 12 to 24 months, to set them up for success ahead of time. When patients need treatment for NTM lung disease, I tell them first of all, we can do this. I think it's really important to understand the medications that will be prescribed, the side effects of those medications, and what to expect during and off treatment. While patients are on treatment, they will need frequent doctor visits, usually at a rate of every 2 to 3 months. They will need frequent sputum samples. They will need frequent blood work, at times. And it is important to let the doctor know about any unwanted or untoward side effects that they may experience.

How important is it for people with NTM to take their medicine?

It is critically important for the patient to take the NTM regimen as prescribed by the physician. Dose adjustments may be needed, as well as medication changes along the way, and it is important to let the provider know of any problems taking NTM medications.

What are important questions people should ask their doctors about their treatment?

Important questions that patients should ask their physicians about the treatment of NTM lung disease include what medications will I be on and how long will I be on these medications? What are the potential side effects of these medications?

What type of monitoring will occur while I'm on therapy? And also, how will I know how I'm doing with treatment and how I'm responding to treatment?

Are there doctors who specialize in NTM diagnosis and treatment?

There are different physicians who do specialize in the treatment and diagnosis of NTM lung disease. Oftentimes, a patient may be diagnosed by a pulmonologist, and they may refer their patient to an infectious disease specialist for treatment with antibiotics. But typically these patients are comanaged by both pulmonologists and infectious disease specialists.

Chapter 4: Lifestyle Changes and Resources

What lifestyle changes should patients with NTM lung disease make?

Lifestyle changes with NTM lung disease usually involve, first of all, understanding the disease itself, utilizing things like airway clearance in order to help expectorate and clear out mucus and infection from the lungs. Oftentimes, people also need to undergo dietary changes—focus on nutrition and how important nutrition is to help the body to fight infections—as well as exercise is recommended for every person with NTM lung disease.

How can people learn more and connect with others?

There are a variety of resources now available for NTM patients to better understand their disease process and even connect with other patients who are undergoing similar circumstances. There's online support groups, there's online resources, there's actually blogs and chat rooms that patients can utilize.

Topic and Speaker Introduction

Hello and welcome. I'm Pat Allen. I'm a registered nurse at New York Presbyterian Hospital in the pulmonary division. We're here today to talk to you about a very serious lung condition called nontuberculous mycobacterial lung disease, also known as NTM. The event is being hosted in recognition of rare disease day to raise awareness of an often overlooked condition, such as NTM lung disease. NTM lung disease is often misdiagnosed because its symptoms are very similar to other more common lung conditions, such as chronic obstructive pulmonary disease, bronchiectasis, and asthma. I'm joined today by Dr. Doreen Addrizzo-Harris, Pulmonologist, and professor of medicine at New York University, Langone Health, and Darielle, a person who's been affected directly by NTM lung disease. We will be discussing signs and symptoms, risk factors, tips for talking to your doctor and much more. Please note, we will not be talking about specific treatments today. Dr. Addrizzo-Harris, could you tell us a little bit about yourself? Hello, I'm Doreen Addrizzo-Harris. I'm a professor of medicine and a pulmonologist at New York University, Langone Health. I've been practicing there for over 20 years, and I currently, direct our bronchiectasis and NTM center. I am with Darielle, one of my patients who has been living with NTM lung disease

Okay. Let me tell you a little bit about myself. I'm retired. My husband and I have been married for 48 years. We have two beautiful daughters and two terrific granddaughters. As far as my NTM lung disease, I was diagnosed more than three years ago in October of 2015, but my journey with my condition began several years before then. Since being diagnosed and through the help of different pulmonologists, I've gotten a better understanding of my lung disease, which in turn has helped me to manage my symptoms.

About NTM Lung Disease

Dr. Addrizzo-Harris, could you explain a little bit about NTM lung disease?

Sure. NTM lung disease is a disease of the lungs that's caused by a common bacterium that we find in the soil and the water. We are exposed to it pretty much every day. However, most patients, most people will not come down with NTM lung disease. It mostly affects patients with preexisting lung diseases, such as bronchiectasis or COPD. There are, multiple causes of NTM lung disease. Um, however, 80% of the cases are caused by Mycobacterium AVM complex, otherwise known as MAC.

Why is it important for people to be aware of NTM lung disease?

NTM lung disease is considered a rare disease. However, it's becoming more common. It's growing 8% each year. In 2018, last year, it was estimated that 75,000 to 105,000 patients were diagnosed with NTM lung disease just in the United States. Symptoms can be, cough fatigue or shortness of breath, and in some cases, an NTM infection can cause severe even permanent damage to the lungs.

Darielle, you said you were diagnosed with this NTM lung disease. Had you heard about it before your diagnosis?

No, I never heard about it before my diagnosis. I first heard about NTM lung disease and bronchiectasis in 2009 when I first saw a pulmonologist. The doctor suspected that I had disease because, some chest discomfort I was having and an abnormal chest cat scan of my lungs. But it wasn't until years later, as we mentioned earlier, that I was diagnosed. NTM lung disease is one of the conditions that not everyone knows about, but it's important that we are talking about it today.

Risk Factors and Symptoms

Doctor, you mentioned that there were certain people who were more at risk for this condition. What are the risk factors?

So, risk factors include, having underlying lung disease such as chronic obstructive pulmonary disease, as you said earlier or otherwise, COPD or having bronchiectasis as in Darielle’s case, those patients at more are more at risk. In COPD patients, you're at 16 times more likely to have NTM lung disease. And in patients with bronchiectasis, about 50% of them have NTM lung disease. If you're older, older than 65, we see an increased risk and that population is increasing. If you have a weak immune system, if you happen to be on chemotherapy or other immune suppressing drugs, it's more common in women than men. And we also know that it's more common if you live in a state that's in a coastal area, seven out of 10 cases come from those states.

Once you're infected by the bacteria doctor, what symptoms would a person have?

So the first thing to note is that not everyone who's infected with NTM lung disease will have symptoms. Many patients can go months, even years without symptoms, and sometimes patients are diagnosed when they're having a chest CAT scan for another reason. Okay. Patients often will develop symptoms, or they will have a common cold that doesn't go away, that brings them to their doctor asking why isn't this cold going away? And those symptoms are the common pulmonary symptoms such as cough, sputum, production, fatigue. They can have weight loss, they can have lack of appetite, low grade fevers, night sweats. They can just have recurrent infections that aren't going away with routine antibiotics. And that may alert them that maybe they should be asking someone about NTM lung disease.

So when you say sputum, you mean they get more phlegm?

Yes. Okay. They get more phlegm.

Darielle, tell us a little bit about the symptoms you experienced that brought you to this point.

Well, I only experienced a few of these symptoms. As I said earlier, I started with a discomfort in my chest and then I had repeated respiratory infections with a non-productive dry cough over the course of the next several months. When I saw my pulmonologist, he initially thought I had organizing pneumonia, but after some additional tests, he suspected that my GERD and my bronchiectasis were contributing factors to the symptoms I was having. When one of the CAT scans I had, had some significant changes, it gave me some concern. So after speaking and consulting with my family and getting their support, I decided to seek some more opinions, on my disease, and my diagnosis was confirmed in 2015.

Conversations with your Doctor

I'm understanding that symptoms worsen over time. Many, many people who were affected with symptoms and respiratory are cared for by primary care physicians. What is your recommendation? If someone's experiencing symptoms like you've described and they're concerned, what should they do and who should they see?

So if you think you might have NTM lung disease or that a loved one might have it, or if you're having chronic pulmonary symptoms, the first thing to do is to go to your primary care doctor and ask them to refer you to a specialist in pulmonary disease. Someone who knows the lung and can treat lung infections. There may be pulmonologists in your area that are also specialists in NTM lung disease and things like bronchiectasis, in certain parts of the country, infectious disease specialists may be able to treat NTM lung disease.

Darielle, what kind of doctors did you see when you were developed these symptoms?

Well, I initially saw my primary care physician, who referred me to a pulmonologist. I ended up seeing four pulmonologists and all. Dr. Addrizzo-Harris was one of them. Since I've seen her, she referred me to a gastroenterologist, who specializes in treating people with gerd. And he was able to narrow in on medications that would help with the gerd symptoms I was experiencing. Even if you've received a diagnosis, it's important that you, continue discussions with your doctors in case you have ongoing symptoms, or your CAT scans are changing.

This is a chronic illness. It requires, as you've both said, real lifestyle changes. As a person who's a patient with this chronic illness, tell us a little bit about how you communicate to your physician, your concerns and how you've learned to manage your illness.

When I first heard I had NTM lung disease and I reviewed the abnormal CAT scan from my first one, I had a lot of concern. I was a little taken back by it, and I had a lot of questions. A few of the questions I asked were, how did I get the disease? Is the disease hereditary? I knew I was going to be looking at possible CAT scans in the future, and I wanted to know how frequently they would be done, because I was concerned with some of the radiation that you hear about. But I was assured that the radiation is low dose. My biggest advice to people and to our viewers is to be your own advocate. The disease is often misdiagnosed or not diagnosed at all. So you have to be proactive.

You have to sit down and talk with your doctor. You need to ask a lot of questions and, take notes. If you feel you need somebody with you, have somebody go with you. It's a disease that's going to be ongoing. You will have periods where you feel worse and it's, it's important you keep an ongoing relationship with your doctor. I sought out three different opinions in my situation until I got a final DI diagnosis, probably about three years ago. I decided I wanted to start seeing somebody closer to home. So I did some research online and I found out that NYU had specialists who treat NTM lung disease and treat bronchiectasis, which led me to seeing Dr. Addrizzo-Harris. So I've now been seeing her since 2016.

Dr. Harris, what would be your recommendation to people that are watching us today in terms of how to manage? My recommendation is to talk to your current physician. If you're having chronic pulmonary symptoms, or if you feel like you might have NTM lung disease, you need to be referred to a specialist who knows about NTM lung disease. So my recommendation is to ask your primary care doctor to refer you to a pulmonologist. And if in your area, there may be pulmonologists who specialize in NTM lung disease, then you might consider going to see one of them. So that's extremely important that you're dealing with someone who knows about NTM lung disease because it is a rare disease.

Testing and Addressing NTM Lung Disease

Doctor, if a doctor suspects or a physician suspects a patient they're caring for, has NTM lung disease, is there something they can do to test for it? And what can they do to treat it?

Well, in addition to a thorough physical exam and a good medical history, in order to diagnose NTM lung disease, you need to have sputum cultures sent to the lab to identify the bacteria. And often that has to be multiple sputum cultures because it doesn't always grow the first time. In addition, you need to have, as Darielle did, a chest cat scan. Often chest x-rays are not sufficient to diagnose this disease. You asked me about treating the disease? right. If you are diagnosed with NTM lung disease, you'll want to speak to your doctor about treatment. You want to make sure you're with someone who is comfortable treating NTM lung disease. You will start with, working on managing the mucus. Most patients have increased mucus. Airway clearance is one of the parts of therapy, and there are a number of ways we can help patients with airway clearance. By giving them some medications to inhale and we give them some devices to help increase their cough. Those things will help with clearing the mucus. And then often patients will need to go on treatment. Unfortunately for treatment in this disease, it requires multiple antibiotics. We use a guideline to help us determine which antibiotics should be used in which patients. But, antibiotics usually have to be continued for 12 to 18 months.

Healthy Lifestyle Changes and Reducing NTM Exposure

So this is a chronic illness? Yeah. And it requires some lifestyle changes, I would think, on a patient's product. Could you talk a little bit about that?

Absolutely. In addition to the airway clearance and the antibiotics, if a patient is on them, we recommend a daily exercise. Exercise is a great way to help with airway clearance, a healthy, balanced diet. And of course, we recommend that patients don't smoke if they are smoking. Since this bacterium is very common in the environment, we recommend lifestyle changes that involve the environment. That may mean increasing their hot water heaters to higher temperatures to 130 degrees to kill the bacteria more efficiently, may involve reducing the number of showers they take. It may involve changing their shower heads so that they use ones with larger holes, so there's less aerosolization of the water. We recommend, changing your water filters based on the manufacturer's instructions. We recommend avoiding humidifiers if at all possible. And we often recommend boiling water for more than 20 minutes, and then using that water to drink in order to kill any mycobacteria that will be in the water. So there are a number of things that we recommend that patients do on a daily basis.

Darielle, tell us a little bit about the changes you've made in your lifestyles and how this NTM lung disease has affected your life. Well, I've made several changes, and after following up with my pulmonologist, they implemented treatment plans to better manage my symptoms. And these did, like Dr. Addrizzo-Harris said, include airway clearance and medications, and I’m soon to start some cardiopulmonary rehab. The other lifestyle changes I've made are diet as Dr. Addrizzo-Harris mentioned. I try to follow a well-planned diet, avoid certain foods that they restrict on, and avoid certain liquids. I carry my own bottled water wherever I go. I try not to even go to restaurants. I cook with purified water. I also eat my largest meal earlier in the evening and try not to lie down after it. I also sleep at the head of my bed elevated. I do, as Dr. Addrizzo-Harris said, take shorter showers. I shower every other day and I have now switched to washing my hair in the sink instead of the shower because as she has mentioned, the bacteria can be found in shower heads. I do very little gardening, but, if I do work outside with some plants and flowers, I wear a mask and I try to make sure that the soil is moist so I don't inhale any of the dust from the dry soil.

Patient Resources

The other thing is to educate yourself, there are some very good websites. You can go to like aboutntm.com to start gathering information, make a list of questions for your doctor. There should be an interaction between you and your doctor. Doctors like to sit with you and answer your questions. It's a very important part of my relationship with Darielle, and I think it helped us get to the final diagnosis knowing that she had NTM lung disease. So that would be my recommendation. And then also looking for patient advocacy groups. In New York City, there are a number, so we refer our patients to a number of the different groups here. Ask your physician what groups are in your area. You can look online to find websites as to where there are advocacy groups.