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Jeffrey Swigris, DO: Welcome to Medscape InDiscussion on the topic of idiopathic pulmonary fibrosis, or IPF. I'm Dr Jeff Swigris, and today we'll be discussing the long-term follow-up of patients with IPF and how remote monitoring may play a role. How and how often should we follow patients? Can we or should we do some of it remotely? If so, what might that look like? With me to delve into these questions is Dr Marlies Wijsenbeek, a pulmonary physician at the Erasmus University Medical Center in Rotterdam, the Netherlands, and a world-class expert in IPF. She is also chair of the Erasmus University Medical Center Multidisciplinary ILD (Interstitial Lung Disease) Center. Her research interests include patient-centered outcome measures in interstitial lung disease, e-health, and new therapies in IPF and sarcoidosis. She's the perfect guest for today's topic. I am so happy to have you here, Marlies. Welcome.
Marlies Wijsenbeek, MD, PhD: Thank you. I'm very happy to be here and excited to talk about this topic.
Swigris: Marlies, tell us how you got interested in interstitial lung disease and IPF.
Wijsenbeek: In 2007, I started creating an ILD center here because that was a time when we really did not have much to offer people with IPF and with older forms of progressive fibrosis. It was mostly supportive care, so there was a major unmet need for these patients, and there was also a major need for better therapies. The field has changed in the past decade. We have antifibrotic drugs that slow down disease decline and improve outcomes for patients. But we should remember that IPF remains a deadly disease, and support is still very much needed for these patients. So, to answer your question, I [got interested in interstitial lung disease and IPF] to help people live as long and as well as possible, do it with a team, and do research to improve the field.
Swigris: Let's talk about how we follow these patients and try to help them live as long and as well as possible. Okay, Marlies, you've just made the diagnosis of IPF in a typical patient. This is a 67-year-old gentleman — a former 30–pack-year smoker. He's had 12-18 months of this insidious shortness of breath upon exertion. You diagnose IPF. How are you going to follow him over time? What's your approach to testing? What do you do and why do you do it?
Wijsenbeek: This is something we all see in our practice in our country. We agreed in the national guideline to see these patients every 3-4 months, which includes pulmonary function tests such as FVC and Dlco at the minimum. Furthermore, we must consider whether patients are just starting on medication that will also involve labs. Then normally, our specialist nurses will call after about 4 weeks to see how it's going with the medication and how the patient's tolerance is. HRCT scans and 6-minute walk tests are performed based mostly on the indication, and nowadays we also offer remote monitoring to patients.
Swigris: That's great. So, to clarify, FVC is forced vital capacity or lung capacity. Dlco is diffusing capacity of the lung for carbon monoxide. HRCT is a high-resolution CT scan. How frequently will you do scans on patients, Marlies? Do you do it as a routine at certain intervals, or do you let the clinical scenario dictate? What's your approach?
Wijsenbeek: In patients with IPF, I usually let the clinical scenario dictate. If there is any unexpected change in a situation and I want, for instance, to exclude a pulmonary embolism if there is a very rapid progression for which I have no explanation, I would order an HRCT. If there is suspicion of a malignancy, I would make certain that an HRCT is performed. We don't standardly do HRCTs, but in practice it ends up that each patient usually gets a CT scan about every 1-2 years.
Swigris: You mentioned remote monitoring. Take us through that. I know you've built a remote monitoring program there in the Netherlands, and I would love to hear more about it. How do you do it? How is it operationalized and so forth?
Wijsenbeek: Now, this already started in 2015 when we saw how much patients struggled to come to the hospital. They had to take oxygen. Their family had to take days off from work to bring them. We thought that maybe this could be done more easily, especially if everything with the patient is stable. So, together with our patients, we developed a system that includes home spirometry. The patients have a small device that measures lung function at home, which sends it via Bluetooth automatically to the hospital. They also keep track of their symptoms, side effects, and health-related quality of life. They have sort of a chatbot where they can send messages to the nurse, and we can also do video consultations if we want. Patients especially like the medication coach a lot because if they rate side effects on a certain level, it gives them a pop-up on how they could deal with these side effects. It asks them afterward whether it helps enough. And if not, the nurses receive a message.
Swigris: So, patients will use their iPad or smart device — whatever it is. A cell phone, perhaps. How frequently are you asking them to do spirometry?
Wijsenbeek: In the beginning we started with daily use because we were learning, but that turned out to be too much. Most patients do it weekly, but some patients keep doing it daily because they like it and others do it monthly. Outside of the research setting, we leave it a bit up to the patients, but we advise three blows once a week, and then it selects the best blow, like in clinic.
Swigris: Across the world, the pandemic has made us think about remote monitoring. Here in the states, it's making its way into our care. If you have patients doing this at home — they're filling out questionnaires on their smart devices and they're doing spirometry — could you share with us how frequently you are looking at their data?
Wijsenbeek: That's a very good question because now we are only looking at the data if there are problems. So, if a patient sends us images or calls us up about a problem, we look at the data. We also look at the data if we have a scheduled remote visit. Then I look back at how the data were in the past month. I'd rather have several measurements because we do see some variance at home. If you have several measurements, you see nice trends; you don't have a technician sitting there and coaching the patients. You have a bit more variability. In studies we used alarms, but that creates quite a lot of work for us because you must then filter out older measurement variations, the grandchild who blew on the machine, or things like that. Nowadays, I only look at the data when patients call for problems or when I have a consultation with them. Obviously they don't complete the questionnaires every week; they complete them at greater intervals.
Swigris: So if a patient is at home and they seem to be doing okay and they're tolerating their medication, you might have a follow-up at 4 months. Then, do you try to bring the patient into your clinic on a routine basis, or are they just monitored at home and you're happy with that?
Wijsenbeek: I think COVID made a big shift here. In the COVID times, we started up with a little bit over 300 patients countrywide, and we saw that we replaced about 50% of in-hospital visits with remote care — obviously forced by COVID. We see that this has gone down a bit now again, but we're going to start an implementation program here to see if you can replace half of the visits with remote monitoring safely and with patient and doctor satisfaction. In the end, we don't want patients to travel to us and have a lot of hurdles to jump if they're stable and well. But if they need us, they should come in even earlier. That is the balance you would like to find: You create space in your clinics for patients who need you at that moment, and the patients who are stable don't need to travel to you.
Swigris: Right. I think that's a great setup. Let's shift gears a little bit and stick with our theme of longitudinal surveillance. There are a handful of comorbid or related conditions in our patients with IPF that we all think about. I don't think there's consensus on when and how or even whether we should look for these things. What I'm talking about are gastroesophageal reflux or GERD, obstructive sleep apnea, and pulmonary hypertension. These three comorbid, or related, conditions to IPF have a bunch of interesting data behind them on whether they may in some way be related to disease progression. We know that each of them is relatively common in patients with IPF. Give us your thoughts on whether you check for them, how you check for them, how frequently, and the like.
Wijsenbeek: Yes, it starts at the first consultation. We ask, for instance, about gastroesophageal reflux. We don't do all kinds of investigations; we're pragmatic about that, except for the patients who go for transplant who undergo a more rigorous workup. In the average 67-year-old male you just talked about, we'll just ask about reflux. If there's reflux, I would treat it. If there are no signs or symptoms of reflux reported, I will not treat it. It's also according to the guideline not to treat reflux for the sake of the pulmonary fibrosis. For obstructive sleep apnea, we also ask about it or use questionnaires because I think it is important to detect. It will improve quality of life for patients if you treat it. Whether this will also make them live longer in the long term, we don't know yet. But improving quality of life is important. As for pulmonary hypertension, that's tricky. It's good to realize the setting in which you are. In the Netherlands, we don't have approved drugs for the treatment of ILD-related pulmonary hypertension. We do screen for it if we have an unexplained decrease in Dlco, especially, or in lung function. We also screen for it in case of transplant. But often we don't have much more to offer to patients than conservative and supportive measures for pulmonary hypertension. So, it doesn't have that many consequences. We always think about the consequences of screening, and if we screen, we usually screen with ultrasound, except for the transplant patients.
Swigris: Let me just clarify. Marlies brought up a great point about gastroesophageal reflux. You know, there was a time when many people thought, "Wow, gastroesophageal reflux. All the way up to the larynx, and then with microaspiration, it could be one thing that causes progression of IPF." With those very weak observational and retrospective data, many of us said, "We're going to put all of our patients on antacid therapy. Any patient with IPF should automatically be on a proton pump inhibitor with the intent to slow disease progression." The thinking was that if we decrease the acid content of the reflux, even if stuff is still getting down into the lungs through the trachea via microaspiration, it's probably better that it's not acidic. But the most recent treatment guidelines that just came out in 2022 suggest that this is not appropriate. So, using a proton pump inhibitor with the intent to slow disease progression in IPF is no longer warranted and is not considered standard of care anymore.
Marlies, you also mentioned the 6-minute walk test. I wrangle with this all the time because it's seemingly a simple test. You tell a patient, "Walk as far or as fast as you can in 6 minutes. Don't run or jog. If you need to slow down, slow down. If you need to stop and rest, stop and rest. We're going to keep the clock going." Pretty simple. But it's become so convoluted whether we measure oxygen saturation or not and what we do with those data. At your center, are you measuring peripheral oxygen saturation during a 6-minute walk test? Do you have stopping rules?
Wijsenbeek: Yes, we do measure it. We have a sensor that goes on the forehead, which sort of reliably measures peripheral oxygen saturation. I mostly do these tests to see if a patient desaturates on exercise. I don't care if it is completely reliable with the number of meters. You use it except in patients who are in trials or go for transplants. For the other patients, the important thing is whether they desaturate on exercise and whether you could give ambulatory oxygen for that to improve their breathlessness and probably their exercise tolerance. This has been shown in a very nice study from a group in the United Kingdom, which reported that patients who desaturated on exercise below a saturation of 88% actually benefited from ambulatory oxygen use. So, that is the approach I take.
Swigris: How about in terms of remote monitoring of oxygen saturation or oxygen needs? Do you incorporate that into your remote monitoring? If so, how?
Wijsenbeek: Yes, we started doing that during COVID because it became so relevant when patients had COVID. But we must acknowledge that we don't have very good data on which is the best pulse oximeter and how to do it. You must at least explain to your patients that you need a good signal — warm hands and see a pulse — so you get reliable data. Otherwise, your patients call you up and say that the measure is at 48%. It's probably not measuring there. The new home spirometry has a field under your thumb where you automatically also get the oxygen saturation. It is useful. Some weeks ago, I had a guy who called me up, saying, "I'm feeling more short of breath. Over the last 3 days, I did my pulmonary function tests and I see that my FVC goes down. I also see that my oxygen saturation goes from normally 94 at rest now to 91 at rest." That was the moment where I got worried about him. I had him come into the clinic and he turned out to have pneumonia. This remote monitoring was quite helpful to use at a distance to monitor and detect.
Swigris: Right. Remote monitoring has the capability for us to be able to detect abnormalities or a decline before a patient has waited 3 or 4 months to come back in and see us. It makes a lot of sense and hopefully leads to improved care.
Well, we are short on time, so let's wrap up with a question. What do you want physicians or practitioners who are treating patients with IPF to consider doing differently now? Is there something we should change about how we monitor our patients over time?
Wijsenbeek: Yes. In the whole trajectory, we can still improve things. It already starts at diagnosis. You must be timely in talking about initiating treatment and the fact that this is a progressive disease, and in referring patients to a transplant center. Also, I think you must be supportive, so patients should always be able reach you — whether it is remote or in person, according to your setting. You must talk about vaccination, symptom relief, measures to improve their quality of life, and exercise. I also think if the end of life is approaching, you need to be honest. If in your head you think, "I'm not sure if I will see this patient the next year," then it's time to talk about advanced care planning. You don't want these patients to end up with a junior doctor in the emergency room deciding whether they go on mechanical ventilation when they have end-stage lung disease and die in an ICU instead of with their family at home. Those are all areas in which we can still improve the care for patients.
Swigris: Absolutely. Marlies, thank you so much for being with us today. This information is incredibly helpful. I think you've highlighted the direction in which our field is hopefully going, and that is to unburden our patients and not make them travel to our centers all the time. We can get reliable data from remote monitoring at that 3- to 4-month interval that we all know is so important in following these patients over time. Thanks for being here. Thank you for sharing your experience with us.
Wijsenbeek: Thank you.
Swigris: To the audience, thank you for listening to this conversation on IPF with our guest, Dr Marlies Wijsenbeek. There's much more ahead in the coming episodes, so please be sure to check us out on the Medscape app and share, save, and subscribe if you enjoyed this episode. I'm Dr Jeff Swigris for Medscape InDiscussion.
Antifibrotic Drugs for Idiopathic Pulmonary Fibrosis: What We Should Know?
Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
An Approach to Interpreting Spirometry
Six-Minute Walk Test in Patients With Idiopathic Pulmonary Fibrosis
Remote Monitoring in Idiopathic Pulmonary Fibrosis: Home Is Where the Bluetooth-Enabled Spirometer Is
Challenges in Pulmonary Fibrosis – 1: Use of High Resolution CT Scanning of the Lung for the Evaluation of Patients With Idiopathic Interstitial Pneumonias
The Impact of SARS-CoV-2 Pandemic on the Management of IPF Patients: Our Narrative Experience
Idiopathic Pulmonary Fibrosis and GERD: Links and Risks
Obstructive Sleep Apnea Is Common in Idiopathic Pulmonary Fibrosis
Pulmonary Hypertension Idiopathic Pulmonary Fibrosis: A Dastardly Duo
Ambulatory Oxygen Improves the Effectiveness of Pulmonary Rehabilitation in Selected Patients With Chronic Obstructive Pulmonary Disease
Pulse Oximetry Saturation Can Predict Prognosis of Idiopathic Pulmonary Fibrosis
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Does stress make pulmonary fibrosis worse? ›
Endoplasmic reticulum (ER) stress is associated with development and progression of fibrotic diseases, including idiopathic pulmonary fibrosis (IPF).Is there a miracle cure for idiopathic pulmonary fibrosis? ›
How is pulmonary fibrosis treated? There is no cure for pulmonary fibrosis.Can stress cause lung scarring? ›
Studies indicate that stress can lead to wear and tear of the lungs thereby deteriorating respiratory health. It is also known to worsen the symptoms of chronic lung conditions such as asthma and chronic obstructive pulmonary disease (COPD).What is the longest survival for idiopathic pulmonary fibrosis? ›
Idiopathic pulmonary fibrosis (IPF) portends a poor prognosis. With regard to idiopathic pulmonary fibrosis life expectancy, the estimated mean survival is 2-5 years from the time of diagnosis.What aggravates pulmonary fibrosis? ›
Long-term exposure to a number of toxins and pollutants can damage your lungs. These include: Silica dust. Asbestos fibers.Is coffee good for pulmonary fibrosis? ›
In the lung, caffeine appears to exhibit its antifibrotic effects through distinct actions on both epithelial cells and fibroblasts, which are two of the key effector cells involved in the pathogenesis of pulmonary fibrosis.How do you stop pulmonary fibrosis from getting worse? ›
Treatments for idiopathic pulmonary fibrosis
Treatments include: self-care measures – such as stopping smoking, eating healthily and exercising regularly. medication to reduce the rate at which scarring worsens – such as pirfenidone and nintedanib.
In patients with idiopathic pulmonary fibrosis (IPF), supplementing with a combination of vitamins C, D, and E may positively affect respiratory function and alleviate inflammation and oxidative stress, according to a study published in Clinical Nutrition ESPEN.Can IPF stop progressing? ›
There is no cure for pulmonary fibrosis, but treatments can slow the progression of the disease in some people. Maintaining a healthy lifestyle and working closely with your care team can help you best manage your PF.Does walking help pulmonary fibrosis? ›
Tips for Staying Active with PF
Pulmonary rehab is a program of exercise, education and support to help you learn to breathe and get stronger. Some activities often done in pulmonary rehab include walking on a treadmill, riding a stationary bike, stretching and light weight training.
Do you feel cold with pulmonary fibrosis? ›
Cold and clubbed fingers and toes: This is one of those symptoms that will appear in the list you Google when looking up PF. Clubbed fingers and toes are a result of chronic under-oxygenation, but of these two effects, feeling cold is a lot more bothersome for me.How can I help myself with pulmonary fibrosis? ›
Practice relaxation techniques like meditation and breathing exercises. Call the Lung HelpLine to talk one-on-one with a medical professional who can offer tips and connect you to resources. Listen to your body. Push yourself to be as active as you can but also rest when your body needs it.Can you live 10 years with IPF? ›
A diagnosis of PF can be very scary. When you do your research, you may see average survival is between three to five years. This number is an average. There are patients who live less than three years after diagnosis, and others who live much longer.What is the most common cause of death in idiopathic pulmonary fibrosis? ›
Death related to IPF is typically respiratory failure related to either progression of the disease or acute exacerbation.What climate is best for pulmonary fibrosis? ›
Find a temperature that is comfortable for you. Most patients find that mid-70's strikes the right balance. Keep the blinds drawn and the windows closed during the day. If your temperatures drop in the evening, then take advantage of a cross breeze and open some windows.What should people with pulmonary fibrosis avoid? ›
Eat a diet low in sodium (salt), added sugars, saturated and trans fat. Try and get most of your calories from lean meats and fish, fruits, whole grains, beans, vegetables and low-fat dairy products.Do you sleep a lot with pulmonary fibrosis? ›
It is caused by the throat muscles occasionally relaxing and blocking the airway during sleep. This can lead to excessive sleepiness during the day, loud snoring and mood changes.What organ does pulmonary fibrosis affect? ›
Idiopathic pulmonary fibrosis (IPF) is a serious chronic (long term) disease that affects the tissue surrounding the air sacs, or alveoli, in your lungs.Which drink is good for lungs? ›
Green tea contains many antioxidants that may help reduce inflammation in the lungs. These compounds may even protect lung tissue from the harmful effects of smoke inhalation.
The reason most pulmonologists advise IPF ( particularly post-transplant) patients against drinking alcohol is because many of the drugs that these patients take put a tremendous stress on the liver and drinking alcohol can exacerbate the problem.
Does the heat make pulmonary fibrosis worse? ›
When the body is hot, it has to work extra hard to cool off, resulting in the use of more oxygen which makes the lungs work harder. Similar to all lung diseases, hot weather is not well tolerated for individuals with Idiopathic Pulmonary Fibrosis (IPF). This is due to both high heat as well as humidity levels.What should oxygen levels be with pulmonary fibrosis? ›
You will aim to maintain your oxygen saturation level above 90 percent throughout the day and night. Getting started with oxygen can be challenging but most PF patients find that oxygen doesn't limit their lives—it actually allows them to do more.What is the new drug for pulmonary fibrosis? ›
An experimental anticancer drug called saracatinib shows promise as a treatment for Idiopathic Pulmonary Fibrosis (IPF), a chronic and often fatal condition that causes scarring or fibrosis of the lungs and makes breathing difficult.Can you fly with pulmonary fibrosis? ›
Many people with pulmonary fibrosis (PF) can safely travel by air, but for some, air travel can be dangerous. The atmosphere is made of 20% oxygen and 80% nitrogen whether you are at sea level or living high up in the mountains.How do you get more energy with pulmonary fibrosis? ›
Regular exercise can increase strength, improve exercise capacity and decrease shortness of breath, which all may improve energy level. Improve sleep habits: Improved sleep may reduce fatigue. Practicing good sleep hygiene may improve quality of sleep. Make sure your bedroom is comfortable for sleeping.What herb is good for pulmonary fibrosis? ›
Introduction: There are many clinical studies in the treatment of idiopathic pulmonary fibrosis (IPF) with herbal medicine including Astragalus mongholicus Bunge, Radix Astragali (RA) and Angelica sinensis (Oliv.) Diels, Radix Angelicae Sinensis (RAS).Does turmeric help with pulmonary fibrosis? ›
Curcumin is well known anti-inflammatory natural compound and is shown to have beneficial effects in many diseases. It is also reported to show antifibrotic activities in pulmonary fibrosis.Does a nebulizer help pulmonary fibrosis? ›
In patients with idiopathic pulmonary fibrosis (IPF), treatment with interferon beta (IFN-β) delivered through a mesh nebulizer is associated with decreased fibroblast proliferation, according to a study published in the Journal of Aerosol Medicine and Pulmonary Drug Delivery.How do you know the end is near with pulmonary fibrosis? ›
Towards the end, you may be sleepy or unconscious much of the time. You may also lose interest in eating and drinking. Your breathing pattern may change and eventually, your skin may become pale and moist, and you will become very drowsy. You may wish to consider end-of-life care.What are the signs of progression in IPF? ›
Progression of IPF is typically reflected in a decline in forced vital capacity (FVC), worsening of dyspnea, a reduction in exercise capacity, and deterioration in health-related quality of life (HRQL) [5, 14, 15].
How to do pulmonary rehab at home? ›
- Sit comfortably with your shoulders back. Breathe in slowly and deeply.
- Lower your chin while breathing out.
- Breathe in, while slowly moving your head back to neutral.
- Nod your head from side to side, moving gently and naturally with your breath.
- Return to neutral and start again.
Breathing exercises are important for pulmonary fibrosis patients as they help to better control breathing if they experience a sudden shortness of breath, strengthen their diaphragm and clear excess mucus.How do you sleep with pulmonary fibrosis? ›
Ensure your head is higher than your body and lie on your side. Use pillows or a wedge to keep you there as this opens up the airway. Sleeping with your head raised may also reduce the symptoms of acid reflux, which can affect your sleep. Keep to a regular sleep schedule.Is pulmonary fibrosis worse in winter? ›
Cold air can tighten your airways making it harder for you to breathe. Try using your reliever inhaler half an hour before going outside. Try a slow drink of lukewarm water. A glass of warm water when returning indoors can help to reduce the burning sensation of the lungs after exposure to the cold.Does weather affect pulmonary fibrosis? ›
Not everybody with pulmonary fibrosis struggles in hot weather. But hot weather combined with humidity can make you feel breathless. Hayfever and pollutants may also aggravate your symptoms of cough and breathlessness.Is a runny nose a symptom of pulmonary fibrosis? ›
Post-nasal drip is a common symptom of many PF patients.How do you get rid of mucus with pulmonary fibrosis? ›
If your pulmonary fibrosis cough is mucus productive, your doctor may suggest a mucolytic therapy. These treatments help to 'break up' mucus, making it runnier and easier to cough up and clear from the lungs.What is the strongest predictor of mortality in IPF patients? ›
Data from the IPF-PRO Registry demonstrated high mortality in patients with IPF, with oxygen use at rest being the strongest predictor of mortality over the follow-up period.How fast can IPF progress? ›
No one can predict how fast a patient's PF will progress. In some people, PF progresses very quickly while others live with the disease for many years.What is the average age at diagnosis for IPF? ›
Introduction. Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease (ILD) characterized by clinical symptoms such as dyspnea, cough and increasing immobility (1). IPF occurs primarily in patients over 60 years of age (1, 2), for which reason a connection with aging processes has been suspected.
Is COVID a risk for IPF? ›
Are patients with idiopathic pulmonary fibrosis (IPF) at increased risk of worse outcomes with COVID-19? COVID-19 has worse outcomes in patients with IPF compared with those without IPF. Outcomes of COVID-19 in patients with IPF are not yet reported in current literature.What is the burden of idiopathic pulmonary fibrosis? ›
Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease primarily affecting older adults, associated with progressive loss of lung function . Prevalence estimates vary widely, ranging from 1.25 to 63 IPF cases per 100,000 persons, depending on definitions and study methodology [2,3,4,5].Has anyone survived idiopathic pulmonary fibrosis? ›
Idiopathic pulmonary fibrosis (IPF) has an overall poor prognosis. The median survival time of patients with IPF was 2–3 years from the time of diagnosis, in several retrospective longitudinal studies (1-6). There are limited data on clinical factors associated with increased mortality (4).Is ocean air good for pulmonary fibrosis? ›
According to the Lung Institute, ocean air improves respiratory functions. Sea air is full of negative ions, which, research suggests, improve the ability to absorb oxygen. Other studies have shown that breathing in ocean air can improve lung function in patients with cystic fibrosis.What is the longest a person can live with pulmonary fibrosis? ›
While many live past three to five years, others experience respiratory failure before the three-year mark, and some become very ill within months.Is humidifier good for IPF? ›
Low humidity: Hot and humid air can create resistance to airflow into the lungs. Using a humidifier to add moisture to the air in your home can boost your comfort.What is sudden worsening of pulmonary fibrosis? ›
Some patients will experience a sudden worsening of their pulmonary fibrosis, which is referred to as an acute exacerbation. This occurs when there is a triggering event that results in a sudden increase in the processes that lead to scarring. As the lung scarring gets worse, patients have a harder time breathing.What slows pulmonary fibrosis? ›
These include nintedanib (Ofev®) and pirfenidone (Esbriet®). These medications are called anti-fibrotic agents, meaning that they have shown in clinical trials to slow down the rate of fibrosis or scarring in the lungs. These drugs are approved for patients with mild, moderate and severe IPF.What is the best exercise for pulmonary fibrosis? ›
Some activities often done in pulmonary rehab include walking on a treadmill, riding a stationary bike, stretching and light weight training. Use your oxygen. Many patients find that using oxygen when they exercise is a game changer. They can be more active with less worry.What are the last days of pulmonary fibrosis like? ›
Towards the end, you may be sleepy or unconscious much of the time. You may also lose interest in eating and drinking. Your breathing pattern may change and eventually, your skin may become pale and moist, and you will become very drowsy. You may wish to consider end-of-life care.
How fast does idiopathic pulmonary fibrosis progress? ›
No one can predict how fast a patient's PF will progress. In some people, PF progresses very quickly while others live with the disease for many years.Does your oxygen level drop with pulmonary fibrosis? ›
People with pulmonary fibrosis can often feel breathless. A build-up of scar tissue in your lungs – as with pulmonary fibrosis – restricts the transfer of oxygen from the tiny air sacs in your lungs into your blood. This causes low levels of oxygen in the blood.Can pulmonary rehab be done at home? ›
But with self-care techniques and exercises, you can still reap the benefits of pulmonary rehab from the comfort of your home.