How to Manage Pulmonary Fibrosis Symptoms 

Pulmonary fibrosis is scarring of the lungs that can cause trouble breathing. Most cases of pulmonary fibrosis have no known cause (called idiopathic pulmonary fibrosis), and it appears that these cases are on the rise. (1) Up to 28 per 100,000 people in the U.S. and up to 23 per 100,000 people in Europe are believed to have the disease, although some estimates suggest the condition is nearly three times as common. (2)

In general, pulmonary fibrosis is chronic (it lasts a long time) and progressive (it gets worse over time). (3) Although the disease can be very different from person to person, it usually makes it hard to breathe during activity and, eventually, even at rest. (4)

By arming yourself with information about the condition and by committing to strategies to manage your symptoms, you may notice some improvement in how you feel. (5)

What Is Pulmonary Fibrosis?

Pulmonary fibrosis is one of a group of lung disorders that cause scar tissue to form in the lungs. In most cases, the cause of pulmonary fibrosis is unknown. These cases are called idiopathic pulmonary fibrosis, or IPF.

Although some people have stable disease for many years, the disease gets worse very quickly in others. (6) There is no known treatment to help the body reverse scarring in the lungs — once it’s there, it stays there. Some treatments may improve symptoms, but there is no cure for pulmonary fibrosis. (7)

Over time, the scarred tissue in the lungs makes it hard to breathe. People with pulmonary fibrosis often feel out of breath and may find it a challenge to do simple physical activities, such as climbing stairs or running errands. As the scarring gets worse, it can become hard to breathe even when you are sitting or resting. (8)

Pulmonary fibrosis is most likely to affect older men, although people of any age, gender, race and background can be affected. (9) It is diagnosed using a combination of blood tests, breathing tests, a chest X-ray and high-resolution CT (HRCT) scan of your chest, and exercise testing. You may also need a lung biopsy. (10)

Pulmonary Fibrosis Stages

There are no universal stages for measuring pulmonary fibrosis. (11) However, you may be told that you have mild, moderate or severe disease. Alternatively, you may hear the stage of your illness referred to as early, advanced or end-stage.

In general, these stage terms are used to let you know how serious your diagnosis is, based on your test results for lung function and scarring. Early and mild cases mean you have less lung damage and less limitation in your breathing compared to someone who may be at the moderate, severe or advanced stages. Your health care provider should be able to tell you about how severe your diagnosis is, even if you haven’t been diagnosed with the disease at a particular stage.

Your disease severity may be measured by your score on a forced vital capacity (FVC) test (with or without other test results). For example, an FVC score of more than 75 percent may be considered mild disease; 50 to 75 percent as moderate; 25 to 49 percent as severe; and anything below 25 as very severe or end-stage. (12) The lower your FVC score, the more difficulty you may have breathing on your own.

In recent years, more information has become available to help a doctor understand how severe a person’s diagnosis may be. Your doctor may use some of the following tests to stage your diagnosis: (13, 14)

• the forced expiratory volume in one second (FEV1) test
• biopsy results showing how much scar tissue you have
• the 6-minute walk test (6MWT)
• pressure levels in your pulmonary artery (pulmonary hypertension)
• imaging (your chest X-ray or HRCT scan)
• pulse oximetry to check the oxygen saturation in your blood
• blood tests to check for oxygen and carbon dioxide levels in your blood, and other signs of disease
• whether you have other lung diseases

Pulmonary Fibrosis Prognosis

When you first receive a diagnosis of IPF, you may have immediate concerns about what it means for your future. Is pulmonary fibrosis a terminal illness (fatal)? How long can you live with pulmonary fibrosis?

There is no simple answer to these important questions. Pulmonary fibrosis life expectancy varies from person to person, as do symptoms. Most people slowly get worse over a period of years, rather than getting worse quickly or staying the same. (15) Most people live less than five years with the diagnosis, although some people live much longer. (16, 17)

Eventually, scarring in the lungs can make it very hard to get enough oxygen, even with oxygen therapy. About 77 percent of people with the disease die from breathing-related complications. This usually happens either after a few weeks or a few months of symptoms getting noticeably worse, rather than coming totally out of the blue as a sudden attack. Although this can be intimidating, it means that people dying of pulmonary fibrosis generally have some warning time to either seek new treatments or to set affairs in order and say their goodbyes. The remaining 23 percent of people with the disease die of other causes, such as cancer, heart attacks, strokes, accidents or illness. (18)

Regardless of the eventual prognosis, there are ways that people with pulmonary fibrosis can improve their quality of life, including how active they are and how well they feel on a daily basis. (19) By working hard to maintain their activity levels and being vigilant for any changes in their health, people with pulmonary fibrosis may have the chance to improve and make the most of their well-being before their health significantly declines. Certain treatments, including lung transplant, may also improve survival and overall health. (20)

Signs & Symptoms

Pulmonary fibrosis symptoms include: (21, 22, 23)

• Breathlessness, especially during exercise
• Taking fast, shallow breaths
• Dry or hacking cough that doesn’t go away
• Feeling tired
• Achy joints or muscles
• Unintended weight loss
• Fingertips and toes that get wider and rounder at the ends (clubbing)
• Swelling in your lower legs

In some cases, symptoms come on quickly but don’t improve. This is rare. Most people have a slow and steady progression of symptoms.

People can also experience an “acute exacerbation” of their pulmonary fibrosis. This means that symptoms get worse very quickly. These exacerbations can last a few days to a few weeks and may require hospitalization. (24) However, once these acute attacks are over, health usually improves.

Causes & Risk Factors

What is the cause of pulmonary fibrosis?

Pulmonary fibrosis is caused by scar tissue. As it builds up, it can block oxygen from passing from your lungs into your blood. (25) Not having enough oxygen in your blood can cause you to feel tired, weak, and breathless.

How you get pulmonary fibrosis is sometimes unknown. In those cases, it is called idiopathic pulmonary fibrosis. However, for some people, a cause can be identified.

Causes of pulmonary fibrosis may include: (26)

• Exposure to environmental or occupational toxins and pollutants, such as silica dust, asbestos, metal dust, coal or grain dust, or animal droppings
• Radiation treatment for lung or breast cancer, even from months or years ago
• Certain medications, such as chemotherapy drugs, drugs to treat irregular heart rhythm, some antibiotics, and certain prescription anti-inflammatory drugs
• Other medical conditions, such as sarcoidosis, scleroderma, pneumonia, lupus, rheumatoid arthritis, or other rare inflammatory conditions that affect the muscles, tissue or skin.

Is pulmonary fibrosis hereditary?

In general, it is not considered a genetic disease. Most people have no family history of the disease. (27) That being said, there are some rare types of idiopathic pulmonary fibrosis that seem to run in families. (28)

There are some known genes that may be risk factors for developing pulmonary fibrosis, but they are not found in everyone with the condition. It is possible that research will find more genetic or hereditary causes to the disease. For now, however, it is believed that even if there is some hereditary aspect to the disease, it simply increases the risk of developing the condition. That means it doesn’t always cause disease on its own. Instead, people who inherit the risk may or may not be triggered by other possible exposures, such as infections, smoking or the causes listed above. (29)

Risk factors for pulmonary fibrosis

You are more likely to be diagnosed with pulmonary fibrosis if you are: (30)

• 50 to 70 years old
• Male
• A smoker or a former smoker
• A current or former farmer, miner, construction worker or other worker who was exposed to certain pollutants that can harm the lungs
• A current or former cancer patient who received radiation to the chest or certain chemotherapy drugs
• Related to someone with certain types of the disease

It is also believed that gastroesophageal reflux disease (GERD) may be a risk factor for idiopathic pulmonary fibrosis. (31) This is because people with GERD may sometimes breathe in stomach acid, which can damage the lungs. (32)

Conventional Treatment

Pulmonary fibrosis treatment aims to relieve symptoms and improve your quality of life. Treatment cannot reverse the course of the disease. (33) There is also no treatment that seems to really stop it from getting worse, but new medicines may slow it down. (34) In addition, new treatments are being developed and studied that may also slow the course of disease.

Your doctor may recommend: (35)

• Pirfenidone or nintedanib to slow down damage to your lung tissue
• Antacids to treat GERD
• Oxygen therapy to make it easier for you to breathe and be active
• Pulmonary rehabilitation to help you manage your daily symptoms
• Taking part in a clinical trial to give you access to drugs that are not yet approved for general use
• Lung transplant, if you are a good candidate

For some people, a lung transplant is a good option, since it may relieve symptoms and help you live longer (about 50 percent of people with a transplant live five years or more). (36, 37) However, there are risks, and there is no guarantee a lung transplant will help. (38)

Your doctor may also choose to give you another medication, such as blood thinners, acetylcysteine (an antioxidant to protect your lung tissue), or combined acetylcysteine and azathioprine and prednisone (to suppress immune system reactions and reduce inflammation). If you have a short-term (acute) worsening of the disease, you may receive corticosteroids or be put on a mechanical ventilator to help you breathe. (39) Your conventional health care provider may also recommend you get annual flu and pneumonia vaccines to help you avoid lung infections that can make your symptoms worse. (40)

10 Natural Ways to Manage Pulmonary Fibrosis Symptoms

Due to the serious nature of pulmonary fibrosis, and the fact that there is no medical cure, natural therapies provide a good option for many people hoping to find symptom relief. Follow these natural tips for managing your symptoms and improving your health if you have pulmonary fibrosis: (41, 42)

1. Stop smoking
2. Get to and maintain a healthy weight
3. Avoid lung infections
4. Do pulmonary rehabilitation
5. Follow a diet to improve your symptoms
6. Monitor your oxygen
7. Keep stress and anxiety under control
8. Stay active
9. Get support
10. Get enough sleep

By actively working to protect your health, you can maintain a better quality of life for as long as possible. Work with your doctor to develop an exercise plan and pulmonary rehabilitation therapy course that works for you.

When the disease progresses to its end stages, you can still take advantage of spiritual, psychosocial and physical support from health professionals, religious leaders and social workers. (43) Palliative care and hospice services can often be tailored to your wishes to incorporate natural and holistic therapies that support your well-being and honor your personal preferences.

1. Stop smoking

You should also avoid secondhand smoke. There has been conflicting research on the importance of stopping smoking for people with pulmonary fibrosis. (44, 45) However, lung health experts generally recommend quitting smoking due to its ability to improve breathing-related symptoms. In most cases, smokers who quit may be observed without other treatments for a time to see if the condition improves on its own. (46)

2. Get to (and stay at) a healthy weight

Excess weight makes it harder for you to breathe comfortably. Also, obesity may keep you from getting a lung transplant. (47) Talk to your doctor about how to safely exercise and whether there are other changes you can make to help achieve a healthy weight.

3. Avoid lung infections

Guard your health when you are near other people who are sick with coughs and colds. You should also practice other natural methods for avoiding colds and the flu, particularly during the winter. (48)

4. Take part in pulmonary rehabilitation

These programs or sessions focus on making it easier for you to breathe. You will learn safe ways to exercise to help increase your stamina; breathing techniques to help your lungs work more efficiently; what to eat; more information about your condition; and other strategies to improve your quality of life. (49)

5. Follow a diet to improve your symptoms

The Lung Institute offers a detailed guide to a special diet for people with pulmonary fibrosis. Their chief recommendations for a good pulmonary fibrosis diet include: (50)

• Get plenty of lean protein
• Focus on healthy fats, such as omega-3s
• Avoid fried foods — try grilling or baking instead
• Prioritize vegetables, then fruits
• Aim for a variety of vegetables and fruits to maximize antioxidants and vitamins, such as kale, sweet potatoes, broccoli, carrots, squash, citrus, mangos, cherries and berries
• Stay hydrated, particularly with water and milk (don’t worry, milk will not lead to extra mucus production!)

6. Monitor your oxygen

You may be able to use a pulse oximeter at home. This is a small device placed over your fingertip to tell you how much oxygen is in your blood. If you have levels below 90 percent, you are not getting enough oxygen and should tell your health care provider. (51)

7. Keep stress and anxiety under control

These can lead to breathlessness. When you feel you can’t breathe well enough, you can also experience panic attacks and anxiety. Since the problems cut both ways, learning relaxation techniques and proactively keeping your stress under control are great ways to improve how you cope with this disease. (52)

8. Stay active

Resting too much because of your symptoms actually works against you. Inactivity makes your lungs weaker, so over time you will be even more out of breath than you would have (for the same activity) had you stayed in shape. (53)

9. Get support

Join a Pulmonary Fibrosis Foundation support group. (54) Talking with others can give you new ideas, lift your spirits, help you cope with stress or depression, and learn how others cope with the same emotions and challenges you are experiencing. If you cannot get involved with a support group, talk to a health care professional, friends, families or other patients you know to get the support you need. Many people also feel better when they help support others in similar situations. (55)

10. Get enough sleep

Eight hours of sleep at night can keep your body energized and better able to fight infection. (56)

Precautions

In addition to its impact on your health long term, pulmonary fibrosis can cause complications. You should be aware of these possible complications so that you can tell your doctor if you have symptoms or suspect you are at risk.

Pulmonary fibrosis complications can include: (57, 58)

• High blood pressure in your lungs, also called pulmonary hypertension
• Lung complications, such as blood clots, lung infections or even a collapsed lung
• Lung cancer
• Side effects from treatments and medications
• Heart failure (on the right side of your heart)
• Respiratory failure, or a lack of enough oxygen in the blood

If you have other health problems, such as chronic obstructive pulmonary disease (COPD), sleep apnea, or emphysema, you may be at risk for other complications as well. (59) Tell your doctor about all of your symptoms and all of the ways you are treating your symptoms, including any supplements or vitamins you are taking. Natural therapies can interact with other medications, which can sometimes cause problems such as low blood pressure.

Some doctors in other countries may offer stem cell therapy as a treatment. It is not an approved treatment for pulmonary fibrosis in the U.S. because it has not yet been thoroughly researched, although trials are underway. (60) Consider taking part in a clinical trial for stem cell therapy rather than taking part in medical tourism (travelling to get the therapy outside of the U.S.), until more is known about cellular therapy’s possible risks and benefits for people with pulmonary fibrosis.

Key Points 

• Pulmonary fibrosis is a serious disease. It causes scarring of the lungs that can cause trouble breathing.
• Most cases of pulmonary fibrosis have no known cause.
• Although some people have stable disease for many years, the disease gets worse very quickly in others.
• Some treatments may improve symptoms, but there is no cure for pulmonary fibrosis.
• By sticking to your treatment plan, working hard to manage your health naturally, and taking care of your emotional health, you can help improve your quality of life and minimize the disease’s impact for as long as possible.

10 Natural Ways to Manage Pulmonary Fibrosis Symptoms

1. Stop smoking
2. Get to and maintain a healthy weight
3. Avoid lung infections
4. Do pulmonary rehabilitation
5. Follow a diet to improve your symptoms
6. Monitor your oxygen
7. Keep stress and anxiety under control
8. Stay active
9. Get support
10. Get enough sleep

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