Pulmonary Fibrosis: Everything You Need to Know

What is Pulmonary Fibrosis?

Pulmonary fibrosis is a kind of interstitial lung disease (ILD), which belongs to a class of more than 200 diseases that affect the interstitial tissue in the lungs. What is interstitial tissue? It's a type of connective tissue found in the lungs between the air sacs, or alveoli.

Pulmonary fibrosis is aptly named after the disease's main detrimental effect: deep scarring in the lungs. The term “fibrosis” is a medical term for scarring, while “pulmonary” refers to the respiratory system.

People with pulmonary fibrosis have severely scarred tissue deep in their lungs that steadily gets worse over time. The scarring causes the lung tissue to lose its elasticity and become thick and stiff, hurting overall lung function and making it difficult to breathe. Unfortunately, this change is permanent and cannot be reversed with medication or any other known treatment.

Although respiratory irritants, medications, and other diseases can cause lung scarring and lead to pulmonary fibrosis, it is still difficult in most cases to determine the cause of the disease. In fact, most cases of pulmonary fibrosis can't be traced back to any specific known cause.

How Does Pulmonary Fibrosis Affect Your Body?

The main effect of pulmonary fibrosis is that it reduces lung function and makes it difficult to breathe. That's because scarred (or “fibrotic”) tissue in the lungs is unable to absorb oxygen efficiently.

When you breathe in, the air travels down to your lungs where lots of tiny air sacs, or alveoli, absorb oxygen from the air. Alveoli have very thin membranes with lots of capillaries underneath, which allows them to absorb oxygen very efficiently.

The thin, supple tissue is a key characteristic that allows oxygen to easily diffuse through the membrane into the blood vessels directly underneath. But when healthy lung tissue becomes scarred, it changes texture and becomes thicker, making it hard for the oxygen to get through.

The more scarring (fibrosis) and the less healthy lung tissue there is, the less oxygen is able to enter the bloodstream, and the lower the blood's oxygen saturation level goes. As a result, the lungs have to work harder and take more total breaths in order to supply the whole body with enough oxygen.

In addition to affecting oxygen absorption, the stiff, fibrotic tissue is less flexible and stretchy than healthy lung tissue. This makes it difficult for the lungs to expand, causing them to use up more energy with each breath.

This leads people with pulmonary fibrosis to feel breathless and fatigued. If left untreated, low blood oxygen levels can lead to more serious complications over time.

Key Facts about Pulmonary Fibrosis

About 140 thousand Americans are currently diagnosed and living with pulmonary fibrosis. It can affect men and women of any age, but it is most common in middle-aged and older adults.

Each case of pulmonary fibrosis is unique, and the disease can progress slowly in some people and more quickly in others. The speed at which the disease progresses can vary based on age, lifestyle, treatment, individual genetic factors, and the disease's root cause.

Besides causing lung damage and respiratory issues, pulmonary fibrosis can lead to other serious complications. It can cause a variety of life-threatening conditions, including lung infections, lung cancer, blood clots, and collapsed lungs.

Most people diagnosed with pulmonary fibrosis have a life expectancy of three to five years, depending on how quickly the disease is discovered and how effectively it's treated. The most common cause of death is respiratory failure, but death can also result from complications like pulmonary hypertension, heart failure, lung cancer, or pneumonia.

Symptoms of Pulmonary Fibrosis

The first and most obvious symptoms of pulmonary fibrosis are shortness of breath and coughing. While these symptoms are associated with many more minor, temporary respiratory illnesses (like the common cold), the symptoms of pulmonary fibrosis are chronic and refuse go away.

In early stages of the disease, many people don't notice any symptoms at all. As their pulmonary fibrosis progresses, however, people with the disease usually start noticing that they tire more easily and have difficulty breathing during heavy physical activity.

As the disease goes on and lung damage worsens, patients can experience weight loss, joint pain, and “clubbing” in their fingers and toes. Eventually, the decline in lung function can leave you feeling breathless during even light daily activities or at rest.

Pulmonary Fibrosis Symptoms:

• Shortness of breath (especially with physical activity)
• Fatigue
• Shallow breathing
• Dry cough
• Unexplained weight loss
• Sore muscles and joints
• “Clubbing” (rounding and widening) of the fingers and toes

Causes of Pulmonary Fibrosis

Many different diseases, conditions, and environmental factors can cause, or contribute to, pulmonary fibrosis. However, in a large number of cases doctors can't find any clear cause.

If a reason or underlying condition is found, then it can improve the patient's treatment and prognosis. It can help your doctor put together a more targeted treatment plan and help you more effectively manage the disease.

The causes and contributing factors for pulmonary fibrosis fall into three general categories: environmental irritants, medications, and other underlying diseases.

Environmental Factors

Exposure to certain environmental factors, such as dust or asbestos, can cause lung scarring over long periods of time. That's why it's very important to avoid respiratory irritants when you can and wear proper protection when you can't.

This is especially important if you work in a field like agriculture or construction, which can expose workers to harmful chemicals and respiratory irritants for hours day after day.

Here are a few of the environmental irritants that can put you at risk for developing pulmonary fibrosis over the long term:

• Asbestos fibers
• Silica dust
• Hard metal dusts
• Grain and other organic dusts
• Bird and other animal droppings

Medication

Research on the risk factors for the disease has identified at least 42 drugs that can cause pulmonary fibrosis as a side effect.

Some medications only have a mild association with the disease, but others are much more risky. The drug bleomycin (often prescribed to treat lung cancer), for example, is known to have a relatively high risk of causing pulmonary fibrosis.

However, it is often difficult to determine whether medications like these are the cause for a specific case of pulmonary fibrosis. Even if a patient has a history of using a high-risk drug it can be impossible to confirm that it was the sole cause.

Radiation and chemotherapy treatments for cancer are another source of lung damage that can lead to pulmonary fibrosis. Because it often takes several months, or even years, before the damage makes itself known, it can creep up out of nowhere.

Luckily, only a small percentage of cancer patients who go through radiation or chemotherapy treatment eventually develop pulmonary fibrosis. The likelihood of getting the disease following radiation therapy depends largely on the severity and scope of radiation exposure, underlying lung conditions, and whether or not the patient underwent chemotherapy as well.

Here are some of the medications known to cause pulmonary fibrosis:

• Heart medications prescribed for heart murmurs
• Certain antibiotics, including notrofurantoin or ethambutol
• Chemotherapy drugs
• Certain anti-inflammatory medications like rituximab and sulfasalazine

Existing Medical Conditions

Some viruses, including hepatitis C, human herpesvirus-8, cytomegalovirus, and Epstein-Barr virus can affect the lungs and leave you prone to developing pulmonary fibrosis. There is also a variety of other conditions, especially autoimmune diseases, that can cause lung damage and fibrosis.

Here are some other medical conditions that can lead to pulmonary fibrosis:

• Dermatomyositis
• Polymyositis
• Mixed connective tissue disease
• Systemic lupus erythematousus
• Rheumatoid Arthritis
• Sarcoidosis
• Scleroderma
• Pneumonia
• Emphysema

Other Causes

Although there are many diseases and environmental factors that can lead to pulmonary fibrosis, a direct cause for the disease is difficult to find. Often, there are simply no underlying illnesses or obvious risk factors in a person's history that would explain how they got the disease.

In this case, a doctor will diagnose Idiopathic Pulmonary Fibrosis (IPF), which is a case of pulmonary fibrosis that happens spontaneously, with no obvious cause. This can be a frustrating diagnosis to get, because it doesn't give patients the answers and explanations that some need to understand and come to terms with the disease.

However, it's important to remember that most people with pulmonary fibrosis never find an obvious cause. Instead of focusing on the “why,” patients should focus what they and their doctor can do in the future to manage the symptoms and prevent disease progression.

In some cases, genetic factors can predispose you to getting a disease like pulmonary fibrosis, which provides an explanation for some spontaneous cases of IPF. It's been found that as many as 10-15 percent of people diagnosed with idiopathic pulmonary fibrosis have at least one other family member with the disease.

Risk Factors for Pulmonary Fibrosis

Some people are more likely than others to develop pulmonary fibrosis. Depending on your lifestyle, habits, and genetic predisposition to the disease, you might be more likely to develop pulmonary fibrosis at some point in your lifetime.

First, pulmonary fibrosis is more likely to affect people who are older, and people who are female. The disease is also far more likely to affect people who smoke or are exposed to respiratory irritants through their occupation.

If you work in certain industries, like farming, mining, or construction, you might be at a higher risk for developing pulmonary fibrosis. These kinds of jobs are a common source of exposure to lung irritants that can cause inflammation and scarring.

Here are all the major factors that can put you at risk for developing pulmonary fibrosis:

• Smoking
• Being above the age of 50
• Being male
• Cancer treatments (radiation and chemotherapy)
• Genetics (having other family members with pulmonary fibrosis)
• Having an occupation that exposes you to respiratory irritants (exposure to silica dust, coal dust, grain dust, and other irritants)

Although all of these things are not always controllable, you can still take steps to prevent pulmonary fibrosis by being aware of potential irritants and risk factors. If you avoid unhealthy habits like smoking and protect yourself from dust and other irritants at home and work, you can greatly reduce your risk for lung diseases like pulmonary fibrosis.

Complications that Can Occur with Pulmonary Fibrosis

While pulmonary fibrosis only directly affects the tissue in the lungs, it can lead to a host of other symptoms and detrimental health conditions. Long-term inflammation also makes lung tissue more prone to infection and even developing cancer.

One of the most serious complications pulmonary hypertension, which puts pressure and strain on the heart and surrounding arteries. In the most severe cases, this can enlarge the heart and even cause right ventricle heart failure and death.

Pulmonary Hypertension: This is a potentially serious condition that occurs when the scarred tissue from pulmonary fibrosis presses against the arteries in the lungs, causing them to narrow. This results in high blood press/ure in the lungs and heart, which causes pressure in the lower right heart chamber as well as the heart's main arteries.

Right-sided Heart Failure: This is a medical emergency that can result from pulmonary hypertension. As the pressure in the heart increases due to constricted arteries, it has to work much harder to pump blood through the lungs. This effects the right ventricle of the heart the most, and over time it can cause  it to fail.

Respiratory Failure: Respiratory failure happens when the lungs simply cannot absorb enough oxygen to supply your vital organs, like your heart and brain. Blood oxygen falls to critically dangerous levels, requiring oxygen therapy and medical intervention.

Pulmonary Embolism: A pulmonary embolism a blood clot that travels through the bloodstream to the lungs, where it gets caught and blocks blood flow. In patients with pulmonary fibrosis, pulmonary embolisms can be much more serious, and even small clots can cause major problems.

Pulmonary embolism can be caused by long periods of inactivity and immobilization, such as during long plane rides and travel. It tends to happen more often in males and to patients after lung transplant surgery.

Lung Cancer: Over time, the inflammation and tissue damage caused by pulmonary fibrosis can leave you at a greater risk of developing lung cancer.

Other Lung Complications: As the disease progresses, pulmonary fibrosis can cause severe problems like infections, blood clots, or even a collapsed lung.

Treatments for Pulmonary Fibrosis

There is no absolute cure for pulmonary fibrosis, but it is possible to increase your lifespan and quality of life through proper treatment. Effective disease management usually involves a variety of lifestyle changes, medication, supplemental oxygen, and taking steps to avoid catching contagious illnesses.

Quit Smoking

Smoking and pulmonary fibrosis do not mix well. In fact, smoking is one of the quickest ways to make symptoms worse and cause the disease to progress even faster.

Quitting smoking can be a very difficult change to make, but you don't have to do it alone. There are countless resources online and in local communities in every state dedicated to providing smokers with information, support, and the medical resources they need to quit.

If you have pulmonary fibrosis, it is very important to protect your lungs from all kinds of smoke, including secondhand smoke and open fires. Make sure you ask your friends and family members who smoke not to smoke around you, and help them understand why it's important for your health.

If you need help quitting smoking, talk to your doctor about quit smoking medications or smoking cessation programs in your area. You can also visit the American Lung Association's website for more advice and support. Www.lung.org/stop-smoking

Pulmonary Rehabilitation

Lung diseases can be scary and difficult to manage, and there is a lot to learn after being diagnosed with pulmonary fibrosis. Many people find that they need some help and support staying healthy and adhering to strict treatment and exercise plans—especially since the disease can make physical activity difficult.

That's what pulmonary rehabilitation is designed for: to help people with lung diseases like pulmonary fibrosis learn the skills and techniques they need to survive and thrive with their disease. It often includes group sessions with licensed medical professionals and therapists who can answer questions, provide support, and teach you how to manage your symptoms.

Pulmonary rehabilitation will teach you how to monitor your symptoms and use medications, supplemental oxygen, and breathing techniques to keep your oxygen levels high. You will learn about healthy diet and nutrition, special lung-friendly exercises, and how to manage your breathing and oxygen levels during physical activity.

Pulmonary rehabilitation is a truly comprehensive approach to health and lung disease management that anyone with pulmonary fibrosis could benefit from. And it's not only a place to learn skills and receive professional advice; one of the best things about pulmonary rehab is the opportunity to meet and connect with other people who have lung diseases, too.

Pulmonary rehab is a great place to build relationships with people who understand what it's like to live with pulmonary fibrosis and are dealing with similar challenges. You can't put a value on support and friendship, and that's what everyone who has to cope with a chronic disease truly needs.

Vaccination

When you have a lung disease like pulmonary fibrosis, even minor illnesses can be a big deal. That's why it's important to keep up with vaccinations, including yearly flu shots.

Getting an illness that affect the respiratory system, like the common flu, can make pulmonary fibrosis symptoms worse. Existing symptoms like shortness of breath and wheezing can become much more severe and require medical intervention.

Since doctors commonly prescribe immune system suppressants for pulmonary fibrosis, people with the disease are even more vulnerable to getting sick than the general population. However, you can protect yourself by getting yearly flu shots and practicing proper hygiene in public places.

Diet & Exercise

Anything that puts extra stress on your heart and lungs can make it difficult to breathe when you have pulmonary fibrosis. That's why proper diet and exercise, and maintaining a healthy weight, is such an important part of treating the disease.

Being overweight can worsen your symptoms and increase breathlessness, and patients who are severely overweight may not be eligible for lung transplant surgery. If you struggle to maintain a healthy weight, talk to your doctor to get advice on healthy ways to lose weight and eat better.

Pulmonary rehabilitation is also a great way to gain the knowledge and support you need to live a healthy, active lifestyle. Talk to your doctor or visit the COPD foundation's website to find a pulmonary rehabilitation program near you.

Lung Transplant Surgery

Depending on personal health and disease history, patients diagnosed with pulmonary fibrosis are often urged to seek a lung transplant. Lung transplant surgery can be a very effective treatment and increase both the length and quality of life for pulmonary fibrosis patients.

Many people with pulmonary fibrosis have benefited from the procedure; in fact, more than 50% of lung transplant surgeries are performed for patients with some form of pulmonary fibrosis. However, in order to be eligible for a lung transplant, you must be relatively healthy and have no other major diseases.

If you are diagnosed with pulmonary fibrosis, it's important to begin the process of becoming a potential transplant candidate as soon as possible. You will have to undergo an intense evaluation and be placed on a waiting list before you can be considered for the operation.

Medications

Although pulmonary fibrosis is not curable, there is still a variety of medications available to help people with the disease live more comfortably. Medication can not only reduce symptoms, but also slow the progression of the disease.

Medications for pulmonary fibrosis are primarily aimed at reducing inflammation. They usually do this by suppressing the immune system, which helps protect the patient's lungs from their body's own immune response.

Reducing inflammation helps to reduce pulmonary fibrosis symptoms and prevent further lung deterioration. Doctors may also prescribe anti-inflammatory drugs and anti-fibrotics alongside immunosuppressants for a more comprehensive approach to controlling the disease.

Here are some of the most common medications prescribed to patients with pulmonary fibrosis:

Cyclophosphamide: Cyclophosphamide (aka Cytoxan) is an immune system suppressant that can help reduce inflammation in the lungs caused by pulmonary fibrosis. The medication can be taken orally or intravenously.

Azathioprine: Azathioprine (aka Imuran) is a heavy immune system suppressant that is traditionally given to patients after an organ transplant. It helps prevent the patient's body from attacking and rejecting the new organ, and it doctors believe it can treat pulmonary fibrosis in a similar way.

Azathioprines suppress the body's immune system in order to keep it from damaging its own organs. In the case of pulmonary fibrosis, the medication could help protect the lungs from immune system attack and further scarring. However, while it does help some patients, the effectiveness of this medication for patients with pulmonary fibrosis has not yet been confirmed.

Mycophenolate Mofetil: This drug is another type of immunosuppressant that doctors commonly prescribe to patients with pulmonary fibrosis. They are sometimes used in combination with corticosteroids.

Nintedanib: This anti-fibrotic medication is another drug often used to treat idiopathic pulmonary fibrosis. When the disease is still in its early stages, this drug can be effective at slowing disease progression and lung function decline.

Pirfenidone: Pirfenidone is both an anti-fibrotic and anti-inflammatory drug. Like Nintedanib, it is often prescribed to people with idiopathic pulmonary fibrosis and can slow down the progression of the disease in its early stages.

Corticosteroids: Corticosteroid medications like Prednisone help treat pulmonary fibrosis by suppressing the immune system. This helps to reduce inflammation in the lungs but requires careful monitoring by a doctor since it can ave many serious side effects.

Proton Pump Inhibitors: Proton pump inhibitors are often used to treat gastrointestinal diseases like peptic ulcers and GERD, but they can also treat types of pulmonary fibrosis. These medications don't target the lungs or immune system, but instead work by blocking excess stomach acid production.

In some cases of pulmonary fibrosis, excess stomach acid leaks into the lungs, causing irritation and scarring. By reducing the amount of acid your stomach produces and quelling acid reflux, proton pump inhibitors help prevent the lungs from getting further inflamed.

Supplemental Oxygen Therapy

Supplemental oxygen therapy is another effective treatment for pulmonary fibrosis. While it doesn't treat the underlying disease, it can reduce symptoms and help people with pulmonary fibrosis stay active and achieve a higher quality of life.

Many pulmonary fibrosis patients have difficulty breathing because their scarred lung tissue is no longer efficient at absorbing oxygen, leading to breathlessness and fatigue. Supplemental oxygen helps damaged lungs take up oxygen more quickly and effectively than they can from ambient air alone.

Some patients only need to use supplemental oxygen during physical activity or while sleeping, while others need it even at rest. Your doctor will help you determine when and how much oxygen you need for different activities.

Supplemental oxygen is one of the most effective ways to reduce breathing problems and ease other symptoms caused by pulmonary fibrosis. As the disease progresses and lung function continues to decline, pulmonary fibrosis patients will need more and more oxygen. If you have been diagnosed with the disease it's important to have regular visits with your doctor to evaluate your blood oxygen levels and determine your personal supplemental oxygen needs.

Conclusion

Pulmonary fibrosis can be a frightening diagnosis to get. Dealing with coughing, breathlessness, and fatigue can take a real toll on your daily life and activities.

But although there is no known cure for pulmonary fibrosis, there are many treatments available that can help you live a fulfilling life with the disease. With proper medication, healthy habits, and support from doctors, friends, and family, many people with pulmonary fibrosis are able to slow the progression of their disease, extend their lives, and live comfortably.

There is such a varied amount of information and advice to learn when you're diagnosed with pulmonary fibrosis that it can seem overwhelming to take in. That's why we created this guide to give you a comprehensive overview of the disease and a place to start.

If you have been diagnosed with pulmonary fibrosis, don't waste any time taking control of your life. With the right treatment plan, practice, and support most patients are able to life healthier, more active lives and manage their symptoms effectively.

It can be challenging to get used to the lifestyle changes and treatment regimen you need to follow in order to manage a disease like pulmonary fibrosis. However, it's a challenge that many people have to take, and the reward for success is a healthier, happier life.