HomeHealth A-ZEverything You Need to Know about Pulmonary Fibrosis

Everything You Need to Know about Pulmonary Fibrosis

What is Pulmonary Fibrosis?

Pulmonary fibrosis is a lung disease that occurs because of lung tissue damage or scarring. As the affected area is the lung, the patient can become short of breath. The body suffers from an oxygen deficit, which can lead to cardiac and respiratory complications. Although the ill-effects caused to the lung are irreversible, medicines and therapies can manage the condition and ease the symptoms.

What are the Symptoms of Pulmonary Fibrosis?

Typically, the symptoms are very mild and even unnoticeable at the beginning. Most commonly, people face difficulty in breathing as the initial symptom. Some other symptoms include:

  • Long dry cough
  • Weakness and muscle ache
  • Weight loss
  • Severe shortness of breath
  • Chest stiffness
  • Curving of nails (nail clubbing)

What are the Causes of Pulmonary Fibrosis?

The primary cause of lung or pulmonary fibrosis can differ from person to person, as it depends on their medical history. But some common causes are:

  • Environmental Factors

Certain pollutants, with time, can have adverse effects on the lungs. Such toxins and pollutants include silica dust, asbestos fibers, coal dust, grain dust, and hard metal dust.

Long-term exposure to these pollutants can cause lung fibrosis.

  • Medicines

Various medicines can damage the lungs as a side effect. They can be chemotherapy drugs, heart medicines, certain antibiotics, and anti-inflammatory drugs. Some medicines, namely, nitrofurantoin, sulfasalazine, amiodarone, and cyclophosphamide, affect the lungs and can cause lung fibrosis.

  • Genetics

Up to 20% of people with lung fibrosis may have another member in the family with the same disease, which is known as ‘familial pulmonary fibrosis’ or ‘familial interstitial pneumonia.

  • Infections

Some viral and bacterial infections affect the lungs. These can originate from hepatitis C, adenovirus, herpes virus, and other viruses.  

  • Autoimmune Diseases

Autoimmune disease can trigger the immune system to attack the lungs. Some autoimmune diseases like rheumatoid arthritis, scleroderma, and vasculitis can cause scarring in the lungs leading to lung fibrosis.

  • Radiation Therapy

Some people experience lung fibrosis because of radiation treatment for cancer. People generally face these problems years after the treatment.

Things to be Careful About With Pulmonary Fibrosis

When a person has been diagnosed with pulmonary fibrosis, several things should be taken care of. Some of them are described below.

  • The patient has a problem in breathing, so a backup oxygen cylinder should always be present. 
  • One must stay away from household sources of fumes like oil, cooking, candles, and even body scents/perfumes. These fumes can also aggravate existing symptoms. 
  • Any type of dust or lint is also harmful to these patients. 
  • Avoid harsh bathroom cleaners as their potent smells can affect the patient’s health. 
  • A beneficial solution is to wear a face mask to prevent these irritants from entering the respiratory system.

What are the Risk Factors for Pulmonary Fibrosis?

Pulmonary fibrosis does not occur in the body within a brief period. Various factors are responsible in the long term for lung health deterioration and any kind of fibrosis in the lungs.  Factors that result in high risk for pulmonary fibrosis are:

  • Age

The disease affects the middle-aged and the elderly adversely.

  • Gender

Men are more likely to have pulmonary fibrosis than women.

  • Smoking

Smoking is highly injurious to health. A person who smokes has a significantly higher chance of having pulmonary fibrosis. 

  • Work Environment

Pollutants in the environment can cause severe lung problems. People who work in mines, farms, industries, and construction sites risk developing pulmonary fibrosis.

When to See the Doctor?

It is hard to diagnose pulmonary fibrosis as it has symptoms similar to various other lung disorders. If you experience any trouble in breathing, consult a doctor immediately. Since it is a disease that is not easy to diagnose, seek a specialist who has expertise in treating lung problems.

The doctor could examine you with a stethoscope and ask you the following questions:

  • How long have you been experiencing these symptoms?
  • Do you smoke?
  • Does your surrounding environment have chemicals or other pollutants present? If yes, which ones?
  • Does anyone in your family also have such problems or any other lung disease?
  • What is your past medical history?

Try to be prepared with answers to these questions beforehand and prefer consulting a pulmonary specialist.

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How is Pulmonary Fibrosis diagnosed?

Your clinical information, coupled with a chest CT scan, can help the doctor diagnose you accurately. Sometimes, when the diagnosis is unclear, the doctor may suggest a tissue sample or a biopsy.

The doctor does a variety of other tests before confirming pulmonary fibrosis. These are:

  • Pulse oximetry test to check blood oxygen levels 
  • Blood test, to check for autoimmune diseases and infections 
  • Arterial blood gas test to check blood oxygen levels more precisely 
  • Sputum sample test for infections 
  • Pulmonary function test for measuring lung capacity
  • Echocardiogram to check for symptoms caused by cardiac problems 

What are the Treatment Options for Pulmonary Fibrosis?

Based on the severity of the disease, doctors can prescribe proper treatment to manage the symptoms. Some of these treatment options are:

  • Medication

Various medicines like pirfenidone and nintedanib are prescribed by doctors for pulmonary fibrosis. The Food and Drug Administration (FDA) has approved these medicines to slow down the scarring process in the lungs.

  • Oxygen Therapy

Oxygen use cannot cure any lung fibrosis, but it can make breathing easier. Also, it reduces complications from low blood oxygen levels. It also helps to keep the heart healthy and improves sleep patterns.

  • Pulmonary Rehabilitation

It can help improve the daily functioning of the lungs. Various physical exercises and breathing techniques are taught in these rehab sessions. 

  • Lung Transplant

Lung transplant is the last alternative if one presents with severe symptoms and faces escalated life threats. A lung transplant increases the quality of life.

What Complications Can Arise from Pulmonary Fibrosis?

Although lung fibrosis is mostly undetectable in the initial stages, later on, it can cause a variety of complications. Some of them are:

  • Pulmonary Hypertension

It affects the arteries in the lungs by compressing them. It occurs because of the scar tissue, which increases the difficulty of normal blood flow due to the compressed arteries. As a result, blood pressure in the right ventricle increases. 

  • Cor pulmonale

Right-sided heart failure is called ‘Cor Pulmonale’. This occurs when the right ventricular function is impaired to due to overwork leading to resistance of blood flow in the lungs.

  • Respiratory Failure and Complications

Various respiratory complications like blood clots and lung infection arise due to pulmonary fibrosis. The last stage of such conditions can be total respiratory failure, which occurs when blood oxygen levels decrease drastically. 

  • Lung Cancer

Prolonged pulmonary fibrosis can cause inflammation and even lung cancer.

Prevention Tips for Pulmonary Fibrosis

Though the effects of pulmonary fibrosis are irreversible, it can be prevented. Various linked aspects of health can cause these problems. The following prevention tips can lower the risk of developing this disease:

  • Avoid smoking and second-hand smoke.
  • Wear a mask if your environment has pollutants.
  • Consult a doctor immediately if you face breathing issues.

Conclusion

Pulmonary fibrosis affects the lungs by scarring and stiffening them. Treatment options are available to ease the symptoms.  Various medications are also available to slow down the scarring of the lungs. There is an option of lung transplantation for pulmonary fibrosis patients.  It can give them a longer life and improve the quality of life. 

Frequently Asked Questions (FAQs)

Can I Exercise With Pulmonary Fibrosis?

Adequate physical exercise is always good. But, if you have pulmonary fibrosis, it is challenging, so consult your doctor for advice about the appropriate exercises and activities for you.  

Should I Carry an Oxygen Cylinder if I am Traveling to a High-altitude Location?

With an increase in altitude, the oxygen level decreases. So, if you are suffering from this disease, you should carry an oxygen cylinder to places at high altitudes.

Is Sleep Apnea Connected to Pulmonary Fibrosis?

It has been observed that a person suffering from pulmonary fibrosis is likely to suffer from sleep apnea. Get sleep apnea treated as well with pulmonary fibrosis.

I Have Pulmonary Fibrosis. Do I Have a Higher Risk of Getting COVID-19?

Yes, the patients affected with pulmonary fibrosis have a higher risk of contracting COVID-19. Both diseases affect the lung and breathing capacity. Try to maintain proper hygiene and sanitize your hands regularly.

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Verified By Dr Chennamchetty Vijay Kumar
MD (Pulmonology), IDCC (Critical Care), FSM (Sleep Medicine), FCCP, Consultant Interventional Pulmonologist & Sleep Disorder Specialist, Apollo Hospitals, Jubilee Hills, Hyderabad

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