What is pulmonary fibrosis?
Pulmonary fibrosis is a disease caused by scarring in the lungs. The tissue inside and between the air sacs becomes thick, stiff and scarred. The scarring is referred to as fibrosis. As the lung tissue becomes scarred, oxygen cannot pass through to the red blood cell, which carries oxygen. Creating hypoxia, a reduction in oxygen to all your body tissue. Once the lung tissue becomes scarred, the damage cannot be reversed.
In some cases, the cause of pulmonary fibrosis can be found. Most cases of pulmonary fibrosis have no known cause.
In most cases, there is no know cause of pulmonary fibrosis. So your doctor my refer to it as idiopathic. In other cases, there maybe so possible causes:
- Cigarette smoking
- Certain viral infections
- Exposure to environmental pollutants, including silica and hard metal dusts, bacteria and animal proteins, gases and fumes
- The use of certain medicines
- Some chemotherapy treatments
- Genetics. Some families have at least two members who have pulmonary fibrosis. At this time no markers have been found.
- Gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.
Sign and symptoms
- Shortness of breath, particularly during exercise
- Dry, hacking cough
- Fast and/or shallow breathing
- Gradual unintended weight loss
- Aching joints and muscles
- Clubbing (widening and rounding) of the tips of the fingers or toes
There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to try and make the right diagnosis. Your doctor will do a physical exam. Your doctor may ask about your history of smoking, things in the air at your job that could irritate your lungs, your medication history, other medical conditions, your family’s medical history, and your hobbies.
Other tests for pulmonary fibrosis may include:
- Chest X-ray
- Breathing tests to determine how much lung damage is present
- Blood tests to check for oxygen levels in your bloodstream, autoimmune disease, and for possible infections
- High resolution CT scan of chest (HRCT)
- Lung biopsy if diagnosis not able to be made by HRCT. The biopsy is done as a surgical procedure and removes small pieces of lung tissue to find a diagnosis.
- Exercise testing to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream. Sometimes referred to as a 6 minute walk.
Once all of these test have been resulted, your doctor will develop a diagnosis.
There is no cure for pulmonary fibrosis. Once the scarring has formed there is no reversal. Current treatments are aimed at slowing the course of the disease, relieving symptoms, including oxygen and helping you stay active and healthy. Treatment cannot reverse lung damage that has already occurred.
Medicine. Depending on the type of pulmonary fibrosis you have, there may be medications to treat you. Only your medical provider can determine if any medications may be beneficial for you.
Many people diagnosed with pulmonary fibrosis will be prescribed a corticosteroid (prednisone) to reduce inflammation. Corticosteroids can have some serious side effects, especially if used long-term, and need to be carefully monitored. Corticosteroids are not indicated for all types of pulmonary fibrosis. Other medicines sometimes prescribed to reduce inflammation are azathioprine and cyclophosphamide, which suppress the immune system.
The FDA has approved two drugs for one type of pulmonary fibrosis, idiopathic pulmonary fibrosis IPF. They are nintedanib (Ofev®) and pirfenidone (Esbriet®, Pirfenex®, Pirespa®). Both have been shown to slow the progressive decline in lung function in patients with IPF. These drugs are approved for patients with mild, moderate, and severe IPF.
Oxygen therapy may be prescribed if the amount of oxygen in your blood gets low. Supplemental oxygen therapy may help reduce your shortness of breath and make it easier for you to stay active.
Pulmonary rehabilitation is a program that teaches you about your lung disease, how to exercise and how to manage your disease, and provides support and counseling.
Lung transplant evaluation should be recommended early after diagnosis by your physician. To be considered eligible for a lung transplant, you will need to be in good physical condition besides your lung function, have no other life-threatening illnesses, and be willing to accept the risks of surgery and be compliant with subsequent medical treatment.